Pediatric neurofibrosarcoma

Holly Neville, Cyndy Corpron, Martin L. Blakely, Richard Andrassy, A. Sandler

Research output: Contribution to journalArticle

35 Citations (Scopus)

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 languageEnglish
Pages (from-to)343-346
Number of pages4
JournalJournal of Pediatric Surgery
Volume38
Issue number3
DOIs
StatePublished - Mar 1 2003
Externally publishedYes

Fingerprint

Neurofibrosarcoma
Pediatrics
Neurofibromatoses
Recurrence
Survival Rate
Radiation
Drug Therapy
Lung
Therapeutics
Rare Diseases
Natural History

Keywords

  • Neurofibroma
  • Outcome
  • Pediatric
  • Soft tissue sarcoma

ASJC Scopus subject areas

  • Surgery

Cite this

Neville, H., Corpron, C., Blakely, M. L., Andrassy, R., & Sandler, A. (2003). Pediatric neurofibrosarcoma. Journal of Pediatric Surgery, 38(3), 343-346. https://doi.org/10.1053/jpsu.2003.50105

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 journalArticle

Neville, H, Corpron, C, Blakely, ML, Andrassy, R & Sandler, A 2003, 'Pediatric neurofibrosarcoma', Journal of Pediatric Surgery, vol. 38, no. 3, pp. 343-346. https://doi.org/10.1053/jpsu.2003.50105
Neville H, Corpron C, Blakely ML, Andrassy R, Sandler A. Pediatric neurofibrosarcoma. Journal of Pediatric Surgery. 2003 Mar 1;38(3):343-346. https://doi.org/10.1053/jpsu.2003.50105
Neville, Holly ; Corpron, Cyndy ; Blakely, Martin L. ; Andrassy, Richard ; Sandler, A. / Pediatric neurofibrosarcoma. In: Journal of Pediatric Surgery. 2003 ; Vol. 38, No. 3. pp. 343-346.
@article{cd968e228e764e26a6fca9577f96e24c,
title = "Pediatric neurofibrosarcoma",
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.",
keywords = "Neurofibroma, Outcome, Pediatric, Soft tissue sarcoma",
author = "Holly Neville and Cyndy Corpron and Blakely, {Martin L.} and Richard Andrassy and A. Sandler",
year = "2003",
month = "3",
day = "1",
doi = "10.1053/jpsu.2003.50105",
language = "English",
volume = "38",
pages = "343--346",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "3",

}

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 -