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Mineral Metabolic Effects Of Thyroidectomy And Long-Term Outcomes In A Family With Men 2a, Henry G. Bone Iii, Leonard J. Deftos, William H. Snyder, Charles Y. C. Pak Sep 1992

Mineral Metabolic Effects Of Thyroidectomy And Long-Term Outcomes In A Family With Men 2a, Henry G. Bone Iii, Leonard J. Deftos, William H. Snyder, Charles Y. C. Pak

Henry Ford Hospital Medical Journal

We have followed a family with multiple endocrine neoplasia type 2A for 18 years. Four members have undergone total thyroidectomy for medullary thyroid carcinoma or C-cell hyperplasia, and one has required bilateral adrenalectomy for pheochromoctyoma. None has developed hypercalcemic hyperparathyroidism, although parathyroid hormone levels were relatively high prethyroidectomy and fell postoperatively in the patients with high calcitonin levels. In three of the four cases, intestinal calcium absorption decreased following thyroidectomy.


Multiple Endocrine Neoplasia Type 2b: Eighteen-Year Follow-Up Of A Four-Generation Family, Glen W. Sizemore, J. Aiden Carney, Hossein Gharib, Charles C. Capen Sep 1992

Multiple Endocrine Neoplasia Type 2b: Eighteen-Year Follow-Up Of A Four-Generation Family, Glen W. Sizemore, J. Aiden Carney, Hossein Gharib, Charles C. Capen

Henry Ford Hospital Medical Journal

Seven members with multiple endocrine neoplasia type 2B from a 15-member family have been followed for 18 years. All affected had the neuroma phenotype in a distribution compatible with autosomal dominant inheritance. The phenotype features have allowed 100% initial and continuing prediction of affected versus nonaffected status in as early as 1.5 years. Among the affected: immunoreactive plasma calcitonin (iCT) concentration was high in 100%; thyroid palpation was false-negative in 71%; and thyroid scintiscan was false-negative in 83%. All had total thyroidectomy, plus lymphadenectomy in three, for bilateral medullary thyroid carcinoma (MTC) or C-cell hyperplasia (in the youngest). None ...


Extracellular Ca2+ Sensing In C-Cells And Parathyroid Cells, Hans Scherubl, Maria Luisa Brandi, Jurgen Hescheler Sep 1992

Extracellular Ca2+ Sensing In C-Cells And Parathyroid Cells, Hans Scherubl, Maria Luisa Brandi, Jurgen Hescheler

Henry Ford Hospital Medical Journal

An essential function of C-cells and parathyroid cells is to monitor the extracellular Ca2+ concentration. The Ca2+-dependent secretion of calcitonin (CT) and parathyroid hormone is known to be mediated by corresponding changes in the intracellular Ca2+ concentration. To address the question of whether Ca2+ influx through voltage-dependent Ca2+ channels couples the extracellular to the intracellular Ca2+, we applied the patch clamp technique to C-cells of the rMTC 44-2 cell line and to parathyroid cells of the PT-r cell line. The rMTC cells displayed dihydropyridine-sensitive, voltage-dependent, high-threshold Ca2+ channels which allowed ion influx ...


The Importance Of Screening For The Men 1 Syndrome: Diagnostic Results And Clinical Management, C. J. M. Lips, H. P. F. Koppeschaar, M. J. H. Berends, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven Sep 1992

The Importance Of Screening For The Men 1 Syndrome: Diagnostic Results And Clinical Management, C. J. M. Lips, H. P. F. Koppeschaar, M. J. H. Berends, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven

Henry Ford Hospital Medical Journal

No abstract provided.


