Small Cell Neuroendocrine Carcinoma of the Vulva: Case Report and Literature Review

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.

Synchronous multiple lung cancers with hilar lymph node metastasis of small cell carcinoma:A case report

BACKGROUND Synchronous multiple lung cancers are rare and refer to the simultaneous presence of two or more primary lung tumors,which present significant challenges in terms of diagnosis and treatment.CASE SUMMARY We report a case of multiple synchronous lung cancers with hilar lymph node metastasis of small cell carcinoma of unknown origin in a 73-year-old man.Transbronchial lung biopsy revealed squamous cell carcinoma.Although enlargement of lymph node 12u was detected,no distant metastases were observed.The patient was preoperatively diagnosed with T1cN0M0 and underwent thoracoscopic right upper lobectomy with nodal dissection(ND2a).Based on histopathological findings,the primary lesion was squamous cell carcinoma.A microinvasive adenocarcinoma was also observed on the cranial side of the primary lesion.Tumors were detected in two resected lymph nodes(#12u and#11s).Both tumors were pathologically diagnosed as small cell carcinomas.The primary lesion of the small cell carcinoma could not be identified even by whole-body imaging;however,chemotherapy was initiated for hilar lymph node metastasis of the small cell carcinoma of unknown origin.CONCLUSION Multiple synchronous lung cancers can be accompanied by hilar lymph node metastasis of small cell carcinomas of unknown origin.

Primary Ovarian Small Cell Carcinoma of Pulmonary Type: Analysis of 6 Cases and Review of 31 Cases in the Literatures

Objective Primary ovarian small cell carcinoma of pulmonary type(SCCOPT)is a rare ovarian tumor with a poor prognosis.The platinum-based chemotherapy is the standard treatment.However,there is little research on the clinical characteristics of SCCOPT and the potential benefits of other treatments due to its low incidence.The study aims to investigate clinicopathological characteristics and treatment of SCCOPT.Methods We summarized the clinical,imaging,laboratorical and pathological characteristics of 37 SCCOPT cases,in which 6 cases were admitted to the Gansu Provincial Hospital from the year of 2008 to 2022 and 31 cases reported in 17 English and 3 Chinese literatures.Results The median age of the studied SCCOPT cases(n=37)was 56.00(range,22-80)years.Almost 80%of them had a stageⅢorⅣtumor.All patients underwent an operation and postoperative chemotherapy.Nevertheless,all cases had a poor prognosis,with a median overall survival time of 12 months.Immunohistochemical y,the SCCOPT of all patients showed positive expressions of epithelial markers,such as CD56 and sex-determining region of Y chromosome-related high-mobility-group box 2(SOX-2),and negative expressions of estrogen receptor,progesterone receptor,vimentin,Leu-7,and somatostatin receptor 2.The tumor of above 80%cases expressed synaptophysin.Only a few cases expressed neuron-specific enolase,chromogranin A,and thyroid transcription factor-1.Conclusions SCCOPT had a poor prognosis.SOX-2 could be a biomarker to be used to diagnose SCCOPT.

SURGICAL TREATMENT OF PRIMARY ESOPHAGEAL SMALLCELL CARCINOMA

Objective: To study the clinical biocharacteristics of primary esophageal small-cell carcinoma (PESC) and factors influencing prognosis and to find rational indications for combination therapy. Methods: To analyze the clinical materials of 47 patients who had undergone an operation with PESC and to compare it with those patients with esophageal squamous-cell carcinoma (ESCC) or primary esophageal adenocarcinoma (PEAC). Results: The overall resectability, morbidity and 30-day mortality rates of PESC were 93.6%, 17.0% and 2.1% respectively, similar to those of ESCC or PEAC. TNM staging and lymph node metastasis were the major determinants influencing long-term survival. Tumor length, depth of tumor invasion and type of operation had little influence on long-term prognosis. The 5-year survival rate of PESC was 7.5%, which was much lower than that of ESCC and PEAC (P<0.01). Among the 42 deceased patents, one died of anastomotic leakage and the others died of remote metastasis and recurrence. Adjuvant chemotherapy did not help improve the patients’ long-term survival. Conclusions: Compared with ESCC and PEAC, PESC is the most malignant type with early lymphatic and hematogenous metastases and poor prognosis. Lymph node metastasis is the major factor influencing the prognosis. Patients in stage 0, I and II a of PESC are indicated for surgical resection, while those in stage II b, III or IV should be managed with non-surgical combined therapy.

