Neuroendocrine tumors of the pancreas

Neuroendocrine tumours are rare and often include insulinoma, gastrinoma and other low frequency tumours that secrete gastrointestinal hormones. Their preoperative localization, despite continuous medical advances, is extremely difficult but helpful in guiding the surgeon towards a proper form of treatment. After presenting their cases, the authors conclude that the treatment of choice for these tumours is surgery due to their anatomopathological features (benign, scarcely malign or invasive, slow growth). On the other hand, medical therapy plays an important role in either preparing the surgical intervention or alleviating symptoms when the patient is inoperable.     

Neuroendocrine tumors of the pancreas

Background: The benefits of associating magnetic resonance imaging (MRI) and laparoscopy for localizing and treating neuroendocrine pancreatic tumors (NEPTs) have been poorly documented. Methods: In a retrospective study, eight patients with a mean age of 48 years were operated on for a NEPT. MRI was used to localize the lesions. In all patients a laparoscopic resection was carried out. Laparoscopic ultrasonography (LUS) was used during most operations. Results: The tumor was clearly localized by MRI in seven patients, and LUS showed the lesion in another patient whose preoperative MRI had been unsuitable. Three enucleations, three spleen-preserving caudal pancreatectomies, and two caudal pancreatectomies with splenectomy were carried out. There were no conversions and the mean operating time was 260 min, with a mean blood loss of 180 ml. The mean hospital stay was 7 days. There were no minor nor major complications during the hospitalization period. However, a pancreatic abscess was diagnosed in one patient 1 month later, requiring an urgent laparotomy. Conclusion: MRI as well as LUS are indeed suitable techniques to localize NEPTs. Moreover, the minimally invasive approach ensured an adequate treatment with a more comfortable and short postoperative recovery.     

Neuroendocrine tumors of the thymus

Neuroendocrine tumors of the thymus (NETTs) are unusual thymic neoplasms that were misdiagnosed as thymomas until the 1970s, when they eventually acquired a distinct identity. No collective large series have been published so far, and information about clinical presentation, diagnosis, histology, and treatment is derived from analysis of the case series and case reports published over a long period. NETTs are more aggressive than their pulmonary and abdominal counterparts, presenting at a more advanced stage, often with distant metastases, and are associated with poor long-term survival. Most patients are symptomatic at presentation as a result of the local invasion. Twenty percent to 30% of the cases are associated with endocrine disorders, mostly Cushing syndrome and multiple endocrine neoplasia syndrome. There is no official staging system for these tumors and investigators rely on the Masaoka staging system used for thymomas. Histologically, 2 classification are used: the World Health Organization and the Armed Forces Institute of Pathology classifications. Histologically, most tumors show moderately to poorly differentiated histologic features, reflecting their aggressive clinical behavior. Surgery is the most effective treatment option, although the aggressiveness of the tumor often requires extensive resection. Chemotherapy and radiotherapy may be used either preoperatively or postoperatively, although the small number of patients does not allow the design of standard guidelines about optimal schedules and doses. Survival depends on stage at presentation, histologic degree of differentiation, associated endocrine syndromes, and resectability rate. Recurrences are frequent after surgery and may be locoregional or distant. Surgery is recommended when feasible in the treatment of locoregional recurrences.     

Neuroendocrine tumors of the gut

Of 75 patients with neuroendocrine tumors of the gut, 50 had functioning lesions, causing endocrine syndromes. These included 16 insulinomas, 13 gastrinomas, 5 vipomas (4 pancreatic, 1 adrenal), 4 corticotrophinomas, and 1 renal enteroglucagonoma. Three patients had mixed endocrine syndromes. Eight or 9 patients with pancreatic lesions had multiple endocrine adenopathy (MEA I), including 3 of 4 patients with multiple insulinomas and all 3 patients with malignant gastrinomas. Localization of the pancreatic insulinomas, vipomas, and mixed tumors by arteriography was excellent, but few gastrinomas were detected before operation. Eight patients developed the malignant carcinoid syndrome with metastases from primary tumors in the ileum (6 patients), bronchus, and stomach. Hepatic artery embolization provided palliative relief for several with this and some other syndromes. Twenty-five patients had tumors that caused nonspecific features only. These included 18 appendicular carcinoids, 5 islet cell carcinomas, and 2 other apudomas (1 hepatic and 1 whose primary site is unknown). The importance of the latter is that they are more responsive to therapy and have a better prognosis than the more common acinar tumors. Over 40% of the patients had lesions that were cured surgically and over 80% obtained some benefit from treatment.

