Abstract
Background/Purpose: Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described. The authors present a 57-year review of their experience. Methods: The charts of children with neurofibrosarcoma were reviewed retrospectively. Statistical analysis was performed using the X2 and unpaired t tests. Results: From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma. Twenty-two were boys. The average age at diagnosis was 13.8 years (range, 3 to 19.9 years). Nineteen patients (50%) had neurofibromatosis. The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients. The average tumor size was 10 cm. The margins after resection were as follows: grossly positive, 9; microscopically positive, 5; negative, 21; and unknown, 3. Patients with positive margins had a 22% survival rate, whereas those with negative or unknown margins had a 34% survival rate. Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy. Twenty-six patients relapsed after a complete response (11 local, 10 distant, 5 both). Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung. Twelve (32%) patients survived with an average follow-up of 14 years (range, 0.3 to 28 years). Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation. Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy. Conclusion: Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy. Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.
| Original language | English |
|---|---|
| Pages (from-to) | 343-346 |
| Number of pages | 4 |
| Journal | Journal of Pediatric Surgery |
| Volume | 38 |
| Issue number | 3 |
| DOIs | |
| State | Published - Mar 1 2003 |
| Externally published | Yes |
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Keywords
- Neurofibroma
- Outcome
- Pediatric
- Soft tissue sarcoma
ASJC Scopus subject areas
- Surgery
Cite this
Pediatric neurofibrosarcoma. / Neville, Holly; Corpron, Cyndy; Blakely, Martin L.; Andrassy, Richard; Sandler, A.
In: Journal of Pediatric Surgery, Vol. 38, No. 3, 01.03.2003, p. 343-346.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pediatric neurofibrosarcoma
AU - Neville, Holly
AU - Corpron, Cyndy
AU - Blakely, Martin L.
AU - Andrassy, Richard
AU - Sandler, A.
PY - 2003/3/1
Y1 - 2003/3/1
N2 - Background/Purpose: Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described. The authors present a 57-year review of their experience. Methods: The charts of children with neurofibrosarcoma were reviewed retrospectively. Statistical analysis was performed using the X2 and unpaired t tests. Results: From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma. Twenty-two were boys. The average age at diagnosis was 13.8 years (range, 3 to 19.9 years). Nineteen patients (50%) had neurofibromatosis. The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients. The average tumor size was 10 cm. The margins after resection were as follows: grossly positive, 9; microscopically positive, 5; negative, 21; and unknown, 3. Patients with positive margins had a 22% survival rate, whereas those with negative or unknown margins had a 34% survival rate. Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy. Twenty-six patients relapsed after a complete response (11 local, 10 distant, 5 both). Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung. Twelve (32%) patients survived with an average follow-up of 14 years (range, 0.3 to 28 years). Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation. Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy. Conclusion: Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy. Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.
AB - Background/Purpose: Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described. The authors present a 57-year review of their experience. Methods: The charts of children with neurofibrosarcoma were reviewed retrospectively. Statistical analysis was performed using the X2 and unpaired t tests. Results: From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma. Twenty-two were boys. The average age at diagnosis was 13.8 years (range, 3 to 19.9 years). Nineteen patients (50%) had neurofibromatosis. The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients. The average tumor size was 10 cm. The margins after resection were as follows: grossly positive, 9; microscopically positive, 5; negative, 21; and unknown, 3. Patients with positive margins had a 22% survival rate, whereas those with negative or unknown margins had a 34% survival rate. Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy. Twenty-six patients relapsed after a complete response (11 local, 10 distant, 5 both). Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung. Twelve (32%) patients survived with an average follow-up of 14 years (range, 0.3 to 28 years). Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation. Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy. Conclusion: Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy. Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.
KW - Neurofibroma
KW - Outcome
KW - Pediatric
KW - Soft tissue sarcoma
UR - http://www.scopus.com/inward/record.url?scp=0037373217&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0037373217&partnerID=8YFLogxK
U2 - 10.1053/jpsu.2003.50105
DO - 10.1053/jpsu.2003.50105
M3 - Article
C2 - 12632346
AN - SCOPUS:0037373217
VL - 38
SP - 343
EP - 346
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 3
ER -