Clinicopathological Features and Prognosis of Small Cell Carcinoma of the Cervix

Small cell carcinoma of cervix (SCCC) is a rare disease with highly aggressive behaviour and is pathologically hard to diagnose.In this study, the clinicopathological features, diagnosis, treatment and prognosis of the condition were examined.Clinical records and follow-up data of 7 cases of SCCC were retrospectively studied.Our results showed that five non-recurrent cases initially presented irregular vaginal bleeding or increased apocenosis of varying degrees.Pathological examination revealed that the stroma was diffusely infiltrated with small monomorphous cells ranging from round to oval shape.Three cases were immunohistochemically confirmed.One case was accompanied with squamous cell cancer.Of the 7 cases, one case was classified as stage Ⅰb 1, two stageⅠ b2, one stage Ⅱ a, one stage Ⅱb , and one stage Ⅲ b.On the basis of their stages of condition, one subject with stage III b underwent chemotherapy, and one with stage Ib2 received extensive hysterectomy plus pelvic lymphadenectomy, while the other 5 cases were treated by extensive hysterectomy and pelvic lymphadenectomy in combination with pre-and/or post-operative adjuvant chemotherapy and radiotherapy.Of the 7 patients, 4 had relapse-free survival of 14, 14, 16 and 28 months respectively.It is concluded that SCCC is an aggressive tumor with propensity for early pelvis lymph node metastases.Early-stage patients should be treated by extensive hysterectomy and pelvic lymphadenectomy in combination with pre-and/or post-operative adjuvant chemotherapy and radiotherapy.

Relevance of EGFR gene mutation with pathological features and prognosis in patients with non-small-cell lung carcinoma

Objective:To study the relevance of EGFR gene mutation with pathological features and prognosis in patients with non-small-cell lung carcinoma.Methods:A total of 297 patients from July 2009 to May 2013 were chosen as objects.EGFR gene mutation were detected with fluorescence quantitative PCR.Relevance of EGFR gene mutation with clinical and pathological features was analyzed,and the prognosis of EGFR- mutant-patients and that of EGFR- wide type-patients was compared.Results:In 297 patients.136(45.79%) showed EGFR gene mutation.EGFR gene mutation had no significant relevance with age.gender,smoking history,family history of cancer and clinical stage(P>0.05);there was significant relevance between EGFR gene mutation and blood type,pathologic types,differentiation and diameter of cancer(P<0.05).The difference between prognosis of EGFR- mutant-patients and that of EGFR- wide type-patients was statistical significance(P<0.05).Conclusions:EGFR gene mutation has significant relevance with pathological features,the prognosis of EGFRmutant-paticnts is better than that of EGFR- wide type-patients.

Primary small cell carcinoma of kidney after renal transplantation:a case report and literature review

Extrapulmonary small cell carcinoma （EPSCC） is a rare neoplasm comprising 2.5％ to 5％ of small cell carcinomas （SCCs）.Bladder SCC is the most common site of genitourinary tract.Primary renal SCC is extremely rare.We report a case of primary SCC of the kidney which is rarely reported in the urinary tract and presents an aggressive clinical picture.A 59-year-old female visited a urologic clinic with complaint of persistent left flank soreness 10 years after undergoing renal transplantation.Abdominal computed tomography showed a left renal pelvis tumor.After the patient received left nephroureterectomy with bladder cuff resection,her pathology results showed SCC.After surgery,she received adjuvant systemic chemotherapy,and her recovery has been uneventful as of 8 months.Primary renal SCC presents with an advanced tumor stage and a short median survival period,therefore early intervention and close follow-up are recommended.

A case of long survival in poorly differentiated small cell carcinoma of the pancreas

Small cell carcinoma (SCC) of the pancreas is rare. It has similar histological features to pulmonary small cell carcinoma and is equally aggressive. Most patients with SCC in the pancreas reported in case studies died within 1 year after diagnosis. We present a case of unusually long-term survival after surgery and combined chemotherapy for SCC of the pancreas. A 62-year-old woman presented with epigastric pain and jaundice. Computed tomography revealed dilated common bile duct caused by external compression of the mass in the pancreatic head. Exploratory laparotomy and pancreaticoduodenectomy (PPPD) was performed with histopathological analysis confirming a primary small cell carcinoma of the pancreas. After an uneventful postoperative recovery, the patient was treated with 6 cycles of combined chemotherapy consisting of cisplantin and ectoposide. During the follow-up, there was no evidence of recurrence and the patient has remained in a good health condition for 36 mo since the diagnosis.

