Immunohistochemical profile of myogenin and MyoD1 does not support skeletal muscle lineage in alveolar soft part sarcoma.
ABSTRACT The histogenesis of alveolar soft part sarcoma remains elusive. Myogenic origin is favored, although conflicting data on immunohistochemical demonstration of muscle-associated markers exist. Myogenin and MyoD1, transcription factors of the myogenic determination family, have crucial roles in commitment and differentiation of mesenchymal progenitor cells to myogenic lineage and in maintenance of skeletal muscle phenotype. Their immunohistochemical detection is specific in characterization of rhabdomyosarcoma.
Antibodies for myogenin, MyoD1, desmin, and muscle-specific actin were employed on a large series of cases (n = 19) of formalin-fixed, paraffin-embedded alveolar soft part sarcoma.
Minimal scattered nuclear staining was seen with myogenin. All cases had pronounced, nonspecific granular cytoplasmic immunostaining with MyoD1; nuclei were negative. All tumors were negative for desmin and muscle-specific actin. Ultrastructural study in 10 cases failed to reveal features of skeletal muscle differentiation.
Cytoplasmic staining with MyoD1 in alveolar soft part sarcoma may correspond to cross-reactivity with an undetermined cytoplasmic antigen. The lack of immunostaining with myogenin, MyoD1, desmin, and muscle-specific actin provides evidence against a myogenic origin for alveolar soft part sarcoma.
SourceAvailable from: Benjamin D Levine[Show abstract] [Hide abstract]
ABSTRACT: Alveolar soft part sarcoma (ASPS) commonly arises in the soft tissue of the lower extremities. Primary bone involvement is rare. We report a 23-year-old male who presented with pathologic fracture of the clavicle, and diagnosis of clavicular ASPS. Workup demonstrated a lumbar meningeal mass, also involving the vertebral bodies. Few cases of primary bone ASPS have been identified. Most common primary bone involvement includes the fibula, ilium and tibia. Likewise, meningeal involvement is quite rare. In summary, primary bone ASPS is rare and may involve the clavicle. Meningeal involvement is likewise rare, and presumably represents metastatic spread.03/2014; 11(1):48-53. DOI:10.1016/j.jor.2013.12.014
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ABSTRACT: Alveolar soft part sarcoma (ASPS) is a rare, aggressive malignancy of uncertain histological origin with propensity of vascular invasion and distant metastasis. ASPS demonstrates strong predilection for adolescents and young adults with a female preponderance. The head and neck region is the commonly affected region in the pediatric population with orbit and tongue being most common. The indolent clinical course and asymptomatic nature often leads to misdiagnosis and delayed treatment. Herein, we present a case of ASPS affecting the tongue in 14-year-old boy which clinically mimicked hemangioma, common benign vascular tumor of tongue.
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ABSTRACT: Alveolar soft tissue sarcoma is a rare tumor with a controversial histiogenesis. We analized a case showing the tipical histopatological image and reviewed series of cases diagnosed between 1996 to 2003.