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Multiple Endocrine Neoplasia Type 2 Syndromes: Historical Perspectives Henry Ford Hospital Medical Journal Volume 32 Number 4 First International Workshop on Article 3 MEN-2 12-1984 Multiple Endocrine Neoplasia Type 2 Syndromes: Historical Perspectives John H. Sipple Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Sipple, John H. (1984) "Multiple Endocrine Neoplasia Type 2 Syndromes: Historical Perspectives," Henry Ford Hospital Medical Journal : Vol. 32 : No. 4 , 219-222. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol32/iss4/3 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp Med J Vol 32, No 4, 1984 Multiple Endocrine Neoplasia Type 2 Syndromes: Historical Perspectives John H. Sipple, MD=' bponyms are flattering, sometimes embarrassing, and So i dug deeper in the library stacks and was able to find certainly not descriptive. When I was asked to give the five other cases of carcinoma of the thyroid in patients historical perspective on the Sipple syndrome at this with pheochromocytoma. All were quite similar to the conference, I was flattered. The paper 1 wrote in 1959 case I had seen, mainly young adults with bilateral was my oniy venture into endocrinology, and 1 have pheochromocytomas and anaplastic-appearing car­ worked in the field of pulmonary disease for the last 25 cinomas of the thyroid. All five cases had been reported years. So much outstanding work has been done by so because of some interesting medical or surgical aspect many investigators in this fieid, and my contribution of pheochromocytoma and not because of the presence was so minimal, that remembering the name MEN-2 is of thyroid carcinoma. At that time, there were slightly more meaningful to a medical student than remem­ more than 500 cases of pheochromocytoma in the litera­ bering an eponym. ture. The six cases of carcinoma of the thyroid were somewhere between 14 and 40 times the expected inci­ dence if it were a chance occurrence. No familial history Discovery of the Sipple Syndrome of pheochromocytoma or carcinoma of the thyroid was i was a medical resident in 1959 when i was asked bythe reported in any of the six cases, although a few cases of neurosurgical service to consult on the case of a 33-year- familial pheochromocytoma had been reported by that old man who was hypertensive after he had surgery for time, it was well known that 5% of the patients with arteriovenous malformation of the brain with cerebral pheochromocytoma had neurofibromatosis. Pheo­ hemorrhage. I thought his hypertension was due to chromocytoma had also been described in some of the increased intracranial pressure; but, as I stood at the inherited neurocutaneous syndromes in which vascular autopsy tabie, 1 was amazed when I saw large, bilateral malformations in the brain occurred. The clue which pheochromocytomas and a 2 cm pale tan mass in each should have led me to suspect that my patient's prob­ lobe of the thyroid gland and nodular enlargement of lem was hereditary was that he had an arteriovenous the oniy parathyroid gland we could find. Micro­ malformation in the brain. It seemed reasonable that scopically, the thyroid tumors, called follicular pheochromocytoma and neurofibromatosis might be adenocarcinoma (1), were invasive and pooriy differ­ an inherited condition because both tissues arise from entiated. Since this was the same year in which Hazard neuroectoderm, but I could not understand how car­ first described medullary carcinoma (2), it was no won­ cinoma of the thyroid gland would fit into that scheme. der that our pathologists did not recognize it as such. The pheochromocytomas had high levels of epi­ In my literature search I found experimental data to nephrine and norepinephrine, so there was no doubt suggest that fluctuating levels of catecholamines might that they were functional. Since we did not have serum cause fluctuating levels of thyroid-stimulating hormone calcium or phosphorus determinations, we did not (TSH), which might cause alternating hyperplasia and know whether the patient had had hyperpara­ involution of thyroid follicular cells, which in turn, thyroidism. might result in carcinoma of the thyroid gland. So I wrote a three-page paper entitied "The Association of As I stood at the autopsy table, I knew 1 was looking at Pheochromocytoma with Carcinoma of the Thyroid something special although I did not understand it. I Gland," submitted it to the American Journal of Med­ doubted very much that it was a chance occurrence. icine, and it was published in 1961 (1). When i went to the literature, I found oniy a 1952 book by DeCourcy and DeCourcy entitled Pheochro­ mocytoma and the General Practitioner (3). They stated Submitted for publication: July 24, 1984 that "a considerable and, we beiieve, a significantly Accepted for publication: December 3, 1984 high