Primary small cell neuroendocrine carcinoma of the right posterior tongue

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.

Small cell carcinoma of the gastric remnant：a case report

Objective Small cell carcinoma(SCC) is mostly found in the lungs. It is extremely rare in the gastric remnant. Here, we report a case and review the literature in order to improve the diagnosis and treatment of SCC of the gastric remnant.Methods We report a case of SCC of the gastric remnant in a 71-year-old male Chinese patient who presented with epigastric pain, acid regurgitation, and belching and who underwent Billroth II gastrectomy more than 38 years ago.Results Physical examination showed no obvious abnormalities. Laboratory data were within normal limits, except for anemia. Pathology of the mass showed a protruded tumor measuring 5.0 × 5.0 × 2.5 cm at the anastomotic edge of the gastric remnant that infiltrated through the full wall of the stomach; this was confirmed by immunohistochemical staining for cytokeratin [CK(-)], leukocyte common antigen(LCA)(+), synaptophysin(+), CD56(+), and Ki-67(+ > 50%).Conclusion SCC of the gastric remnant is extremely rare, although the pathology, symptoms, diagnosis, treatment, and prognosis of SCC are similar to those of gastric SCC. Although the standard treatment of SCC of the gastric remnant remains unclear, effective surgical resection and subsequent multiagent chemotherapy should be performed for long-term survival. Our case shows the efficacy of tegafurgimeracil-oteracil-potassium capsule chemotherapy. Examination of a large series is required to determine the optimal treatment strategy for SCC of the gastric remnant.

Value of ^(18)F-FDG PET/CT and MRI in diagnosing primary endometrial small cell carcinoma

Primary small cell carcinoma（SCC） is a group of aggressive neoplasms that mainly arise from the lung and digestive tract. Endometrial small cell carcinoma（ESCC） is extremely rare. To our knowledge, less than 90 cases have been reported, and most of these reports were dedicated to describing the clinicopathologic or immunochemical features of ESCC. Herein, we present a new case of ESCC involving a 51-year-old woman and mainly focus on the magnetic resonance imaging（MRI） and positron emission tomography/computed tomography（PET/CT） findings. MRI showed that the uterus was significantly enlarged（11.6 cm × 11.1 cm × 14.4 cm）, and a giant irregular mass（7.5 cm × 8.4 cm × 8.5 cm） was observed in the uterine cavity. The lesion demonstrated an extremely low apparent diffusion coefficient（ADC） value [（0.553±0.088）×10^–3 mm^2/s] and a high FDG uptake value（22.7）. Multiple metastatic lymph nodes（LNs） were identified at different positions, with diameters ranging from 0.3 to 2.8 cm and a maximum standardized uptake value（SUV max） ranging from 6.9 to 19.3.

Squamous Cell Carcinoma of Small Intestine: a Case Report

NEOPLASMS derive from small intestine are rareand most cases are adenocarcinomas andcarcinoid.1 Squamous cell carcinoma of smallintestine is even rarer and only few casesreported in literature.1 In this article, we report a case of a68-year-old male who underwent a laparotomy due toperforation of the small intestine and was diagnosed withsquamous cell carcinoma of the small intestine.

Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater

Small cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare and different from the common ampullary adenocarcinoma. The ampullary adenoma is also a rare neoplasm and has the potential to develop an adenocarcinoma. Their coexistence has been rarely reported in the literature. We herein describe an unusual case of a small cell neuroendocrine carcinoma associated with a villous adenoma in the ampulla of Vater with emphasis on computed tomography (CT) and histopathological findings. We also discuss their clinical, histopathological and radiological features as well as possible histogenesis.

Small cell carcinoma of the liver and biliary tract without jaundice

An 80-year-old woman presenting with chest pain was found to have a large,lobulated soft tissue mass in the liver and nearby tissues on abdominal computed tomography(CT).The tumor had invaded the common hepatic artery and main portal vein.Jaundice developed 4 wk later,at which point,a pancreas and biliary CT scan revealed a large mass in the right lobe of the liver and a hilar duct obstruction,which was found to be a small cell carcinoma.Despite its rarity,liver and bile duct small cell carcinoma should be considered in the differential diagnosis of atypical chest pain without jaundice.

