What comprises appropriate therapy for children/adolescents with rhabdomyosarcoma arising in the abdominal wall? A report from the Intergroup Rhabdomyosarcoma Study Group.
ABSTRACT The aim of this study was to define clinical features and determine the best therapy for patients with rhabdomyosarcoma (RMS) of the abdominal wall.
We examined the demographic, clinical features, therapy (especially surgical), and outcome of 34 patients. Patients received combination chemotherapy after complete surgical resection (group I, n = 14; 41%); resection with microscopic residual followed by local irradiation (RT; group II, n = 8; 24%); partial resection or biopsy only plus RT with gross locoregional residual tumor (group III, n = 4; 12%); or biopsy only plus RT with metastatic disease (group IV, n = 8; 24%). Patients with group I or group II tumors had undergone partial abdominal wall resection (ie, involved muscle only with preservation of peritoneum, n = 11) or complete abdominal wall resection (n = 7). Four additional patients had groin lesions.
Thirty-four children or adolescents with abdominal wall RMS (about 1% of all patients) were treated on Intergroup Rhabdomyosarcoma Study I (IRS-I) through IRS-IV. Overall, adolescents comprised 14 of 34 eligible patients (41%), and 10 of 14 (71%) adolescents had alveolar or undifferentiated tumors versus 8 of 20 (40%) younger children (P= .07). Failure-free survival (FFS) rate and survival rate at 5 years was 65%. Treatment outcome was poorer for patients with group III-IV tumors (P = .01), adolescents (P = .09) and patients with alveolar or undifferentiated sarcomas (P = .12).
Patients with localized tumors appear to fare better if they undergo complete abdominal wall resection (long-term survival rate, 100%) versus partial resection (long-term survival rate, 62% [P = .12]).
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ABSTRACT: Intensive chemotherapy and high-dose radiation with complete excision of the tumor are the treatment of choice for rhabdomyosarcoma (RMS). However, because there are so few reports of perianal RMS, a mainstream treatment has not been established. We report two cases of perianal RMS and review 13 cases documented in Japan since 1990. Case 1 was a 7-year-old boy first treated by tumor excision followed by chemotherapy and external irradiation. He has since been tumor-free but suffered severe perianal erosion and ulceration. Case 2 was a 17-year-old girl first treated by preoperative chemotherapy to reduce the tumor size, after which the tumor was completely excised with anatomical guidance using three-dimensional computed tomography via the posterior sagittal approach, followed by open intraoperative irradiation. She has since been tumor-free with preserved anal function. These case reports show the importance of cogitated treatments for preservation of anal function and optimal therapeutic outcome in patients with perianal RMS.Surgery Today 02/2004; 34(8):719-24. · 0.96 Impact Factor
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ABSTRACT: There has been only one reported case of an intrasellar rhabdomyosarcoma, the origin of which was in the para-nasal sinus. The authors encountered a patient with an intrasellar rhabdomyosarcoma with no evidence of tumor at any additional sites. A 28-year-old otherwise healthy man complaining of headache exhibited left abducent nerve palsy and left visual disturbance. The patient was diagnosed as having a sellar tumor invading the left cavernous sinus. Near total removal of the tumor was achieved via a trans-sphenoidal approach. Histologically, the tumor was composed of small, round-to-elongated undifferentiated cells and large spindle cells with myoblastic features. Immunohistochemically, tumor cells were positive for antibodies against desmin, myoglobin, and alpha-smooth muscle actin. The tumor was identified as an embryonal rhabdomyosarcoma on the basis of the above pathological findings. Systemic investigation, including the nasal and para-nasal regions, failed to detect any additional tumors. Postoperative local radiation therapy and chemotherapy with the use of ifosfamide, etoposide, and vincristine brought about complete initial remission. Rhabdomyosarcoma should be considered in the differential diagnosis of a primary intrasellar neoplasm.Neurosurgery 04/2001; 48(3):677-80. · 2.53 Impact Factor
- Sarcoma 01/2002; 6(4):111-22.