Adult neuroblastoma of the retroperitoneum and abdomen - Clinicopathologicdistinction from primitive neuroectodermal tumor

Citation
T. Hasegawa et al., Adult neuroblastoma of the retroperitoneum and abdomen - Clinicopathologicdistinction from primitive neuroectodermal tumor, AM J SURG P, 25(7), 2001, pp. 918-924
Citations number
14
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Research/Laboratory Medicine & Medical Tecnology","Medical Research Diagnosis & Treatment
Journal title
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
ISSN journal
0147-5185 → ACNP
Volume
25
Issue
7
Year of publication
2001
Pages
918 - 924
Database
ISI
SICI code
0147-5185(200107)25:7<918:ANOTRA>2.0.ZU;2-E
Abstract
Adult neuroblastoma (ANE) is a rare and poorly recognized entity among a hi stologically defined group of small, round-cell tumors arising in the retro peritoneum and abdomen. Eight cases of ANB were compared with seven cases o f primitive neuro-ectodermal tumor (PNET) in these locations to identify cl inicopathologic features that could be used to distinguish between the two lesions. The ANB study group included four men and four women 22-74 years o f age (mean 38 years). Five patients with ANB presented with inflammatory s ymptoms or elevated levels of catecholamines and their metabolites. Five of the ANB tumors were classified as undifferentiated and three as poorly dif ferentiated with a background of neuropil. These cases often showed immunor eactivity for multiple neural markers such as CD56, chromogranin A, synapto physin, neurofilament, and neuron-specific enolase, but were negative for C D99, cytokeratins, desmin, myogenin, smooth muscle actin, muscle-specific a ctin, CD34, S-100 protein, and CD45. In contrast, all of the PNETs were pos itive for CD99, and four (57%) were also positive for cytokeratins. Two cas es of ANB of the undifferentiated subtype had ultrastructural features char acteristic of neuroblastoma and racked a chimeric transcript (EWS-FLI1or ER G), which is specific for PNET. All five patients with the undifferentiated subtype of ANB and six of the seven patients with PNET died of their disea se within 3 years of discovery of the lesion. Our results show that ANB, al though rare, should be considered in the differential diagnosis of patients with small, round-cell tumors in the retroperitoneum and abdomen. Appropri ate immunohistochemical studies and laboratory examination enable pathologi sts to distinguish ANB from other differential diagnoses, especially PNET.