Undifferentiated sarcomas of children: Pathology and clinical behavior - An intergroup rhabdomyosarcoma study

B. R. Pawel, A. B. Hamoudi, L. Asmar, Jr Newton, F. B. Ruymann, S. J. Qualman, B. L. Webber, H. M. Maurer

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Undifferentiated soft tissue sarcoma (UND-STS) is the most poorly defined tumor eligible for Intergroup Rhabdomyosarcoma Studies (IRS). Recent IRS UND-STS experience was reviewed to assess the histologic characteristics and clinical behavior of undifferentiated sarcomas. Of the 1,527 patients entered on IRS-III and IRS pilot-IV, 96 had tumors classified by the IRS Pathology Committee as UND-STS. Of these, 52 had adequate histologic material for this study. After application of immunohistochemistry, 18 tumors were reclassified, mostly as embryonal rhabdomyosarcomas (RMS), primitive neuroectodermal tumors, and intraabdominal desmoplastic small round cell tumors. The remaining 34 UND-STS had a diffuse hypercellular histologic pattern made up of sheets of medium-sized cells. The tumor cells had a minimal to moderate amount of cytoplasm and a variable nuclear morphology, predominately vesicular with finely granular chromatin. Except for reactivity with antibodies against vimentin, most tumors had a negative immunohistochemical profile. The 5 year Kaplan-Meier survival estimate for patients with non-metastatic disease was 72%, a significant improvement when contrasted with patients diagnosed to have UND-STS in IRS-I and IRS-II.

Original languageEnglish (US)
Pages (from-to)170-180
Number of pages11
JournalMedical and Pediatric Oncology
Volume29
Issue number3
DOIs
StatePublished - Jul 23 1997

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Clinical Pathology
Rhabdomyosarcoma
Sarcoma
Neoplasms
Desmoplastic Small Round Cell Tumor
Embryonal Rhabdomyosarcoma
Primitive Neuroectodermal Tumors
Kaplan-Meier Estimate
Vimentin
Chromatin
Cytoplasm
Immunohistochemistry
Pathology
Survival
Antibodies

Keywords

  • Immunohistochemistry
  • Pediatric tumors
  • Rhabdomyosarcoma
  • Survival
  • Undifferentiated soft tissue sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

Cite this

Undifferentiated sarcomas of children : Pathology and clinical behavior - An intergroup rhabdomyosarcoma study. / Pawel, B. R.; Hamoudi, A. B.; Asmar, L.; Newton, Jr; Ruymann, F. B.; Qualman, S. J.; Webber, B. L.; Maurer, H. M.

In: Medical and Pediatric Oncology, Vol. 29, No. 3, 23.07.1997, p. 170-180.

Research output: Contribution to journalArticle

Pawel, B. R. ; Hamoudi, A. B. ; Asmar, L. ; Newton, Jr ; Ruymann, F. B. ; Qualman, S. J. ; Webber, B. L. ; Maurer, H. M. / Undifferentiated sarcomas of children : Pathology and clinical behavior - An intergroup rhabdomyosarcoma study. In: Medical and Pediatric Oncology. 1997 ; Vol. 29, No. 3. pp. 170-180.
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