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Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation.
Pediatr Dev Pathol. 2006 May-Jun; 9(3):229-33.PD

Abstract

We report the case of an 11-year-old girl with a retroperitoneal tumor in the left upper quadrant. The girl was admitted to hospital with weight loss and a painless abdominal mass that on biopsy was diagnosed as a peripheral primitive neuroectodermal tumor/Ewing sarcoma (pPNET/EWS) of the soft tissue. The patient underwent chemotherapy followed by surgical resection of the tumor 5 months after diagnosis. The posttreatment residual viable tumor showed a morphologic appearance resembling a neuroblastoma. Interphase and metaphase fluorescent in situ hybridization (FISH) studies performed on the pretreatment and posttreatment samples showed the presence of a t(11;22) rearrangement resulting in EWSR1/FLI1 gene fusion consistent with pPNET/EWS in both specimens. This case is unusual in the sense of showing the typical gene fusion for pPNET/EWS both in the pretherapy sample with the typical morphological appearance of this tumor and in the posttherapy specimen showing neural differentiation suggestive of a neuroblastoma.

Authors+Show Affiliations

Birmingham Children's Hospital, Department of Histopathology, West Midlands, UK. aweissferdt@doctors.org.ukNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

16944972

Citation

Weissferdt, Annikka, et al. "Peripheral Primitive Neuroectodermal Tumor With Postchemotherapy Neuroblastoma-like Differentiation." Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society, vol. 9, no. 3, 2006, pp. 229-33.
Weissferdt A, Neuling K, English M, et al. Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. Pediatr Dev Pathol. 2006;9(3):229-33.
Weissferdt, A., Neuling, K., English, M., Arul, S., McMullan, D., Ely, A., Bründler, M. A., & Brown, R. (2006). Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society, 9(3), 229-33.
Weissferdt A, et al. Peripheral Primitive Neuroectodermal Tumor With Postchemotherapy Neuroblastoma-like Differentiation. Pediatr Dev Pathol. 2006 May-Jun;9(3):229-33. PubMed PMID: 16944972.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. AU - Weissferdt,Annikka, AU - Neuling,Kim, AU - English,Martin, AU - Arul,Suren, AU - McMullan,Dominic, AU - Ely,Alison, AU - Bründler,Marie-Anne, AU - Brown,Rachel, PY - 2005/10/23/received PY - 2006/01/18/accepted PY - 2006/9/2/pubmed PY - 2006/10/18/medline PY - 2006/9/2/entrez SP - 229 EP - 33 JF - Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society JO - Pediatr. Dev. Pathol. VL - 9 IS - 3 N2 - We report the case of an 11-year-old girl with a retroperitoneal tumor in the left upper quadrant. The girl was admitted to hospital with weight loss and a painless abdominal mass that on biopsy was diagnosed as a peripheral primitive neuroectodermal tumor/Ewing sarcoma (pPNET/EWS) of the soft tissue. The patient underwent chemotherapy followed by surgical resection of the tumor 5 months after diagnosis. The posttreatment residual viable tumor showed a morphologic appearance resembling a neuroblastoma. Interphase and metaphase fluorescent in situ hybridization (FISH) studies performed on the pretreatment and posttreatment samples showed the presence of a t(11;22) rearrangement resulting in EWSR1/FLI1 gene fusion consistent with pPNET/EWS in both specimens. This case is unusual in the sense of showing the typical gene fusion for pPNET/EWS both in the pretherapy sample with the typical morphological appearance of this tumor and in the posttherapy specimen showing neural differentiation suggestive of a neuroblastoma. SN - 1093-5266 UR - https://www.unboundmedicine.com/medline/citation/16944972/Peripheral_primitive_neuroectodermal_tumor_with_postchemotherapy_neuroblastoma_like_differentiation_ L2 - http://journals.sagepub.com/doi/full/10.2350/06-01-0018.1?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -