1/8. Primary carcinoid of the testis associated with carcinoid syndrome.Testicular carcinoid is a rare disease accounting for less than 1% of all testicular neoplasms. It rarely manifests symptoms of carcinoid syndrome. Recent reports have noted that only 1.1-3.1% of testicular carcinoid tumors are complicated by carcinoid syndrome. In general, large tumor size and the presence of carcinoid syndrome are features associated with a malignant course. In the present case, pathological findings revealed pure carcinoid of the testis without metastasis. Moreover, watery diarrhea due to carcinoid syndrome disappeared and the serum serotonin level normalized following orchiectomy. The patient was followed up for 12 months with whole body computed tomography scan and assessment of serotonin levels. To date, there is no evidence of tumor recurrence. These findings suggest that monitoring serum serotonin levels may be useful as a marker during follow up of this type of tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome.Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome is a rare disease. Recent evidence suggests that these tumors form part of a continuous spectrum ranging from well-differentiated carcinomas to small cell carcinomas. We report two new cases and review the 23 cases reported in the literature since 1972. The different diagnostic modalities are discussed, and an algorithm for the diagnosis of ectopic secretion of adrenocorticotropin (ACTH) is presented. In the future, the advent of radiologic and nuclear imaging as well as more accurate workup should help to diagnose these tumors at an earlier stage and improve the long-term outcome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record -- zollinger-ellison syndrome: pathophysiology, diagnosis and therapy.BACKGROUND: zollinger-ellison syndrome is a very rare disease caused by tumor with gastrin producing cells accompanied by hypergastrinemia leading to gastric hypersecretion and peptic ulcers and their complications.CASE STUDY: female case of gastrinoma (zollinger-ellison syndrome; Z-E) with a record of 38 yrs of survival. Acute gastro-duodenal ulcers started at 28 yr of age and Z-E was diagnosed by using gastrin assays. Basal and maximal acid outputs and ratio of basal/maximal outputs were away over normal limits. Because of ulcer recurrence and complications, patient was subjected to several gastric surgeries but refused total gastrectomy. She was also treated with many H2-receptor (R) antagonists and proton-pump inhibitors (PPI), each new drug being initially highly effective but then showing declining efficacy except when PPI, lansoprazole was used. The gastrin level rose in the course of disease from initial high value of 2000 pg/mL to the extreme 4500 ng/mL at present. During the last 2 yrs, metastasis mainly to liver developed and they were successfully treated by synthetic octapeptide derivative of somatostatin and, as a result, metastatis partly reduced and plasma gastrin drasticly decreased. biopsy taken from liver metastasis showed the presence of typical gastrinoma cells with gastrin and chromogranin, while that from oxyntic mucosa revealed the ECL-cell hyperplasia with carcinoid tumors and unexpected gastric atrophy.CONCLUSIONS: This phenomenal case described in this article might be the new proven evidence needed by gastroenterologists to overturn the traditional treatment using total gastrectomy as a treatment of choice to the partial gastrectomy combined with proton pump inhibitors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Endoscopic mucosal resection in the management of gastric carcinoid tumors.BACKGROUND AND STUDY AIMS: Gastric carcinoid tumors are a rare disease. Previously, total gastrectomy was regarded as the treatment of choice. However, differences in biological malignancy have recently led to the increased use of endoscopic mucosal resection (EMR) for treatment. We studied the outcome of EMR in patients with gastric carcinoids who were treated at our hospital and discuss the indications for endoscopic treatment. patients AND methods: Between 1986 and 1999 we carried out gastric mucosal resection in five patients with gastric carcinoid tumors. The procedure used for EMR was either strip biopsy or endoscopic aspiration mucosectomy. RESULTS: The carcinoid tumors measured 10 mm or less in four of the five patients. Two patients had type A gastritis, and all had hypergastrinemia. There was no evidence of recurrence during follow-up (range 6 - 66 months; mean 32.6 months). CONCLUSION: EMR is useful in the management of type 1 gastric carcinoids as classified by Rindi (hypergastrinemia; tumor diameter of 10 mm or less).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Ampullary carcinoid tumors: rationale for an aggressive surgical approach.Two cases of ampullary carcinoid tumor are reported. These tumors are among the most rare of GI tract carcinoids and appear to have a distinct presentation and biological behavior from carcinoids arising in the duodenum. The existing literature is reviewed with attention to the implications for surgical management of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. carcinoid tumor of the kidney presenting as a large abdominal mass: report of a case.Carcinoid tumors are known to occur frequently in the gastrointestinal tract and they can develop in nearly every organ system. However, a carcinoid tumor of the kidney is an extremely rare entity and only 38 cases have been reported in the English literature. We herein describe the 39th case of primary renal carcinoid tumor, found in a young Japanese woman 2 months after delivery. The pathologic similarities of our case with those of previously reported cases are briefly reviewed together with some aspects of clinical features and prognostic factors of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome.Carcinoid tumors derived from neuroendocrine cells can release serotonin and other vasoactive substances into the systemic circulation, resulting in carcinoid syndrome. Testicular carcinoid, a rare disease accounting for less than 1% of all testicular neoplasms, rarely manifests symptoms of carcinoid syndrome. We describe a case of carcinoid syndrome arising from a primary testicular carcinoid tumor. A 21-year-old male patient presented with facial flushing and diarrhea for 5 years. He had an enlarged left testis and a 1-cm, ill-defined, hard, non-tender mass in his right testis. His 24 h urinary excretion of 5-hydroxyindoleacetic acid was elevated (16.1 mg/day). somatostatin receptor scintigraphy correlated with carcinoid tumor in both testes. Following bilateral orchiectomy, the patient's facial flushing and diarrhea disappeared.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Advanced endocrine cell carcinoma of the gallbladder: a patient with 12-year survival.gallbladder carcinoid is a rare disease. In previous reports, classical carcinoid, an entity with a good prognosis, has not been distinguished from endocrine cell carcinoma, a tumor associated with marked cell atypia and mitosis, and a poor prognosis. The patient was a 66 year old woman who presented to our hospital with a chief complaint of jaundice. Pre-operatively, she was diagnosed as having advanced gallbladder carcinoma invading the liver and the hepatic hilus. The patient underwent right hepatic trisegmentectomy with en bloc resection of the caudate lobe and extrahepatic bile ducts, extended lymph node clearance and left hepaticojejunostomy. Histopathological examination showed positive Grimelius staining, marked mitosis, and intense atypism, hence, the tumor was diagnosed as an endocrine cell carcinoma. Twelve years after surgery, the patient is healthy, without any sign of recurrence. We present this novel case of long-term survival and review the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |