Pediatric intracerebral histiocytic sarcoma with rhabdoid features: Case report and literature review.

Autor: Kim YH; Department of Pathology, Gachon University Gil Hospital, Incheon, South Korea., Yie GT; Department of Neurosurgery, Gachon University Gil Hospital, Incheon, South Korea., Kim NR; Department of Pathology, Gachon University Gil Hospital, Incheon, South Korea., Jeon IS; Department of Pediatrics, Gachon University Gil Hospital, Incheon, South Korea., Cho HY; Department of Pathology, Gachon University Gil Hospital, Incheon, South Korea., Seok JY; Department of Pathology, Gachon University Gil Hospital, Incheon, South Korea., Kim EY; Department of Radiology, Gachon University Gil Hospital, Incheon, South Korea., Lee KC; Department of Radiation Oncology, Gachon University Gil Hospital, Incheon, South Korea.
Jazyk: angličtina
Zdroj: Neuropathology : official journal of the Japanese Society of Neuropathology [Neuropathology] 2017 Dec; Vol. 37 (6), pp. 560-568. Date of Electronic Publication: 2017 Jul 27.
DOI: 10.1111/neup.12396
Abstrakt: A 16-year-old boy presented with marked weight loss, weakness of the left extremities and dizziness of 2 months duration and vomiting for 2 days. Brain MRI showed an approximately 6.5 × 5.3 cm-sized huge heterogeneous enhancing mass located in the corpus callosum, extending into the lateral ventricle. Open biopsy showed that the lesion consisted of lymphoplasmacytes and plump histiocytes with rhabdoid morphology, which were stained with S-100 protein, CD68 (KP1) and negative for CD1a. Histiocytic tumor was initially diagnosed. Chemotherapy using methotrexate, 6-mercaptopurine, vinblastine, interferon-alpha and dexamethasone was performed. After 5 months, partial removal was done. Microscopically, plump and bizarre tumor cells as well as rhabdoid features were found. Occasional spindle cells and necrosis were also found. These cells were positive for CD163, CD68, lysozyme, CD4, INI-1 and BRG1. BRAF V600E mutation was detected. The lesion was finally diagnosed as histiocytic sarcoma. Radiotherapy (6000 cGy in 30 fractions) was done. Both cerebral and extracerebral histiocytic sarcomas have long been diagnosed by unclarified criteria; its rarity as well as previously unclarified criteria can easily lead to a misinterpretation. Histiocytic sarcoma of the CNS is exceptionally rare in children, associated with an exceptionally poor prognosis. To date, only seven cases of pediatric cerebral histiocytic sarcomas have been reported. The present case is the first pediatric case showing BRAF V600E-mutated intracerebral histiocytic sarcoma.
(© 2017 Japanese Society of Neuropathology.)
Databáze: MEDLINE
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