Neuroendocrine tumours of the urinary system and male genital organs: clinical significance

Two basic types of neuroendocrine (NE) tumours with diverse clinicopathological features and outcome are identified in the urinary system and male genital organs: carcinoid tumour and neuroendocrine carcinoma. Carcinoid, a rare tumour, occurs in the kidney, bladder, prostate and testis. It is morphologically, histochemically, immunohistochemically and ultrastructurally similar to its counterpart in other organs, such as lung or gastrointestinal tract. Metastases can be detected at the initial evaluation, although they have been reported up to several years after removal, emphasizing the need for a long‐term follow‐up. NE carcinoma occurs in the kidney, bladder and prostate, and includes small cell carcinoma (SCC) and large cell NE carcinoma (LCNEC), the latter being exceedingly rare. Both show the morphology and immunophenotype of NE carcinoma originating in other organs. Although the occurrence is rare, it is highly aggressive.     

Use of octreotide and lanreotide in the treatment of symptomatic non-resectable carcinoid tumours

Background: Carcinoid tumours are rare neoplasms that secrete hormones and biogenic amines, most commonly serotonin. Octreotide and long acting lanreotide are found to be useful in the management of carcinoid syndrome by its interaction with somatostatin receptor, found on the carcinoid tumour. The aim of this study is to look at the efficacy of octreotide and long acting lanreotide in the treatment of symptomatic non‐resectable carcinoid tumours. Method: The effects of octreotide and long‐acting lanreotide were studied in 10 patients with symptomatic non‐resectable carcinoid tumours. Results: Symptom improvement occurred in nine of 10 patients. Three patients responded only to octreotide, three patients responded to both octreotide and long‐acting lanreotide and three patients only responded to long‐acting lanreotide. Slight reductions in 24‐h urine 5‐hydroxyindoleacetic acid levels occurred in three of six patients but no patients were found to have objective tumour regression on computed tomography scan. Conclusions: Octreotide and long‐acting lanreotide are useful palliative treatments for the control of symptoms in patients with non‐resectable carcinoid tumours but there is no evidence of tumour stasis.     

Carcinoma of the ampulla of vater

Summary Endoscopically placed biliary endoprostheses were used to treat obstructive jaundice in 71 patients with ampullary carcinoma. Successful placement of an endoprosthesis was achieved in 68 patients (95.8%). Bilirubin declined in 67 patients (98.5%). There was no procedure-related mortality. Twenty-two patients (31%) received further surgical therapy, and 47 received an endoprosthesis as their only therapeutic intervention. In the latter group, bilirubin normalized in 44 of 46 patients surviving longer than 30 days (95.7%). Mean survival was 466 days (median 410, range 23-1515), which compares favorably with surgical palliation. Complications mainly involved clogging of the endoprosthesis, which was easily treated endoscopically and, more significantly, duodenal stenoses secondary to continued tumor growth in almost 25% of the patients. Although endoscopic drainage is a safe and effective method of relieving biliary obstruction in patients with ampullary carcinoma, we feel it should be reserved for poor surgical candidates and for those patients with a limited life expectancy due to metastatic disease.     

Systemic treatment of pancreatic neuroendocrine tumors

Pancreatic neuroendocrine tumours (pNET) are a group of heterogeneous tumours. Despite being rare, their incidence is rising. While localized lesions can be cured with surgical resection, most patients present with metastatic disease. Systemic therapy is thus integral for the management of this rare entity. Numerous advancements have been made in the past decade in the systemic treatment of unresectable, metastatic pNET. Treatment options for pNET include somatostatin analogues, cytotoxic chemotherapy, targeted agents and peptide receptor radionuclide therapy. In this Review, we present the latest evidence for these treatment options.     

Neuroendocrine carcinoma of the sigmoid colon: Report of a case

(Received for publication on Apr. 27, 1998; accepted on Mar. 11, 1999)     

Neuroendocrine neoplasms of the sinonasal region

Neuroendocrine neoplasms of the sinonasal region, which are relatively uncommon but clinically very important, are reviewed here in the light of current knowledge. Using a definition for neuroendocrine based on phenotypic, histologic, immunohistochemical, and electron microscopic features rather than histogenetic criteria, sinonasal neuroendocrine carcinomas are examined with a particular emphasis on the small‐cell and large‐cell subtypes. This is followed by revisiting olfactory neuroblastoma because it is also a tumor that shows a neuroendocrine phenotype. Kadish clinical and Hyams histologic grading systems as prognosticators of olfactory neuroblastoma are also considered in detail. Finally, controversies regarding sinonasal undifferentiated carcinoma as a neuroendocrine tumor are discussed and a possible relationship with high‐grade olfactory neuroblastoma is explored. Genetic events and current management of these tumors are also outlined. © 2015 Wiley Periodicals, Inc. Head Neck 38 : E2259–E2266, 2016     

Pulmonary Neuroendocrine Tumors

Pulmonary neuroendocrine tumors represent a morphologic spectrum of tumors from the well-differentiated typical carcinoid tumor, to the intermediate-grade atypical carcinoid tumor, to the high-grade neuroendocrine carcinomas composed of small-cell carcinoma and large-cell neuroendocrine carcinoma. The addition of immunohistochemistry in diagnostics is helpful and often essential, especially in the classification of large-cell neuroendocrine carcinoma. The importance of the intermediate-grade atypical carcinoid group is underscored by the impact of this diagnosis on therapy. The distinction of pulmonary small-cell carcinoma from large-cell neuroendocrine carcinoma, despite both being in the high-grade group, is of relevance to the therapeutic approach to these tumor types.     

