BACKGROUND Early adenocarcinoma mixed with a neuroendocrine carcinoma(NEC)component arising in the gastroesophageal junctional(GEJ)region is rare and even rarer in young patients.Here,we report such a case in a 29-yea...BACKGROUND Early adenocarcinoma mixed with a neuroendocrine carcinoma(NEC)component arising in the gastroesophageal junctional(GEJ)region is rare and even rarer in young patients.Here,we report such a case in a 29-year-old Chinese man.CASE SUMMARY This patient presented to our hospital with a 3-mo history of dysphagia and regurgitation.Upper endoscopy revealed an elevated nodule in the distal esophagus 1.6 cm above the GEJ line,without Barrett’s esophagus or involvement of the gastric cardia.The nodule was completely resected by endoscopic submu-cosal dissection(ESD).Pathological examination confirmed diagnosis of intra-mucosal adenocarcinoma mixed with an NEC component,measuring 1.5 cm.Immunohistochemically,both adenocarcinoma and NEC components were positive for P53 with a Ki67 index of 90%;NEC was positive for synaptophysin and chromogranin.Next-generation sequencing of 196 genes demonstrated a novel germline mutation of the ERCC3 gene in the DNA repair pathway and a germline mutation of the RNF43 gene,a common gastric cancer driver gene,in addition to pathogenic somatic mutations in P53 and CHEK2 genes.The patient was alive without evidence of the disease 36 mo after ESD.CONCLUSION Early adenocarcinoma with an NEC component arising in the distal esophageal side of the GEJ region showed evidence of gastric origin.展开更多
Objective: There is an ongoing debate about whether the management of gastroenteropancreatic(GEP)neuroendocrine carcinoma(NEC) should follow the guidelines of small-cell lung cancer(SCLC). We aim to identify the genet...Objective: There is an ongoing debate about whether the management of gastroenteropancreatic(GEP)neuroendocrine carcinoma(NEC) should follow the guidelines of small-cell lung cancer(SCLC). We aim to identify the genetic differences of GEPNEC and its counterpart.Methods: We recruited GEPNEC patients as the main cohort, with lung NEC and digestive adenocarcinomas as comparative cohorts. All patients undergone next-generation sequencing(NGS). Different gene alterations were compared and analyzed between GEPNEC and lung NEC(LNEC), GEPNEC and adenocarcinoma to yield the remarkable genes.Results: We recruited 257 patients, including 99 GEPNEC, 57 LNEC, and 101 digestive adenocarcinomas.Among the mutations, KRAS, RB1, TERT, IL7R, and CTNNB1 were found to have different gene alterations between GEPNEC and LNEC samples. Specific genes for each site were revealed: gastric NEC(TERT amplification),colorectal NEC(KRAS mutation), and bile tract NEC(ARID1A mutation). The gene disparities between small-cell NEC(SCNEC) and large-cell NEC(LCNEC) were KEAP1 and CDH1. Digestive adenocarcinoma was also compared with GEPNEC and suggested RB1, APC, and KRAS as significant genes. The TP53/RB1 mutation pattern was associated with first-line effectiveness. Putative targetable genes and biomarkers in GEPNEC were identified in22.2% of the patients, and they had longer progression-free survival(PFS) upon targetable treatment [12.5 months vs. 3.0 months, HR=0.40(0.21-0.75), P=0.006].Conclusions: This work demonstrated striking gene distinctions in GEPNEC compared with LNEC and adenocarcinoma and their clinical utility.展开更多
BACKGROUND Neuroendocrine neoplasms of the female genital tract are rare.AIM To enhance our clinical understanding of neuroendocrine carcinoma(NEC)of the ovary.METHODS A retrospective review was conducted on 12 patien...BACKGROUND Neuroendocrine neoplasms of the female genital tract are rare.AIM To enhance our clinical understanding of neuroendocrine carcinoma(NEC)of the ovary.METHODS A retrospective review was conducted on 12 patients diagnosed with NEC of the ovary,analyzing clinicopathological characteristics,treatment modalities,and survival status.RESULTS The median age at diagnosis was 34.5 years(range:20 to 62 years).Among the 12 cases,9 were small cell carcinoma of the ovary and 3 were large cell NEC.Five cases were stage I tumors,one case was stage IV,and six cases were stage III.Eleven patients underwent surgery as part of their treatment.All patients received adjuvant chemotherapy.Among the 12 patients,one patient received radiotherapy,and one patient with a BRCA2 mutation was administered PARP inhibitor maintenance after chemotherapy.The median progression-free survival was 13 months,and the median overall survival was 19.5 months.Four cases remained disease-free,while eight cases experienced tumor recurrence,including three cases that resulted in death due to disease recurrence.CONCLUSION NEC of the ovary is a rare condition that is more common in women of childbearing age and is associated with aggressive behavior and poor clinical outcomes.Surgical resection remains the mainstay of treatment,with some patients benefiting from adjuvant chemoradiation therapy.展开更多
In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the ga...In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential,rapid growth,and poor prognosis.Due to the rarity of these cancers,the standard therapy is poorly defined.The diagnosis of these tumors is based on combination of morphological features,immunohistochemical and neuroendocrine and epithelial cell markers.Both endocrine and epithelial cell components can act independently of each other and thus,careful grading of each component separately is required.These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response.Regardless of the organ of origin,these tumors portend poor prognosis with increased proportion of neuroendocrine component.Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes.The etiopathogenesis of these mixed tumors remains obscure but poses interesting question.We briefly discuss a few salient points in this editorial.展开更多
BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the bladder is a rare nonurothelial tumor of the bladder.The treatment of LCNEC of the bladder is different from that of urothelial carcinoma(UC);therefore,early...BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the bladder is a rare nonurothelial tumor of the bladder.The treatment of LCNEC of the bladder is different from that of urothelial carcinoma(UC);therefore,early and accurate diagnosis is particularly important.As LCNEC of the bladder is rare and its clinical symptoms and radiographic features are similar to those of urothelial tumors,the clinical diagnosis of the disease remains challenging.CASE SUMMARY We report a 72-year-old female patient who presented with gross hematuria for 3 mo.A solitary tumor located in the anterior wall of the bladder was found by cystoscopy.Pathological examination after biopsy suggested UC of the bladder in the absence of immunohistochemical assessment.The patient underwent partial cystectomy and was finally diagnosed with LCNEC(pT2bN0M0)based on the results of postoperative immunohistochemical examination.During the 10-mo follow-up,no signs of tumor recurrence or metastasis were found.CONCLUSION Immunohistochemical examination is essential for diagnosing LCNEC of the bladder.Accurate diagnosis and multidisciplinary treatment in the early stage of the disease are crucial for improving the prognosis.展开更多
