Pediatric neurofibrosarcoma☆
Section snippets
Materials and methods
A comprehensive search of the patient files at MD Anderson Cancer Center was performed by requesting the charts of all patients under the age of 21 years with the diagnosis of neurogenic sarcoma, neurofibrosarcoma, malignant peripheral nerve sheath tumor, or malignant schwannoma. The charts then were evaluated to ensure that patients selected were diagnosed accurately. These charts were reviewed retrospectively for patient demographics, presentation, tumor location, size and grade, therapy
Results
From 1944 to 2001, 38 patients under the age of 21 years had neurofibrosarcoma diagnosed at MD Anderson Cancer Center. Twenty-two patients were boys. The average age at diagnosis was 13.8 years (range, 4 months to 19.9 years). Fifty percent of the children had (19) neurofibromatosis. The extremity was the most common site of the tumor with 19 tumors located either in the arm, leg, or thigh. The neurofibrosarcoma was located in the trunk in 8 patients, the head and neck region in 7 patients, and
Discussion
Neurofibrosarcoma remains a rare disease in children, constituting only 4% to 10% of all soft tissue sarcomas. Unlike other soft tissue sarcomas, which are of mesenchymal cell origin, neurofibrosarcomas are of neuroectodermal origin. Historically, this tumor has shown a significant variation in tumor morphology; however, immunohistochemical evaluation and electron microscopy may now aid in confirmation of diagnosis.5
Despite its rarity, neurofibrosarcoma should be included in the differential
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Cited by (36)
Pediatric soft tissue sarcomas
2011, Seminars in Ultrasound, CT and MRICitation Excerpt :Most patients with neurofibromatosis type I (NF1) have an 8%-13% risk of developing MPNSTs, but in those with an unusual mutation of the tumor suppressor gene, as many as 26% develop MPNSTs.112 Previous irradiation is also a risk factor.110 The lifetime risk of MPNST in the general population is 0.001%.113
Skeletal malignancies and related disorders
2011, Textbook of Pediatric RheumatologyCervicobrachial neuralgia revealing neurosarcoma
2008, Revue NeurologiqueMalignant peripheral nerve sheath tumor arising from the colon in a newborn: Report of a case and review of the literatures
2006, Journal of Pediatric SurgeryMicrosurgical approach to peripheral nerve tumors of the upper limb
2023, Minerva Orthopedics
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Address reprint requests to Richard Andrassy, MD, 6431 Fannin MSB 4.020, Houston, TX 77030.