Myogenic Markers in the Evaluation of Embryonal Botryoid Rhabdomyosarcoma of the Female Genital Tract

Department of Pathology, Children's Hospital Boston, MA 02115, USA.
Pediatric and Developmental Pathology (Impact Factor: 0.87). 06/2005; 8(4):427-34. DOI: 10.1007/s10024-005-0006-y
Source: PubMed


Rhabdomyosarcoma presents special diagnostic problems when it involves the uterine cervix in young children because tumor cells may lack marked atypia and may blend with the normal, immature, condensed, cellular stroma, rendering diagnosis difficult. Myogenic makers are a valuable ancillary technique for establishing a diagnosis of rhabdomyosarcoma. However, desmin positivity has been reported in cervical stromal cells, which can confound diagnosis. To determine whether immunohistochemical markers of skeletal muscle differentiation are helpful in the diagnosis of uterine botryoid rhabdomyosarcoma, we compared the immunohistochemical staining pattern of cervical rhabdomyosarcoma from 3 patients with that of normal uteri from age-matched autopsy controls by using antibodies for desmin, smooth muscle actin, muscle-specific actin, myoD1, myogenin, and WT-1. All tumors demonstrated at least focal immunopositivity for desmin, muscle-specific actin, smooth muscle actin, myoD1, and WT-1, and 1 tumor was also positive for myogenin. Autopsy controls showed only scattered subepithelial stromal immunoreactivity for desmin, muscle-specific actin, smooth muscle actin, and WT-1 and showed cytoplasmic, but not nuclear, immunopositivity for myoD1 and myogenin. Myometrium was diffusely positive for desmin and muscle-specific actin. We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. Nuclear staining for myoD1 and myogenin appears not to occur in normal tissue, but it may be absent or sparse in embryonal rhabdomyosarcoma. Our findings indicate that, in this anatomic site, the diagnosis of rhabdomyosarcoma and in particular determination of tumor margins remain very reliant on histomorphology.

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    • "Because of the glycogen and myofibrils contained, those tumor cells stain positively with the periodic acid-Schiff reagent and phosphotungstic acid [11]. It expresses a DNA binding protein, MyoD1, which may turn out to be a lineage marker for rhabdomyosarcomas [10] [15]. "
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    ABSTRACT: Rhabdomyosarcoma is a malignant tumor of the skeletal muscle cells. Those tumors mainly affect children and arise in primitive fetal mesenchyme even at sites that do not contain skeletal muscle. However, very few intracranial rhabdomyosarcomas have been reported. No previous case of primary intrasellar rhabdomyosarcoma except for 1 case has been reported in the literature. A 44-year-old woman presented with fatigue and diplopia associated with amenorrhea and galactorrhea. Visual field evaluation revealed a superior temporal deficit in both eyes. A right afferent pupillary defect was present. Magnetic resonance imaging revealed an intrasellar tumor with suprasellar extension. The tumor involved the sella turcica and right cavernous sinus. The patient was preoperatively diagnosed as pituitary adenoma, which was confirmed pathologically as rhabdomyosarcoma after the operation. Our findings suggest that rhabdomyosarcoma should be considered in the differential diagnosis of a primary intrasellar neoplasm.
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