title: Primitive neuroectodermal tumor as a differential diagnosis of CD56-positive tumors in adults.
abstract: A 33-year-old Japanese man was referred to our hospital after a huge intrapelvic tumor with bilateral hydronephrosis was found following persistent lumbago. Natural killer/T-cell lymphoma was suspected due to positive immunostaining for CD56, but CHOP therapy was ineffective. Re-evaluation of the tumor cells showed that they were positive for CD99, neuron-specific enolase, and synaptophysin and had a t(11 ; 22) (q24 ; q12) translocation, leading to the revised diagnosis of primitive neuroectodermal tumor (PNET). Systemic chemotherapies and radiation therapy were added to surgical resection, and no recurrence has been detected for 3 years. Taken together, PNET may be considered in adult patients with CD56-positive tumors.
authors: Department of Internal Medicine, Matsumoto Medical Center, Matsumoto.
authors: Tadanobu
authors: T
authors: Nagaya
authors: Naoki
authors: N
authors: Tanaka
authors: Atsushi
authors: A
authors: Kamijo
authors: Satoru
authors: S
authors: Joshita
authors: Koh
authors: K
authors: Nakazawa
authors: Hideharu
authors: H
authors: Miyabayashi
authors: Suguru
authors: S
authors: Yoneda
authors: Tetsuya
authors: T
authors: Ito
authors: Michiharu
authors: M
authors: Komatsu
authors: Eiji
authors: E
authors: Tanaka
authors: Kiyoshi
authors: K
authors: Kitano
mesh_terms: D000328:Adult; D019002:CD56 Antigen / Q000378:metabolism*; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis / Q000276:immunology; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology*; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics; D034802:RNA-Binding Protein EWS; D012512:Sarcoma, Ewing / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000276:immunology; D014178:Translocation, Genetic
chemical_list: D019002:CD56 Antigen; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS