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Journal of Endocrinological Investigation

Erschienen in:

01.06.2016 | Review

Update on parathyroid carcinoma

verfasst von: F. Cetani, E. Pardi, C. Marcocci

Erschienen in: Journal of Endocrinological Investigation | Ausgabe 6/2016

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Abstract

Introduction

Parathyroid carcinoma (PC) is a rare endocrine disorder, commonly causing severe primary hyperparathyroidism (PHPT). PC is mainly a sporadic disease, but it may occur in familial PHPT. Patients with PC usually present markedly elevated serum calcium and PTH. The clinical features are mostly due to the effects of the excessive secretion of PTH rather than to the spread of tumor. At times, the diagnosis can be difficult.

Purpose

The aim of this work is to review the available data on PC, and focus its molecular pathogenesis and the clinical utility of CDC73 genetic testing and immunostaining of its product, parafibromin. The pathological diagnosis of PC is restricted to lesions showing unequivocal growth into adjacent tissues or metastasis. Inactivating mutations of the cell division cycle 73 (CDC73) gene have been identified in up to 70 % of apparently sporadic PC and in one-third are germline. Loss of parafibromin immunostaining has been shown in most PC. The association of CDC73 mutations and loss of parafibromin predicts a worse clinical outcome and a lower overall 5- and 10-year survival.

Conclusions

The treatment of choice is the en bloc resection of the tumor. The course of PC is variable; most patients have local recurrences or distant metastases and die from unmanageable hypercalcemia.

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Titel: Update on parathyroid carcinoma
verfasst von: F. Cetani
E. Pardi
C. Marcocci
Publikationsdatum: 01.06.2016
Verlag: Springer International Publishing
Erschienen in: Journal of Endocrinological Investigation / Ausgabe 6/2016
Elektronische ISSN: 1720-8386
DOI: https://doi.org/10.1007/s40618-016-0447-3

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Springer Medizin

Update on parathyroid carcinoma

Abstract

Introduction

Purpose

Conclusions

Leitlinien kompakt für die Innere Medizin

Neu im Fachgebiet Innere Medizin

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

Adjuvante Immuntherapie verlängert Leben bei RCC

Alectinib verbessert krankheitsfreies Überleben bei ALK-positivem NSCLC

Update Innere Medizin

Springer Medizin

Abstract

Introduction

Purpose

Conclusions

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