Background: Neuroendocrine neoplasms are those that develop from a neuroendocrine cell. They most commonly affect the lungs, gastrointestinal tract, and pancreas, being rare conditions in the female genital tract. Whe...Background: Neuroendocrine neoplasms are those that develop from a neuroendocrine cell. They most commonly affect the lungs, gastrointestinal tract, and pancreas, being rare conditions in the female genital tract. When present, these neoplasms often manifest with nonspecific signs and symptoms such as pain, itching, swelling, single-focus lesions, bleeding, and enlargement of inguinal lymph nodes, in addition to the presence of progressively enlarging vulvar nodules. Consequently, the diagnostic investigation involves histopathological examination and confirmation through immunohistochemistry. Objective: To present a comprehensive understanding of this rarely studied pathology. The primary objective is to provide valuable insights that could aid in the future development of universally applicable treatment guidelines. Case Presentation: A 57-year-old female, with no prior comorbidities, menopause at 36, who presented with a left vulvar nodule accompanied by intense pain and swelling, later diagnosed with small cell neuroendocrine carcinoma in the vulva. Conclusion: This case report highlights the importance of enhancing our knowledge regarding small cell neuroendocrine carcinoma in the vulva, given its scarcity in medical literature. The information presented here underscores the need for standardized diagnostic and treatment approaches, paving the way for future consensus on managing this uncommon but challenging neoplasm.展开更多
BACKGROUND Large-cell neuroendocrine carcinoma(NEC)is an uncommon type of tumor that can occur in the endometrium.This aggressive cancer requires definitive management.Here,we describe the clinical characteristics and...BACKGROUND Large-cell neuroendocrine carcinoma(NEC)is an uncommon type of tumor that can occur in the endometrium.This aggressive cancer requires definitive management.Here,we describe the clinical characteristics and treatment of a postmenopausal woman with large cell NEC of the endometrium.CASE SUMMARY A 55-year-old Asian female presented with a 1-year history of postmenopausal vaginal bleeding.Transvaginal ultrasound revealed a thickened endometrium(30.2 mm)and a hypervascular tumor.Computed tomography revealed that the tumor had invaded more than half of the myometrium and spread to the pelvic lymph nodes.The tumor marker,carcinoembryonic antigen,was elevated(3.65 ng/mL).Endocervical biopsy revealed high-grade endometrial carcinoma.She underwent radical hysterectomy,bilateral salpingo-oophorectomy,omentectomy,and bilateral pelvic and para-aortic lymph node dissection.Pathological examination revealed mixed neuroendocrine and endometrioid adenocarcinoma,pT2N0M0,grade 3,and International Federation of Gynecology and Obstetrics stage 2.Immunohistochemistry showed moderate estrogen and progesterone receptor expressions(20%and 1%,respectively),focal CD56 expression(NEC marker),positive staining for vimentin,p53(wild type),and ki67(90%),and loss of expression of PMS2(Lynch syndrome marker).The patient received five cycles of cisplatin and etoposide after surgery.No recurrence was noted after 5 mo.CONCLUSION We report the characteristics and successful management of a rare case of large cell endometrial NEC concomitant with Lynch syndrome.展开更多
BACKGROUND Primary hepatic neuroendocrine carcinoma(NEC)is rare,and a combination with hepatocellular carcinoma(HCC)and cholangiocarcinoma(CCA)is extremely rare.To date,only four combination cases have been reported.T...BACKGROUND Primary hepatic neuroendocrine carcinoma(NEC)is rare,and a combination with hepatocellular carcinoma(HCC)and cholangiocarcinoma(CCA)is extremely rare.To date,only four combination cases have been reported.The present paper describes the fifth patient.CASE SUMMARY A 32-year-old Chinese man with chronic hepatitis B was hospitalized for persistent upper abdominal pain.Abdominal computed tomography(CT)examination revealed a liver mass.The tumor was located in the 7th and 8th segments of the liver,and CT and magnetic resonance imaging findings were consistent with the diagnosis of HCC.Laboratory examinations revealed the following:Alanine aminotransferase,243 U/L;aspartate aminotransferase,167 U/L;alpha-fetoprotein,4519μg/L.Laparoscopic right lobe hepatectomy was performed on the liver mass.Postoperative pathology showed low differentiation HCC plus medium and low differentiation CCA combined with NEC.One month after the surgery,the patient suffered from epigastric pain again.Liver metastasis was detected by CT,and tumor transcatheter arterial chemoembolization was performed.Unfortunately,the liver tumor was progressively increased and enlarged,and after 1 mo,the patient died of liver failure.CONCLUSION This is a rare case,wherein the tumor is highly aggressive,grows rapidly,and metastasizes in a short period.Imaging and laboratory tests can easily misdiagnose or miss such cases;thus,the final diagnosis relies on pathology.展开更多
BACKGROUND Collision carcinoma is a rare histological pattern,and includes two or more different types of tumors coexisting in the same organ as one neoplasm.Different to the combined type,the two adjacent tumors of c...BACKGROUND Collision carcinoma is a rare histological pattern,and includes two or more different types of tumors coexisting in the same organ as one neoplasm.Different to the combined type,the two adjacent tumors of collision carcinoma are histologically distinct.Collision carcinoma may occur from any origin or organ,including the cecum,liver,cervix,thyroid,stomach,kidney,and esophagus.In the rectum,adenocarcinoma is the most common pathological type,the combined type is rare,and collision tumors are even rarer.To date,only a limited number of collision carcinoma cases originating from the rectum have been reported.Due to the scarcity of rectal collision carcinoma,more cases need to be reported to fully understand the clinico-pathological features and biological behavior of the tumor.CASE SUMMARY Here we report a 40-year-old female who presented with the chief complaints of persistent changes in bowel habits and hematochezia for 10 d.She underwent Miles'operation which revealed a collision carcinoma of the rectum,showing a“side by side”pattern,composed of a high grade neuroendocrine carcinoma,(small cell carcinoma)and moderately differentiated adenocarcinoma,based on its clinico-pathological features and biological behavior.The patient remained disease-free at 12 mo follow-up.We also focused on the related literature and expert opinion.CONCLUSION Collision carcinoma is a rare tumor with ambiguous biological behavior.Greater attention should be paid to its clinico-pathologic diagnosis.Regular and adequate follow-up is essential to help rule out metastasis and assess the prognosis.展开更多
BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized.We report a case of pure endometrial LCNEC and review ...BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized.We report a case of pure endometrial LCNEC and review the current literature of similar cases to raise awareness of the histological features,treatment,and prognosis of this tumor.CASE SUMMARY We report the case of a 73-year-old woman who presented with irregular postmenopausal vaginal bleeding.Ultrasonography showed an enlarged uterus and a 5.1 cm×3.3 cm area of medium and low echogenicity in the uterine cavity.Biopsy by dilatation and curettage suggested poorly differentiated carcinoma.Magnetic resonance imaging revealed a heterogeneously enhanced uterine tumor with diffuse infiltration of the posterior wall of the uterine myometrium and enlarged pelvic lymph nodes.The patient underwent a hysterectomy and bilateral adnexal resection.Gross observation revealed an ill-defined white solid mass of the posterior wall of the uterus infiltrating into the serosa with multiple solid nodules on the serous surface.Microscopically,the tumor cells showed neuroendocrine morphology(organoid nesting).Immunohistochemistry revealed the tumor cells were diffusely positive for the neuroendocrine markers CD56,chromogranin A,and synaptophysin.Thus,the tumor was diagnosed as stage IIIC endometrial LCNEC.CONCLUSION Pathologic findings and immunohistochemistry are essential in making a diagnosis of endometrial LCNEC.展开更多
BACKGROUND There are many disputes about the definition,diagnosis,therapy,and prognosis of collision tumors.CASE SUMMARY We describe a rare patient with a collision tumor consisting of neuroendocrine carcinoma(NEC)and...BACKGROUND There are many disputes about the definition,diagnosis,therapy,and prognosis of collision tumors.CASE SUMMARY We describe a rare patient with a collision tumor consisting of neuroendocrine carcinoma(NEC)and squamous cell carcinoma(SCC)in the nasal cavity and paranasal sinus.She received operation,concurrent chemoradiotherapy,and then two cycles of palliative chemotherapy.Follow-up at 12 mo after diagnosis showed that this patient experienced a complete response with no signs of recurrence or metastasis.A literature review of previous 26 cases diagnosed with collision tumor of NEC and SCC in the head and neck was also undertaken.CONCLUSION It is challenging to manage collision tumors because there are two morphologically and etiologically distinct tumors.Well-designed multimodality therapy including surgery and chemoradiotherapy might lead to a long survival in these patients.展开更多
BACKGROUND Combined tumors comprising large-cell neuroendocrine carcinoma and hepatocellular carcinoma have been rarely reported in the literature.CASE SUMMARY We report a case of a 73-year-old woman with chronic hepa...BACKGROUND Combined tumors comprising large-cell neuroendocrine carcinoma and hepatocellular carcinoma have been rarely reported in the literature.CASE SUMMARY We report a case of a 73-year-old woman with chronic hepatitis B suspected to have a malignant hepatic mass(segment 3;size,4.5 cm)and lymph node metastasis based on computed tomography and magnetic resonance imaging.Despite being Child-Pugh class A,esophageal varices were present.She underwent left lateral sectionectomy and lymph node dissection.Pathological examination revealed a collision tumor consisting of large-cell neuroendocrine(90%)and hepatocellular(10%)carcinomas.The combined carcinoma had metastasized to one of the three lymph nodes excised.The patient recovered without any postoperative complications and was discharged in good condition on postoperative day 13.Adjuvant chemotherapy was not performed.No recurrence occurred during a follow-up period of 24 mo.CONCLUSION To improve the therapeutic management of combined tumors in the liver,it is necessary to discuss each clinical experience and consider an appropriate method for the preoperative diagnosis and treatment.展开更多
BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)accounts for about 0.25%of colorectal cancer patients.Furthermore,synchronous LCNEC and adenocarcinoma coexistence in the colon is very rare.LCNEC are usually aggre...BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)accounts for about 0.25%of colorectal cancer patients.Furthermore,synchronous LCNEC and adenocarcinoma coexistence in the colon is very rare.LCNEC are usually aggressive and have a poor prognosis.Usually,colorectal LCNEC patients complain of abdominal symptoms such as pain,diarrhea or hematochezia because it is often diagnosed as an advanced disease that accompanies metastatic lesions.CASE SUMMARY We describe a case of relatively asymptomatic synchronous LCNEC and colon adenocarcinoma.A 62-year-old male patient visited our hospital due to anemia detected by a local health check-up.He did not complain of melena,hematochezia or abdominal pain.Physical examination was unremarkable and his abdomen was soft,nontender and nondistended with no palpable mass.Laboratory tests revealed anemia with hemoglobin 5.1 g/dL.Colonoscopy revealed an ulcerofungating lesion in the ascending colon and about a 1.5 cm-sized large sessile polyp in the sigmoid colon.Endoscopic biopsy of the ascending colon lesion revealed the ulcerofungating mass that was LCNEC and endoscopic mucosal resection at the sigmoid colon lesion showed a large polypoid lesion that was adenocarcinoma.Multiple liver,lung,bone and lymph nodes metastasis was found on chest/abdominal computed tomography and positron emission tomography.The patient was diagnosed with advanced colorectal LCNEC with liver,lung,bone and lymph node metastasis(stage IV)and synchronous colonic adenocarcinoma metastasis.In this case,no specific symptom except anemia was observed despite the multiple metastases.The patient refused systemic chemotherapy and was discharged after transfusion.CONCLUSION We report a case of silent LCNEC of the colon despite the advanced state and synchronous adenocarcinoma.展开更多
Small cell neuroendocrine carcinoma(SNEC)is an extremely aggressive tumor and mainly occurs in the lung.Primary extra-pulmonary SNEC is rare.To date,only 11 primary SNECs occurring in the oral cavity have been reporte...Small cell neuroendocrine carcinoma(SNEC)is an extremely aggressive tumor and mainly occurs in the lung.Primary extra-pulmonary SNEC is rare.To date,only 11 primary SNECs occurring in the oral cavity have been reported in the English literature.We describe a case of primary SNEC of the right posterior tongue in a 46-year-old man.The patient had stage IVA disease and received adjuvant chemotherapy,followed by radical surgery and radiotherapy.He remained tumor-free for 20 mo before death due to gastrointestinal metastasis.The relevant literature on the 11 previously reported patients was reviewed,and the clinical features,histopathological characteristics,differential diagnosis and therapeutic strategies of this rare tumor were analyzed.展开更多
A 57 year-old male patient was found to have a lesion in the middle lobe of his right lung using chest computed tomography(CT).Tumor cells were detected,and surgical excision was performed.The patient was diagnosed wi...A 57 year-old male patient was found to have a lesion in the middle lobe of his right lung using chest computed tomography(CT).Tumor cells were detected,and surgical excision was performed.The patient was diagnosed with mixed large cell neuroendocrine carcinoma,and underwent six cycles of a chemotherapy regimen comprising etoposide combined with cisplatin.Genetic testing revealed an EGFR mutation,which prompted oxitinib-targeted therapy.To date,no signs of recurrence or metastasis have been reported.展开更多
BACKGROUND: Primary hepatic neurocridocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English/Chinese-language literature, therefore it is difficult to make a proper diagn...BACKGROUND: Primary hepatic neurocridocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English/Chinese-language literature, therefore it is difficult to make a proper diagnosis and determine a therapeutic approach. METHODS: Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008. Laboratory examination, digestive endoscopy, B-ultrasonography, CT, MRI, or PET-CT were performed on the patients for preoperative diagnosis. All patients received liver resection. Some patients received transcatheter arterial chemoembolization (TACE), percutaneous ethanol injection treatment (PEIT), or octreotide injection when a recurrence was found. The patients' clinical data were recorded and all patients were followed up. RESULTS: The patients were confirmed pathologically as having PHNEC. Their median follow-up time was 33 months (12-107 months). All patients survived, and the longest postoperative survival time was 107 months, the longest disease-free survival time was 98 months, the 1-year survival rate was 100%, and the 1-year recurrence rate was 45.5% (5/11). CONCLUSIONS: Since PHNEC is easy to confuse with hepatocellular carcinoma, careful screening of symptoms is needed to avoid misdiagnosis. Resection is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival. Other treatment such as TACE and PEIT can be considered as well, especially when a tumor recurs.展开更多
AIM To provide more information and therapeutic methods about gastric neuroendocrine carcinomas(G-NECs) which occur rarely but are highly malignant and clinically challenging.METHODS We retrospectively analyzed the cl...AIM To provide more information and therapeutic methods about gastric neuroendocrine carcinomas(G-NECs) which occur rarely but are highly malignant and clinically challenging.METHODS We retrospectively analyzed the clinicopathological characteristics, treatments, and prognosis of 43 G-NEC patients at our hospital between January 2007 and December 2014. The diagnosis was based on the 2010 World Health Organization criteria.RESULTS Forty-three G-NECs containing 39 small cell carcinomas and 4 large cell NECs with Ki67 > 60% were included in this study, accounting for only 0.95% of all gastric carcinomas. The median patient age was 62 years (range, 33-82) and the male-to-female ratio was 4.4:1. All patients underwent surgery, including 38 curative resections and 5 palliative resections. Among these 43 patients, nearly half(48.84%) of these tumors were located in the cardiac region of the stomach, regional lymph node metastasis was found in 31 cases(72.09%), and liver metastasis was found in 6 cases(13.95%). Follow-up information was got for 40 patients. Twentythree die of this disease with a median survival of 31 mo(range 1-90). The 1-year, 2-year, 3-year, and 5-year survival rate was 77.50%, 57.04%, 44.51%, and 35.05%, respectively. Survival was better in patients with tumor located in the cardiac region of the stomach, less than 7 lymph nodes metastasis and no liver metastasis. Five patients did not undergo postoperative chemotherapy, and the median survival time for these patients was 15 mo. For the remaining 34 patients who received postoperative chemotherapy, the median survival time was 44 mo and those received etoposide, cisplatin, and Paclitaxel survived the best. One patient with resected liver metastasis who received postoperative Capecitabine plus Oxaliplatin and Paclitaxel systemic chemotherapy plus octreotide LAR(30 mg intramuscularly, every 4 wk, for 2 years) has survived for 74 mo with no recurrence.CONCLUSION G-NECs are mostly nonfunctioning, which lead to a delay in detection. Local and/or distant metastases were noticed in most patients when diagnosed, and they required postoperative medical treatment. Adjuvant etoposide, cisplatin plus Paclitaxel systemic chemotherapy is recommended for these patients.展开更多
Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third...Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.展开更多
Neuroendocrine carcinoma(NEC) of the gallbladder is a rare subtype of gallbladder tumor. Here, we report two cases of NEC in two patients initially suspected to have gallbladder carcinoma. No specific symptoms or abno...Neuroendocrine carcinoma(NEC) of the gallbladder is a rare subtype of gallbladder tumor. Here, we report two cases of NEC in two patients initially suspected to have gallbladder carcinoma. No specific symptoms or abnormal blood test results were observed preoperatively. Abdominal computed tomography scans indicated intraluminal masses in the gallbladder and lymph node enlargement in the hepatic hilum. Radical cholecystectomy and regional lymphadenectomy were performed. The first patient also presented with liver invasion and therefore underwent resection of liver segment Ⅳ. A diagnosis of NEC was made upon postoperative pathological examination and immunohistochemical staining according to the WHO Classification of Tumors of the Digestive System(2010). One tumor was identified as poorly differentiated NEC and the other as poorly differentiated mixed adenoneuroendocrine carcinoma. Immunohistochemical staining data from both tumors showed positivity for chromogranin A and synaptophysin. The first patient received 4 cycles of chemotherapy consisting of cisplatin and etoposide. No metastases or recurrence were observed 12 mo following surgery. The second patient refused chemotherapy and presentedwith tumor recurrence 4 mo after surgery. In conclusion, NEC of the gallbladder is an aggressive tumor and the identification of a standardized optimal treatment still requires further research. Our experience together with published studies suggests that radical surgery and adjuvant chemotherapy may improve the prognosis.展开更多
BACKGROUND Neuroendocrine neoplasms are rarely located in the gallbladder(GB),and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.CASE SUMMARY We report a case of GB neuroendocrine ...BACKGROUND Neuroendocrine neoplasms are rarely located in the gallbladder(GB),and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.CASE SUMMARY We report a case of GB neuroendocrine carcinoma(GB-NEC)in a 65-year-old man,who presented with flushing for 2 mo.Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis.Computed tomography and magnetic resonance imaging indicated neoplasm in the GB with liver invasion and enlarged lymph nodes in the portacaval space.High fluorodeoxyglucose uptake was detected in lymph nodes in the portacaval space,but distant metastasis was not seen by positron emission tomography.Ultrasound-guided needle biopsy of the GB neoplasm was suggestive of high-grade NEC.Because of the functional characteristics of poorly differentiated NEC,en bloc cholecystectomy,resection of hepatic segments IVb and V,pancreaticoduodenectomy,and regional lymphadenectomy were performed.A diagnosis of poorly differentiated NEC was made by pathological findings and immunohistochemical staining data.Ki-67 index was>80%.The patient refused adjuvant therapy and passed away in the 7th month.CONCLUSION Distinctive manifestation combined with imaging helps make correct preoperative diagnosis.Radical surgery and adjuvant chemotherapy might improve prognosis.展开更多
基金Changzhou High-level Medical Talents Training Project of the Health Commission of the Changzhou City,No.2022CZBJ079Changzhou Sci&Tech Program,No.CE20235064.
