Primary cerebellar histiocytic sarcoma in a 17-month-old girl

Case report

Kiyoshi Gomi M.D., Ph.D. 1 , Mio Tanaka M.D. 1 , Mariko Yoshida M.D. 1 , Susumu Ito M.D., Ph.D. 2 , Masaki Sonoda M.D. 2 , Fuminori Iwasaki M.D. 3 , Tetsu Niwa M.D., Ph.D. 4 , Noriko Aida M.D., Ph.D. 4 , Hisato Kigasawa M.D., Ph.D. 3 , and Yukichi Tanaka M.D., Ph.D. 1
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  • 1 Divisions of Pathology,
  • 2 Neurosurgery,
  • 3 Oncology, and
  • 4 Radiology, Kanagawa Children's Medical Center, Yokohama, Japan
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The authors report on a case of histiocytic sarcoma (HS) in a pediatric patient presenting with a solitary tumor in the cerebellum, with the aim of providing insight into primary HS in the CNS, which is especially rare. A 17-month-old Japanese girl presented with a 2-week history of progressive gait disturbance. Brain MRI revealed a 4.7 × 4.3 × 4.3–cm well-demarcated solitary mass in the right hemisphere of the cerebellum, initially suggestive of medulloblastoma, ependymoma, or anaplastic astrocytoma. On intraoperative inspection the cerebellar tumor showed intensive dural attachment and was subtotally removed. Histological and immunohistochemical findings were consistent with HS. The patient subsequently received chemotherapy, and her preoperative neurological symptoms improved. Primary HS in the CNS usually demonstrates an aggressive clinical course and is currently considered to have a poor prognosis. The possibility of this rare tumor should be included in the differential diagnosis of localized cerebellar tumors in the pediatric age group.

Abbreviation used in this paper:HS = histiocytic sarcoma.

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Contributor Notes

Address correspondence to: Kiyoshi Gomi, M.D., Ph.D., Division of Pathology, Kanagawa Children's Medical Center, Mutsukawa 2-138, Minami-ku, Yokohama City, Kanagawa Prefecture, 232-8555 Japan. email: gomikiyoshi@yahoo.co.jp.

Please include this information when citing this paper: published online June 29, 2012; DOI: 10.3171/2012.5.PEDS11270.

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