MEN Type 1 Associated with Mediastinal Carcinoid Producing Parathyroid Hormone, Calcitonin and Chorionic Gonadotropin

Takayama, Toshimasa; Kameya, Toru; Inagaki, K; Nonaka, Makoto; Miyazawa, Hideki; Ogawa, Norio; Yano, Makoto; Morita, Takatomo; Arai, Takashi; Niino, S; Tajima, Hiroshi; Yamaguchi, Ken

doi:10.1016/s0344-0338(11)80689-8

Cited by 11 publications

(7 citation statements)

References 17 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%

“…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%

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

et al. 2013

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“…Another case report of MEN 1 has shown ectopic parathyroid tissue in the mediastinal mass [10] and another has reported thymic carcinoid with atypical course in such patients [11]; however, very limited data are reported on the concomitant presence of parathyroid adenoma and asymptomatic metastatic mediastinal atypical carcinoid other than thymus. A single study has described a clear distinction between such a neuroendocrine tumor originating as ectopic tissue in the mediastinum rather than from a thymus gland [1].…”

Section: Discussionmentioning

confidence: 99%

Incidental metastatic mediastinal atypical carcinoid in a patient with parathyroid adenoma: a case report

et al. 2017

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BackgroundAtypical carcinoid arising from the mediastinal tissue is a rare neuroendocrine tumor and an association with parathyroid adenoma is very unusual. We report an unusual case of atypical carcinoid of mediastinum with metastasis in a patient presenting with parathyroid adenoma, which is the first case to be reported from Pakistan.Case presentationA 51-year-old Pakistani man was seen in postoperative intensive care after right parathyroidectomy and mediastinal mass resection for the management of postoperative hypocalcaemia. He had a background history of dyspnea. Examination was unremarkable. Preoperative laboratory evaluation revealed a calcium level of 12.7 mg/dl, phosphate of 1.9 mg/dl, serum albumin of 4.8 g/dl, alkaline phosphate of 94 U/L, and serum intact parathyroid hormone level 413.8 pg/ml. A technetium-99m sestamibi parathyroid scan showed right parathyroid increased tracer uptake. Further workup revealed a large mediastinal mass which was diagnosed as atypical carcinoid after Tru-Cut biopsy. He underwent right-sided parathyroidectomy and resection of the mediastinal mass. The histopathology confirmed it to be a parathyroid adenoma and atypical carcinoid tumor of his mediastinum with metastasis in his lymph node and parathyroid gland. Somatostatin receptor scintigraphy revealed a well-defined focus in his left hypochondriac region consistent with a somatostatin receptor scintigraphy-avid tumor. He was started on everolimus and planned for octreotide therapy. ConclusionsWe describe an incidental finding of atypical carcinoid of the mediastinum in a patient diagnosed as having parathyroid adenoma. Clinical manifestations of neuroendocrine syndromes are challenging. Some tumors cluster in a non-classic description with other common neoplasms. They rarely present in isolation, remain clinically silent, and need aggressive workup with the aid of imaging and histopathology.

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Choroidal Metastasis as the Initial Manifestation of a Pigmented Neuroendocrine Tumor

Eagle¹

2000

Arch Ophthalmol

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W e report the case of a 77-year-old woman in whom choroidal metastasis was the initial manifestation of a primary neoplasm presumed to be a pigmented pulmonary carcinoid tumor. The tumor initially was misdiagnosed cytologically and pathologically as a choroidal melanoma because it contained intrinsic melanin pigment. Positive immunoreactivity for cytokeratin, synaptophysin, chromogranin, and calcitonin and the presence of dense-core neurosecretory vesicles disclosed by electron microscopy established that the metastasis was a neuroendocrine tumor. Findings from systemic evaluation suggested that the primary tumor was located in the lung. The patient subsequently developed an intradural paraspinal metastasis, which also contained melanin pigment. The latter observation confirmed that the melanin in the uveal metastasis was intrinsic and did not represent secondary phagocytosis by tumor cells. Metastases from pigmented tumors of nonmelanocytic derivation are exceedingly rare but present a major diagnostic challenge to ocular pathologists and cytopathologists if the diagnosis is not suspected. Confirmatory immunohistochemical analysis should be obtained when a pigmented choroidal tumor thought to be a melanoma has atypical features.

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MEN Type 1 Associated with Mediastinal Carcinoid Producing Parathyroid Hormone, Calcitonin and Chorionic Gonadotropin

Cited by 11 publications

References 17 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

Incidental metastatic mediastinal atypical carcinoid in a patient with parathyroid adenoma: a case report

Choroidal Metastasis as the Initial Manifestation of a Pigmented Neuroendocrine Tumor

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