文摘BACKGROUND Early adenocarcinoma mixed with a neuroendocrine carcinoma(NEC)component arising in the gastroesophageal junctional(GEJ)region is rare and even rarer in young patients.Here,we report such a case in a 29-year-old Chinese man.CASE SUMMARY This patient presented to our hospital with a 3-mo history of dysphagia and regurgitation.Upper endoscopy revealed an elevated nodule in the distal esophagus 1.6 cm above the GEJ line,without Barrett’s esophagus or involvement of the gastric cardia.The nodule was completely resected by endoscopic submu-cosal dissection(ESD).Pathological examination confirmed diagnosis of intra-mucosal adenocarcinoma mixed with an NEC component,measuring 1.5 cm.Immunohistochemically,both adenocarcinoma and NEC components were positive for P53 with a Ki67 index of 90%;NEC was positive for synaptophysin and chromogranin.Next-generation sequencing of 196 genes demonstrated a novel germline mutation of the ERCC3 gene in the DNA repair pathway and a germline mutation of the RNF43 gene,a common gastric cancer driver gene,in addition to pathogenic somatic mutations in P53 and CHEK2 genes.The patient was alive without evidence of the disease 36 mo after ESD.CONCLUSION Early adenocarcinoma with an NEC component arising in the distal esophageal side of the GEJ region showed evidence of gastric origin.
基金supported by the Major Program of National Natural Science Foundation of China (No. 91959205)National Natural Science Foundation of China (No. 82141117)+3 种基金The Capital’s Funds for Health Improvement and Research (CFH) (No. 2022-2-1023)Beijing Xisike Clinical Oncology Research Foundation Ypierrefabre (No. 202101-0099)Beijing Municipal Administration of Hospitals Incubating Program (No. PX2020045)Science Foundation of Peking University Cancer Hospital (No. 2020-4)。
文摘Objective: There is an ongoing debate about whether the management of gastroenteropancreatic(GEP)neuroendocrine carcinoma(NEC) should follow the guidelines of small-cell lung cancer(SCLC). We aim to identify the genetic differences of GEPNEC and its counterpart.Methods: We recruited GEPNEC patients as the main cohort, with lung NEC and digestive adenocarcinomas as comparative cohorts. All patients undergone next-generation sequencing(NGS). Different gene alterations were compared and analyzed between GEPNEC and lung NEC(LNEC), GEPNEC and adenocarcinoma to yield the remarkable genes.Results: We recruited 257 patients, including 99 GEPNEC, 57 LNEC, and 101 digestive adenocarcinomas.Among the mutations, KRAS, RB1, TERT, IL7R, and CTNNB1 were found to have different gene alterations between GEPNEC and LNEC samples. Specific genes for each site were revealed: gastric NEC(TERT amplification),colorectal NEC(KRAS mutation), and bile tract NEC(ARID1A mutation). The gene disparities between small-cell NEC(SCNEC) and large-cell NEC(LCNEC) were KEAP1 and CDH1. Digestive adenocarcinoma was also compared with GEPNEC and suggested RB1, APC, and KRAS as significant genes. The TP53/RB1 mutation pattern was associated with first-line effectiveness. Putative targetable genes and biomarkers in GEPNEC were identified in22.2% of the patients, and they had longer progression-free survival(PFS) upon targetable treatment [12.5 months vs. 3.0 months, HR=0.40(0.21-0.75), P=0.006].Conclusions: This work demonstrated striking gene distinctions in GEPNEC compared with LNEC and adenocarcinoma and their clinical utility.
文摘BACKGROUND Neuroendocrine neoplasms of the female genital tract are rare.AIM To enhance our clinical understanding of neuroendocrine carcinoma(NEC)of the ovary.METHODS A retrospective review was conducted on 12 patients diagnosed with NEC of the ovary,analyzing clinicopathological characteristics,treatment modalities,and survival status.RESULTS The median age at diagnosis was 34.5 years(range:20 to 62 years).Among the 12 cases,9 were small cell carcinoma of the ovary and 3 were large cell NEC.Five cases were stage I tumors,one case was stage IV,and six cases were stage III.Eleven patients underwent surgery as part of their treatment.All patients received adjuvant chemotherapy.Among the 12 patients,one patient received radiotherapy,and one patient with a BRCA2 mutation was administered PARP inhibitor maintenance after chemotherapy.The median progression-free survival was 13 months,and the median overall survival was 19.5 months.Four cases remained disease-free,while eight cases experienced tumor recurrence,including three cases that resulted in death due to disease recurrence.CONCLUSION NEC of the ovary is a rare condition that is more common in women of childbearing age and is associated with aggressive behavior and poor clinical outcomes.Surgical resection remains the mainstay of treatment,with some patients benefiting from adjuvant chemoradiation therapy.
