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.

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.

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.

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.

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.

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.

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 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.

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.

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.

Neuroendocrine carcinoma of the extrahepatic bile duct: Case report and literature review

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.

Prostatic Small Cell Carcinoma: Diagnosis and Management

Prostatic small cell carcinoma (PSCC) is a distinct clinical phenotype of prostate cancer. Although rare, this phenotype is highly aggressive with very high mortality. Due to this, it is imperative for clinicians to be aware of it, diagnose it early and treat it appropriately. In this article we discuss the current literature, outline a plan for its diagnosis and management, and highlight latest research on this topic.

Androgen-deprivation therapy-induced aggressive prostate cancer with neuroendocrine differentiation

Most prostate cancers （PCas） are classified as acinar type （conventional） adenocarcinoma which are composed of tumor cells with luminal differentiation including the expression of androgen receptor （AR） and prostate-specific antigen （PSA）. There are also scattered neuroendocrine （NE） cells in every case of adenocarcinoma. The NE cells are quiesecent, do not express AR or PSA, and their function remains unclear. We have demonstrated that IL8-CXCR2-P53 pathway provides a growth-inhibitory signal and keeps the NE cells in benign prostate and adenocarcinoma quiescent. Interestingly, some patients with a history of adenocarcinoma recur with small cell neuroendocrine carcinoma （SCNC） after hormonal therapy, and such tumors are composed of pure NE cells that are highly proliferative and aggressive, due to P53 mutation and inactivation of the IL8-CXCR2-P53 pathway. The incidence of SCNC will likely increase due to the widespread use of novel drugs that further inhibit AR function or intratumoral androgen synthesis. A phase II trial has demonstrated that platinum-based chemotherapy may be useful for such therapy-induced tumors.

Neuroendocrine carcinomas arising in ulcerative colitis:Coincidences or possible correlations?

Patients with inflammatory bowel disease （IBD） are at increased risk of colorectal malignancies. Adenocarcinoma is the commonest type of colorectal neoplasm associated with ulcerative colitis （UC） and Crohn＇s disease, but other types of epithelial and non-epithelial tumors have also been described in inflamed bowel. With regards to non-epithelial malignancies, lymphomas and sarcomas represent the largest group of tumors reported in association with IBD, especially in immunosuppressed patients. Carcinoids and in particular neuroendocrine neoplasms other than carcinoids （NENs） are rare tumors and are infrequently described in the setting of IBD. Thus, this association requires further investigation. We report two cases of neoplasms arising in mild left-sided UC with immuno- histochemical staining for neuroendocrine markers： a large cell and a small cell neuroendocrine carcinoma of the rectum. The two patients were different in age （35 years vs 77 years） and disease duration （11 years vs 27 years）, and both had never received immunosuppressant drugs. Although the patients underwent regular endoscopic and histological follow-up, the two neoplasms were locally advanced at diagnosis. One of the two patients developed multiple liver metastases and died 15 mo after diagnosis. These findings confirm the aggressiveness and the poor prognosis of NENs compared to colorectal adenocarcinoma. While carcinoids seem to be coincidentally associated with IBD, NENs may also arise in this setting. In fact, long-standing inflammation could be directly responsible for the development of pancellular dysplasia involving epithelial, goblet, Paneth and neuroendocrine cells. It has yet to be established which IBD patients have a higher risk of developing NENs.

以小细胞神经内分泌癌和鳞癌为表现的同时同叶双原发肺癌1例

多原发性肺癌(MPLC)是一种较为罕见的肺癌类型,小细胞肺癌及非小细胞肺癌同时存在于同一肺叶的MPLC更为罕见。我们报道一例63岁男性,既往无吸烟史,无恶性肿瘤家族史,在左肺上叶同时发现两个结节,一个结节以分叶状为特征,另一个以毛刺、空泡、胸膜牵拉为特征,术后病理提示小细胞神经内分泌癌和鳞癌,诊断为同时同侧同叶双原发肺癌(pT1bN0M0,IA2期),并对其CT特征进行分析,以提高临床医生对该病的认识。

宫颈小细胞神经内分泌癌预后因素分析及列线图模型构建

目的:分析宫颈小细胞神经内分泌癌(SCNECC)患者预后的重要风险因素,构建列线图预测患者总生存率。方法:从SEER数据库收集2000年至2018年诊断为SCNECC的553例患者数据,按7∶3分成建模组和验证组。采用最小绝对值收缩和选择算子(Lasso)回归和Cox回归分析筛选预后相关因素,构建列线图预后模型。通过一致性指数、校准曲线、ROC曲线和决策曲线对模型性能进行分析,比较模型与FIGO分期系统的预测性能。结果:年龄、FIGO分期、化疗、检出淋巴结状态和肝转移是SCNECC患者预后的独立影响因素。建模组和验证组模型的C指数分别为0.756和0.724,高于FIGO分期。ROC曲线结果表明,两组患者的1、3、5年AUC均大于0.75。校准图显示模型与实际一致性较好,决策曲线结果提示列线图模型临床收益更高。结论:年龄、FIGO分期、化疗、检出淋巴结和肝转移是SCNECC患者预后的独立影响因素,所构建的列线图模型准确且具有临床实用性,能为SCNECC患者提供可视化和量化的预后评估,为临床医生治疗决策提供参考。

