Retroperitoneal rhabdomyosarcoma in children. Results of multimodality therapy

The clinical course of 18 consecutive children treated for primary retroperitoneal rhabdomyosarcoma was reviewed. At diagnosis, 8 patients had regional unresected tumor and 10 patients had disseminated tumor, including 3 patients with documented bone marrow infiltration by tumor. Following combined...

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Bibliographic Details
Published in:Cancer 1980-03, Vol.45 (5), p.845-850
Main Authors: Ransom, J. Laurence, Pratt, Charles B., Hustu, H. Omar, Mahesh Kumar, A. P., Howarth, Cathryn B., Bowles, Deborah
Format: Article
Language:English
Subjects:
Adolescent
Adult
Antineoplastic Agents - administration & dosage
Child
Child, Preschool
Drug Therapy, Combination
Female
Humans
Infant
Male
Retroperitoneal Neoplasms - mortality
Retroperitoneal Neoplasms - pathology
Retroperitoneal Neoplasms - therapy
Rhabdomyosarcoma - mortality
Rhabdomyosarcoma - pathology
Rhabdomyosarcoma - therapy
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Summary:The clinical course of 18 consecutive children treated for primary retroperitoneal rhabdomyosarcoma was reviewed. At diagnosis, 8 patients had regional unresected tumor and 10 patients had disseminated tumor, including 3 patients with documented bone marrow infiltration by tumor. Following combined modality therapy, 14 of 18 patients achieved a greater than 50% tumor response (11 complete and 3 partial responses); 4 patients failed to respond and died of progressive disease within eight months of diagnosis. Among the 14 patients responding, 7 patients had subsequent reextension of active tumor three to 16 months (median, 9 months) following the onset of therapy. Three of the 7 remaining patients died of treatment complications, 2 of intestinal obstruction and 1 of disseminated histoplasmosis, within the first year of therapy and at post‐mortem examination had no demonstrable tumor. Four patients are alive and free of active tumor for 10+, 10+, 32+ and 33+ months from diagnosis. Treatment complications have included hematopoietic depression, mucositis, enteritis, intestinal obstruction, excessive weight loss, malnutrition, and life‐threatening infection. These results illustrate limitations in current combined modality therapy of retroperitoneal rhabdomyosarcoma and the necessity for future treatment modifications to both reduce morbidity and to improve survival.
ISSN:0008-543X
1097-0142
DOI:10.1002/1097-0142(19800301)45:5<845::AID-CNCR2820450504>3.0.CO;2-H