title: Congenital peripheral primitive neuroectodermal tumor refractory to treatment.
abstract: The authors report an unusual case of an infant presenting with a congenital peripheral primitive neuroectodermal tumor (PPNET) of the left hand refractory to treatment. A newborn girl was born with a large bluish-red mass of 4.5 cm diameter protruding into the palm and the dorsum of the left hand. Tumor biopsy confirmed the diagnosis of PPNET. The initial metastatic workup for the detection of metastases was negative. Four cycles of chemotherapy according to CCSG 7881/POG 8850 regimen B were given. Despite this aggressive chemotherapy the tumor grew to involve the entire left hand. The left hand was amputated, and then two cycles of topotecan/cyclophosphamide were given. Five months later extensive metastases developed, involving the brain, lungs, liver, and skeleton, and the child died at the age of 2.5 years. PPNET presenting at birth is uncommon; presentation in the hand is unusual, and the fact that it did not respond to treatment is still more uncommon.
authors.affiliation: Department of Pediatric Hematology/Oncology, Tawam University Hospital and Faculty of Medicine and Health Sciences, Al Ain, United Arab Emirates. mohhayek@emerates.net.ae
authors.forename: Mohammed
authors.initials: M
authors.lastname: El Hayek
authors.forename: Omar
authors.initials: O
authors.lastname: Trad
authors.forename: Sherief
authors.initials: S
authors.lastname: Islam
mesh_terms: D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D017809:Fatal Outcome; D005260:Female; D006225:Hand; D006801:Humans; D007223:Infant; D009362:Neoplasm Metastasis; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy*; D017211:Treatment Failure