Metástasis ganglionar mesentérica de neoplasia neuroendocrina: una causa infrecuente de obstrucción de intestino delgado / Small bowel obstruction caused by lymph nodes of a neuroendocrine tumor: a clinical case report

Small intestine neuroendocrine tumors (NET-SI) are relatively rare neoplasms. If encountered, the most common location is the ileum. Symptoms are usually non-specific, delaying the tumors diagnosis. NET-SI are often small in size and can be challenging to recognize on imaging studies. However, they have a tendency to induce a pronounced fibrotic reaction in the mesentery, often accompanied by large calcified mesenteric adenopathies. In some cases, the fibrotic reaction can produce rare complications, such as intestinal obstruction or vascular congestion with occasional secondary ischemia. This case report presents a 79-year-old male with a partial small bowel obstruction caused by a fibrotic reaction and mesenteric adenopathies of a well-differentiated neuroendocrine tumor of the ileum. The patient also presented multiple peritoneal metastases at diagnosis. Characteristic imaging findings of the tumor, allowed an accurate and early diagnosis. Once the acute episode was resolved, the diagnosis was confirmed with an image guided biopsy.

Neuroendocrine tumors of the urinary bladder - Report of two cases

Primary paraganglioma and small cell neuroendocrine carcinoma of the urinary bladder are rare tumors, comprising 0.05% of all bladder tumors and <1% of all malignant bladder tumors, respectively. These tumors can be the cause of a diagnostic dilemma or misdiagnosis on morphology. Paraganglioma is often mistaken for urothelial carcinoma and small cell carcinoma for poorly differentiated carcinoma or lymphoma. Herein, we report a case of primary paraganglioma and another of a small cell carcinoma of the urinary bladder and discuss their closest differential diagnoses. The diagnostic pitfalls should be kept in mind so that correct, timely diagnosis of these entities can be made due to implications in the management and prognosis.

Use of cinacalcet and sunitinib to treat hypercalcaemia due to a pancreatic neuroendocrine tumor

SUMMARY Neuroendocrine tumors (NETs) can secrete hormones, including ectopic secretions, but they have been rarely associated with malignant hypercalcemia. A 52-year-old man with a history of diabetes mellitus was diagnosed with a pancreatic tumor. A pancreatic biopsy confirmed a well-differentiated pancreatic NET (pNET). The patient subsequently developed liver metastasis and hypercalcemia with high 1,25 OH vitamin D and suppressed parathyroid hormone (PTH) levels. Hypercalcemia was refractory to chemotherapy, intravenous saline fluids, diuretics, calcitonin and zoledronate. Cinacalcet administration (120 mg/day) resulted in a significant calcium reduction. Hypocalcemia was observed when sunitinib was added three months later and cinacalcet was stopped. Subsequently, the calcium and PTH levels normalized. After six months, we observed 20% shrinkage of the pancreatic tumor and necrosis of a liver metastasis. Cinacalcet is an allosteric activator of the calcium receptor agonist, and it is used for severe hypercalcemia in patients with primary (benign and malignant) hyperparathyroidism. In this patient, cinacalcet demonstrated a calcium lowering effect, normalized hypophosphatemia, and improved the clinical condition of the patient. The mechanism through which cinacalcet improved PTH-rp mediated hypercalcemia is still unclear, but studies have suggested that a potential mechanism is the activation of calcitonin secretion. Sunitinib is an oral multi-targeted tyrosine kinase inhibitor used to treat advanced pNETs. The hypocalcemic effects of sunitinib have not been previously described in a patient with pNET. Here, we report for the first time the successful combination of cinacalcet and sunitinib in the treatment of a pNET patient presenting with malignant hypercalcemia.

Secreción ectópica de ACTH en un paciente con antecedentes de enfermedad de Cushing / ACTH's ectopic secretion in a patient with precedents of Cushing's disease

Hombre de 54 años con antecedentes de enfermedad de Cushing 32 años antes de la consulta. Ingresó por edemas asociados a astenia y adinamia. En el laboratorio se constató hipopotasemia y alcalosis metabólica. Se realizó diagnóstico humoral de síndrome de Cushing secundario a secreción ectópica de hormona adrenocorticotropa (ACTH). En la tomografía de tórax se halló un tumor de 3 × 3 cm en el mediastino anterior. La anatomía patológica de la pieza quirúrgica fue compatible con un carcinoide tímico. Este paciente sufrió en dos oportunidades un síndrome de Cushing, la primera por enfermedad (adenoma hipofisiario) y la segunda vez por secreción ectópica de ACTH (SEA) una asociación no descripta, en nuestro conocimiento, en la literatura médica.