percentage of patients with pheochromocytoma •Department of Medicine, State University of New York, Upstate Medical have had coexistent diffuse or nodular goiter or thyroid Center, Syracuse carcinoma." That was interesting, but it provided no Address reprint requests to Dr. J.H. Sipple, Department of Medicine, State University of New York, Upstate Medical Center, 766 Irving Avenue, Syracuse, facts. New York 13210, 219 Sipple Medullary Carcinoma of the Thyroid proven and 15 probable cases of pheochromocytoma, The association I reported in 1961 was developed into a five cases of MTC, and two cases with parathyroid chief- well-defined syndrome by Cushman from Rochester, cell hyperplasia. The mix of cases differed somewhat in New York, in 1962 when he reported a family in which the 256-person kindred reported by a group from the the father had pheochromocytoma, medullary car­ National institutes of Health in 1973 (7). They found 25 cinoma of the thyroid, and parathyroid adenoma; the cases of MTC, 16 cases of parathyroid disease, and 11 son had pheochromocytoma and medullary carcinoma cases of pheochromocytoma. The increased number of of the thyroid; and the granddaughter had medullary MTC cases is a result of better detection through the use carcinoma of the thyroid gland (4). Cushman correctly of serum calcitonin as a marker for MTC. concluded that the syndrome was a Mendelian domi­ nantly inherited disorder and that the carcinoma of the The fascinating work of defining the nature of MTC thyroid gland was medullary (MTC), not a carcinoma of began in 1959 when Hazard described this entity as a the follicular cells. solid and nonfollicular carcinoma (2). Amyloid couid be In our case, the pathology had been reviewed by Dr. identified consistentiy in the stroma. The incidence of Schimke, and the carcinoma was medullary (5), as it was lymph node metastases is high, and the carcinoma in ail subsequent reports. appears undifferentiated under the microscope but is not highly malignant. Usually called papillary or an­ Dr. Anton Joachimpalli and Dr. Phillip Speller in aplastic carcinoma before Hazard's description, MTC Syracuse are the endocrinologists who studied the rela­ accounts for only about 5% of thyroid carcinomas. tives of the patient i reported. The family pedigree was About 20% of cases are familial, and most of the familial prepared by Dr. Joachimpalli (Figure). Both children of cases occur in MEN-2 kindreds (8). the propositus had pheochromocytoma and MTC, as did one of his brothers. Two of the brother's children had MTC, and one had C-ceii hyperplasia. A seven-year- old grandchild has elevated calcitonin levels and is awaiting thyroid surgery. Discovery of Serum Calcitonin as a Marker for MTC [pt84 (j)t78 [pt83 (pt3 In 1966, Williams reported thatthe parafollicular cells in the thyroid are the cells of origin of medullary car­ cinoma (9). in his studies, Pearse (10) found evidence Dt38 itis itas A* n that, in response to elevated serum calcium, these cells secreted a serum calcium-lowering hormone which he 65 /-N 063 called thyrocalcitonin. In 1968, three reports confirmed calcitonin secretion by MTC (11-13), and by the early 1970s, Tashjian and associates developed a radio­ immunoassay for the hormone in serum (14-16). Serum |i]i6 (^14 is 07 calcitonin was measured before and after calcium in­ fusion in 170 members of kindreds with MEN-2. In 47 subjects whose calcitonin tests were positive, 42 had Figure thyroidectomies, and ali had C-ceil abnormalities; Pedigree of Sipple's original case of pheochromocytoma and medullary 35, medullary carcinoma; and 7, C-ceil hyperplasia. carcinoma of the thyroid (courtesy of Dr. Anton Joachimpalli). Thyroid nodules or palpable lymph nodes couid be demonstrated in only half of the cases. Thereafter, Medullary Thyroid Carcinoma and Pheochromocytoma other families with MEN-2 were discovered by cal­ W\ Medullary Thyroid Carcinoma citonin tests performed on relatives of patients thought ^ C-cell Hyperplasia of Thyroid to have sporadic MTC or sporadic or familial pheo­ r*1 Tested and No Disease chromocytoma. Thus was discovered a very sensitive t Died and specific tumor marker. 33 Age in 1984 or at time of deatn ? Possible cases Calcitonin testing and thyroidectomy in patients with ^ Propositus MEN-2 disclosed that the C-cell abnormality is a rather diffuse process. The carcinomas are almost always bi­ By 1968, 41 cases of pheochromocytoma and MTC had lateral, and areas of C-ceil hyperplasia are frequently been reported in the literature, as reviewed by Steiner, adjacent to areas of carcinoma. Occasionally, in Goodman, and Powers in Medicine (6). They also re­ younger patients, oniy C-cell hyperplasia is present, ported a kindred of 186 persons, which included 10 indicating that it is a precursor of medullary carcinoma.
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