title: Spindle cell variants of embryonal rhabdomyosarcoma in the paratesticular region. A report of the Intergroup Rhabdomyosarcoma Study.
abstract: We reviewed 173 cases of paratesticular rhabdomyosarcoma (RMS) of Intergroup Rhabdomyosarcoma Studies (IRS)-I, -II, and -III for evaluation of possible histological factors that might account for the good prognosis of these patients. Almost all cases (161 of 173 cases, 93.1%) occurring in this site were of embryonal histology. A spindle-cell subtype of embryonal RMS was identified that presented a storiform growth pattern with abundant collagen between the tumor cells in most cases. Other tumors of this subtype showed an arrangement of tumor cells in bundles with a low to moderate amount of collagen, resembling a leiomyosarcoma. The other embryonal RMS in this site had the classical embryonal cytology. The spindle-cell subtype was highly differentiated by immunohistochemistry and electron microscopy. Lymph node metastasis was found in seven of 43 patients (16.3%) with a RMS of spindle-cell subtype, compared with 40 of 112 patients (35.7%) with RMS of non-spindle-cell type. Clinical data from patients with spindle-cell subtypes of the paratesticular lesions revealed that they almost always had an association with clinical groups of limited disease (32 patients, 74.4%, with Group I; 10 patients, 23.3%, with Group II disease) and a significantly better prognosis (95.5% survival at 5 years) when compared with patients with the classic embryonal variant of RMS (80% survival at 5 years, p < 0.035). The incidence and anatomic distribution of this spindle cell subtype of embryonal RMS was estimated on 800 randomly selected patients from IRS-II. It was found in the head and neck, extremities, orbit, and some other sites, but 30.6% were located in the paratesticular area. Patients with spindle cell RMS of nonparatesticular sites usually had more extensive disease compared with patients having paratesticular lesions; two thirds of the cases had gross residual tumor after surgery or metastatic tumor at diagnosis. We conclude that spindle-cell RMS is a subtype of embryonal RMS with a very favorable prognosis. The site factor of the paratesticular localization may allow earlier diagnosis of the spindle-cell lesions compared with other sites. Other unknown factors may also play a role.
authors.affiliation: Children's Cancer Study Group, Children's Hospital, Columbus, Ohio 43205.
authors.forename: I
authors.initials: I
authors.lastname: Leuschner
authors.forename: W A
authors.initials: WA
authors.lastname: Newton
authors.forename: D
authors.initials: D
authors.lastname: Schmidt
authors.forename: N
authors.initials: N
authors.lastname: Sachs
authors.forename: L
authors.initials: L
authors.lastname: Asmar
authors.forename: A
authors.initials: A
authors.lastname: Hamoudi
authors.forename: D
authors.initials: D
authors.lastname: Harms
authors.forename: H M
authors.initials: HM
authors.lastname: Maurer
mesh_terms: D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000032:analysis; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008207:Lymphatic Metastasis; D008297:Male; D008637:Mesenchymoma / Q000401:mortality / Q000473:pathology* / Q000648:ultrastructure; D009367:Neoplasm Staging; D011379:Prognosis; D016019:Survival Analysis; D013736:Testicular Neoplasms / Q000401:mortality / Q000473:pathology* / Q000648:ultrastructure
chemical_list: D003094:Collagen