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.

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 of the prostate with negative CD56,NSE,Syn,and CgA indicators:A case report

BACKGROUND Small-cell carcinoma of the prostate(SCCP)is a clinically rare malignant tumor,accounting for<1%of all prostate tumors.However,negativity for all SCCP neuroendocrine markers is rare.Herein,we report a case of SCCP with completely negative neuroendocrine markers and explore its clinicopathologic features,thus improving the understanding of its clinical diagnosis and management.CASE SUMMARY We report the case of a 48-year-old patient with SCCP negative for common sensitive neuroendocrine-staining indicators.Dysuria was the first symptom,and rectal examination revealed a hard prostate,palpable nodules,diffuse prostate enlargement,no pressure pain,no blood staining in the finger sleeve,1.33 ng/mL total prostate-specific antigen level,and a free-to-total prostate-specific antigen ratio of 0.21 ng/mL.Ultrasound suggested a prostate size of 5.3 cm×5.8 cm×5.6 cm,and magnetic resonance imaging suggested prostate cancer.The lower posterior bladder wall,rectal mesentery,and bilateral seminal vesicles were invaded,with multiple lymph node metastases in the pelvis.A whole-body bone scan suggested an abnormally active multiple bone metabolism and possible bone metastases.Head and lungs computed tomography revealed no significant nodal shadow.Following a pathological diagnosis of SCCP after a prostate puncture,with negative indicators of common sensitive neuroendocrine staining,chemotherapy was administered;the patient died 4-5 mo after SCCP diagnosis.CONCLUSION SCCP is a rare disease characterized by atypical clinical symptoms,limited treatment options,a short survival period,and a poor prognosis.

Small-cell neuroendocrine carcinoma of the prostate： are heterotransplants a better experimental model？

Small-cell neuroendocrine carcinoma of the prostate （SCNCP） is an uncommon type of prostate cancer. However, it is of clinical importance because it is one of the most aggressive tumors of the prostate with a very poor prognosis. There exist few artificially cultured tumor cell lines to study SCNCE Then, another approach to that study consists in the use of fresh tumor tissue obtained from patients and its heterotransplantation into host mice. The purpose of this review is to integrate data from more than 20 years of heterotransplantation research in the study of small-cell neuroendocrine carcinoma of the prostate （SCNCP）. Heterotransplantation has provided data regarding the histopathology, karyotype, DNA content, cell cycle frequency, tumor markers, androgen receptor expression, metastasis and take rate of this prostate disease. When possible, comparisons between original in situ specimens removed from patients and heterotransplanted tissue from host mice have been made. There are advantages, as well as limitations, that have been identified for SCNCP heterotransplants versus xenotransplantation of cultured cells. Overall, heterotransplanted tumors are better than conventional tumor xenografts at retaining tumor morphology, pathology, secretory activity and expression of tumor markers of the patient＇s original specimen. Furthermore, heterotransplanted tissue preserves the three-dimensional tumor architecture of the prostate to maintain critical stromal-epithelial cell interactions.

Pang Fuwan Uses Yao Medicine to Observe the Therapeutic Effects on the Physical and Mental Symptoms of Patients with Advanced Non-Small Cell Lung Cancer

Objective: Investigate the efficacy and safety of Yao Medicine in the treatment of advanced non-small-cell lung carcinoma, and explore the best therapeutic measure for clinical benefit. Methods: From July 2020 to July 2022, 84 patients with advanced non-small-cell lung carcinoma were selected and randomly divided into the Observation Group and control group, and the control group was treated with routine Western medicine, with 42 cases in each group. The activity of daily living (ADL) was assessed before and after treatment, meanwhile, the self-rating depression scale (SDS) and self-rating anxiety SAS (SAS) were used to assess the improvement of a bad mood, and quality of life SF-36 was used to assess the quality of life, to judge the efficacy and safety. Results: The effective rate of observation group was 91.67%. The effective rate of the control group was 76.19%. The effective rate of the observation group was significantly higher than that of the control group (P 0.05). There were no significant differences in the scores of SDS, SAS and quality of life between the two groups before treatment (P > 0.05), and after treatment, the scores of SDS, SAS and quality of life in the two groups were compared with those in the control group (P > 0.05), the scores of VAS, SDS and SAS decreased significantly, while ESCV, angle of straight leg elevation, ADL, physiological score, emotional score, social score and health status score increased significantly, the difference was statistically significant (P 0.05). Conclusion: Yao Medicine can improve the psychosomatic symptoms of patients with advanced non-small-cell lung carcinoma better, with better efficacy and higher safety.

