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The case of a 47-year-old patient is described with a carcinoid of the middle ear. Initial symptoms were hearing impairment, feeling of pressure and dizziness. Mastoidectomy was carried out for mastoid shadowing with space encroachment in the auditory canal. The histological examination initially revealed an atypical cholesteatoma and the subsequent immunohistochemical investigation revealed a carcinoid. A radical excavation with complete excision of the tumor and tympanoplasty was carried out. A carcinoid of the middle ear is definitely a rare finding and the primary treatment is complete surgical removal. If metastases are suspected octreotide scintigraphy has proved to be the best option in analogy to intestinal carcinoids. Radiation therapy has not proved successful but the use of the somatostatin analog octreotide, interferon-alpha or palliative chemotherapy (e.g. streptozotosine, 5-fluorouracil) for metastases are further therapy options.

Intussusception of the intestine is a frequent reason for intestinal obstruction in infants; the course is acute but the causes are often not recognizable. In adults intussusception is the cause for bowel obstruction in only 1% of cases. The course is subacute and in over 90% a benign or malignant process in this part of the intestine is found. We report the case of a 45-year-old male with abdominal discomfort, increasing over weeks. The preoperative ultrasonography and computed tomography of the abdomen revealed the typical finding of ileocoecal intussusception. An ileocoecal resection was performed. The histopathological workup showed a small carcinoid tumour of the terminal ileum.

Aim: To evaluate intra-arterial CT mesentericography (CTM) in the diagnostics of severe obscure overt intestinal bleeding in comparison with conventional mesentericography (MG) and surgery., Methods: In 8 patients (23 - 82 years, mean 59 years), CTM was performed via the catheter left in the superior mesenteric artery after MG to detect the source of bleeding. Early and late-phase spiral CT scans were acquired after administration of contrast medium. Active bleeding was considered to be present if extravasation of contrast medium into the bowel was found. The results of MG and CTM were compared with the results of surgery., Results: With MG active bleeding was found in one patient, with CTM in five patients. In three patients, both MG and CTM were negative. Six patients underwent surgery. Five cases of bleeding detected with CTM were confirmed by surgery. In one case, bleeding found with CTM was not confirmed by surgery. One patient underwent surgery although all imaging procedures were negative. The source of bleeding remained unknown. Surgically, the site of bleeding was located in the jejunum in 3 patients (jejunitis, jejunal ulcers, carcinoid), one patient had a diverticulum in the ascending colon. The colonic bleeding site was correctly localized with CTM, whereas the small bowel bleeding could only roughly be assigned to the proximal or distal jejunum or jejunoileal transition area., Conclusion: Preliminary results indicate that selective CTM is superior to MG in the evaluation of severe obscure overt intestinal bleeding.

A 74-year-old male suffering from Recklinghausen's fibromatosis (NvR) is reported. He presented with weight loss, cholestasis, endocrine and exocrine pancreatic insufficiency. These symptoms were caused by a neuroendocrine tumor of the ampulla of Vater containing somatostatin. The tumor induced an obstruction of both the common bile and the pancreatic duct. In addition to this uncommon tumor, a silent pheochromocytoma was found. The patient was treated by endoscopic papillotomy, substitution of pancreatic enzymes and additional enteral nutrition. After recovery no progression of the disease was observed over one year. A review of the literature shows that patients with neurofibromatosis are at high risk for periampullar tumors. In particular, somatostatin-rich carcinoids were previously documented. Pheochromocytomas are also quite prevalent in NvR. However the combination of NvR, pheochromocytoma and somatostatin-rich neuroendocrine tumors of the duodenum has only been reported a few times. An explanation for the high prevalence of neuroendocrine tumors in NvR might be the loss of neurofibromin, a tumor suppressor protein, which is the main product of the neurofibromatosis-l-gene.

Meckel's diverticulum is the most common anomaly of the gastrointestinal tract. Neoplasms are extremely rare. We report a case of carcinoid tumor of Meckel's diverticulum in a patient with acute abdominal pain. So far, 111 cases have been reported. Review of the literature reveals that carcinoids are the most common malignant tumors of Meckel's diverticulum. Two thirds of the patients remain asymptomatic. Metastases are seen in 25% at diagnosis. The probability of metastases depends on the size of the primary tumor. Because of the early metastases rate even in small tumors, aggressive surgical management is justified.

History and Findings: A 69-year-old woman reported marked restriction of voluntary movements of the hands in the preceding 6 months. She had also experienced loss of motivation, memory and concentration. Her skin was pale yellow, and scratches on her skin indicated marked pruritus., Investigations: Neurological examination revealed decreased vibratory sense in both legs. Haemoglobin concentration was 8.3 g/dl, mean corpuscular volume 114 fl, vitamin B12 level < 100 ng/l, folic acid level normal. Antibody titre against parietal cells was increased, vitamin B12 resorption diminished. Gastroscopy revealed small raised lesions, made up of hyperplastic cells which stained with chromogranin, indicating a diagnosis of microcarcinoid of the gastric mucosa., Treatment and Course: On administration of cobalamine (1,000 micrograms i.m. daily for 2 weeks, twice weekly for 6 weeks, then once per week for the last 7 months) the blood picture returned to normal, but the microcarcinoids, the psychological symptoms and the apraxia of the hands were unchanged.

