Alveolar Soft Part Sarcoma Presenting as a Pulsating Tumour of the Arm in a Young Woman H. Rowley, A. Connolly, M. P. Brady
Department ofSurgery. Regional Hospital and University College. Cork. Summary We present the case or an alveolar soft part sarcoma presenting as a pulsating tumour of the arm in a young woman. Case Report
A nineteen year old female presented with swelling of the posterior aspect of her left arm. There was no history of trauma and she noticed the slightly tender swelling only two days previously. On examination there was a 10 etn x 11 em smooth, fIrm, mobile swelling extending from the axillary fold down to mid-humeral level. This was clinically attached to the triceps and was pulsatile with a bruit over it. There was no neurological or vascular defIcit. Laboratory investigations were normal. Left branchial angiography followed by contrast cr scan was done. The tumour was well demonstrated posterior to the main branchial vessels. There was no evidenceofbony involvement though appearances were suggestive oflocal invasion into triceps. At operation, after vascular control and demonstration of all nerves the tumour was excised with one cm margin. Subsequent histology stated the tumour to be completely excised. Post operativelya year long Contrast - enhanced CT scan showing large tumour mass.
chemotherapy regime was commenced. This consisted of: Actinomycin D 0.4 mg/m2 iv. Cyclophosphamide 300 mg/m 2 iv. Vincristine 1.0 mg/m2 iv. This was given weekly for six weeks, then three weekly for one year. She subsequently went abroad and was lost to follow up for a period of one year. Recent follow up by CT scan revealed no evidence of local recurrence (2 years and 3 months from time of original diagnosis). Unfortunately CT scan of chest revealed findings consistent with multiple metastases in both lung fIelds. Her future management is currently under review with the probability of further chemotherapy. Discussion Alveolar soft part sarcoma is an uncommn neoplasm accounting for less than 1% of all soft tissue sarcomas and is uniformly malignant!. The ultimate prognosis is poor despite the relatively slow growth of the tumoW. Many cases develop metastatic lesions even before detection of the primary tumour. The main sites for metastases are lung and brain. Treatment is not particularly promising. Most authorities recommend radical surgical excision followed by radiotherapy or chemotherapy3,4. At the present time most patients undergo limb-sparing surgery. This involves complete removal of the tumour with 2-3 em of surrounding tissue if
Left brachial angiogram showing vascularity of mass. Reprints available from: Ms. H. Rowley. F.R.C.S.I., Beaumont Hospital, Dublin.
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possible. Since extensive margins of normal tissue are not always possible, the use of radiotherapy and chemotherapy continues to be evaluate
Department ofSurgery. Regional Hospital and University College. Cork. Summary We present the case or an alveolar soft part sarcoma presenting as a pulsating tumour of the arm in a young woman. Case Report
A nineteen year old female presented with swelling of the posterior aspect of her left arm. There was no history of trauma and she noticed the slightly tender swelling only two days previously. On examination there was a 10 etn x 11 em smooth, fIrm, mobile swelling extending from the axillary fold down to mid-humeral level. This was clinically attached to the triceps and was pulsatile with a bruit over it. There was no neurological or vascular defIcit. Laboratory investigations were normal. Left branchial angiography followed by contrast cr scan was done. The tumour was well demonstrated posterior to the main branchial vessels. There was no evidenceofbony involvement though appearances were suggestive oflocal invasion into triceps. At operation, after vascular control and demonstration of all nerves the tumour was excised with one cm margin. Subsequent histology stated the tumour to be completely excised. Post operativelya year long Contrast - enhanced CT scan showing large tumour mass.
chemotherapy regime was commenced. This consisted of: Actinomycin D 0.4 mg/m2 iv. Cyclophosphamide 300 mg/m 2 iv. Vincristine 1.0 mg/m2 iv. This was given weekly for six weeks, then three weekly for one year. She subsequently went abroad and was lost to follow up for a period of one year. Recent follow up by CT scan revealed no evidence of local recurrence (2 years and 3 months from time of original diagnosis). Unfortunately CT scan of chest revealed findings consistent with multiple metastases in both lung fIelds. Her future management is currently under review with the probability of further chemotherapy. Discussion Alveolar soft part sarcoma is an uncommn neoplasm accounting for less than 1% of all soft tissue sarcomas and is uniformly malignant!. The ultimate prognosis is poor despite the relatively slow growth of the tumoW. Many cases develop metastatic lesions even before detection of the primary tumour. The main sites for metastases are lung and brain. Treatment is not particularly promising. Most authorities recommend radical surgical excision followed by radiotherapy or chemotherapy3,4. At the present time most patients undergo limb-sparing surgery. This involves complete removal of the tumour with 2-3 em of surrounding tissue if
Left brachial angiogram showing vascularity of mass. Reprints available from: Ms. H. Rowley. F.R.C.S.I., Beaumont Hospital, Dublin.
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Vol. 161 No.1
possible. Since extensive margins of normal tissue are not always possible, the use of radiotherapy and chemotherapy continues to be evaluate
