Adult-type rhabdomyosarcoma: analysis of 57 cases with clinicopathologic description, identification of 3 morphologic patterns and prognosis.
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.
Stock
Nathalie
N
Bergonié Institute and INSERM U 916, Bordeaux, France.
Chibon
Frederic
F
Binh
Matthieu Bui Nguyen
MB
Terrier
Philippe
P
Michels
Jean Jacques
JJ
Valo
Isabelle
I
Robin
Yves Marie
YM
Guillou
Louis
L
Ranchère-Vince
Dominique
D
Decouvelaere
Anne-Valérie
AV
Collin
Francoise
F
Birtwisle-Peyrottes
Isabelle
I
Gregoire
Fleur
F
Aurias
Alain
A
Coindre
Jean-Michel
JM
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D055028:Comparative Genomic Hybridization; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D011237:Predictive Value of Tests; D012208:Rhabdomyosarcoma / Q000737:chemistry / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D014408:Biomarkers, Tumor