Adult-type rhabdomyosarcoma: analysis of 57 cases with clinicopathologic description, identification of 3 morphologic patterns and prognosis.

Details

Serval ID
serval:BIB_99A41CF60402
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Adult-type rhabdomyosarcoma: analysis of 57 cases with clinicopathologic description, identification of 3 morphologic patterns and prognosis.
Journal
The American journal of surgical pathology
Author(s)
Stock N., Chibon F., Binh M.B., Terrier P., Michels JJ., Valo I., Robin YM., Guillou L., Ranchère-Vince D., Decouvelaere AV., Collin F., Birtwisle-Peyrottes I., Gregoire F., Aurias A., Coindre JM., Michels J.J., Valo I., Robin Y.M., Guillou L., Ranchère-Vince D., Decouvelaere A.V., Collin F., Birtwisle-Peyrottes I., Gregoire F., Aurias A., Coindre J.M.
ISSN
1532-0979[electronic]
Publication state
Published
Issued date
2009
Volume
33
Number
12
Pages
1850-9
Language
english
Abstract
Adult-type rhabdomyosarcoma (RMS) has been classically defined as a pleomorphic sarcoma with desmin expression occurring in adult patients. To reevaluate this entity, we analyzed a series of 57 cases using immunohistochemistry for desmin, myogenin, alpha smooth muscle actin, h-caldesmon, pankeratin AE1/AE3, epithelial membrane antigen (EMA), S100 protein, CD34, MDM2, and CDK4. In this series, there were 36 men and 21 women aged from 22 to 87 years (median: 59). Tumors were mainly located in the lower limbs (27 cases), trunk wall (15 cases), and upper limbs (10 cases). Most tumors were deeply located (51/54) with a size from 1 to 30 cm (median: 8 cm). Cases were classified in 3 histologic categories: spindle cell RMS (25 cases), pleomorphic RMS (16 cases), and mixed type (16 cases). Forty-one tumors were grade 3 and 16 grade 2. Immunohistochemistry showed that every case was positive for desmin and myogenin. Alpha smooth muscle actin was positive in 21%, pankeratin AE1/AE3 in 20%, and CD34 in 13.2%. Treatment modalities and follow-up were available in 46 cases. Median follow-up was 60.9 months. Eight patients developed a local recurrence and 16 a distant metastasis with a 5-year overall survival rate of 52.6% and a 5-year metastasis-free survival of 62.9%. The only predictive factor for metastasis was histologic grade. In conclusion, adult-type RMS is a rare sarcoma occurring mainly in the extremities and trunk wall with 2 main histologic patterns, spindle cell, and pleomorphic patterns, which represent the end of the spectrum of a single entity.
Keywords
Adult, Aged, Aged, 80 and over, Comparative Genomic Hybridization, Diagnosis, Differential, Disease-Free Survival, Female, Gene Expression Regulation, Neoplastic, Humans, Immunohistochemistry, Immunophenotyping, In Situ Hybridization, Fluorescence, Kaplan-Meiers Estimate, Male, Middle Aged, Neoplasm Recurrence, Local, Neoplasm Staging, Predictive Value of Tests, Rhabdomyosarcoma/chemistry, Rhabdomyosarcoma/genetics, Rhabdomyosarcoma/</QualifierName> <QualifierName MajorTopicYN="N">, Soft Tissue Neoplasms/chemistry, Soft Tissue Neoplasms/genetics, Soft Tissue Neoplasms/</QualifierName> <QualifierName MajorTopicYN="N">, Time Factors, Treatment Outcome, Tumor Markers, Biological/analysis, Tumor Markers, Biological/genetics, Young Adult
Pubmed
Web of science
Create date
25/01/2010 11:18
Last modification date
20/08/2019 15:01
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