Regulatory peptides and other neuroendocrine markers in medullary carcinoma of the thyroid.

Medullary thyroid carcinoma (MTC) is an APUDoma (APUD refers to amine precursor uptake and decarboxylation) arising from the parafollicular cells. Diarrhoea has been reported in some 30% of patients, variously attributed to excess production of calcitonin (CT), serotonin (5-HT), vasoactive intestinal peptide (VIP) or other factors. The regulatory factors in MTC were examined employing immunocytochemistry and RIA to tumours and their extracts. The patients were followed up for more than 15 years. CT and calcitonin gene-related peptide were universally expressed in all the tumours. The neuroendocrine markers chromogranin A (and its fragments pancreastatin and WE-14), neurone-specific enolase, protein gene product 9.5 and carcino-embryonic antigen were found in the majority of MTCs and might be useful as immunocytochemical markers. 5-HT, substance P, neurokinin A, glucagon and VIP could not be detected, excluding them as candidates in the diarrhoea of MTC.

Mixed type Zollinger-Ellison syndrome in a florid case of multiple endocrine neoplasia type I. A case report.

An unusual and unreported mixed type Zollinger-Ellison syndrome, including features of a Type I and II syndrome, is presented as a component of a neoplastic profile constituting a highly diverse multiple endocrine neoplasia Type I syndrome.

Carcinoid apudoma arising in a glomus jugulare tumor: review of endocrine activity in glomus jugulare tumors.

Previous reports of endocrine activity of glomus jugulare tumors have been of pheochromocytomas but not carcinoid apudomas. This paper is to alert the otolaryngologist to the presence of carcinoid apudomas in glomus jugulare tumors. A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. The diagnosis of carcinoid syndrome is made with a good history and confirmed with a 24 hour urine collection for 5 Hydroxy indole acetic acid (5 Hiaa). a series of patients treated at the Johns Hopkins Hospital for glomus jugulare tumors is reviewed for the presence of endocrine activity and the literature on endocrine active glomus jugulare tumors is presented. Preoperative evaluation for the presence of both pheochromocytoma and carcinoid apudoma is recommended for all patients with glomus jugulare tumors.

Clinical significance of the association of the Von Hippel-Lindau disease with pheochromocytoma and pancreatic apudoma.

Von Hippel-Lindau disease (VHLD) is a rare disorder known to occur with pheochromocytoma. We report a case with this association and also with pancreatic apudoma. We believe that this is the fifth reported case of this triad, which represents an overlap in the multiple endocrine neoplasia (MEN) classification. Many recent reports of overlap of MEN syndromes challenge the validity of this classification. New understanding of the embryologic origin of cells of endocrine organs has led to the elucidation of the APUD (amine precursor uptake and decarboxylation)-neuroendocrine system. Integration of the APUD system may be important to understanding the pathogenesis of the MEN-like syndromes as well as their relationship to the neurocutaneous syndromes. Any endocrine tumor or neurocutaneous syndrome should be evaluated to exclude a spectrum of possible associations.

Invasive pituitary adenoma presenting with nasal obstruction.

We report two cases of massive invasive pituitary adenoma with the unusual presentation of nasal obstruction. In both cases the diagnosis was only made when the obstructing tissue was removed. Cases reported previously have usually presented with severe focal neurological signs or a long history of neurological symptoms. These two cases are unusual in having minimal neurological deficits despite the extension of tumour into the nasopharynx and the middle and posterior cranial fossae. These extensions were well illustrated by computerised tomography. This unusual presentation is discussed with reference to the literature.

[Steatorrhea and its treatment with pancreatic enzymes in endocrine tumors of the pancreas (pancreatic adenomas)]. / Esteatorrea y su tratamiento con fermentos pancreáticos en los tumores endocrinos del páncreas (apudomas pancreáticos).

We report the treatment of steatorrhea with pancreatic enzymes in four pancreatic endocrine tumors treated in our hospital. All four patients were males, with a mean age of 50 +/- 4.6, a basal steatorrhea of 10.8 +/- 3.7 g/24 h (N: less than 5 g/24 h), and a defecation rate of 1.8 +/- 0.8 (mean +/- SD). They were treated for one week with Pankreon 700 in the form of pills of 700 mg pancreatin (28,000 U lipase PIF, 22,000 U amylase PIF and 1,500 U protease PIF). They were given 15 pills/day divided into three doses. The mean steatorrhea dropped to 7.1 +/- 1.5 g/24 h. Then the patients were given Creon capsules with 300 mg pancreatin in pellets (8,000 U lipase PIF, 9,000 U amylase PIF and 450 U protease PIF). They were given six capsules/day divided into three doses, and the steatorrhea dropped to 7.2 +/- 4.1 g/24 h, while the rate of defecation dropped to 1/day. On comparison of the two products (Pankreon-- a classic form of pancreatin, and Creon-- a modern galenical in pellets), it was found that Creon achieved the same results as Pankreon 700 in a doses 2.5 times lower. This pilot study demonstrates that exocrine pancreatic insufficiency can be frequent in endocrine tumors of the pancreas, and although underestimated, it can be partially corrected with pancreatic enzymes.

