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.

Gastric metastasis of small cell lung carcinoma:Three case reports and review of literature

BACKGROUND Small cell lung carcinoma(SCLC)is highly susceptible to metastasis in the early stages of the disease.However,the stomach is an uncommon site of metastasis in SCLC,and only a few cases of this type of metastasis have been reported.Therefore,SCLC gastric metastases have not been systematically characterized and are easily missed and misdiagnosed.CASE SUMMARY We report three cases of gastric metastasis from SCLC in this article.The first patient presented primarily with cough,hemoptysis,and epigastric fullness.The other two patients presented primarily with abdominal discomfort,epigastric distension,and pain.All patients underwent gastroscopy and imaging examinations.Meanwhile,the immunohistochemical results of the lesions in three patients were suggestive of small cell carcinoma.Finally,the three patients were diagnosed with gastric metastasis of SCLC through a comprehensive analysis.The three patients did not receive appropriate treatment and died within a short time.CONCLUSION Here,we focused on summarizing the characteristics of gastric metastasis of SCLC to enhance clinicians'understanding of this disease.

Small cell lung carcinoma with KIF5B-RET fusion partially responded to the 4^(th)-line therapy with anlotinib:A case report

BACKGROUND Small cell lung cancer(SCLC)exhibits a pronounced tendency for metastasis and relapse,and the acquisition of resistance to chemotherapy and radiotherapy,leading to complexity in treatment outcomes.It is crucial to tackle these challenges by advancing targeted therapeutic approaches in ongoing research endeavors.Variant RET fusions have been reported in several solid tumors,but are rarely reported in SCLC.CASE SUMMARY We present the first case of a KIF5B-RET fusion in a 65-year-old male patient with SCLC.To date,the patient has received the 4th line chemotherapy with anlotinib for one year and has shown a sustained favorable partial response.According to the results of next generation sequencing,this SCLC patient harbors the KIF5BRET fusion,suggesting that RET fusion could serve as a promising molecular target for SCLC treatment.Next-generation sequencing(NGS)plays a critical rolein comprehensively assessing the genotype and phenotype of cancer.CONCLUSION NGS can provide SCLC patients with personalized and targeted therapy options,thereby improving their likelihood of survival.

Gastric metastasis of small cell lung carcinoma:A rare but noteworthy entity to consider

Small cell lung carcinoma(SCLC)is an aggressive malignancy known for its propensity for early and extensive metastatic spread.Gastric metastasis,where cancer cells disseminate from the lung to the stomach,is a rare but increasingly recognized complication of SCLC.This review provides a comprehensive overview of gastric metastasis in SCLC,addressing its clinical significance,diagnostic challenges,management strategies,and prognosis.Additionally,it examines the broader metastatic patterns of SCLC and compares them with other malignancies known for gastric metastasis.Gastric metastasis in SCLC,though infrequent,is clinically significant and often indicates advanced disease with a poor prognosis.SCLC typically metastasizes to the liver,brain,bones,and adrenal glands,with the stomach being an unusual site.The incidence of gastric meta-stasis ranges from 1%to 5%in autopsy studies,although this may be underes-timated due to diagnostic difficulties and asymptomatic early lesions.Diagnosing gastric metastasis presents several challenges,including the asymptomatic nature of many cases,limitations of conventional imaging techniques,and difficulties in distinguishing metastatic lesions from primary gastric cancer via endoscopy.Histopathological diagnosis requires careful examination to identify SCLC cells through their characteristic small cell morphology and neuroendocrine markers.Management of gastric metastasis in SCLC typically involves a multidisciplinary approach.Systemic therapy,pri-marily chemotherapy,remains the cornerstone of treatment,with palliative care addressing symptoms and complications.Surgical intervention is usually reserved for specific cases requiring symptomatic relief.The prognosis for patients with gastric metastasis from SCLC is generally poor,reflecting the advanced stage of the disease.Median survival is significantly re-duced compared to patients without gastric metastasis.This review emphasizes the need for enhanced awareness and early detection to improve patient outcomes and highlights the importance of ongoing research into better diagnostic and therapeutic strategies.

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.

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.

