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Am J Clin Pathol. 2015 May;143(5):728-33. doi: 10.1309/AJCP9ZON4ZIHODIG.

Primary rhabdomyosarcoma of the pineal gland.

Author information

1
From the Departments of Surgery, steven.lau@utsouthwestern.edu.
2
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX; and Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston.
3
From the Departments of Surgery.
4
Pathology, and.
5
Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston.
6
Hematology & Oncology, Straub Clinic & Hospital, Honolulu, HI;

Abstract

OBJECTIVES:

To report a case of primary rhabdomyosarcoma (RMS) of the pineal gland in an adult, as well as review the literature on this rare entity.

METHODS:

The case is compared with previous reports of similar entities, with emphasis on this patient's characteristics and clinical presentation, investigations, and management.

RESULTS:

Diagnosis of primary RMS of the pineal gland was based on the presence of strap cells and multinucleated myotube-like structures, as well as tumor cell expression of skeletal muscle markers consistent with myogenic differentiation. Multimodality treatment was initiated based on pediatric protocols. Unfortunately, the disease progressed on treatment, and the patient survived only 5 months from diagnosis.

CONCLUSIONS:

Pineal RMS is a rare disease with poor prognosis. Optimal management is unknown but likely to involve aggressive multimodality therapy.

KEYWORDS:

Primary intracranial rhabdomyosarcoma; Primary pineal rhabdomyosarcoma; adult rhabdomyosarcoma

PMID:
25873508
DOI:
10.1309/AJCP9ZON4ZIHODIG
[Indexed for MEDLINE]

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