Somatostatinoma of the ampulla of vater in celiac sprue

The increased incidence of gastrointestinal lymphoma and adenocarcinoma in patients with celiac sprue is well recognized, with 10% to 15% developing a gastrointestinal malignancy. Somatostatinomas are rare neuroendocrine tumors that occur most commonly within the pancreatic head or duodenum. Although fewer than 100 cases have been reported, somatostatinomas are often associated with multiple endocrine neoplasia-1 syndrome and yon Recklinghausen’s disease. The unusual case of a 43-year-old woman with celiac sprue in which a somatostatinoma involving the ampulla of Vater was identified and resected is presented. To our knowledge, somatostatinomas have not been previously reported in patients with celiac sprue. Presented at the 1999 Americas Hepato-Pancreato-Biliary Congress, Ft. Lauderdale, Fla., February 18–21, 1999 (poster presentation).     

膀胱小细胞神经内分泌癌1例报告并文献复习

目的：探讨膀胱小细胞神经内分泌癌的病理及临床特征,提高对该病的认识和诊洽水平。方法：介绍1例94岁高龄膀胱小细胞神经内分泌癌患者的诊治情况及病理特点,并复习有关文献,分析该肿瘤的组织来源、病理和免疫组化特征、诊治及预后情况。结果：本例行经尿道膀胱肿瘤电切术,术后病理诊断为小细胞神经内分泌癌。免疫组化染色示肿瘤细胞表达神经内分泌标记物NSE和CgA,肿瘤标志物Ki-67、C-erB-2、P53表达阳性,PSA、Syn表达阴性。患者术后1个月死亡。结论：膀胱小细胞神经内分泌癌是一种比较少见的高度恶性肿瘤,主要依靠病理及免疫组化诊断,早期膀胱镜检查及活检有助于诊断。     

原发性肝脏神经内分泌癌3例报道

目的总结原发性肝脏神经内分泌癌的诊治经验。方法回顾性分析我院2010年6月至2011年6月1年间约1000例肝切除病例中发现的3例原发性肝脏神经内分泌癌的临床病理资料。结果 3例病例术前结合临床资料难以诊断原发性神经内分泌癌,治疗方式均采用手术切除,经病理检查及免疫组织化学报告3例均属于低分化神经内分泌癌,术后均使用奥曲肽辅助治疗,患者康复出院,随访1年肿瘤无明显复发。目前3例患者仍在随访中。结论原发性肝脏神经内分泌癌的术前诊断较为困难,治疗和疗效的评估目前在探索中。     

Neuroendocrine Neoplasms of the Larynx: A Clinicopathologic Analysis of 27 Neuroendocrine Tumors and Neuroendocrine Carcinomas

Laryngeal neuroendocrine neoplasms (NENs) are rare and heterogeneous, encompassing well-differentiated neuroendocrine tumors (NETs; grade 1, 2, and 3), neuroendocrine carcinomas (NECs, small cell and large cell types), and mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN). We aimed to study the clinicopathologic spectrum of these neoplasms. A retrospective review of all primary laryngeal NENs diagnosed from 2005 to 2017 was undertaken. Mitotic index was divided into < 2, ≥ 2–10, and > 10 mitoses/2 mm², with a Ki-67 labelling index of < 2%, ≥ 2–20%, and > 20% for the NET grade 1, 2 and 3 categories, respectively. A total of 27 patients were included. The median age at presentation was 60 years; the male-to-female ratio was 8:1. Supraglottis (n = 22) was the most frequently affected subsite. There were 9 NETs grade 2 (G2), and 18 NECs cases. There were no NET grade 1 or 3 cases in our cohort. Among the NETs G2, the morphology was epithelioid (2), plasmacytoid (3), clear (2), oncocytic (1), and rhabdoid (1). Unique ‘glomeruloid structures’ (n = 5), calcification (n = 3), lymphoid aggregates (n = 5), intranuclear inclusions (n = 2), hyaline globules (n = 3), and Leisegang rings (n = 2) were identified. NECs comprised 16 small cell neuroendocrine carcinoma and 2 large cell neuroendocrine carcinoma. On immunohistochemistry, tumor cells expressed AE1/AE3 (86%), synaptophysin (100%), chromogranin (100%), INSM1 (100%), calcitonin (33.3%). In the NEC group, p53 aberrant expression (87.5%), Retinoblastoma (Rb) loss (88.2%), and diffuse p16 immunoreactivity (66.7%) were additionally observed. Lymph-node metastasis was detected in 62.5% and 85.7%, while distant metastasis in 55.6% and 76.9%, respectively in NET G2 and NEC. Laryngeal NENs are aggressive neoplasms with a high rate of nodal and distant metastasis. Awareness of the wide pathologic spectrum of laryngeal NENs and appropriate use of IHC is needed to render an accurate diagnosis. Ki67 assessment is strongly recommended for laryngeal NEN prognosticationSupplementary InformationThe online version contains supplementary material available at 10.1007/s12105-021-01367-9.