Metastatic pancreatic polypeptide producing tumour presenting with diabetes mellitus.

A metastatic pancreatic polypeptide tumour is described which presented clinically with diabetes mellitus. This is the first case to present this way.

Carcinoide pancreático maligno de larga evolución tratado con octeotrida / No disponible

Se presenta un varón con un carcinoide pancreático maligno, con metástasis, secretor de serotonina plasmática (5-HIAA urinario elevado) con síndrome carcinoide-like, evaluado mediante ecografía (US), tomografía computarizada (TAC), resonancia magnética (RM), ultrasonografía endoscópica (USE) y Octreoscan, tratado con quimioterapia, Interferón y Octeotrida, de forma secuencial, con supervivencia prolongada de 12 años después del diagnóstico. A propósito de este caso inusual, el segundo publicado desde nuestro país, se revisa la literatura mundial(AU)

A male presented with a metastatic, plasma serotonin-secreting (high 5-HIAA in urine), malignant pancreatic carcinoid with a carcinoid-like syndrome, and was assessed using ultrasounds (US), computerized tomography (CT), magnetic resonance imaging (MRI), endoscopic ultrasonography (EUS) and Octreoscan; he sequentially received chemotherapy, interferon and octreotide, with long-term, 12-year survival after diagnosis. Given this unusual case, the second reported in our country, the overall literature is reviewed(AU)

[Follow-up study of non-neoplastic Cushing's syndrome followed by paraneoplastic Cushing's syndrome after removal of a malignant pulmonary tumor. Role of the diffuse endocrine system and APUD (amine precursor uptake decarboxylase) cells]. / Syndrome de cushing non tumoral succédant a un cushing paranéoplasique traité avec succès par l'exérèse d'une tumeur pulmonaire maligne. Recul de onze ans. Rôle de la responsabilité du système endocrinien diffus et des cellules dites APUD.

A 27 year old woman had in 1965, a paraneoplastic Cushing's syndrome was cured by removal of a pulmonary tumour. The case was published in June 1968. After a normal pregnancy, a recurrence of the Cushing's syndrome in 1969 led in 1970 to bilateral total adrenalectomy in two stages. Pathology showed hyperplastic adrenal glands. After 11 years follow up, we have not noted any relapse of the tumour nor of the Cushing's syndrome. The pathological appearance of the lung carcinoma removed in 1965 (endocrinoid or neuroid structures) suggests that this was an "apudoma" and that the recurrence of the Cushing's syndrome was perhaps an "apudomatosis" involving the pituitary ACTH cells.

[Steiner-type neuroapudomatosis presented a pseudo-Hirschsprung disease (author's transl)]. / Neuroapudomatose de type Steiner révélée par une pseudo-maladie de Hirschsprung.

A case of Steiner type neuroapudomatosis presented with a pseudo-Hirschsprung disease in an infant. Secondarily, the facies became typical, while hyperthyrocalcitoninaemia led to the discovery at the age of 2 years and 8 months of a medullary carcinoma of the thyroid. Despite early surgery in the absence of any clinical signs, it was not possible to avoid cervical lymphatic spread.

Apudoma and subcorneal pustular dermatosis (Sneddon-Wilkinson disease).

The subcorneal pustular dermatosis (SPD) is a rare dermatosis. IgA monoclonal gammapathy is the most frequently associated disease with the SPD. We report a case of SPD in a patient with metastatic apudoma, an association not previously described. The rare nature of both diseases suggests that this association is not fortuitous.

Gastrin-secreting tumor of the duodenum (G-cell apudoma) associated with secondary biliary cirrhosis.

A middle-aged woman with evidence of chronic cholestasis of several years and no previous abdominal surgery was initially thought to have primary biliary cirrhosis. Clinical evaluation disclosed a well-developed secondary biliary cirrhosis apparently caused by extrahepatic obstruction due to a 1 X 2 cm neoplasm of the periampullary duodenum. Electron microscopy and immunofluorescent studies showed the neoplasm to be a G-cell adenoma. Wide local excision has resolved the biliary obstruction. Bening or slow-growing duodenal tumors, if they involve the ampulla of Vater, may produce prolonged partial extrahepatic obstruction and secondary biliary cirrhosis.

[Segmental portal hypertension as a rare cause of gastric hemorrhage--case report]. / Segmentalna portalna hipertenzija kao retki uzrok krvavljenja iz zeluca--prikaz bolesnika.

Segmental portal hypertension is a rare pathologic condition, which produce gastric bleeding. Spleen vein thrombosis is more often caused by pancreatic disease (inflammations, tumors). Diagnosis is difficult to perform. Initial treatment is conservative. After successful conservative treatment early surgery should be planned. Unsuccessful conservative treatment indicates surgery. Splenectomy absolutely eliminates risk from rebleeding. Prognosis of these patients depends from etiology of pancreatic disease. We present a 46 old woman who successfully operated in our Department of Surgery due to massive upper gastrointestinal bleeding caused by isolated portal hypertension.