A case of multiple endocrine neoplasia, type 1, with parathyroid adenoma, multinodular adrenal cortical hyperplasia, islet cell tumor and hyperplasia of thyroid with concomitant hypersecretion of gastrin and total glucagon-like immunoreactivity.

Shigematsu, S; Shimoyama, Nobuo; Sato, Yasufumi; Horita, Masakazu; Oribe, Yasuhiro; Ono, Junko; Takaki, Ryosaburo; Ishimatsu, Toshiyuki; Kodama, Mitsuhiro; Iwashita, Koichi

doi:10.2169/naika.78.357

Cited by 3 publications

(5 citation statements)

References 4 publications

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“…For the first comparison, 18 series were found that reported the non-peptic cause of death in ≥7 MEN1 patients with or without a PET 15,54,57,78,88,129,150,217,224,226,254,289,367,378,379,444,448,465 . For the second comparison with small series/case reports of MEN1 with PETs with at least 1 reported no-peptic ulcer disease related death, 108 separate reports containing 227 patients were identified of which 62 reports contained a single case 4–6,25,28,32,68,70,82,86,97,109,111,112,119,125,142,156,162,165,202,206,207,213,216,229,236,249,264,296,297,300,301,304–306,311,313,333,334,358,359,361,369,381,386,387,391,393,396,398,409,411,414,426,430,438,449,460,462,464,467,468 and 40 contain more than one case (mean-4.3 cases/report) 21,22,30,40,40,41,46,94,113,…”

Section: Methodsmentioning

confidence: 99%

“… Abbreviations: lit=literature; MEN1 = multiple endocrine neoplasia type-1; ZES = Zollinger-Ellison syndrome; HPT = hyperparathyroidism; PET=Pancreatic endocrine tumor: CNS=central nervous system. (1) Adrenal abnormality refers to presence of an abnormality detected on imaging studies (NIH[n=45],lit[n=9]),and/or clinically adrenal Cushing syndrome (NIH[n=3],lit[n=1]), or pheochromocytoma (NIH[n=2],lit[n=1]). (2) Other functional PETs includes insulinoma (NIH[n=10],lit[n=27])), glucagonoma (NIH[n=1], lit[n=8]), carcinoid syndrome (NIH[n=1],lit[n=1]), GRFoma ([lit=1]), SSoma ([lit=1]), ACTHoma ([lit=1]), and VIPoma ([lit=3]), (3) CNS tumor includes meningiomas (NIH[n=4]), ependymonas (NIH[n=1],lit[n=2]),schwanomas (NIH[n=1]), and neurolemmona (NIH[n=1],. (4) Smooth muscle tumors includes leiomyomas (NIH[n=1],lit[n=1]), and leiomyosarcomas(NIH[n=2]). (5) Onset of MEN1 was time of first symptom compatible with MEN1 or time of detection at screening if asymptomatic and time of diagnosis was time patient first told had MEN1. (6) Age at death was for 24 patients and for last follow-up was 82 patients [NIH]. (7) The 227 patients with MEN1 and PETs from the literature were identified as described in METHODS and included patients who died during followup of other than peptic disease due to ZES reported in 108 small series (<7 cases) or case reports of which 62 reports contained a single case 4–6, 25, 28, 32, 68, 70, 82, 86, 97, 109, 111, 112, 119, 125, 142, 156, 162, 165, 202, 206, 207, 213, 216, 229, 236, 249, 264, 296, 297, 300, 301, 304–306, 311, 313, 333, 334, 358, 359, 361, 369, 381, 386, 387, 391, 393, 396, 398, 409, 411, 414, …”

Section: Figurementioning

confidence: 99%

“…NF-PET-nonfunctional pancreatic endocrine tumor. (1) Type of adrenal abnormality, carcinoid tumor, other PET, other functional PET, CNS tumor, skin tumor or smooth muscle tumor are specified in legend of Table 1. (2) Other carcinoids refers to 7 patients with gastric, 3 with lung and 1 with intestinal carcinoids. (3) Percentage of the reported patients with or without an MEN-1 related death that had the indicated MEN1 characteristic. (4) The 227 patients with MEN1 and PETs from the literature were identified as described in METHODS and included patients who died during followup of other than peptic disease due to ZES reported in 108 small series (<7 cases) or case reports of which 62 reports contained a single case 4,–6, 25, 28, 32, 68, 70, 82, 86, 97, 109, 111, 112, 119, 125, 142, 156, 162, 165, 202, 206, 207, 213, 216, 229, 236, 249, 264, 296, 297, 300, 301, 304–306, 311, 313, 333, 334, 358, 359, 361, 369, 381, 386, 387, 391, 393, 396, 398, 409, 411, 414, 426, 430,438,449,460,462,464,467,468 and 40 contain more than one case (mean-4.3 cases/report) 21,22,30,40,40,41,46,94,113,120,153,…”

Section: Figurementioning

confidence: 99%

“…Literature review included 108 small series (<7 cases) or case reports of which 62 reports contained a single case 4–6,25,28,32,68,70,82,86,97,109,111,112,119,125,142,156,162,165,202,206,207,213,216,229,236,249,264,296,297,300,301,304–306,311,313,333,334,358,359,361,369,381,386,387,391,393,396,398,409,411,414,426,430,438,449,460,462,464,467,468 and 40 contain more than one case (mean-4.3 cases/report) 21,22,30,40,40,41,46,94,113,120,153,166,180,203,223,226,230,238,244,246,247,303,346,353,362,372,375,389,398,406,407,413,425,…”

