共 50 条
Pediatric intracerebral histiocytic sarcoma with rhabdoid features: Case report and literature review
被引:13
作者:
Kim, Young Hye
[1
]
Yie, Gie-Taek
[2
]
Kim, Na Rae
[1
]
Jeon, In-Sang
[3
]
Cho, Hyun Yee
[1
]
Seok, Jae Yeon
[1
]
Kim, Eung Yeop
[4
]
Lee, Kyu Chan
[5
]
机构:
[1] Gachon Univ, Gil Hosp, Dept Pathol, 21 Namdong Daero,774 Beon Gil, Incheon 21565, South Korea
[2] Gachon Univ, Gil Hosp, Dept Neurosurg, Incheon, South Korea
[3] Gachon Univ, Gil Hosp, Dept Pediat, Incheon, South Korea
[4] Gachon Univ, Gil Hosp, Dept Radiol, Incheon, South Korea
[5] Gachon Univ, Gil Hosp, Dept Radiat Oncol, Incheon, South Korea
关键词:
BRAF;
central nervous system;
children;
histiocytic sarcoma;
rhabdoid tumor;
CENTRAL-NERVOUS-SYSTEM;
LYMPHOMA;
MUTATIONS;
DIAGNOSIS;
D O I:
10.1111/neup.12396
中图分类号:
R74 [神经病学与精神病学];
学科分类号:
摘要:
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 6.5x5.3cm-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 5months, 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.
引用
收藏
页码:560 / 568
页数:9
相关论文
共 50 条