6533b853fe1ef96bd12ace07
RESEARCH PRODUCT
Extra-Adrenal Adult Neuroblastoma With Aberrant Germ Cell Marker Expression: Maturation After Chemotherapy as an Important Clue to a Challenging Diagnosis
Carolina Martínez-ciarpagliniRosa NogueraAkihiko YoshidaAna P. BerbegallMaria Teresa Blanquer-maceírasIsidro MachadoSamuel NavarroGema NietoAntonio Ferrándezsubject
AdultMalePathologymedicine.medical_specialtymedicine.medical_treatmentInguinal CanalBiologyPathology and Forensic MedicineDiagnosis DifferentialLesionNeuroblastomaSALL4NeuroblastomaAntineoplastic Combined Chemotherapy ProtocolsBiomarkers TumormedicineHumansIfosfamideCyclophosphamideEtoposideHomeodomain ProteinsChemotherapyExtra-AdrenalNuclear ProteinsChemoradiotherapymedicine.diseaseGerm CellsHomeobox Protein Nkx-2.2medicine.anatomical_structureVincristineAbdominal NeoplasmsDactinomycinSurgeryAnatomymedicine.symptomAdrenal medullaOctamer Transcription Factor-3Germ cellAfter treatmentTranscription Factorsdescription
Adult neuroblastoma is an extremely infrequent neoplasm, usually occurring in the adrenal medulla or in the paraspinal sympathetic ganglia, as its childhood counterpart. We report a very unusual case of a Schwannian stroma-poor adult neuroblastoma of inguinal location, showing aberrant expression of germ cell markers: SALL4 and OCT4. This aberrant marker expression, the unusual positivity for NKX2.2 and the very scattered (instead of diffuse strong) PHOX2B expression, complicated the initial diagnosis. In this case, the posttreatment histological evaluation revealed the neuroblastic nature of the lesion. Neuroblastoma maturation after treatment is an unusual finding in adults, and in this case, added an important clue for the final diagnosis. Germs cells markers expression in neuroblastoma is an interesting feature to explore and may define a subset of neuroblastomas with a different biological nature.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2019-03-24 | International Journal of Surgical Pathology |