Two Different Pituitary Adenomas in a Patient with Multiple Endocrine Neoplasia Type 1 Associated with Growth Hormone-Releasing Hormone-Producing Pancreatic Tumor: Clinical and Genetic Features.

Shintani, Yoshihiro; Yoshimoto, Katsuhiko; Horie, Hitoshi; Sendo, Toshiaki; Kanesaki, Yoshiko; Hosoi, Eiji; Yokogoshi, Yutaka; Bando, Hiroshi; Iwahana, Hiroyuki; Kannuki, Seiji; Matsumoto, Keizō; Itakura, Mitsuo; Saito, Shiro

doi:10.1507/endocrj.42.331

Cited by 40 publications

(32 citation statements)

References 30 publications

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“…These findings are in agreement with the hypothesis that pituitary tumors develop from clonal expansion of transformed somatic cells [28,29]. They are also consistent with observations in patients with MAS [14][15][16] and some patients with MEN 1 [30]. CNC and MAS are genetic conditions that share skin pigmentation abnormalities, adrenocortical hyperplasia, thyroid tumors and even myxomas.…”

Section: Overviewsupporting

confidence: 92%

Pituitary Pathology in Carney Complex Patients

et al. 2004

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Carney complex (CNC) is a familial multiple neoplasia syndrome with features overlapping those of McCune-Albright syndrome (MAS) and multiple endocrine neoplasia (MEN) type 1 (MEN 1). Like MAS and MEN 1 patients, patients with CNC develop growth hormone (GH)-producing pituitary tumors. Occasionally, these tumors are also prolactin-producing, but there are no isolated pro-lactinomas or other types of pituitary tumors. In at least some patients with CNC, the pituitary gland is characterized by hyperplastic areas; hyperplasia appears to involve somatomammotrophs only. Hyperplasia most likely precedes the formation of GH-producing adenomas in CNC, as has been suggested in MAS-related somatotropinomas, but has never been seen in MEN 1 patients. In at least one case of a patient with CNC and advanced acromegaly, a GH-producing macroadenoma showed extensive genetic changes at the chromosomal level. So far, half of the patients with CNC have germline inactivating mutations in the PRKAR1A gene; in their pituitary tumors, the normal allele of the PRKAR1A gene is lost. Loss-of-hererozygosity suggests that PRKAR1A, which codes for the regulatory subunit type 1α of the cAMP-dependent protein kinase A (PKA) may act as a tumor-suppressor gene in CNC somatomammotrophs. These data provide evidence for a PRKAR1A-induced somatomammotroph hyperpasia in the pituitary tissue of CNC patients; hyperplasia, in turn may lead to additional genetic changes at the somatic level, which then cause the formation of adenomas in some, but not all, patients.

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Section: Overviewsupporting

confidence: 92%

Pituitary Pathology in Carney Complex Patients

et al. 2004

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“…Six patients were classified as having MEN-1. In the latter patients, the development of a GH-secreting pituitary adenoma after removal of the ectopic source of GHRH should be considered as a possible explanation for persisting acromegaly, as reported in one of these cases (Shintani et al, 1995).…”

Section: Clinical Aspectsmentioning

confidence: 87%

“…Confirmation of the ectopic nature of acromegaly requires, nevertheless, the demonstration of an elevated plasma GHRH level. Acromegalic patients with a known or family history of the MEN I syndrome may either have a sporadic GH-secreting pituitary adenoma (Oberg et al, 1989) or secrete GHRH from a carcinoid tumour (Aida et al, 1977;Sano et al, 1987;Ramsay et al, 1988;Yamasaki et al, 1988, Shintani et al, 1995. Again, the only method to distinguish reliably between the two possibilities is to measure the plasma GHRH concentration.…”

Section: Differential Diagnosismentioning

confidence: 99%

Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Losa¹,

Werder²

1997

Clinical Endocrinology

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“…There is also evidence to suggest that persistent activation of G S amediated signaling stimulate proliferation of certain cell types and can contribute to invasive tumor development in humans (Gaiddon et al, 1994;Muca and Vallar, 1994;Shintani et al, 1995;Zeiger and Norton, 1993;Drews et al, 1992;Reubi, 1995;Juarranz et al, 1994;Wu et al, 1996;Hoosein et al, 1993;Shah et al, 1994;Lefkowitz, 1993;Clapham, 1993). However, the role of G S a-mediated signal transduction in prostate tumorigenesis and tumor progression has not been investigated.…”

Section: Discussionmentioning

confidence: 99%