Section: Figurementioning

confidence: 99%

“… (1) Oral cancer was a case of carcinoma of the tongue in two series 54,150 . (2) GI diseases include one patient in two series with complications of diverticulitis 54,88 . (3) Other includes “old age” 54 in 1 case; 11 cases in which cause of death not specified 78 ; one case 367 due to peritonitis of unknown origin; in study 448 1 due to Cor pulmonale, 1 to chronic pericarditis, and 1 to mesenteric artery thrombosis; 1 death due to small bowel obstruction 254 ; 1 case of accidental drug overdose in our series. (4) Lung diseases include one death due to respiratory failure 54 ; 5 to pulmonary embolus in three studies 88,129,150,448 ; one to emphysema/COPD 88 ; 3 to pneumonia 78 . (5) Neurological diseases include Alzheimers in 1 patient 150 ; 1 patient with multiple sclerosis 88 ; 1 patient with Lewy body dementia 448 . (6) Literature review includes 108 small series (<7 cases) or case reports of which 62 reports contained a single case 4–6,25,28,32,68,70,82,86,97,109,111,112,119,125,142,156,162,165,202,206,207,213,216,229,236,249,264,296,297,300,301,304–306,311,313,333,334,358,359,361,369,381,386,387,391,393,396,398,…”

Section: Figurementioning

confidence: 99%

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Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

et al. 2013

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Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess state, which was a major cause of death in these patients in the past, coupled with the recognition that nonendocrine tumors increasingly develop late in the disease course, the natural history of the disease has changed. An understanding of the current causes of death is important to tailor treatment for these patients and to help identify prognostic factors; however, it is generally lacking.To add to our understanding, we conducted a detailed analysis of the causes of death and prognostic factors from a prospective long-term National Institutes of Health (NIH) study of 106 MEN1 patients with pancreatic endocrine tumors with Zollinger-Ellison syndrome (MEN1/ZES patients) and compared our results to those from the pooled literature data of 227 patients with MEN1 with pancreatic endocrine tumors (MEN1/PET patients) reported in case reports or small series, and to 1386 patients reported in large MEN1 literature series. In the NIH series over a mean follow-up of 24.5 years, 24 (23%) patients died (14 MEN1-related and 10 non-MEN1-related deaths). Comparing the causes of death with the results from the 227 patients in the pooled literature series, we found that no patients died of acute complications due to acid hypersecretion, and 8%–14% died of other hormone excess causes, which is similar to the results in 10 large MEN1 literature series published since 1995. In the 2 series (the NIH and pooled literature series), two-thirds of patients died from an MEN1-related cause and one-third from a non-MEN1-related cause, which agrees with the mean values reported in 10 large MEN1 series in the literature, although in the literature the causes of death varied widely. In the NIH and pooled literature series, the main causes of MEN1-related deaths were due to the malignant nature of the PETs, followed by the malignant nature of thymic carcinoid tumors. These results differ from the results of a number of the literature series, especially those reported before the 1990s. The causes of non-MEN1-related death for the 2 series, in decreasing frequency, were cardiovascular disease, other nonendocrine tumors > lung diseases, cerebrovascular diseases. The most frequent non-MEN1-related tumor deaths were colorectal, renal > lung > breast, oropharyngeal. Although both overall and disease-related survival are better than in the past (30-yr survival of NIH series: 82% overall, 88% disease-related), the mean age at death was 55 years, which is younger than expected for the general population.Detailed analysis of causes of death correlated with clinical, laboratory, and tumor characteristics of patients in the 2 series allowed identification of a number of prognostic factors. Poor prognostic factors included higher fasting gastrin levels, presence of othe...

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Section: Methodsmentioning

confidence: 99%

Section: Figurementioning

confidence: 99%

Section: Figurementioning

confidence: 99%

Section: Figurementioning

confidence: 99%

Section: Figurementioning

confidence: 99%

See 3 more Smart Citations

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

et al. 2013

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Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

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Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1.

Skogseid¹,

Larsson²,

Lindgren³

et al. 1992

The Journal of Clinical Endocrinology & Metabolism

126

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In multiple endocrine neoplasia type 1 (MEN-1), benign enlargement of the adrenal cortex has been found in about one third of necropsy cases. To elucidate the clinical and genetic characteristics of the MEN-1 adrenal lesion, we have investigated 33 MEN-1 patients. Twelve individuals (37%) demonstrated adrenal enlargement, which was bilateral in 7 of them. Histopathology revealed diffuse and nodular cortical hyperplasia, adenomas, and a single case of adrenocortical carcinoma. The apparently benign adrenal enlargements were not associated with presently ascertainable biochemical disturbances in the hypothalamic-pituitary-adrenocortical axis, and they were without radiological signs of progression during follow-up. The individual developing unilateral adrenocortical carcinoma showed rapid adrenal expansion, feminization, and an abnormal urinary steroid profile after 4 yr of observation for bilateral minor adrenal enlargements. Pancreatic endocrine tumors were significantly overrepresented and present in all MEN-1 individuals with adrenal involvement. In agreement with findings in sporadic cases, the MEN-1 adrenocortical carcinoma genome showed loss of constitutional heterozygosity for alleles at 17p, 13q, 11p, and 11q. The benign adrenal lesions retained heterozygosity for the MEN-1 locus at chromosome 11 q 13. Despite its prevalence and malignant potential, the pituitary-independent adrenocortical proliferation does not appear to be a primary lesion in MEN-1, but might represent a secondary phenomenon, perhaps related to the pancreatic endocrine tumor.

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A case of multiple endocrine neoplasia, type 1, with parathyroid adenoma, multinodular adrenal cortical hyperplasia, islet cell tumor and hyperplasia of thyroid with concomitant hypersecretion of gastrin and total glucagon-like immunoreactivity.

Cited by 3 publications

References 4 publications

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1.

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