A 48-year-old woman with type II diabetes developed fatigue, arthralgia and myalgia. A few weeks later she was found to have hepatomegaly. The erythrocyte sedimentation rate was raised (53/93 mm), as were liver enzyme activities (GOT 186 U/l; GPT 240 U/l; gamma-GT 199 U/l), the gamma-globulin levels (40.7%;IgG 4470 mg/dl, IgA 698 mg/dl, IgM 245 mg/dl), antinuclear antibodies and antibodies against double-strand DNA, smooth muscles and actin. Laparoscopy revealed small-nodular liver cirrhosis. The autoimmune hepatitis was treated with prednisolone (initially 60 mg daily, then reduced to 10 mg daily) and azathioprine (initially 100 mg daily, reduced to 50 mg daily). The symptoms markedly improved. But one year later, during follow-up examination, gastric polyps were found, excised and histologically found to be carcinoid. The gastrin level was raised to 765 pg/ml. Another year later the liver cirrhosis had advanced further and the type A gastritis was still present, but there was no sign of carcinoid recurrence.

Thrombosis of the left subclavian vein occurred in a 44-year-old man. It was found to be caused by an atypical thymus carcinoid of the anterior mediastinum without carcinoid syndrome. Primary resection was not possible, but it was removed after three cycles of neoadjuvant chemotherapy with doxorubicin, cisplatin, vincristine and cyclophosphamide. Increased concentrations of alkaline phosphatase and parathormone were then noted. Subtotal parathyroidectomy revealed hyperplastic parathyroids. A gastrinoma was suspected from a history of peptic ulcer for many years which had persisted despite a Billroth II gastric resection 10 years ago. Serum gastrin, analysis of gastric secretion and a secretin-stimulating test confirmed the diagnosis. Recurrent episodes of weakness and syncope, in the presence of low blood sugar levels and a positive C-peptide suppression test, were interpreted as due to an insulinoma. There was no evidence of increased hypophyseal or adrenal function. Finally, in the absence of a family history, multiple endocrine neoplasia type 1 (MEN 1) was diagnosed with co-existing primary hyperparathyroidism, gastrinoma, insulinoma and thymus carcinoid. Somatostatin-receptor scintigraphy provided localization of the MEN 1 with enrichment in the thorax and abdomen.

A collection of 13 tumors causing an ectopic Cushing's syndrome was composed of four bronchial carcinoids, one small cell carcinoma of the bronchus, one thymic carcinoid, two islet cell tumors of the pancreas, one pheochromocytoma, two medullary carcinomas of the thyroid, one prostatic cancer and one intrasellar choristoma. By immunohistochemistry, ACTH in combination with CRH was found in one bronchial carcinoid and in one islet cell carcinoma. ACTH but not CRH was demonstrable in one bronchial carcinoid, in both medullary carcinomas of the thyroid, in the thymic carcinoid and in the pheochromocytoma. CRH without ACTH was present in the small cell carcinoma of the bronchus, one bronchial carcinoid, the prostatic cancer and the choristoma of the sellar region. Neither ACTH nor CRH could be found in one islet cell carcinoma. In the pituitary (n = 7) Crooke's cells were found except in one case with islet cell carcinoma which was treated with adrenostatic drugs. The pituitary besides the intrasellar choristoma harbored an ACTH cell adenoma. The pathophysiological correlations are discussed.

A 36-year-old patient developed marked pigmentation, marked myopathy and severe hypokalaemic alkalosis which at first pointed towards an ectopic ACTH syndrome. The dexamethasone test at a high dose indicated cortisol suppression. A mediastinal tumour was seen radiologically, but the sella was of normal size. Computed tomography provided indirect signs of a sellar space-occupying lesion which suggested an ectopic production of corticotropin-releasing factor (CRF) as cause of the Cushing's syndrome. CRF concentration in antecubital venous blood was markedly elevated to 280 ng/l. The mediastinal tumour was excised and proved to be a carcinoid histologically. Postoperatively the CRF concentration fell to 70 ng/l. An extract of the carcinoid contained 15.5 ng/g wet-weight of CRF and 254 ng/g wet-weight of beta-endorphin. The patient died 5 weeks postoperatively of sepsis with bilateral pneumonia. At autopsy the hypophysis was of normal size but showed nodular ACTH-cell hyperplasia. This was thus a case of Cushing's syndrome resulting from ectopic CRF production in a mediastinal carcinoid tumour.