Localization Of The Gene For Men 2a, Jay B. Lichter, Michael Difilippantonio, Jingshi Wu, Diane Miller, David C. Ward, Paul J. Goodfellow, Kenneth K. Kidd Sep 1992

Localization Of The Gene For Men 2a, Jay B. Lichter, Michael Difilippantonio, Jingshi Wu, Diane Miller, David C. Ward, Paul J. Goodfellow, Kenneth K. Kidd

Henry Ford Hospital Medical Journal

The search for the gene that causes the multiple endocrine neoplasia type 2A (MEN 2A) syndrome is entering a new phase. Genetic linkage studies have localized the gene to the pericentromeric region of chromosome 10. The statistical portion of mapping the gene for MEN 2A is nearly complete and now classical molecular biological/gene mapping techniques will be employed. We have used fluorescence in situ hybridization to estimate the size of the MEN2A region to be about 2 to 5 mb, using some liberal assumptions; at worst the region should contain no more than about 10 mb of non-alphoid DNA ...


Front Matter Sep 1992

Front Matter

Henry Ford Hospital Medical Journal

No abstract provided.


A Preliminary Analysis Of Consortium Data For Markers Tightly Linked To Multiple Endocrine Neoplasia Type 2a, J. B. Lichter, S. M. Hackleman, B. A. J. Ponder, D. Easton, S. A. Narod, G. M. Lenoir, R. F. Gagel, N. E. Simpson, E. Gardner, P. J. Goodfellow, S. Takai, A. J. Pakstis, K. K. Kidd Sep 1992

A Preliminary Analysis Of Consortium Data For Markers Tightly Linked To Multiple Endocrine Neoplasia Type 2a, J. B. Lichter, S. M. Hackleman, B. A. J. Ponder, D. Easton, S. A. Narod, G. M. Lenoir, R. F. Gagel, N. E. Simpson, E. Gardner, P. J. Goodfellow, S. Takai, A. J. Pakstis, K. K. Kidd

Henry Ford Hospital Medical Journal

We have analyzed DNA marker typing data contributed by six independent groups to estimate the pairwise genetic distances between these markers and the locus for multiple endocrine neoplasia type 2A (MEN 2A). We used LIPED to calculate these distances for female, male, and sex-average linkage maps and to determine the corresponding LOD scores. The preliminary analyses of this large data set (89 MEN 2A families and five non-MEN 2A references families, with 1,934 total individuals) are reported here. These refined estimates of the genetic map in this region will aid in the assignment of presymptomatic diagnoses. This study clearly ...


Somatostatin Acts Via A Pertussis Toxin-Sensitive Mechanism On Calcitonin Secretion In C-Cells, Angela Zink, Hans Scherubl, Friedhelm Raue, Reinhard Ziegler Sep 1992

Somatostatin Acts Via A Pertussis Toxin-Sensitive Mechanism On Calcitonin Secretion In C-Cells, Angela Zink, Hans Scherubl, Friedhelm Raue, Reinhard Ziegler

Henry Ford Hospital Medical Journal

The effect of the somatostatin analog octreotide on cAMP-mediated calcitonin (CT) secretion and cAMP accumulation in C-cells was investigated. Glucagon stimulated cAMP accumulation and CT secretion with a maximal effect at a concentration of 10-6 M. The cAMP antagonist RpcAMPs blocked the glucagon-induced CT secretion down to control levels. Therefore, no other second messengers seem to be involved in glucagon-stimulated CT secretion. Octreotide in increasing doses (10-9 to 10-6 M) inhibited cAMP accumulation and CT secretion with a maximal effect at a concentration of 10-7 (40% and 29% of control values, respectively). Pretreatment of the cells ...


Primary Hyperparathyroidism In Patients With Multiple Endocrine Neoplasia Type 1: Experience By A Single Surgical Team In Japan, Takao Obara, Yoshihide Fujimoto, Yukio Ito Sep 1992

Primary Hyperparathyroidism In Patients With Multiple Endocrine Neoplasia Type 1: Experience By A Single Surgical Team In Japan, Takao Obara, Yoshihide Fujimoto, Yukio Ito

Henry Ford Hospital Medical Journal

Nineteen patients were surgically treated for hyperparathyroidism associated with multiple endocrine neoplasia type 1 syndrome. Fourteen patients (74%) had removal of three or more parathyroid glands at the first operation, and five (26%) by removal of 2 1/2 or fewer glands. Two patients had recurrent hypercalcemia during the mean follow-up period of 65 months. One had a recurrence 10 years after subtotal parathyroidectomy. Reexploration in this patient revealed enlargement of the remaining tissue in the neck and an enlarged supernumerary gland in the aorticopulmonary window. The other patient had persistent hypercalcemia after removal of two hyperplastic parathyroid glands until ...