文摘In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential,rapid growth,and poor prognosis.Due to the rarity of these cancers,the standard therapy is poorly defined.The diagnosis of these tumors is based on combination of morphological features,immunohistochemical and neuroendocrine and epithelial cell markers.Both endocrine and epithelial cell components can act independently of each other and thus,careful grading of each component separately is required.These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response.Regardless of the organ of origin,these tumors portend poor prognosis with increased proportion of neuroendocrine component.Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes.The etiopathogenesis of these mixed tumors remains obscure but poses interesting question.We briefly discuss a few salient points in this editorial.
文摘BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the bladder is a rare nonurothelial tumor of the bladder.The treatment of LCNEC of the bladder is different from that of urothelial carcinoma(UC);therefore,early and accurate diagnosis is particularly important.As LCNEC of the bladder is rare and its clinical symptoms and radiographic features are similar to those of urothelial tumors,the clinical diagnosis of the disease remains challenging.CASE SUMMARY We report a 72-year-old female patient who presented with gross hematuria for 3 mo.A solitary tumor located in the anterior wall of the bladder was found by cystoscopy.Pathological examination after biopsy suggested UC of the bladder in the absence of immunohistochemical assessment.The patient underwent partial cystectomy and was finally diagnosed with LCNEC(pT2bN0M0)based on the results of postoperative immunohistochemical examination.During the 10-mo follow-up,no signs of tumor recurrence or metastasis were found.CONCLUSION Immunohistochemical examination is essential for diagnosing LCNEC of the bladder.Accurate diagnosis and multidisciplinary treatment in the early stage of the disease are crucial for improving the prognosis.
文摘Background: Neuroendocrine neoplasms are those that develop from a neuroendocrine cell. They most commonly affect the lungs, gastrointestinal tract, and pancreas, being rare conditions in the female genital tract. When present, these neoplasms often manifest with nonspecific signs and symptoms such as pain, itching, swelling, single-focus lesions, bleeding, and enlargement of inguinal lymph nodes, in addition to the presence of progressively enlarging vulvar nodules. Consequently, the diagnostic investigation involves histopathological examination and confirmation through immunohistochemistry. Objective: To present a comprehensive understanding of this rarely studied pathology. The primary objective is to provide valuable insights that could aid in the future development of universally applicable treatment guidelines. Case Presentation: A 57-year-old female, with no prior comorbidities, menopause at 36, who presented with a left vulvar nodule accompanied by intense pain and swelling, later diagnosed with small cell neuroendocrine carcinoma in the vulva. Conclusion: This case report highlights the importance of enhancing our knowledge regarding small cell neuroendocrine carcinoma in the vulva, given its scarcity in medical literature. The information presented here underscores the need for standardized diagnostic and treatment approaches, paving the way for future consensus on managing this uncommon but challenging neoplasm.
文摘BACKGROUND Large-cell neuroendocrine carcinoma(NEC)is an uncommon type of tumor that can occur in the endometrium.This aggressive cancer requires definitive management.Here,we describe the clinical characteristics and treatment of a postmenopausal woman with large cell NEC of the endometrium.CASE SUMMARY A 55-year-old Asian female presented with a 1-year history of postmenopausal vaginal bleeding.Transvaginal ultrasound revealed a thickened endometrium(30.2 mm)and a hypervascular tumor.Computed tomography revealed that the tumor had invaded more than half of the myometrium and spread to the pelvic lymph nodes.The tumor marker,carcinoembryonic antigen,was elevated(3.65 ng/mL).Endocervical biopsy revealed high-grade endometrial carcinoma.She underwent radical hysterectomy,bilateral salpingo-oophorectomy,omentectomy,and bilateral pelvic and para-aortic lymph node dissection.Pathological examination revealed mixed neuroendocrine and endometrioid adenocarcinoma,pT2N0M0,grade 3,and International Federation of Gynecology and Obstetrics stage 2.Immunohistochemistry showed moderate estrogen and progesterone receptor expressions(20%and 1%,respectively),focal CD56 expression(NEC marker),positive staining for vimentin,p53(wild type),and ki67(90%),and loss of expression of PMS2(Lynch syndrome marker).The patient received five cycles of cisplatin and etoposide after surgery.No recurrence was noted after 5 mo.CONCLUSION We report the characteristics and successful management of a rare case of large cell endometrial NEC concomitant with Lynch syndrome.
基金Supported by Zunyi City Science and Technology Department HZ(2021),No.44。
文摘BACKGROUND Primary hepatic neuroendocrine carcinoma(NEC)is rare,and a combination with hepatocellular carcinoma(HCC)and cholangiocarcinoma(CCA)is extremely rare.To date,only four combination cases have been reported.The present paper describes the fifth patient.CASE SUMMARY A 32-year-old Chinese man with chronic hepatitis B was hospitalized for persistent upper abdominal pain.Abdominal computed tomography(CT)examination revealed a liver mass.The tumor was located in the 7th and 8th segments of the liver,and CT and magnetic resonance imaging findings were consistent with the diagnosis of HCC.Laboratory examinations revealed the following:Alanine aminotransferase,243 U/L;aspartate aminotransferase,167 U/L;alpha-fetoprotein,4519μg/L.Laparoscopic right lobe hepatectomy was performed on the liver mass.Postoperative pathology showed low differentiation HCC plus medium and low differentiation CCA combined with NEC.One month after the surgery,the patient suffered from epigastric pain again.Liver metastasis was detected by CT,and tumor transcatheter arterial chemoembolization was performed.Unfortunately,the liver tumor was progressively increased and enlarged,and after 1 mo,the patient died of liver failure.CONCLUSION This is a rare case,wherein the tumor is highly aggressive,grows rapidly,and metastasizes in a short period.Imaging and laboratory tests can easily misdiagnose or miss such cases;thus,the final diagnosis relies on pathology.
文摘BACKGROUND Collision carcinoma is a rare histological pattern,and includes two or more different types of tumors coexisting in the same organ as one neoplasm.Different to the combined type,the two adjacent tumors of collision carcinoma are histologically distinct.Collision carcinoma may occur from any origin or organ,including the cecum,liver,cervix,thyroid,stomach,kidney,and esophagus.In the rectum,adenocarcinoma is the most common pathological type,the combined type is rare,and collision tumors are even rarer.To date,only a limited number of collision carcinoma cases originating from the rectum have been reported.Due to the scarcity of rectal collision carcinoma,more cases need to be reported to fully understand the clinico-pathological features and biological behavior of the tumor.CASE SUMMARY Here we report a 40-year-old female who presented with the chief complaints of persistent changes in bowel habits and hematochezia for 10 d.She underwent Miles'operation which revealed a collision carcinoma of the rectum,showing a“side by side”pattern,composed of a high grade neuroendocrine carcinoma,(small cell carcinoma)and moderately differentiated adenocarcinoma,based on its clinico-pathological features and biological behavior.The patient remained disease-free at 12 mo follow-up.We also focused on the related literature and expert opinion.CONCLUSION Collision carcinoma is a rare tumor with ambiguous biological behavior.Greater attention should be paid to its clinico-pathologic diagnosis.Regular and adequate follow-up is essential to help rule out metastasis and assess the prognosis.