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.

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.

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.

The 2002 AJCC TNM classification is a better predictor of primary small cell esophageal carcinoma outcome than the VALSG staging system

Small cell carcinoma of the esophagus (SCCE) is a rare and aggressive malignant tumor with a poor prognosis. The optimal disease staging system and treatment approaches have not yet been defined. This study aimed to evaluate the prediction of different staging systems for prognosis and treatment options of SCCE. We retrospectively accessed the clinicopathologic characteristics, treatment strategy, and prognosis of 76 patients diagnosed with primary SCCE between 2001 and 2011. The 1-, 2-, 3-, and 5-year overall survival rates were 58%, 31%, 19%, and 13%, respectively. Univariate analysis showed that the 2002 American Joint Committee on Cancer (AJCC) tumor-node-metastasis (TNM) classification (P=0.002), Veterans Administration Lung Study Group (VALSG) stage (P=0.001), predisposing factors (P<0.001), T category (P=0.023), and M category (P<0.001) were prognostic factors for overall survival. Multivariate analysis showed that the 2002 AJCC TNM stage (P<0.001) was the only independent prognostic factor for survival. The value of the area under the receiver operator characteristic (ROC) curve (AUC) of the 2002 AJCC TNM staging system was larger than that of VALSG staging system with regard to predicting overall survival (0.774 vs. 0.620). None of the single treatment regimens showed any benefit for survival by Cox regression analysis. Thus, the 2002 AJCC TMN staging system improved the prediction of SCCE prognosis; however, the optimal treatment regimen for SCCE remains unclear.

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.

A case report of primary small cell carcinoma of the breast and review of the literature

Primary small cell carcinoma (SCC) of the breast, an exceedingly rare and aggressive tumor, is often characterized by rapid progression and poor prognosis. We report a case of primary SCC of the breast that was diagnosed through pathologic and immunohistochemical examinations. Computed tomography (CT) scans failed to reveal a non-mammary primary site. Due to the scant number of relevant case summaries, this type of tumor is proved to be a diagnostic and therapeutic challenge. Therefore, we also reviewed relevant literature to share expertise in diagnosis, clinicopathologic characteristics, treatment, and prognosis of this type of tumor. Future studies with more cases are required to define more appropriate treatment indications for this disease.

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.

Review of the treatment of metastatic non small cell lung carcinoma:A practical approach

In recent years,as we have a better knowledge and understanding of the biology of non small cell lung carcinoma(NSCLC),which leads us to targeting biomarkers driving the NSCLC carcinogenesis and metastatic potential,we now have an increased number of options to offer our patients with NSCLC.We also realize the importance of distinguishing squamous and non squamous histology to guide our treatment decisions of NSCLC.The palliative care concomitant with therapies from the very start of the treatment also showed an impact on survival.This review examines the treatment options in all lines of therapy for metastatic NSCLC that have been approved in Canada,the United States,or Europe.

Progress in Diagnosis and Treatment of Small Cell Carcinoma of the Cervix

Small cell carcinoma of the cervix （SCCC） belongs to the neuroendocrine carcinomas, and it is a rare gynecological tumor of high-potential malignancy. It has a poorer prognosis compared to cervical squamous cancer or adenocarcinoma, and the therapeutic regimen of the disease differs. Diagnosis is based on pathomorphological characteristics, i.e., the small and round cancer cells （oat cell） which are uniform in shape and size, with the immunohistochemical marker helpful for diagnosis. Combined therapy is first recommended, Postoperative chemotherapy with platinum/etoposide （PE）, vincristine/adriamycin/cyclophosphamide （VAC） and taxel/carboplatin （TP） can markedly improve the prognosis of early SCCC patients.