High-Sensitivity Serum Calcitonin Assays Applied To Screening For Thyroid C-Cell Disease In Multiple Endocrine Neoplasia Type 2a, Michael M. Kaplan, Glenn M. Stall, Timothy Cummings, Andrew Macaulay, Philippe Motté, Hubert J. Wolfe, Seymour Reichlin, Arman J. Tashjian Jr. Sep 1992

High-Sensitivity Serum Calcitonin Assays Applied To Screening For Thyroid C-Cell Disease In Multiple Endocrine Neoplasia Type 2a, Michael M. Kaplan, Glenn M. Stall, Timothy Cummings, Andrew Macaulay, Philippe Motté, Hubert J. Wolfe, Seymour Reichlin, Arman J. Tashjian Jr.

Henry Ford Hospital Medical Journal

Two serum calcitonin assays with sensitivities ≤ 10 pg/mL were compared to our standard radioimmunoassay (sensitivity 100 pg/mL) in multiple endocrine neoplasia type 2A (MEN 2A) screening. Values from the Nichols displacement radioimmunoassay averaged 38% higher than values from the CIS immunoradiometric assay; values from both were highly correlated, r = 0.845. In three individuals, both of the newer assays revealed abnormalities in pentagastrin tests three to four years before abnormalities were detected by the standard assay. Pentagastrin tests after total thyroidectomy were assayed by the newer methods in patients with medullary thyroid carcinoma (MTC) diagnosed at initial testing ...


Cutaneous Lesion Associated With Multiple Endocrine Neoplasia Type 2a: Lichen Amyloidosis Or Notalgia Paresthetica?, O. Chabre, F. Labat, N. Pinel, F. Berthod, V. Tarel, I. Bachelot Sep 1992

Cutaneous Lesion Associated With Multiple Endocrine Neoplasia Type 2a: Lichen Amyloidosis Or Notalgia Paresthetica?, O. Chabre, F. Labat, N. Pinel, F. Berthod, V. Tarel, I. Bachelot

Henry Ford Hospital Medical Journal

Three patients of a French family demonstrated an association of multiple endocrine neoplasia type 2A (MEN 2A) with a pruritic scapular skin lesion. The lesions are similar to those described as familial cutaneous lichen amyloidosis in unrelated MEN 2A and medullary thyroid carcinoma families, but histological, immunohistochemical, and ultrastructural analysis of skin biopsies from each patient in the French family did not show amyloid deposition. The topography of the lesion follows dermatomes C8-D3. The patients report not only pruritus but also paresthesia and hyperalgesia, and one showed touch hypoesthesia and pain hyperesthesia in the area of the lesion. Such an ...


Long-Term Follow-Up In Four Large Men 2 Families In The Netherlands, C. J. M. Lips, M. J. H. Berends, J. Brouwers-Smalbraak, R. A. Geerdink, W. H. L. Hackeng, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven Sep 1992

Long-Term Follow-Up In Four Large Men 2 Families In The Netherlands, C. J. M. Lips, M. J. H. Berends, J. Brouwers-Smalbraak, R. A. Geerdink, W. H. L. Hackeng, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven

Henry Ford Hospital Medical Journal

Results of follow-up studies in four large multiple endocrine neoplasia type 2A families (total of 95 patients affected) have shown a positive effect on the course of the disease since early screening and intervention were initiated in 1974.


Proceedings Of The Fourth International Workshop On Multiple Endocrine Neoplasia: Introduction, Robert F. Gagel, Charles E. Jackson Sep 1992

Proceedings Of The Fourth International Workshop On Multiple Endocrine Neoplasia: Introduction, Robert F. Gagel, Charles E. Jackson

Henry Ford Hospital Medical Journal

No abstract provided.


Sublocalization Of The Multiple Endocrine Neoplasia Type 1 Gene, Catharina Larsson, Gunther Weber, Marie Janson Sep 1992

Sublocalization Of The Multiple Endocrine Neoplasia Type 1 Gene, Catharina Larsson, Gunther Weber, Marie Janson

Henry Ford Hospital Medical Journal

Tumorigenesis in multiple endocrine neoplasia type 1 (MEN 1) involves the unmasking of a recessive mutation at the MEN 1 locus which has been mapped to chromosomal region 11q11-13. By analyzing 58 DNA markers on a panel of radiation-reduced somatic cell hybrids, the region encompassing the MEN 1 gene was divided into nine subregions. Pulsed field gel electrophoresis analysis of markers within subgroups showed that the recombination rate around the MEN 1 locus is high. Combined linkage analysis in MEN 1 families and deletion mapping in MEN 1-related tumors suggest the MEN 1 gene is located centromeric to D11S807 and ...


Molecular Genetic Mapping Of The Multiple Endocrine Neoplasia Type 1 Locus, Joanna T. Pang, Mark A. Pook, James H. Eubanks, Carol Jones, Veronica Van Heyningen, Glen A. Evans, Rajesh V. Thakker Sep 1992

Molecular Genetic Mapping Of The Multiple Endocrine Neoplasia Type 1 Locus, Joanna T. Pang, Mark A. Pook, James H. Eubanks, Carol Jones, Veronica Van Heyningen, Glen A. Evans, Rajesh V. Thakker

Henry Ford Hospital Medical Journal

Familial multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disorder characterized by the combined occurrence of tumors of the parathyroid glands, the endocrine pancreas, and the pituitary gland. MEN 1 tumors have previously been shown to be associated with the loss of alleles on chromosome 11, and deletion mapping studies together with family linkage studies have localized the MEN 1 gene to 11q13. A detailed genetic map around the MEN 1 locus is required to facilitate further characterization and cloning of the gene (MEN1). We have characterized a panel of seven rodent-human somatic cell hybrids which contain ...


Prospective Screening In Multiple Endocrine Neoplasia Type 1, Britt Skogseid, Kjell Oberg Sep 1992

Prospective Screening In Multiple Endocrine Neoplasia Type 1, Britt Skogseid, Kjell Oberg

Henry Ford Hospital Medical Journal

To assess the age of clinically detectable onset of multiple endocrine neoplasia type 1 (MEN 1), 88 members of four families were invited to participate in a ten-year biochemical screening program. Evidence for clinically detectable MEN 1 was found in adolescence. Pancreatic endocrine dysfunction constituted the presenting lesion in a majority of these individuals. The age at diagnosis of pancreatic endocrine tumors averaged 25 years and was lowered by almost two decades by prospective investigation. Furthermore, the penetrance of the pancreatic endocrine and parathyroid lesions equaled the penetrance found in autopsy studies. The use of a standardized meal stimulation test ...


Practical Guidelines For Dna-Based Testing In Multiple Endocrine Neoplasia Type 1, Catharina Larsson, Magnus Nordenskjold, Britt Skogseid, Kjell Oberg Sep 1992

Practical Guidelines For Dna-Based Testing In Multiple Endocrine Neoplasia Type 1, Catharina Larsson, Magnus Nordenskjold, Britt Skogseid, Kjell Oberg

Henry Ford Hospital Medical Journal

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant predisposition to neoplastic lesions of the parathyroid glands, the neuroendocrine pancreas, and the anterior pituitary gland. The predisposing genetic defect was localized to the long arm of chromosome 11 by genetic linkage analysis in three affected families. By analyzing six MEN 1 families with 14 DNA marker systems located close to the MEN 1 gene, we have developed a method to identify carriers of the MEN 1 predisposition. We describe practical aspects of such DNA-based diagnostic procedures.


