Peripheral Primitive Neuroectodermal Tumor with Postchemotherapy Neuroblastoma-Like Differentiation
| Autor: | Marie Anne Bründler, Dominic McMullan, Alison Ely, Suren G. Arul, Rachel M. Brown, Kim Neuling, Martin English, Annikka Weissferdt |
|---|---|
| Rok vydání: | 2006 |
| Předmět: |
Pathology
medicine.medical_specialty Time Factors Chromosomes Human Pair 22 medicine.medical_treatment Biology Disease-Free Survival Translocation Genetic Pathology and Forensic Medicine Neuroblastoma Antineoplastic Combined Chemotherapy Protocols Biopsy medicine Humans Oncogene Fusion Neuroectodermal Tumors Primitive Peripheral Child Cyclophosphamide In Situ Hybridization Fluorescence Chemotherapy medicine.diagnostic_test Peripheral Primitive Neuroectodermal Tumor Chromosomes Human Pair 11 Soft tissue Cell Differentiation General Medicine medicine.disease Abdominal mass Vincristine Karyotyping FLI1 Pediatrics Perinatology and Child Health Dactinomycin Female Radiotherapy Adjuvant Sarcoma medicine.symptom Tomography X-Ray Computed Follow-Up Studies |
| Zdroj: | Pediatric and Developmental Pathology. 9:229-233 |
| ISSN: | 1615-5742 1093-5266 |
| DOI: | 10.2350/06-01-0018.1 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|