Multiple Endocrine Neoplasia Type 1 with Unusual Concomitance of Various Neoplastic Disorders

Nishimura, Yoshihiro; Yamashita, Koh; Yumita, Wataru; Yamazaki, Masanori; Katai, Miyuki; Sakurai, Akihiro; Hashizume, Kiyoshi

doi:10.1507/endocrj.51.75

Cited by 9 publications

(3 citation statements)

References 33 publications

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“…Recently it is increasingly recognized that MEN1 patients have an increased occurrence of other endocrine and non-endocrine tumors including carcinoid tumors (thymic [0–8%], gastric [7–35%], bronchial [0–8%], rarely intestinal); skin tumors [angiofibromas (88%), collagenomas (72%), lipomas (34%), melanomas]; central nervous system tumors(meningiomas, ependymonas, schwanomas)[0–8%]; and smooth muscle tumors (leiomyomas, leiomyosarcomas) [1–7%] 13,46,48,49,74,131,176,209,228,261,274,354,388,393,413,465 . In other reports small numbers of other tumors are also described, although it is unclear if they are increased in frequency or aggressiveness in MEN1 patients [lymphoma, renal cancer, hematological disorders (thrombotic thrombocytopenic purpura, myeloma), ovarian tumors, gastrointestinal stromal tumors, seminomas, chondrosarcoma, mesothelioma, thymomas] 1,77,84,89,150,214,216,256,312,341,410,432 .…”

Section: Introductionmentioning

confidence: 99%

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: Introductionmentioning

confidence: 99%

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

et al. 2013

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“…In addition, other nonendocrine malignant tumors are also being reported to occur in MEN-1 (11). These include lymphomas, renal cell cancer, melanoma, leiomyosarcoma, thrombotic thrombocytopenia purpura, myeloma, ovarian tumors, gastrointestinal stromal tumor (28), seminoma, chondrosarcoma, mesothelioma and thymomas (11) (29) (30) (31) (32) (33) (34) (35) (36,37). However, whether the incidence of these nonendocrine tumors is truly increased or not is unclear.…”

Section: Multiple Endocrine Neoplasia Type 1 (Men-1)mentioning

confidence: 99%

Multiple Endocrine Neoplasia

Norton¹,

Krampitz²,

Jensen³

2015

Surgical Oncology Clinics of North America

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“…Associated with this syndrom, other endocrine and non-endocrine lesions, such as adrenal cortical tumours, carcinoids of the bronchi, gastrointestinal tract and thymus, meningiomas, lipomas, angiofibromas, collagenomas can also occur in varying combinations (3)(4)(5)(6). Parathyroid gland hyperplasias or adenomas are the most common clinical manifestation of MEN1.…”

Section: Introductionmentioning

confidence: 99%

Unusual Pathologic Findings Associated with Multiple Endocrine Neoplasia Syndrome-1: Undescended Parathyroid Gland Hyperplasia and Mesenteric Cyst

Abdülrezzak

Kula²,

Akçan

et al. 2012

Erciyes Med J

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ÖZETAlthough gastro-entero-pancreatic tumors constitute main clinical features of multiple endocrine neoplasia type 1 (MEN1), mesenteric cyst has not previously been recognized clinically as part of this syndrome. We report here a woman who presented with combination of mesenteric cyst that was removed surgically and undescended hyperplastic parathyroid gland located in the superior cervical region. This case illustrates that mesenteric cyst, like other gastro-entero-pancreatic tumors, may be part of MEN1. Superior cervical and submandibular areas should be investigated carefully for any uptake when evaluating patients with sestamibi scintigraphy for parathyroid diseases because of potential conflict with salivary glands uptake of sestabimi.

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Multiple Endocrine Neoplasia Type 1 with Unusual Concomitance of Various Neoplastic Disorders

Cited by 9 publications

References 33 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

Unusual Pathologic Findings Associated with Multiple Endocrine Neoplasia Syndrome-1: Undescended Parathyroid Gland Hyperplasia and Mesenteric Cyst

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