Multiple metastases to the small bowel from large cell bronchial carcinomas

AIM: Metastases from lung cancer to gastrointestinal tract are not rare at postmortem studies but the development of clinically significant symptoms from the gastrointestinal metastases is very unusual. METHODS: Formalin-fixed, paraffin-embedded tissues were cut into 5 urn thick sections and routinely stained with hematoxylin and eosin. Some slides were also stained with Alcian-PAS. Antibodies used were primary antibodies to pancytokeratin, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, vimentin, smooth muscle actin and CD-117. RESULTS: We observed three patients who presented with multiple metastases from large cell bronchial carcinoma to small intestine. Two of them had abdominal symptoms (sudden onset of abdominal pain, constipation and vomiting) and in one case the tumor was incidentally found during autopsy. Microscopically, all tumors showed a same histological pattern and consisted almost exclusively of strands and sheets of poorly cohesive, polymorphic giant cells with scanty, delicate stromas. Few smaller polygonal anaplastic cells dispersed between polymorphic giant cells, were also observed. Immunohistochemistry showed positive staining of the tumor cells with cytokeratin and vimentin. Microscopically and immunohistochemically all metastases had a similar pattern to primary anaplastic carcinoma of the small intestine. CONCLUSION: In patients with small intestine tumors showing anaplastic features, especially with multiple tumors, metastases from large cell bronchial carcinoma should be first excluded, because it seems that they are more common than expected.

Extrapulmonary small cell carcinoma of lymph node: Pooled analysis of all reported cases

AIM: To study clinical outcomes and management of lymph nodes extrapulmonary small cell carcinoma(LNEPSCC). METHODS: Herein, we perform a systematic search of published literature in the PubMed and EMBASE databases for studies describing LNEPSCC. For uniformity of reporting, LNEPSCC was staged as limited if it involved either single lymph node station or if surgery with curative intent had been undertaken. The disease was staged extensive if it involved two or more lymph node regions.RESULTS: The systematic literature review yielded eight descriptions(n = 14) involving cervical, submandibular and inguinal lymph nodes. Eleven(64.7%) patients had limited disease(LD) and six(35.3%) had extensive disease(ED) at presentation. Chemotherapy(n = 6, 35.3%) or surgery(n = 4, 23.5%) were the most common form of treatment given to these patients. Complete response was achieved in 12(70.6%) of the patients. Median(interquartile range) progression free survival and overall survival was 15(7-42) mo and 22(12.75-42) mo respectively. Of the three illustrative cases, two patients each had ED at presentation and achieved complete remission with platinum based combination chemotherapy.CONCLUSION: LNEPSCC is a rare disease with less than 15 reported cases in world literature. Surgical resection with curative intent is feasible in those with LD while platinum based combination chemoradiation is associated with favorable outcomes in patients with ED. Prognosis of LNEPSCC is better than that of small cell lung cancer in general.

Development and validation of a prognostic nomogram model for Chinese patients with primary small cell carcinoma of the esophagus

BACKGROUND Primary small cell carcinoma of the esophagus(PSCE)is a highly invasive malignant tumor with a poor prognosis compared with esophageal squamous cell carcinoma.Due to the limited samples size and the short follow-up time,there are few reports on elucidating the prognosis of PSCE,especially on the establishment and validation of a survival prediction nomogram model covering general information,pathological factors and specific biological proteins of PSCE patients.AIM To establish an effective nomogram to predict the overall survival(OS)probability for PSCE patients in China.METHODS The nomogram was based on a retrospective study of 256 PSCE patients.Univariate analysis and multivariate Cox proportional hazards regression analysis were used to examine the prognostic factors associated with PSCE,and establish the model for predicting 1-,3-,and 5-year OS based on the Akaike information criterion.Discrimination and validation were assessed by the concordance index(C-index)and calibration curve and decision curve analysis(DCA).Histology type,age,tumor invasion depth,lymph node invasion,detectable metastasis,chromogranin A,and neuronal cell adhesion molecule 56 were integrated into the model.RESULTS The C-index was prognostically superior to the 7th tumor node metastasis(TNM)staging in the primary cohort[0.659(95%CI:0.607-0.712)vs 0.591(95%CI:0.517-0.666),P=0.033]and in the validation cohort[0.700(95%CI:0.622-0.778)vs 0.605(95%CI:0.490-0.721),P=0.041].Good calibration curves were observed for the prediction probabilities of 1-,3-,and 5-year OS in both cohorts.DCA analysis showed that our nomogram model had a higher overall net benefit compared to the 7th TNM staging.CONCLUSION Our nomogram can be used to predict the survival probability of PSCE patients,which can help clinicians to make individualized survival predictions.

