Solid alveolar rhabdomyosarcoma of the hand in adolescence: a clinical, histologic, immunologic, and ultrastructural study.
Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local invasiveness, and occurrence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/NO/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, alpha-sarcomeric actin, muscle-specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.
Bianchi
L
L
Department of Dermatology, Tor Vergata University, Rome, Italy.
Orlandi
A
A
Iraci
S
S
Spagnoli
L G
LG
Nini
G
G
D000293:Adolescent; D005260:Female; D006225:Hand; D006801:Humans; D007150:Immunohistochemistry; D019042:Muscle Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D009124:Muscle Proteins / Q000032:analysis; D018232:Rhabdomyosarcoma, Alveolar / Q000737:chemistry / Q000473:pathology* / Q000556:secondary / Q000648:ultrastructure
D009124:Muscle Proteins