Sternocleidomastoid flap for reconstruction of tongue small cell carcinoma: A case report

BACKGROUND The management of tongue carcinoma is excision and radical neck dissection followed with reconstruction.This is a case report of a patient with tongue squamous cell carcinoma(SCC)who underwent the procedure with sternocleidomastoid(SCM)flap reconstruction.CASE SUMMARY A 52-year-old woman without smoking history complained tongue ulcer since 3 years ago.Based on the histopathological examination,the patient was diagnosed with T2N2M0 right tongue SCC and underwent wide excision of tumor;right mandibular;neck dissection and were reconstructed with SCM flap.CONCLUSION SCC of the tongue requires wide excision and dissection of the neck and mandible if infiltration into the surrounding lymph nodes has been found.The SCM flap reconstruction could be used post-surgery.

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.

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.

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.

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.

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.

Advance in the Studies on Small Cell Neuroendocrine Carcinoma of the Paranasal Sinuses

Small cell neuroendocrine carcinoma (SCNEC) of the paranasal sinuses is extremely rare,with an unclear pathogenesis.The presence of neuroendocrine granules is suggestive of neuroendocrine differentiation.It was reported that this disease relates to the presence of accessory salivary glands,and some basic research has shown that it might originate from the multi-potent stem cells.There are no specific clinical symptoms but rhinal and ophthalmological symptoms are found in most cases.Diagnosis mainly depends on histopathological manifestations,immunohistochemical results and features of the electron microscopic ultra-structure.Pathological differentiation from poorly differentiated squamous carcinoma,melanoma,esthesioneuroblastoma and neuroglioma etc.is needed.No unified regimen has been employed in treating the disease.At present,combined therapy has a manifest therapeutic effect,such as success with the 2003 French regimen.Tumor relapse is common and prognosis is poor.A complete combined treatment plan will be helpful to improve the prognosis.

Endoscopic diagnosis of early-stage primary esophageal small cell carcinoma: Report of two cases

BACKGROUND Primary esophageal small cell carcinoma(PESCC)is a highly aggressive malignancy,and its detailed clinical behaviors have remained virtually unknown.Because of the rapid tumor progression,the diagnosis of esophageal small cell carcinoma at early stage is extremely difficult in clinical practice.Currently,only a handful of PESCC cases have been reported.CASE SUMMARY Case 1:A 62-year-old man was diagnosed with an esophageal submucosal tumor by endoscopy.Endoscopic ultrasonography showed a 0.8 cm low echo nodule in the muscularis mucosa.As the patient refused to undergo endoscopic resection,neoplasia was detected by endoscopy 1 year later.Case 2:A 68-year-old woman was diagnosed as having an esophageal submucosal tumor by endoscopy at a local hospital.About 2 wk later,we performed endoscopic ultrasonography and found a 1 cm low echo nodule in the muscularis mucosa;the submucosal was thinner than normal but still continuous;mucosal hyperemia and erosion were found on the surface of the tumor.Endoscopic submucosal dissection(ESD)was performed and the histopathological finding showed a small cell carcinoma invading the submucosal layer.CONCLUSION Early esophageal small cell carcinoma shows submucosal infiltrating growth with a hypoechoic mass in the muscularis mucosa as diagnosed by endoscopic ultrasonography.It is easily misdiagnosed as submucosal masses.Endoscopic manifestations should be identified and pathological biopsies should beemployed. ESD may be performed to provide an opportunity for early treatmentof PESCC.

Glabridin and Anti-Non-Muscle Myosin IIA Therapy Disrupts Non-Small Cell Lung Carcinoma Motility

Lung cancer is the leading cause of cancer related death in the United States killing over 130,000 people each year. While a combination of chemo and radiation therapy may be effective, surgery is still required for many patients. Without surgery, the disease may progress and lead to metastases. We sought to determine if treatment with anti-non-muscle myosin IIA antibody would inhibit movement of the cells in the presence and absence of glabridin (an isoflavonoid compound shown to inhibit cell migration by inhibiting myosin). We compared inhibition by glabridin to that of an anti-non-muscle myosin IIA antibody and a combination therapy of both at 12 and 24 hours post wound creation. Cells that took up the anti-non-muscle myosin IIA antibody were greatly inhibited in motility and exhibited no significant change in wound healing. Glabridin treatment resulted in a dramatic increase in wound size within 12 hours and regeneration within 24 hours. The greatest decrease in motility was observed in cells treated with the combination of both glabridin and anti-non-muscle myosin IIA antibody. By 24 hrs, cell migration had halted due to death of the cells resulting from this combination. Further testing needs to be done to determine a safe mode of delivery of the combination therapy to ensure only local distribution. Controlled release drug delivery depot systems have been used as a means to provide local release of drugs intra-tumorally or adjacent to the cancerous tissue after surgical resection and have great potential.