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Resumen La serie incluye todos los pacientes con tumores neuroendocrinos intestinales, o lesiones relacionadas, que fueron referidos a cirujanos del Hospital Hammersmith para consulta o manejo, entre 1937 y 1983. De 75 pacientes con tumores neuroendocrinos intestinales, 50 presentaron lesiones funcionales causantes de síndromes endocrinos. Estos incluyeron 16 insulinomas, 13 gastrinomas, 5 vipomas (4 pancreáticos, 1 adrenal), 4 corticotrofinomas y 1 enteroglucagonoma renal. Tres pacientes presentaron síndromes endocrinos mixtos. Ocho o nueve pacientes con lesiones pancreáticas exhibieron el síndrome de adenomas endocrinos múltiples tipo I (AEM I), incluyendo 3 de 4 pacientes con insulinomas múltiples y la totalidad de los 3 pacientes con gastrinomas malignos. La localización de los insulinomas pancreáticos, vipomas y tumores mixtos por medio de la arteriografía fué excelente, pero sólo unos pocos de los gastrinomas pudieron ser detectados antes de la operación. Ocho pacientes desarrollaron el síndrome carcinoide maligno con metástasis del tumor primario ubicado en el íleon (6), bronquio y estómago. La embolización de la arteria hepática significó mejoría paliativa para varios pacientes con éste y otros síndromes. Veinticinco pacientes presentaron tumores que sólo causaban manifestaciones no específicas. Estos incluyeron 18 carcinoides apendiculares, 5 carcinomas de células insulares y otros 2 apudomas (1 hepático y 1 cuya ubicación primaria permaneció desconocida). La importancia de estos últimos radica en que exhiben mayor capacidad de respuesta a la terapia y que poseen un mejor pronóstico que los mas comunes tumores acinares. Más del 40% de los pacientes presentaron lesiones que fueron curadas quirúrgicamente y más de 80% obtuvieron algún beneficio del tratamiento.

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Résumé Chez 75 sujets porteurs de tumeurs neuroendocriniennes du tube digestif 50 présentaient des lésions fonctionnelles se manifestant par des syndromes endocriniens. Parmi ces derniers, on comptait 16 insulinomes, 13 gastrinomes, 5 vipomes (4 pancréatique, 1 rénal), 4 corticotrophinomes et 1 entéroglucagonome. Trois malades présentaient des syndromes endocriniens mixtes. Huit ou neuf malades porteurs de lésions pancréatiques présentaient un syndrome MEA I comprenant 3 malades avec des insulinomes multiples sur 4 et 3 sujets avec des gastrinomes malins. La localisation des insulinomes pancréatiques, des vipomes et des tumeurs mixtes par l'artériographie fut excellente alors que peu des gastrinomes furent découverts avant l'intervention. Huit sujets présentèrent un syndrome carcinoide malin avec des métastases hépatiques alors que la lésion primitive siègeait au niveau de l'iléon, des bronches ou de l'estomac. L'embolisation de l'artère hépatique permis plusieurs fois d'obtenir une rémission de l'affection. Sur les 75 sujets observés, 25 ne présentaient pas de troubles fonctionnels spécifiques: les lésions répondant à 18 carcinoides appendiculaires, 5 carcinomes langheransiens et 2 apudomes (1 hépatique et 1 de siège inconnu). Ces formes sont plus sensibles au traitement et présentent un meilleur pronostic que les tumeurs acineuses plus fréquentes. Plus de 40% des malades furent guéris chirugicalement et au total 80% bénéficierent du traitement.

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The Hammersmith Surgical Series, presented at the International Association of Endocrine Surgeons at Hamburg, September, 1983.     

Non-functional or silent endocrine tumors of the pancreas. Apropos of a case of cystic form

The authors report a new case of silent cystic pancreatic endocrine tumour discovered by chance on abdominal ultrasonography. This tumour was treated surgically by simple enucleation-resection. On the basis of the histological appearance and the absence of metastases, this tumour was considered to be benign, but only the long-term course will confirm the diagnosis.     

Neuroendocrine tumors of the larynx.

A review of the international literature has revealed 68 paragangliomas, 42 large cell, and 74 small cell tumors of the larynx. Paragangliomas are usually benign, although malignant cases have been reported. Large cell tumors are malignancies associated with a high incidence of early cervical metastasis. Small cell tumors are aggressive cancers characterized by early, diffuse metastatic disease. All three neoplasms demonstrate a propensity for the supraglottic larynx. While surgery remains the treatment of choice for paragangliomas and large cell cancers, small cell cancers are best treated by radiotherapy and chemotherapy. The determinate 5-year survival for patients with paragangliomas, large cell, and small cell cancers is 60%, 34%, and 14%, respectively. While representing distinct clinical entities, these neoplasms demonstrate similar ultrastructural and histochemical features and should be classified as neuroendocrine tumors of the larynx (NETL). A comprehensive analysis of these laryngeal tumors is presented herein. Their clinical behavior and management options are reviewed and a scheme for their nomenclature and classification is proposed.     

Neuroendocrine carcinomas of the pancreas with 'Rhabdoid' features

Neuroendocrine carcinomas of the pancreas are rare neoplasms whose morphologic features generally mirror those seen in neuroendocrine tumors in other organs. Rarely, however, they may display unusual morphologic appearances that can introduce difficulties for diagnosis. We report four cases of primary neuroendocrine carcinomas of the pancreas (islet cell tumors) that were characterized by prominent "rhabdoid" features of the tumor cells. The lesions occurred in two men and two women 37-79 years of age who presented with symptoms of biliary obstruction and epigastric pain; one patient had recurrent gastric ulcers and an elevated gastrin level. The tumors were located in the head and tail of the pancreas and measured 2.5-4.5 cm in greatest diameter. Histologic examination revealed sheets of monotonous tumor cells with uniform round nuclei showing dispersed chromatin and containing abundant densely eosinophilic cytoplasmic inclusions that displaced the nuclei toward the periphery. In all cases, the rhabdoid elements appeared to merge with areas showing a more conventional neuroendocrine morphology. Immunohistochemical studies in all cases showed strong cytoplasmic positivity of the rhabdoid tumor cells for chromogranin, synaptophysin, and cytokeratin. Recognition of this unusual morphologic appearance is of importance to avoid mistaking these lesions for other types of malignant neoplasm.