Rhabdomyosarcoma with unknown primary tumor site: A report from European pediatric Soft tissue sarcoma Study Group (EpSSG).
Date
2022-11-01ICR Author
Author
Affinita, MC
Merks, JHM
Chisholm, JC
Haouy, S
Rome, A
Rabusin, M
Brennan, B
Bisogno, G
Type
Journal Article
Metadata
Show full item recordAbstract
BACKGROUND: Rhabdomyosarcoma (RMS) is an aggressive malignancy, and 20% of children present with metastases at diagnosis. Patients presenting with disseminated disease very occasionally have no clear evidence of a primary tumor mass. As these patients have rarely been investigated, we report on a series of patients with RMS and unknown primary tumor site registered in the Metastatic (MTS) RMS 2008 protocol (October 2008 to December 2016) coordinated by the European pediatric Soft tissue sarcoma Study Group. METHODS: Patients were administered nine cycles of induction chemotherapy, and 48 weeks of maintenance chemotherapy. Surgery and/or radiotherapy were planned after the first assessment of tumor response, and implemented after six cycles of chemotherapy. If feasible, radiotherapy to all sites of metastasis was recommended. RESULTS: We identified 10 patients with RMS and unknown primary site, most of them adolescents (median age 15.8 years, range: 4.6-20.4). Nine had fusion-positive alveolar RMS. Multiple organ involvement was identified in seven patients, two only had bone marrow disease, and one only had leptomeningeal dissemination. All patients were given chemotherapy, four were irradiated, and none had surgery. Three patients underwent allogeneic bone marrow transplantation. At the time of this analysis, only two patients are alive in complete remission: one had received radiotherapy; and one had a bone marrow transplant. CONCLUSIONS: RMS with unknown primary tumor occurs mainly in adolescents and is typically fusion-positive alveolar. Radiotherapy may be important, but survival is poor and patients should be offered enrollment in investigational trials.
Collections
Subject
bone marrow
rhabdomyosarcoma
unknown origin
Child
Adolescent
Humans
Neoplasms, Unknown Primary
Antineoplastic Combined Chemotherapy Protocols
Rhabdomyosarcoma
Rhabdomyosarcoma, Embryonal
Rhabdomyosarcoma, Alveolar
Language
eng
Date accepted
2022-08-05
License start date
2022-11-01
Citation
Pediatric Blood and Cancer, 2022, 69 (12), pp. e29967 -
Publisher
WILEY
Except where otherwise noted, this item's license is described
as
https://creativecommons.org/licenses/by-nc-nd/4.0/
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