Rhabdomyosarcoma (RMS) is a rare and aggressive neoplasm characterised by rapid growth and metastatic invasion. The most frequent localisation is the skeletal musculature of the limbs. The head and the neck are rarely involved. A 50-year-old woman presented to our attention because of a progressively increasing headache, ataxia and vomiting. MRI showed a lesion at the right cerebellopontine angle. Thereafter, the patient was submitted to a piece-meal removal of the neoplasm. Despite the postoperative MRI showed no signs of remnant, 7 months after the surgery, the disease recurred with multiple localisations, and the patient died a few days later. This report is the first description in the literature of a pleomorphic RMS of the cerebellopontine angle. This particular tumour carries a bad prognosis because of the vicinity of nervous structures and of the impossibility of achieving a one-piece resection. More than ever, the adjunctive treatments had to be effective against a potential remnant and in controlling recurrences.©2014 BMJ Publishing Group. All rights reserved.

Pleomorphic rhabdomyosarcoma of the cerebellopontine angle in an adult: a review of literature / Caporlingua, Federico; Lapadula, Gennaro; Antonelli, Manila; Missori, Paolo. - In: BMJ CASE REPORT. - ISSN 1757-790X. - ELETTRONICO. - 2014:jan30 1(2014), pp. 1-4. [10.1136/bcr-2013-203257]

Pleomorphic rhabdomyosarcoma of the cerebellopontine angle in an adult: a review of literature.

CAPORLINGUA, FEDERICO;LAPADULA, GENNARO;ANTONELLI, MANILA;MISSORI, Paolo
2014

Abstract

Rhabdomyosarcoma (RMS) is a rare and aggressive neoplasm characterised by rapid growth and metastatic invasion. The most frequent localisation is the skeletal musculature of the limbs. The head and the neck are rarely involved. A 50-year-old woman presented to our attention because of a progressively increasing headache, ataxia and vomiting. MRI showed a lesion at the right cerebellopontine angle. Thereafter, the patient was submitted to a piece-meal removal of the neoplasm. Despite the postoperative MRI showed no signs of remnant, 7 months after the surgery, the disease recurred with multiple localisations, and the patient died a few days later. This report is the first description in the literature of a pleomorphic RMS of the cerebellopontine angle. This particular tumour carries a bad prognosis because of the vicinity of nervous structures and of the impossibility of achieving a one-piece resection. More than ever, the adjunctive treatments had to be effective against a potential remnant and in controlling recurrences.©2014 BMJ Publishing Group. All rights reserved.
2014
brain; cerebellopontine; malignant; tumor
01 Pubblicazione su rivista::01i Case report
Pleomorphic rhabdomyosarcoma of the cerebellopontine angle in an adult: a review of literature / Caporlingua, Federico; Lapadula, Gennaro; Antonelli, Manila; Missori, Paolo. - In: BMJ CASE REPORT. - ISSN 1757-790X. - ELETTRONICO. - 2014:jan30 1(2014), pp. 1-4. [10.1136/bcr-2013-203257]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/650098
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