文摘BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized.We report a case of pure endometrial LCNEC and review the current literature of similar cases to raise awareness of the histological features,treatment,and prognosis of this tumor.CASE SUMMARY We report the case of a 73-year-old woman who presented with irregular postmenopausal vaginal bleeding.Ultrasonography showed an enlarged uterus and a 5.1 cm×3.3 cm area of medium and low echogenicity in the uterine cavity.Biopsy by dilatation and curettage suggested poorly differentiated carcinoma.Magnetic resonance imaging revealed a heterogeneously enhanced uterine tumor with diffuse infiltration of the posterior wall of the uterine myometrium and enlarged pelvic lymph nodes.The patient underwent a hysterectomy and bilateral adnexal resection.Gross observation revealed an ill-defined white solid mass of the posterior wall of the uterus infiltrating into the serosa with multiple solid nodules on the serous surface.Microscopically,the tumor cells showed neuroendocrine morphology(organoid nesting).Immunohistochemistry revealed the tumor cells were diffusely positive for the neuroendocrine markers CD56,chromogranin A,and synaptophysin.Thus,the tumor was diagnosed as stage IIIC endometrial LCNEC.CONCLUSION Pathologic findings and immunohistochemistry are essential in making a diagnosis of endometrial LCNEC.
基金Supported by National Natural Science Foundation of China,No.81802732.
文摘BACKGROUND There are many disputes about the definition,diagnosis,therapy,and prognosis of collision tumors.CASE SUMMARY We describe a rare patient with a collision tumor consisting of neuroendocrine carcinoma(NEC)and squamous cell carcinoma(SCC)in the nasal cavity and paranasal sinus.She received operation,concurrent chemoradiotherapy,and then two cycles of palliative chemotherapy.Follow-up at 12 mo after diagnosis showed that this patient experienced a complete response with no signs of recurrence or metastasis.A literature review of previous 26 cases diagnosed with collision tumor of NEC and SCC in the head and neck was also undertaken.CONCLUSION It is challenging to manage collision tumors because there are two morphologically and etiologically distinct tumors.Well-designed multimodality therapy including surgery and chemoradiotherapy might lead to a long survival in these patients.
文摘BACKGROUND Combined tumors comprising large-cell neuroendocrine carcinoma and hepatocellular carcinoma have been rarely reported in the literature.CASE SUMMARY We report a case of a 73-year-old woman with chronic hepatitis B suspected to have a malignant hepatic mass(segment 3;size,4.5 cm)and lymph node metastasis based on computed tomography and magnetic resonance imaging.Despite being Child-Pugh class A,esophageal varices were present.She underwent left lateral sectionectomy and lymph node dissection.Pathological examination revealed a collision tumor consisting of large-cell neuroendocrine(90%)and hepatocellular(10%)carcinomas.The combined carcinoma had metastasized to one of the three lymph nodes excised.The patient recovered without any postoperative complications and was discharged in good condition on postoperative day 13.Adjuvant chemotherapy was not performed.No recurrence occurred during a follow-up period of 24 mo.CONCLUSION To improve the therapeutic management of combined tumors in the liver,it is necessary to discuss each clinical experience and consider an appropriate method for the preoperative diagnosis and treatment.
文摘BACKGROUND Large cell neuroendocrine carcinoma(LCNEC)accounts for about 0.25%of colorectal cancer patients.Furthermore,synchronous LCNEC and adenocarcinoma coexistence in the colon is very rare.LCNEC are usually aggressive and have a poor prognosis.Usually,colorectal LCNEC patients complain of abdominal symptoms such as pain,diarrhea or hematochezia because it is often diagnosed as an advanced disease that accompanies metastatic lesions.CASE SUMMARY We describe a case of relatively asymptomatic synchronous LCNEC and colon adenocarcinoma.A 62-year-old male patient visited our hospital due to anemia detected by a local health check-up.He did not complain of melena,hematochezia or abdominal pain.Physical examination was unremarkable and his abdomen was soft,nontender and nondistended with no palpable mass.Laboratory tests revealed anemia with hemoglobin 5.1 g/dL.Colonoscopy revealed an ulcerofungating lesion in the ascending colon and about a 1.5 cm-sized large sessile polyp in the sigmoid colon.Endoscopic biopsy of the ascending colon lesion revealed the ulcerofungating mass that was LCNEC and endoscopic mucosal resection at the sigmoid colon lesion showed a large polypoid lesion that was adenocarcinoma.Multiple liver,lung,bone and lymph nodes metastasis was found on chest/abdominal computed tomography and positron emission tomography.The patient was diagnosed with advanced colorectal LCNEC with liver,lung,bone and lymph node metastasis(stage IV)and synchronous colonic adenocarcinoma metastasis.In this case,no specific symptom except anemia was observed despite the multiple metastases.The patient refused systemic chemotherapy and was discharged after transfusion.CONCLUSION We report a case of silent LCNEC of the colon despite the advanced state and synchronous adenocarcinoma.
文摘Small cell neuroendocrine carcinoma(SNEC)is an extremely aggressive tumor and mainly occurs in the lung.Primary extra-pulmonary SNEC is rare.To date,only 11 primary SNECs occurring in the oral cavity have been reported in the English literature.We describe a case of primary SNEC of the right posterior tongue in a 46-year-old man.The patient had stage IVA disease and received adjuvant chemotherapy,followed by radical surgery and radiotherapy.He remained tumor-free for 20 mo before death due to gastrointestinal metastasis.The relevant literature on the 11 previously reported patients was reviewed,and the clinical features,histopathological characteristics,differential diagnosis and therapeutic strategies of this rare tumor were analyzed.