Rare Tumors of Ovary: Case Report and Literature Review

Introduction: Large cell neuroendocrine carcinoma (LCNEC) or non small cell neuroendocrine carcinoma the ovary is a rare entity and is frequently associated with ovarian surface epithelial tumors. However, its association with serous carcinoma is rarer, in our knowledge tree cases has been described in international literature and the first case in Moroccan literature. Case Report: A 54-year-old woman presented with a pelvic mass measuring 15 cm in diameter. She underwent an exploratory laparotomy with resection of the pelvic mass. Diffuse and nodular intra-abdominal disseminations were observed. Immunohistochemistry stain confirmed the diagnosis of large cell neuroendocrine carcinoma with serous carcinoma. The patient received three courses of carboplatin and paclitaxel and she's still alivewith a decline of 6 months. Its clinicopathologic association is discussed and the literature is reviewed. Conclusion: In summary, ovarian LCNEC is an aggressive tumor with a tendency to present at advanced stage and cause death within a mean of 17 months after diagnosis;however, some patients, particularly those with stage I disease and/or those who have received platinum-based therapy, may have a more favorable prognosis.

原发性膀胱小细胞神经内分泌癌临床病理分析

目的 探讨原发性膀胱小细胞神经内分泌癌临床病理特征、免疫组织化学特点、诊断及与偶发前列腺癌的关系。方法 回顾性分析2013年1月至2022年9月在首都医科大学附属北京朝阳医院就诊的12例原发性膀胱小细胞神经内分泌癌患者的临床资料,经病理科2位副主任医师重新阅片后,按照2022版泌尿系统及男性生殖器官肿瘤世界卫生组织分类进行复核。通过检索电子病历及电话进行随访,随访自病理确诊开始至患者死亡或最后一次随访结束,截止日期2023年1月31日。结果 12例患者中,7例为pT3期,1例为pT4期;8例合并膀胱高级别尿路上皮癌、鳞状细胞癌等其他类型肿瘤,5例合并偶发前列腺癌。免疫组织化学染色结果显示,12例患者中,12例(100.0%)CD56阳性,10例(83.3%)Syn阳性,8例(66.7%)CgA阳性,Ki67增殖指数介于80%~90%;5例尿路上皮癌CK20、GATA3和CK7均为阳性;5例前列腺癌P504S均为阳性,P63和34βE12均为阴性。随访时间3~60个月,8例患者在随访期间死亡,中位生存时间15.5个月;4例患者生存。结论 原发性膀胱小细胞神经内分泌癌是罕见的泌尿系统肿瘤,侵袭性强,预后差。男性膀胱前列腺切除患者,前列腺组织应全部取材。如发现具有临床意义的前列腺癌,术后应监测前列腺特异性抗原水平。

宫颈小细胞神经内分泌癌的临床及影像表现分析

目的:总结宫颈小细胞神经内分泌癌的临床及影像表现。方法:回顾性分析经病理学确诊为宫颈小细胞神经内分泌癌的33例患者的临床资料与MRI及CT图像特征。结果:33例宫颈小细胞神经内分泌癌患者中淋巴结转移者占42.4%,HPV阳性者占82.4%。33例患者中81.8%为外生性,18.2%为内生性,外生性肿块中5例呈类圆形及类椭圆形,22例呈不规则形及分叶形。肿块的CT表现呈不均匀的轻度到中度强化,MRI表现呈等T 1稍长T 2信号且T 2信号均匀,病灶DWI呈高信号,ADC值平均值为(0.54±0.11)×10^(-3)mm^(2)/s。结论:宫颈小细胞神经内分泌癌影像表现多为外生性肿块,CT及MRI增强呈不均匀强化,其中CT多呈轻中度强化,MRI呈等T 1稍长T 2信号,信号较为均匀,DWI明显扩散受限。

宫颈小细胞神经内分泌癌的磁共振表现

目的分析宫颈小细胞神经内分泌癌(small cell neuroendocrine carcinoma of the uterine cervix,SCNECC)的磁共振(MRI)表现。方法收集SCNECC患者22例、宫颈鳞癌20例,分析SCNECC的临床及病理特点、MRI表现,并测量表观扩散系数(ADC),比较两组之间的ADC值差异。结果SCNECC组平均发病年龄明显低于宫颈鳞癌(48.68 vs 62.65,P=0.00)。MRI上表现为T2WI均匀/不均匀高信号,部分病灶(9/22)内可见小片状更高T2WI信号区,增强扫描呈不均匀中等强化,以病灶周围强化明显,可见明显强化的纤维间隔。弥散加权成像(DWI)上呈明显高信号,鳞癌平均ADC值为(0.99±0.21)×10^(-3)mm^(2)/s,SCNECC平均ADC值为(0.63±0.20)×10^(-3)mm^(2)/s,两者的差异具有统计学意义(P=0.00)。复合性SCNECC与单纯性SCNECC的ADC值无明显差异(P=0.19)。结论SCNECC发病年龄较鳞癌低,MRI上T2WI及增强序列具有一定的特征性,ADC值有助于鉴别SCNECC与宫颈鳞癌。