PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 779598

Multiple endocrine neoplasia type II.

S A Wells·D A Ontjes
View original source ↗Export metadata ↓

Abstract

The clinical characteristics, methods of diagnosis, and treatment of MEN-II have been described. Attention in the future should be focused on diagnosing MCT at the earliest possible time in MEN-II family members. The development of chemotherapeutic or other antitumor agents for the treatment of metastitic MCT should prove a fertile field for investigation.

Explore related subjects

Adrenal Gland NeoplasmsAnimalsCarcinomaChildEndocrine System DiseasesFemaleHumansNeoplasmsNeoplasms, Multiple PrimaryParathyroid NeoplasmsPheochromocytomaSyndromeThyroid NeoplasmsJournal ArticleResearch Support, U.S. Gov't, P.H.S.Review

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

S A Wells, D A Ontjes. 1976. Multiple endocrine neoplasia type II.. https://doi.org/10.1146/annurev.me.27.020176.001403

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2008

Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses.

Opsoclonus-myoclonus-ataxia-syndrome (OMS) represents a rare neuroblastoma-associated paraneoplastic syndrome that commonly results in neurologic deficits despite tumor resection and immunosuppressive therapy. We describe the response of five such children to high-dose dexamethasone pulses including two patients in whom previous glucocorticoids, rituximab, and cytostatic drugs were not successful. All patients had MYCN non-amplified tumors that were detected 1 to 7 months after the onset of the OMS or ataxia. This treatment resulted in a good partial response in three and in complete remission in two patients. Our results show that dexamethasone pulses are likely to be useful for both, first-line- and salvage-therapy for OMS-patients.

Adrenal Gland Neoplasms↗
PubMed2007

Malignant paragangliomas associated with mutations in the succinate dehydrogenase D gene.

INTRODUCTION: Malignant paragangliomas have been well described in carriers of mutations of the succinate dehydrogenase B (SDHB) gene, but have rarely been associated with mutations in the succinate dehydrogenase D (SDHD) gene. AIM: The aim of the study was to report the different clinical expression patterns of malignant paragangliomas in five patients with SDHD (D92Y) mutations observed in approximately 200 SDHD (D92Y) mutation carriers followed in our institution. RESULTS: Metastasis and/or local tumor invasion was documented 0 (n=2), 1, 18, and 30 yr after the initial diagnosis of paraganglioma. Malignancy was proven by paraganglioma bone metastases (n=2), intrathoracic paraganglioma with lymph node metastases, locally invasive head-and-neck paraganglioma with destruction of the petrosal bone, and locally invasive paraganglioma of the bladder with lymph node metastases. Four of the five patients developed catecholamine excess during follow-up due to intraadrenal paraganglioma (pheochromocytoma) (n=1), extra adrenal paraganglioma (n=2), and presumed subclinical disease (n=1). CONCLUSION: SDHD mutations (D92Y) are associated with malignant paragangliomas and catecholamine excess with remarkable interindividual variations despite the same mutation. We estimate that the prevalence of malignancy in carriers of D92Y mutations is at least 2.5%.

Adrenal Gland Neoplasms↗