SMALL CELL CARCINOMA OF THE URINARY BLADDER: THREE CASES REPORT

To study the clinical features of patients with primary small cell carcinoma (SCC) of the bladder and to improve the diagnosis and treatment. Methods: Clinical data of 3 cases with primary SCC of the bladder were discussed and the pathology, diagnosis, treatment and prognosis were reviewed. Results: 3 cases of primary SCC of the bladder were presented. Of them the diagnosis was confirmed by pathological examination after operation (2 cases) and biopsy (1 case). One case with stage T4M1 died after three months?chemotherapy. One case with stage T2M0 underwent partial cystectomy and was treated with chemotherapy and one year later died of miocardial infarction. Another case with stage T4M0 underwent radical cystectomy and postoperative irradiation therapy. The patient was alive and had no recurrence of symptoms during two years follow-up. Conclusion: Primary SCC of the urinary bladder is highly malignant. Radical cystectomy combined with radiotherapy appears to be the efficient treatment. Chemotherapy seems to be of no significant effect.

Is There Any Indication for Prophylactic Brain Irradiation in the Management of Small Cell Prostate Cancer?

Small cell prostate carcinoma (SCPC) is an extremely rare pathology with an aggressive behavior, characterized by early brain metastases. We describe three cases of SCPC where brain metastases occurred despite response to chemotherapy. The benefit of prophylactic brain irradiation (PBI), as part of the management of SCPC, is discussed and compared to its indications in small cell lung cancer.

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.

Bladder-Sparing Approach with Radiotherapy in Patients with Small Cell Carcinoma of the Bladder

Small cell carcinoma (SCCB) of the bladder is rare and has a poor prognosis. Because of its rarity, gold standard care has not been established. The purpose of this study was to analyze the feasibility of a bladder-sparing approach. Data for treatments and outcomes in patients with a diagnosis of SCCB who received bladder-sparing treatment in our facility in the period from February 2007 to August 2012 were retrospectively analyzed. Six eligible patients received definitive radiotherapy in the cancer network of Tohoku region. Mean age of the patients was 69.5 years (range: 44 - 83 years), and median follow-up period was 29.2 months (range: 4.1 - 54.5 months). The mean dose was 60 Gy (range: 60 to 63 Gy), and 1.2 - 2.0 Gy was given per fraction. The initial CTV was the whole pelvis or the small pelvis, and it was shrunk to the bladder or tumor as a boost. The 1-year and 3-year overall survival rates were 83.3% and 55.6%, respectively. The 1-year and 3-year local control rates were both 80.0%. Only one patient had local failure. Recurrence or metastasis in sites other than the brain occurred in three (50%) of the patients after primary treatment. Two of those patients did not receive any chemotherapy. None of the patients had serious toxicities, and the bladder could be preserved in all patients. In this study, radiotherapy was effective for patients with SCCB. There was no recurrence in the bladder, and it was possible to preserve the bladder in all patients. Distant metastases were observed frequently, and chemotherapy was considered to be essential. Local treatment with radiotherapy has an important role from the point of view of the patient’s QOL.

Expressions of FEZ1 and Survivin, and their significance in small cell lung cancer and squamous cell lung carcinoma

Objective： To detect the expressions of FEZ1 and Survivin in small cell lung cancer （SOLO） and poorly differentiated squamous cell carcinoma （PDSCC）, and to approach a theoretical basis for clinical diagnosis and treatment. Methods： Immunohistochemical and flow cytometry method were used to detect the expressions of FEZ1 and Survivin. Apoptosis ratio and cell proliferation index in normal lung tissue, SCLC and PDSCC were analyzed. Results： The expressions of FEZ1 and Survivin were significantly different between SCLC and PDSCC （P 〈 0.05）. The apoptosis ratio and proliferation index of normal lung tissue were lower than those of PDSCC and SOLO, with a significant difference （P 〈 0.05）. Conclusion： The expressions of FEZ1 and Survivin are significantly different between SCLC and PDSCC, indicating that detecting the expressions of the two indexes may be helpful for clinical diagnosis.