Detection of androgen receptor (AR) and AR-V7 in small cell prostate carcinoma: Diagnostic and therapeutic implications

Objective:Small cell prostate carcinoma(SCPC)is a rare and highly malignant subtype of prostate cancer.SCPC frequently lacks androgen receptor(AR)and prostate-specific antigen(PSA)expression,and often responds poorly to androgen deprivation therapy(ADT).AR splice variant-7(AR-V7)is a truncated AR protein implicated in resistance to AR-targeting therapies.AR-V7 expression in castration-resistant prostate cancers has been evaluated extensively,and blood-based detection of AR-V7 has been associated with lack of response to abiraterone and enzalutamide.However,whether AR-V7 is expressed in SCPC is not known.Methods:Using validated antibodies,we performed immunohistochemistry(IHC)assay for the full-length AR(AR-FL)and(AR-V7)on post-ADT surgical SCPC specimens.Results:Seventy-five percent(9/12)of the specimens showed positive staining for the AR-FL with various intensities.Thirty-three percent(4/12)of the specimens showed positive staining for AR-V7.Among the specimens with positive AR-V7 staining,two samples displayed very weak staining,one sample showed weak-to-moderate staining,and one sample showed strong staining.All positive specimens displayed a heterogeneous pattern of AR-FL/AR-V7 staining.All specimens positive for AR-V7 were also positive for AR-FL.Conclusion:The study findings support the existence of measurable AR-FL and AR-V7 proteins in SCPC specimens.The results also have implications in detection of AR-V7 in specimens obtained through systemic sampling approaches such as circulating tumor cells.A positive AR-V7 finding by blood-based tests is not impossible in patients with SCPC who often demonstrate low PSA values.

Small Cell Carcinoma:a Rare Subtype of Thyroid Cancer with Unanticipated Prognosis

The prognosis of small cell thyroid carcinoma(SCTC)in a large cohort has not been well reported in the literature.In this study,we analyzed the mortality of SCTC,in comparison to medullary thyroid cancer(MTC)and anaplastic thyroid cancer(ATC),based on the Surveillance,Epidemiology,and End Results(SEER)Program of the National Cancer Institute,to determine the prognosis of SCTC.Information regarding patients with a diagnosis of MTC,ATC,or SCTC,between 2004 and 2013,was acquired from the SEER database.Patient survival curves were assessed by Cox proportional hazards regression analyses,Kaplan-Meier analyses,and log-rank tests.In a Kaplan-Meier analysis of the entire cohort of thyroid cancer patients,cancer-specific survival declined sharply for patients with SCTC,but it declined more modestly for patients with MTC.The cancer-specific survival was not significantly different between SCTC and ATC.Unadjusted Cox regression analysis showed that SCTC had a higher cancer-specific mortality than MTC but a similar prognosis as ATC.SCTC showed a higher cancer-specific mortality than MTC and ATC after adjustments for various confounding factors.SCTC was found to have a more highly lethal clinical course than MTC and had a similar death rate to ATC.Therefore,we recommend that aggressive,radical treatment like surgery or radiation should be performed tor these patients.

Case Report:Breast metastasis from small cell lung carcinoma

Breast metastases from extramammary neoplasms are very rare. We presented a 66 year-old female with metastasis of small cell lung carcinoma to the breast. She presented with consolidation over the left upper lobe of her lung undetermined after endobronchial or video-assisted thoracoscopic surgery (VATS) biopsy,and this was treated effectively after antibiotic therapy at initial stage. The left breast lumps were noted 4 months later,and she underwent a modified radical mastectomy under the im-pression of primary breast carcinoma. However,the subsequent chest imaging revealed re-growing mass over the left mediastinum and hilum,and cells with the same morphological and staining features were found from specimens of transbronchial brushing and biopsy. An accurate diagnosis to distinguish a primary breast carcinoma from metastatic one is very important because the therapeutic planning and the outcome between them are different.