Multiple Endocrine Neoplasia Type I and Cushing's Syndrome Due to an Aggressive ACTH Producing Thymic Carcinoid

Takagi, Junko; Otake, Kazuo; Morishita, Munehiko; Kato, Harumichi; Nakao, Naoki; Yoshikawa, Kazuhiro; Ikeda, Hiroshi; Hirooka, Y; Hattori, Yoshinobu; Larsson, Catharina; Nogimori, T

doi:10.2169/internalmedicine.45.1427

Cited by 29 publications

(16 citation statements)

References 16 publications

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“…However, GH production by a thymic carcinoid causing acromegaly has been reported 44 as also Cushing syndrome due to ACTH-producing thymic carcinoids. 45,46 The clinical course of carcinoid tumors is often indolent, but it can also be aggressive and resistant to therapy. 47 Thymic carcinoid tumors are generally a late manifestation of MEN1 syndrome because no affected individuals had thymic carcinoid as the initial manifestation of MEN1 syndrome.…”

Section: Carcinoid Tumorsmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 (MEN1): Not only inherited endocrine tumors

Falchetti

Marini

Luzi

et al. 2009

Genetics in Medicine

100

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Section: Carcinoid Tumorsmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 (MEN1): Not only inherited endocrine tumors

Falchetti

Marini

Luzi

et al. 2009

Genetics in Medicine

100

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“…In recent years, the thymic carcinoid has been clearly related to the MEN-1 phenotype and therefore constitutes an important indicator for genetic screening [13][14][15]. Nonetheless, only 4% of patients with MEN-1 present a carcinoid thymic tumor [15,16].…”

Section: Discussionmentioning

confidence: 99%

“…Nonetheless, only 4% of patients with MEN-1 present a carcinoid thymic tumor [15,16]. However, in these cases it is the primary cause of death, given local invasiveness, recurrence, and metastasis; therefore, early thoracic screening is required in all MEN-1 cases [15].…”

Section: Discussionmentioning

confidence: 99%

Carcinoid tumor of the thymus associated with Cushing′s syndrome and dysgeusia: case report and review of the literature

Claret

Chillarón

Flores

et al. 2009

Endocr

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A 30-year-old man was hospitalized with edema, polyuria, and abnormalities in taste. ACTH and cortisol levels at admission were markedly elevated, even after attempted suppression with 8 mg dexamethasone. A thoracic-abdominal CT revealed an anterior mediastinal lesion and hyperplasia of both adrenal glands. After excision of the mediastinal mass, which confirmed the presence of a carcinoid thymic tumor, the patient became totally asymptomatic, with normal ACTH and cortisol levels. A carcinoid thymic tumor has a poor prognosis, especially when it is associated with Cushing's syndrome. Most patients will present recidivism or metastasis within 5 years after surgery. However, the low number of cases available for analysis makes it difficult to establish optimum therapeutic approaches.

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“…Carcinoid syndrome resulting from primary thymic or bronchial NET is rare. Adrenocorticotropic hormone (ACTH) secreting and growth hormone-releasing hormone (GHRH) secreting thymic tumors have been reported in MEN1 patients resulting in ectopic Cushing’s syndrome and acromegaly respectively (Boix, et al 2002; Li, et al 2014; Takagi, et al 2006). In patients with advanced disease, symptoms and signs can develop from local invasion, mediastinal lymphadenopathy, pulmonary and bone metastases (Otake, et al 2010; Thomas de Montpreville, et al 2013).…”

Section: Bronchial and Thymic Neuroendocrine Tumors In Men1mentioning

confidence: 99%

Endocrine neoplasms in familial syndromes of hyperparathyroidism

Li¹,

Simonds²

2016

Endocrine-Related Cancer

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Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor (HPT-JT), comprise 2 to 5% of primary hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also associated with a range of endocrine and non-endocrine tumors, including potential malignancies. Complications of the associated neoplasms are the major causes of morbidities and mortalities in these familial syndromes, e.g., parathyroid carcinoma in HPT-JT syndrome, thymic, bronchial and enteropancreatic neuroendocrine tumors in MEN1, and medullary thyroid cancer and pheochromocytoma in MEN2A. Because of the different underlying mechanisms of neoplasia, these familial tumors may have different characteristics compared to their sporadic counterparts. Large-scale clinical trials are frequently lacking due to the rarity of these diseases. With technological advances and the development of new medications, the natural history, diagnosis and management of these syndromes are also evolving. In this article, we summarize the recent knowledge on endocrine neoplasms in three familial hyperparathyroidism syndromes, with an emphasis on disease characteristics, molecular pathogenesis, recent developments in biochemical and radiological evaluation, and expert opinions on surgical and medical therapies. Because these familial hyperparathyroidism syndromes are associated with a wide variety of tumors in different organs, this review is focused on those endocrine neoplasms with malignant potential.

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Multiple Endocrine Neoplasia Type I and Cushing's Syndrome Due to an Aggressive ACTH Producing Thymic Carcinoid

Abstract: Abstract

Cited by 29 publications

References 16 publications

Multiple endocrine neoplasia type 1 (MEN1): Not only inherited endocrine tumors

Multiple endocrine neoplasia type 1 (MEN1): Not only inherited endocrine tumors

Carcinoid tumor of the thymus associated with Cushing′s syndrome and dysgeusia: case report and review of the literature

Endocrine neoplasms in familial syndromes of hyperparathyroidism

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