A 54-year-old man, with a history of Cushing’s disease diagnosed 32 years earlier, presented with edema, asthenia and general malaise. Abnormal laboratory studies depicted hypokalemia and metabolic alkalosis. A CT scan of the chest revealed a 3 × 3 cm tumor in the anterior mediastinum. The pathology was consistent with a thymic carcinoid. These findings led to a diagnosis of biochemical Cushing’s syndrome secondary to ectopic secretion of ACTH. Thus, this patient suffered twice of Cushing’s syndrome. The first instance was the consequence of an ACTH - secreting pituitary adenoma and the second of an ectopic secretion of ACTH. To the best of our knowledge this is the first such case reported in the medical literature.

A Case of Alpha-cell Nesidioblastosis and Hyperplasia with Multiple Glucagon-producing Endocrine Cell Tumor of the Pancreas / 대한소화기학회지

Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells. It also means maldistribution of islet cells within the ductules of exocrine pancreas. Generally, nesidioblastosis occurs in beta-cell and causes neonatal hyperinsulinemic hypoglycemia or adult noninsulinoma pancreatogenous hypoglycemia syndrome. Alpha-cell nesidioblastosis and hyperplasia is an extremely rare disorder. It often accompanies glucagon-producing marco- and mircoadenoma without typical glucagonoma syndrome. A 35-year-old female was referred to our hospital with recurrent acute pancreatitis. On radiologic studies, 1.5 cm sized mass was noted in pancreas tail. Cytological evaluation with EUS-fine-needle aspiration suggested serous cystadenoma. She received distal pancreatectomy. The histologic examination revealed a 1.7 cm sized neuroendocrine tumor positive for immunohistochemical staining with glucagon antibody. Multiple glucagon-producing micro endocrine cell tumors were scattered next to the main tumor. Additionally, diffuse hyperplasia of pancreatic islets and ectopic proliferation of islet cells in centroacinar area, findings compatible to nesidioblastosis, were seen. These hyperplasia and almost all nesidioblastic cells were positive for glucagon immunochemistry. Even though serum glucagon level still remained higher than the reference value, she has been followed-up without any evidence of recurrence or hormone related symptoms. Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review.

Síndrome de Cushing secundario a secreción ectópica: un desafío diagnóstico / Cushing syndrome secondary to ectopic: diagnostic challenge

The diagnosis of Cushing Syndrome secondary to Ectopic ACTH secretion constitutes a challenge to the endocrinologist. The goal is to make a differential diagnosis of Cushing’s disease and localize the ACTH-secreting tumor, to achieve quick and effective management of a disease that can be fatal. The mainly diffuculties are the limited data due to their low prevalence and the wide variety of the origin tumors. Therefore, a comprehensive and multidisciplinary study is needed, analyzing each particular case. This article reviews the diagnostic alternatives, their strengths and weaknesses, proposing an algorithm that contributes to our clinical practice...

Dilatación quística del conducto de wirsung por carcinoma neuroendocrino no funcionante de páncreas / Quística expansion of the conduit of wirsung by nonfuncionante neuroendocrino carcinoma of páncreas

Antecedentes: La dilatación quística del Wirsung frecuentemente se asocia con neoplasia intraductual papilomucinosa tipo 1. Pero este tipo de dilatación también puede asociarse a tumores neuroendocrinos. Objetivos: Presentar una nuevaa forma de dilatación quística del conducto de Wirsung observada en pacientes con tumoes neuroendocrinos no funcionantes de páncreas. Lugar de aplicación: Centro Terciario de Referencia. Diseño: Retrospectivo. Población: Pacientes con tumores neuroendocrinos nu funcionantes de páncreas. Método: Mediante un análsis retrospectivo de una base de datos se estudió la incidencia de la dilatación quística del Wirsung en los tumores neuroendocrinos. Resultados: De 35 pacientes con resecciones pancreáticas por tumores neuroendocrinos no funcionantes, tres presentaron dilatación quística del conducto de Wirsung. Fue observado en los estudios por imágen y corroborado en la cirugía. Conclusiones: La dilatación quística del Wirsung por tumor neuroendocrino es una forma de presentación poco frecuente en estos timores pero que debe ser tenida en cuenta para un tratamiento quirúrgico correcto.

