title: Aberrant immunohistochemical expression in nonrhabdomyosarcoma soft tissue sarcomas of infancy: retrospective review of clinical material.
abstract: Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma.
authors.affiliation: Department of Histopathology, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK. njsebire@homail.com
authors.forename: Neil James
authors.initials: NJ
authors.lastname: Sebire
authors.forename: Alan Drummond
authors.initials: AD
authors.lastname: Ramsay
authors.forename: Gillian
authors.initials: G
authors.lastname: Levitt
authors.forename: Marian
authors.initials: M
authors.lastname: Malone
authors.forename: Rupert Anthony
authors.initials: RA
authors.lastname: Risdon
mesh_terms: D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000378:metabolism* / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000378:metabolism* / Q000473:pathology*