In 36 patients with neoplastic diseases 72 episodes of hypercalcaemia with serum-calcium levels greater than or equal to 2.75 mmol/l were treated (19 breast carcinoma; 9 bronchial or lung carcinoma; 5 multiple myeloma; 1 each jejunal carcinoid, malignant lymphoma, phaeochromocytoma). Cardinal symptoms were mental, neuromuscular and renal during the hypercalcaemic episodes. Mithramycin is preferred to other methods (infusion of sodium chloride and frusemide, prednisone, sodium-potassium-phosphate infusion) of treating acute or subacute hypercalcaemia. Mithramycin in a single injection of 20-25 microgram/kg body-weight intravenously is usually sufficient to counteract a hypercalcaemic phase for at least 7-10 days, often much longer. There was a highly significant fall in serum-calcium levels from two days onwards after mithramycin injection. Toxic side-effects were minimal and restricted to transitory increase in transaminase levels, initially 5-6 times normal with a maximum on the third day and normalisation on the fifth day after mithramycin administration.

Acanthosis nigricans maligna (a. n. m.) is a well known paraneoplastic syndrome of the skin in 90 p. c. associated with tumours of the gastrointestinal tract (mostly gastric carcinomas) in contrast to pseudoacanthosis. Metastases are detected at the time of first diagnosis usually. Therefore, the a. n. m. may be the first sign of the tumour but does not represent an early symptom. The a. n. m. typically disappears with eradication of the responsible tumour and may reappear on its recurrence or metastasizing. In all cases an intensive search for gastrointestinal cancers is absolutely necessary. The etiopathology of the a. n. m. is still under advisement and the current state of knowledge is discussed in detail.

We have reported on a 43 years old woman patient, who had fallen ill with an extremely seldom carcinoid tumor arising in the trachea histologically confirmed. Our observations of this woman-patient had been compared with those of 14 patients known from the Anglo-American literature upto 1978. In regard to one French and one Russian collective statistics altogether we come to 24 cases of trachea carcinoids documented in the literature. These are the main symptoms: dyspnea, wheezing, hemoptysis and stridor. Radiation tried at the inoperable patient has proved as ineffective. Today operation seems to be the chosen therapy.

In a 32-year-old woman presenting with fever, vomiting and right upper abdominal pain a tumor of the papilla of Vater was detected endoscopically and removed surgically. Histologically it was a carcinoid shown immunohistochemically to produce calcitonin. The tumor had metastasized to a lymph node in the hepatoduodenal ligament. Clinically the patient showed typical signs of von Recklinghausen neurofibromatosis. A survey of the literature suggests that carcinoid of the papilla of Vater may be more frequent in patients with neurofibromatosis. Possible relationships between neurofibromatosis and carcinoid are discussed in the light of the pertinent literature.

19 consecutive patients admitted with severe chronic diarrhoea which had failed to respond to standard therapeutic regimen were treated with 4 to 8 mg of loperamide daily for up to 50 weeks. A marked improvement was achieved in 13 out of the 19 patients (68%). The best results were observed in patients with ulcerative colitis and Crohn's disease. Patients with secretory diarrhoea did not improve. No major side effects were observed. It is concluded that loperamide is a highly effective and safe new drug in the treatment of patients with chronic inflammatory bowel diseases.

In a 53-year-old female patient with typical clinical Cushing's syndrome increased blood sugar levels, hypokalaemia, increased non-dexamethasone suppressable plasma cortisol levels and increased ACTH values in serum were observed. CAT scanning revealed bilateral adrenocortical hyperplasia. The endocrine anterior pituitary function was normal. Radiographically and bronchoscopically a rightsided parahilar tumour was found. Histology showed an atypical metastatic bronchial carcinoid with ectopic ACTH production. Conservative treatment with metyrapone suppressed the Cushing syndrome. The ACTH producing tumour responded to combined chemo- and radiotherapy. The patient is symptom free two years after onset of treatment.

A 63-year-old man developed a carcinoid tumor of the cardia with unusual metastases of the brain and a rapid lethal course. Clinical aspects of these tumors, their attachment to apudomas and their new morphologic respectively functional--morphologic aspects are discussed.

Casuistically is reported on two patients with an endocrine inactive ileum carcinoid, in whom the anamnesis with 5 weeks or 10 months was very short and atypical. Continuously increasing abdominal pain and gradual formation of a lower ileus of the small intestine or profuse watery diarrhoeas were the leading symptoms. Intraoperatively or autoptically in each case a very small (less than 1.5 cm) ileum carcinoid was found, which had led to a stenosing of high degree, there were no fibroses of the endocardium, no liver metastases--accordingly the secretion of serotonine metabolites (5-HIES) in the urine had been normal. The possibility of a so-called endocrine-nervous enteropathy in the female patient with the diarrhoea symptomatology and the necessity of a rapid diagnostic clarification in suspicion of a tumour in the lower small intestine are discussed, since such a suspicion in most cases corresponds to a carcinoid and thus there exists a great chance of cure.

The case report is presented of a thirty five year-old female with haemangioblastoma of the right cerebellum, hämangioblastoma of the retina, phaeochromocytoma, and additional malignant carcinoid, liver metastases, portal hypertension and bleeding from oesophageal varices.

The case of a 67 year old patient with ileus caused by multiple carcinoids of ileum without endocrine symptoms is reported. Frequency, localisation and ways of metastasation , so as diagnostic and therapeutic possibilities are discussed.