(1) These factors are also associated with multiple endocrinological neoplasms type II of Sipple's syndrome.
(2) In a case of familial Sipple disease, follicular lining cells were replaced in areas with ACTH-containing cells.
(3) We recommend the calcium infusion test for: (a) screening for MCT in all Sipple relatives with normal or only slightly elevated basal S-CT, (b) postoperative control in both sporadic and hereditary MCT, (c) investigation of supposed non-MCT tumours with calcitonin production.
(4) Particular attention is drawn to two cases with bilateral sites (one male child, one woman with Sipple's syndrome) and the detection of possible local and distant malignancy.
(5) One hundred and fourteen cases of Sipple's syndrome have been reported in Japan.
(6) MCT would thus appear to be more frequently visualized by MIBG in cases of Sipple's syndrome than in sporadic cases.
(7) Sipple's syndrome (bilateral phaeochromocytoma and medullary thyroid carcinoma) is a multiple endocrinopathy with a familial occurrence.
(8) Approximately twice as often an isolated variant of the familial type of medullary carcinoma is involved, as compared with association with another endocrine affection, in particular pheochromocytoma (Sipple's syndrome), but associated forms will increase in number perspectively (multiple endocrine neoplasia 2A).
(9) Whether this is due to a real geographical variation of incidence, to a larger number of unreported cases in the Northern parts, or to presence of families with Sipple's syndrome in the South is unclear.
(10) Extensive studies revealed that the patient was suffering from a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome) with bilateral pheochromocytomas and a medullary thyroid carcinoma.
(11) Of the multiple endocrine syndromes, the association between pheocromochytoma, medullary thyroid carcinoma (MTC) and parathyroid-hyperplasia (Sipple's syndrome, MEN IIa), and the association of MTC, with multiple monocutaneous neuromas and with a characteristic facial appearance (MEN IIb syndrome) are well known.
(12) In our opinion MR may replace both CT and nuclear scans in the work up of Sipple disease.
(13) Twenty-nine patients had the familial form of MTC; they were subclassified, on the basis of the phenotype, into a group of 15 patients with Sipple syndrome (or multiple endocrine neoplasia (MEN) Type 2A) and a group of 14 patients with mucosal-neuroma phenotype (or MEN type 2B).
(14) Although an exact reason for this remained unknown, our data could suggest that the adrenal tumors in Sipple's syndrome are primarily different from other types of pheochromocytoma, and thorough examinations for detecting the thyroid carcinoma should be necessary when the adrenal tumor is rich in epinephrine.
(15) This calcium infusion test was used in a prospective screening for medullary carcinoma of the thyroid (MCT) in 4 families with Sipple's syndrome as well as in 3 sporadic cases of MCT.
(16) Our patient had a strong family history for Sipple syndrome, which is considered to be transmitted as an autosomal dominant.
(17) However, such other symptoms of Sipple syndrome as presence of endocrine tumors are absent.
(18) This case report describes the localization and categorization of tumors using 99mTc(V)-dimercaptosuccinic acid and [131I]metaiodobenzylguanidine scans in a very uncommon case of medullary thyroid carcinoma associated with pheochromocytoma (Sipple's syndrome) and hepatocellular carcinoma.
(19) This study deals with 11 cases of thyroid medullary carcinoma of which 7 were familial cases including 3 cases of Sipple's syndrome and 4 cases of sporadic cases.
(20) A parallel between this situation and Sipple's disease in humans has previously been suggested.