Hyperparathyroidism With Normal Albumin-Corrected Total Calcium In Patients With Multiple Endocrine Neoplasia Type 1, J. J. Shepherd, Bin Tean Teh, V. Parameswaran, R. David Sep 1992

Hyperparathyroidism With Normal Albumin-Corrected Total Calcium In Patients With Multiple Endocrine Neoplasia Type 1, J. J. Shepherd, Bin Tean Teh, V. Parameswaran, R. David

Henry Ford Hospital Medical Journal

In the largest reported family of patients with multiple endocrine neoplasia type 1 (MEN 1), hyperparathyroidism was expressed at first screening in 33 patients by elevation of ionized calcium (IC) (30 cases) or parathyroid hormone (three cases) without elevation of albumin-corrected total calcium (ACTC). Three of these 33 patients have shown a progressive rise in IC and later an elevation of ACTC. However, the age distribution suggests that in others the level of IC may remain stable at a minimally elevated level throughout life with ACTC remaining normal except for transient rises at the times of intercurrent illness or surgical ...


Expression Of The Ret Proto-Oncogene In Human Medullary Thyroid Carcinomas And Pheochromocytomas Of Men 2a, Akihiro Miya, Masayuki Yamamoto, Hideki Morimoto, Norifumi Tanaka, Esei Shin, Katsu Karakawa, Kumao Toyoshima, Yukihito Ishizaka, Takesada Mori, Shin-Ichiro Takai Sep 1992

Expression Of The Ret Proto-Oncogene In Human Medullary Thyroid Carcinomas And Pheochromocytomas Of Men 2a, Akihiro Miya, Masayuki Yamamoto, Hideki Morimoto, Norifumi Tanaka, Esei Shin, Katsu Karakawa, Kumao Toyoshima, Yukihito Ishizaka, Takesada Mori, Shin-Ichiro Takai

Henry Ford Hospital Medical Journal

We studied the expression of the ret proto-oncogene (proto-ret) in human medullary thyroid carcinomas (MTCs) and pheochromocytomas of multiple endocrine neoplasia type 2A (MEN 2A) by Northern blot analysis. Expression of the normal-sized transcripts was detected in all 12 MTCs and in 6 of 8 pheochromocytomas. In situ localization of proto-ret mRNA revealed that the signal was confined to the cytoplasm of MTC cells. By Southern blot analysis neither amplification nor gross genetic changes of proto-ret were found in the tumors. Although no transcripts were detected in the normal portion of the thyroid from one MEN 2A patient,faint signals ...


Medullary Thyroid Carcinoma: Australian Experience With Genetic Testing, Janet L. Ward, Valentine J. Hyland, David S. Andrew, Debbie J. Marsh, Bruce G. Robinson Sep 1992

Medullary Thyroid Carcinoma: Australian Experience With Genetic Testing, Janet L. Ward, Valentine J. Hyland, David S. Andrew, Debbie J. Marsh, Bruce G. Robinson

Henry Ford Hospital Medical Journal

Linkage analysis has been performed in four pedigrees with multiple endocrine neoplasia type 2A (MEN 2A) or familial medullary thyroid carcinoma (MTC) using pericentromeric chromosome 10 probes. Important information regarding carrier status has been provided in 10 individuals, many of whom would not have been identified by pentagastrin stimulation testing. We have also used pulsed field gel electrophoresis (PFGE) to link the probes H4JRBP and pMCK2 to a 150 kb fragment. Using PFGE, no evidence was found in DNA from lymphocytes of a major DNA rearrangement in two individuals affected with MEN 2A and an individual with MEN 2B compared ...


Screening For Multiple Endocrine Neoplasia Type 2a With Dna-Polymorphism Analysis, Estelle M.-F. Lamothe, Steven A. Narod, Shari Miller, Paul J. Goodfellow, David E. C. Cole, Dawna Gilchrist, Zdenka Pausova, David Goltzman, Geoffrey N. Hendy Sep 1992

Screening For Multiple Endocrine Neoplasia Type 2a With Dna-Polymorphism Analysis, Estelle M.-F. Lamothe, Steven A. Narod, Shari Miller, Paul J. Goodfellow, David E. C. Cole, Dawna Gilchrist, Zdenka Pausova, David Goltzman, Geoffrey N. Hendy

Henry Ford Hospital Medical Journal

Nine chromosome 10 DNA markers (FNRB, D10S34, D10Z1, MEN203, D10S94, RBP3, D10S15, MBP [48.11], D10S22) were typed in two large Canadian pedigrees with multiple endocrine neoplasia type 2A (MEN 2A). These markers and the gene for MEN 2A (MEN2A) are believed to be in one linkage group spanning approximately 15 cM (male). MEN203 and D10S94 were informative and tightly linked to MEN2A with no recombinants observed in 26 meiotic events. D10S15 (MCK2), widely used in DNA genotyping predictions, demonstrated two recombinants in these two families. The use of multiple flanking markers increases both the likelihood of informativeness and the ...


Genetics Of The Multiple Endocrine Neoplasia Type 2b Syndrome, Charles E. Jackson, Robert A. Norum Sep 1992

Genetics Of The Multiple Endocrine Neoplasia Type 2b Syndrome, Charles E. Jackson, Robert A. Norum

Henry Ford Hospital Medical Journal

Multiple endocrine neoplasia type 2B (MEN 2B) is similar to MEN 2A in that both autosomal dominant syndromes include medullary thyroid cancers and pheochromocytomas. It is distinct in that MEN 2B patients have much earlier age of onset with more aggressive tumors and mucosa/neuromas of the lips and tongue. The neuromas allow ascertainment generally before age 5. Studies of two and three generations of 14 MEN 2B families disclosed close linkage of the MEN 2B gene to DNA markers to which MEN2A had been linked. Multipoint analysis utilizing additional results in three generations of a 15th family have disclosed ...


Characterization Of The Clinical Features Of Five Families With Hereditary Primary Cutaneous Lichen Amyloidosis And Multiple Endocrine Neoplasia Type 2, Marion F. Robinson, Eric J. Furst, Vincenzo Nunziata, Maria Luisa Brandi, Jorge P. Ferrer, Maria J. G. Martins Bugalho, Guiseppe Di Giovanni, Richard J. H. Smith, Donald T. Donovan, Bobby R. Alford, James F. Hejtmancik, Vittorio Colantuoni, Loredana Quadro, Edward Limbert, Irene Halperin, Enric Vilardell, Robert F. Gagel Sep 1992

Characterization Of The Clinical Features Of Five Families With Hereditary Primary Cutaneous Lichen Amyloidosis And Multiple Endocrine Neoplasia Type 2, Marion F. Robinson, Eric J. Furst, Vincenzo Nunziata, Maria Luisa Brandi, Jorge P. Ferrer, Maria J. G. Martins Bugalho, Guiseppe Di Giovanni, Richard J. H. Smith, Donald T. Donovan, Bobby R. Alford, James F. Hejtmancik, Vittorio Colantuoni, Loredana Quadro, Edward Limbert, Irene Halperin, Enric Vilardell, Robert F. Gagel

Henry Ford Hospital Medical Journal

The hereditary conditions of primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2 (MEN 2) are rare clinical entities. The initial reports of two families in which the two conditions coincided have led to the identification of at least eight additional families with this clinical syndrome. In this report we describe the clinical features in five of these eight families. The salient feature in these five families is the presence of unilateral (46%) or bilateral (64%) pruritic and lichenoid skin lesions located over the upper portion of the back. Family members describe these skin lesions as intermittently intensely pruritic ...


Statistical Analysis Of Histomorphological Findings In Medullary Thyroid Carcinoma: Distinction Between The Different Familial Forms Of The Disease, B. Franc, M. Rosenberg-Bourgin, B. Auvert, B. Caillou, N. Dutrieux-Berger, J. Floquet, M. Houcke-Lecomte, E. Justrabo, F. Labat-Moleur, M. F. Le Bodic, A. Pages, M. Patey, G. Viennet, F. Vilde, J. P. Saint-Andre Sep 1992

Statistical Analysis Of Histomorphological Findings In Medullary Thyroid Carcinoma: Distinction Between The Different Familial Forms Of The Disease, B. Franc, M. Rosenberg-Bourgin, B. Auvert, B. Caillou, N. Dutrieux-Berger, J. Floquet, M. Houcke-Lecomte, E. Justrabo, F. Labat-Moleur, M. F. Le Bodic, A. Pages, M. Patey, G. Viennet, F. Vilde, J. P. Saint-Andre

Henry Ford Hospital Medical Journal

A multifactorial analysis of morphological findings was performed on 153 cases of medullary thyroid carcinoma (MTC). The aim of the study was to utilize histological criteria to discriminate between MTC associated with multiple endocrine neoplasia type 2A (MEN 2A) and that associated with the inherited MTC only syndrome. The presence of fusiform cells associated with several other markers seemed to he more predictive of MEN 2A. A comparison of inherited MTC only and sporadic MTC only showed fusiform cells to be significantly less common in inherited MTC only. These results suggest that the inherited MTC only syndrome is a distinct ...


Microsurgical Lymph Node Dissection For Metastatic Asymptomatic C-Cell Carcinoma, Heinz J. Buhr, Friedrich Kallinowski, Friedhelm Raue, Christian Herfarth Sep 1992

Microsurgical Lymph Node Dissection For Metastatic Asymptomatic C-Cell Carcinoma, Heinz J. Buhr, Friedrich Kallinowski, Friedhelm Raue, Christian Herfarth

Henry Ford Hospital Medical Journal

In persistent, clinically inapparent medullary thyroid carcinoma, microsurgical dissection of all lymph node compartments of the neck was performed. Between August 1988 and September 1991, 28 cases (mean age 43.3 years) were treated with 38 surgical interventions. Twenty patients had the sporadic form and eight patients the familial form. Unilateral neck dissection resulted in normalization of serum calcitonin (CT) levels even after pentagastrin stimulation in two patients whereas 16 patients exhibited abnormal CT stimulation tests. Eight of ten patients who had bilateral neck dissections had positive pentagastrin test results after surgery. The main postoperative complications included loss of local ...


Should Patients With Medullary Thyroid Carcinoma Undergo Extensive Lymph Node (Re)Operation To Improve Long-Term Survival?, Caro Brumsen, Harm R. Haak, Bernard M. Goslings, Cornelis J. H. Van De Velde Sep 1992

Should Patients With Medullary Thyroid Carcinoma Undergo Extensive Lymph Node (Re)Operation To Improve Long-Term Survival?, Caro Brumsen, Harm R. Haak, Bernard M. Goslings, Cornelis J. H. Van De Velde

Henry Ford Hospital Medical Journal

To investigate the role of primary and/or secondary modified radical neck dissection (MRND) in patients with medullary thyroid carcinoma (MTC), follow-up data on 43 patients operated on between 1972 and 1983 were analyzed retrospectively; patients included 16 with sporadic MTC (group 1) and 27 with multiple endocrine neoplasia type 2 (group 2). Primary surgical therapy consisted of (near-) total thyroidectomy and limited lymph node dissection: one patient in group 1 and two in group 2 underwent primary MRND. Positive nodes were found in nine patients of group 1 and in 12 of group 2. Calcitonin (CT) was raised postoperatively ...


Oncogene And Growth Factor Expression In Men 2 And Related Tumors, Jeffrey F. Moley, Göran K. Wallin, Michele B. Brother, Michael Kim, Samuel A. Wells Jr., Garrett M. Brodeur Sep 1992

Oncogene And Growth Factor Expression In Men 2 And Related Tumors, Jeffrey F. Moley, Göran K. Wallin, Michele B. Brother, Michael Kim, Samuel A. Wells Jr., Garrett M. Brodeur

Henry Ford Hospital Medical Journal

Pheochromocytomas occur sporadically or in individuals affected by inherited syndromes including multiple endocrine neoplasia (MEN) type 2A and 2B, neurofibromatosis, and the von Hippel-Lindau syndrome (vHL). Medullary thyroid carcinomas (MTCs) also occur sporadically or as part of MEN 2A, MEN 2B, and familial MTC. Little is known of the molecular genetic background of these tumors. We have shown previously that activation of the N-ras, H-ras, and K-ras oncogenes does not occur in these tumors, but that deletions of the short arm of chromosome 1 are extremely common (> 60%) and may indicate loss of a suppressor gene in the chromosomal region ...


Evaluation Of Children With Medullary Thyroid Carcinoma, Alfonso Libroia, Fabrizio Muratori, Uberta Verga, Gianleone Di Sacco, Anna Grattieri, Dario Gelli Sep 1992

Evaluation Of Children With Medullary Thyroid Carcinoma, Alfonso Libroia, Fabrizio Muratori, Uberta Verga, Gianleone Di Sacco, Anna Grattieri, Dario Gelli

Henry Ford Hospital Medical Journal

Early diagnosis and surgical treatment of medullary thyroid carcinoma (MTC) in children is essential to decrease the likelihood of metastatic spread. From 1981 to 1991, eight children under 18 years of age (five girls and three boys) with MTC were seen and seven underwent total thyroidectomy. Followup ranged from 14 months to 10 years after surgery. Four of the seven presented with a neck mass and elevated basal levels of calcitonin (CT). After surgery, three had recurrent disease. In the other three, the diagnosis was made after several years of screening (normal basal values of CT but increased CT levels ...


Role Of Insulin-Like Growth Factor-I In The Autocrine Regulation Of Cell Growth In Tt Human Medullary Thyroid Carcinoma Cells, Kuo-Pao P. Yang, Naguib A. Samaan, Yi-Fan Liang, Socorro G. Castillo Sep 1992

Role Of Insulin-Like Growth Factor-I In The Autocrine Regulation Of Cell Growth In Tt Human Medullary Thyroid Carcinoma Cells, Kuo-Pao P. Yang, Naguib A. Samaan, Yi-Fan Liang, Socorro G. Castillo

Henry Ford Hospital Medical Journal

Since the TT human medullary thyroid carcinoma cell line required fewer exogenous growth factors (serum), we investigated whether this line has an autocrine mechanism by examining the effects of antibodies directed toward insulin-like growth factor I (IGF-I) and its receptor on TT cell growth in serum-free conditions. Treating cells with anti-IGF-I antibody for four days reduced the cell number by more than 50% compared with a nonimmune IgG control. Furthermore, a monoclonal antibody to the IGF-I receptor suppressed DNA synthesis when determined by a [3H]thymidine incorporation assay. Exogenous IGF-I (20 ng/mL) stimulated [3H]thymidine incorporation in ...


Pdn-21 (Katacalcin) And Chromogranin A: Tumor Markers For Medullary Thyroid Carcinoma, Friedhelm Raue, Eberhard Blind, Andreas Grauer Sep 1992

Pdn-21 (Katacalcin) And Chromogranin A: Tumor Markers For Medullary Thyroid Carcinoma, Friedhelm Raue, Eberhard Blind, Andreas Grauer

Henry Ford Hospital Medical Journal

The malignant C-cell releases several markets of potential clinical significance into the circulation. To determine the usefulness of these markers for management of medullary thyroid carcinoma (MTC), it is necessary to compare the usefulness of these markers with calcitonin (CT), the classical tumor marker for MTC. Measurement of serum concentrations of the peptide PDN-21 (katacalcin), a carboxyterminal cleavage product of procalcitonin. showed a high correlation with serum CT levels (r = 0.99, P < 0.01, n = 65 patients with MTC). The presence of equimolar concentrations of CT and PDN-21 (CT/PDN-21 molar ratio = 0.95 ± 0.33) indicates the peptide is cosecreted with CT. Stimulation of CT release by intravenous pentagastrin was associated with a parallel increase of PDN-21, providing further evidence of cosecretion of these two peptides. Finally, measurement of either PDN-21 or CT in selective venous catheterization specimens was useful for localization of MTC. Chromogranin A (CgA) levels were also measured in patients with MTC. Circulating levels were elevated in most patients with advanced disease. There was a moderate correlation between CgA and CT serum levels (r = 0.87, P < 0.01. n = 61 patients with MTC). Pentagastrin did not stimulate CgA, and the long half-life of CgA in the circulation did not make it possible to use this peptide for tumor localization by selective venous catheterization. We conclude that measurement of PDN-21 provides an independent assay system for diagnosis, localization, and postoperative management of MTC. whereas CgA measurement is not useful in early diagnosis of MTC and is of limited value for localization or management of progressive disease.