Peripheral medulloepithelioma: an immunohistochemical, ultrastructural, and cytogenetic study of a rare, chemotherapy-sensitive, pediatric tumor.
A case of peripheral medulloepithelioma, a rapidly growing tumor involving the pelvic cavity of a 12-year-old girl, is presented. The diagnosis was supported by expression of vimentin, nestin, alpha-internexin, neurofilaments, and microtubule-associated protein 5 and by characteristic ultrastructure that included absence of cilia or microvilli. Trisomy of chromosomes 2 and 8 was the only detectable chromosomal abnormality. Combination chemotherapy resulted in complete remission. Because some of these rare tumors are sensitive to chemotherapy, their recognition and separation from other neuroectodermal tumors are advisable for better understanding of their biology and determination of optimal treatment.
Donner
Ludvik R
LR
Department of Pathology, Memorial Hospital, Texas A&M University College of Medicine, Temple Texas 76508, USA. ldonner@swmail.sw.org
Teshima
Ikuko
I
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D002889:Chromosomes, Human, Pair 2; D002898:Chromosomes, Human, Pair 8; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D003896:Desmosomes / Q000648:ultrastructure; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007150:Immunohistochemistry; D009363:Neoplasm Proteins / Q000032:analysis; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D010386:Pelvic Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D012074:Remission Induction; D032681:Spectral Karyotyping; D016896:Treatment Outcome; D014314:Trisomy; D014750:Vincristine / Q000008:administration & dosage
D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins; D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D007069:Ifosfamide