Clinical Features of Neuroendocrine Tumor of the Pancreas: Single Center Study / 대한소화기학회지

BACKGROUND/AIMS: Pancreatic neuroendocrine tumors (PNET) are rare and manifest as functioning tumor (FT) or non-functioning tumor (NFT). Although malignant changes are observed in some cases, its prognosis is better than pancreatic cancer. We evaluated clinicoradiologic features and prognosis of FT and NFT. In addition, we tried to find the predictive factors for the recurrence of NFT after resection. METHODS: Between October 1994 and June 2004, we retrospectively evaluated the clinicopathologic features and prognosis of 12 cases of FT and 31 cases of NFT diagnosed by surgical pathology at single medical center in Korea. RESULTS: PNET included 6 insulinomas, 4 gastrinomas, 1 glucagonoma, 1 somatostatinoma and 31 NFT. The major clinical manifestations were neuroglycopenic symptoms (100%) in insulinoma, abdominal ulcer symptoms (75%) in gastrinoma, dermatitis (100%) in glucagonoma, steatorrhea (100%) in somatostatinoma, and abdominal discomfort or pain (45%) in NFT. NFT was located more proximally when compared to FT (p=0.023). NFT showed more malignant (64.5%) behavior compared to FT (41.7%) despite the lack of statistical significance. Curative resections were done without postoperative death in 38 cases. Six cases of NFT (21.4%) and 1 case of FT (10%) recurred with an average of 26.5 months. In the recurrent NFT, the findings of diabetes mellitus (p=0.010), abnormal pancreatic duct (p=0.026), Whipple's operation (p=0.013) and tumor emboli (p=0.03) were more common than in non-recurrent NFT. CONCLUSIONS: FT and NFT showed different clinicoradiologic features. In addition, NFT should be monitored more carefully because of frequent recurrence.

Gastric outlet obstruction and cutaneous metastasis in adenocarcinoid tumor of stomach - unusual presentations with cytologic and ultra structural findings.

Neuroendocrine tumors, including carcinoids account for less than 1% of gastric tumors. Various subtypes of gastric carcinoids have been reported earlier. The present case deals with two unusual presentations, diagnosis and course of a gastric neuroendocrine tumor in an adult patient. A 35-years-old male initially presented with gastric outlet obstruction for an antral growth in the emergency ward. He underwent radical gastrectomy and was diagnosed with a gastric carcinoid tumor, on histopathology. After 6 months, he developed hepatic along with nodular cutaneous lesions over the scalp. Aspiration cytology (FNAC) from these metastatic lesions showed two distinct cell types with rosette formation. Ultrastructural findings showed neurosecretory granules in some cells. Subsequently, he underwent 2 cycles of chemotherapy. After a total duration of 9 months, he finally succumbed to the disease. We present a case of a gastric adenocarcinoid tumor, with 2 rare presentations. The metastatic lesions exhibited neuroendocrine features on cytology and electron microscopy.

Tumor neuroendocrino de células pequeñas de anorrecto / Small-cell neuroendocrine tumor of the anorectal area

Se presenta el caso clínico de una paciente de sexo femenino de 44 años con un tumor de la región rectoanal, de tipo neuroendocrino (NE) indiferenciado de células pequeñas. La paciente presentaba al momento del diagnóstico metástasis hepáticas y ganglionares inguinales e intraabdominales múltiples. El tratamiento incluyó el uso de quimioterapia agresiva con cisplatino y etopósido con buena respuesta inicial. Luego de 8 meses de iniciado el tratamiento, se detecta recidiva tumoral en canal anal y pelviana. La radioterapia pelviana logra el control macroscópico completo. Recidiva metastásica hepática múltiple 2 meses después. La paciente fallece luego de 18 meses de detectada su enfermedad.