文摘A 57 year-old male patient was found to have a lesion in the middle lobe of his right lung using chest computed tomography(CT).Tumor cells were detected,and surgical excision was performed.The patient was diagnosed with mixed large cell neuroendocrine carcinoma,and underwent six cycles of a chemotherapy regimen comprising etoposide combined with cisplatin.Genetic testing revealed an EGFR mutation,which prompted oxitinib-targeted therapy.To date,no signs of recurrence or metastasis have been reported.
文摘BACKGROUND: Primary hepatic neurocridocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English/Chinese-language literature, therefore it is difficult to make a proper diagnosis and determine a therapeutic approach. METHODS: Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008. Laboratory examination, digestive endoscopy, B-ultrasonography, CT, MRI, or PET-CT were performed on the patients for preoperative diagnosis. All patients received liver resection. Some patients received transcatheter arterial chemoembolization (TACE), percutaneous ethanol injection treatment (PEIT), or octreotide injection when a recurrence was found. The patients' clinical data were recorded and all patients were followed up. RESULTS: The patients were confirmed pathologically as having PHNEC. Their median follow-up time was 33 months (12-107 months). All patients survived, and the longest postoperative survival time was 107 months, the longest disease-free survival time was 98 months, the 1-year survival rate was 100%, and the 1-year recurrence rate was 45.5% (5/11). CONCLUSIONS: Since PHNEC is easy to confuse with hepatocellular carcinoma, careful screening of symptoms is needed to avoid misdiagnosis. Resection is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival. Other treatment such as TACE and PEIT can be considered as well, especially when a tumor recurs.
基金Supported by Chinese Society of Clinical Oncology,No.Y-N2013-014
文摘AIM To provide more information and therapeutic methods about gastric neuroendocrine carcinomas(G-NECs) which occur rarely but are highly malignant and clinically challenging.METHODS We retrospectively analyzed the clinicopathological characteristics, treatments, and prognosis of 43 G-NEC patients at our hospital between January 2007 and December 2014. The diagnosis was based on the 2010 World Health Organization criteria.RESULTS Forty-three G-NECs containing 39 small cell carcinomas and 4 large cell NECs with Ki67 > 60% were included in this study, accounting for only 0.95% of all gastric carcinomas. The median patient age was 62 years (range, 33-82) and the male-to-female ratio was 4.4:1. All patients underwent surgery, including 38 curative resections and 5 palliative resections. Among these 43 patients, nearly half(48.84%) of these tumors were located in the cardiac region of the stomach, regional lymph node metastasis was found in 31 cases(72.09%), and liver metastasis was found in 6 cases(13.95%). Follow-up information was got for 40 patients. Twentythree die of this disease with a median survival of 31 mo(range 1-90). The 1-year, 2-year, 3-year, and 5-year survival rate was 77.50%, 57.04%, 44.51%, and 35.05%, respectively. Survival was better in patients with tumor located in the cardiac region of the stomach, less than 7 lymph nodes metastasis and no liver metastasis. Five patients did not undergo postoperative chemotherapy, and the median survival time for these patients was 15 mo. For the remaining 34 patients who received postoperative chemotherapy, the median survival time was 44 mo and those received etoposide, cisplatin, and Paclitaxel survived the best. One patient with resected liver metastasis who received postoperative Capecitabine plus Oxaliplatin and Paclitaxel systemic chemotherapy plus octreotide LAR(30 mg intramuscularly, every 4 wk, for 2 years) has survived for 74 mo with no recurrence.CONCLUSION G-NECs are mostly nonfunctioning, which lead to a delay in detection. Local and/or distant metastases were noticed in most patients when diagnosed, and they required postoperative medical treatment. Adjuvant etoposide, cisplatin plus Paclitaxel systemic chemotherapy is recommended for these patients.
文摘Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.
文摘Neuroendocrine carcinoma(NEC) of the gallbladder is a rare subtype of gallbladder tumor. Here, we report two cases of NEC in two patients initially suspected to have gallbladder carcinoma. No specific symptoms or abnormal blood test results were observed preoperatively. Abdominal computed tomography scans indicated intraluminal masses in the gallbladder and lymph node enlargement in the hepatic hilum. Radical cholecystectomy and regional lymphadenectomy were performed. The first patient also presented with liver invasion and therefore underwent resection of liver segment Ⅳ. A diagnosis of NEC was made upon postoperative pathological examination and immunohistochemical staining according to the WHO Classification of Tumors of the Digestive System(2010). One tumor was identified as poorly differentiated NEC and the other as poorly differentiated mixed adenoneuroendocrine carcinoma. Immunohistochemical staining data from both tumors showed positivity for chromogranin A and synaptophysin. The first patient received 4 cycles of chemotherapy consisting of cisplatin and etoposide. No metastases or recurrence were observed 12 mo following surgery. The second patient refused chemotherapy and presentedwith tumor recurrence 4 mo after surgery. In conclusion, NEC of the gallbladder is an aggressive tumor and the identification of a standardized optimal treatment still requires further research. Our experience together with published studies suggests that radical surgery and adjuvant chemotherapy may improve the prognosis.
基金Supported by The National Natural Science Foundation of China,No.81572975Key Research and Development Project of Science and Technology Department of Zhejiang,China,No.2015C03053
文摘BACKGROUND Neuroendocrine neoplasms are rarely located in the gallbladder(GB),and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.CASE SUMMARY We report a case of GB neuroendocrine carcinoma(GB-NEC)in a 65-year-old man,who presented with flushing for 2 mo.Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis.Computed tomography and magnetic resonance imaging indicated neoplasm in the GB with liver invasion and enlarged lymph nodes in the portacaval space.High fluorodeoxyglucose uptake was detected in lymph nodes in the portacaval space,but distant metastasis was not seen by positron emission tomography.Ultrasound-guided needle biopsy of the GB neoplasm was suggestive of high-grade NEC.Because of the functional characteristics of poorly differentiated NEC,en bloc cholecystectomy,resection of hepatic segments IVb and V,pancreaticoduodenectomy,and regional lymphadenectomy were performed.A diagnosis of poorly differentiated NEC was made by pathological findings and immunohistochemical staining data.Ki-67 index was>80%.The patient refused adjuvant therapy and passed away in the 7th month.CONCLUSION Distinctive manifestation combined with imaging helps make correct preoperative diagnosis.Radical surgery and adjuvant chemotherapy might improve prognosis.