Primary pediatric intraspinal sarcomas

Report of 3 cases

Paul Klimo Jr. Wright-Patterson Air Force Base, Ohio; and

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 M.D., M.P.H.
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Patrick J. Codd Departments of Neurosurgery, Children's Hospital and

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 M.D.
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Holcombe Grier Neuro-Oncology, Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts

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 M.D.
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Liliana C. Goumnerova Departments of Neurosurgery, Children's Hospital and

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 M.D., F.R.C.S.C., F.A.C.S.
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Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery.

Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.

Abbreviations used in this paper:

EES = extraskeletal Ewing sarcoma; ESFT = Ewing sarcoma family of tumors; MPNST = malignant peripheral nerve sheath tumor; NF1 = neurofibromatosis Type 1.
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