Abstract
Current guidelines are vague for the management of soft tissue sarcomas, specifically malignant peripheral nerve sheath tumors (MPNST), regarding staging the disease with the use of routine abdominal imaging. The most recent guidelines from the National Comprehensive Cancer Network (NCCN) recommends to "consider" abdominal/pelvic CT imaging for certain sub groups of sarcomas (e.g., myxoid/round cell liposarcoma, epithelial sarcoma, angiosarcoma, leiomyosarcoma), but provide no guidance on other sarcoma subtypes regardless of tumor size. We report a case of a very large large MPNST in a 40 year-old-female with neurofibromatosis type 1 who was incidentally found to have adrenal metastasis.
Highlights
Soft tissue sarcomas of the extremity are a rare disease process, comprising less than 1% of all malignancies[1]
A similar recommendation is made by the European Society of Medical Oncology (ESMO)/European Sarcoma Network Working Group[7]
We report a case of a malignant peripheral nerve sheath tumor of the thigh, without evidence of concomitant pulmonary metastases, which was found to have metastasized to the adrenal gland
Summary
Soft tissue sarcomas of the extremity are a rare disease process, comprising less than 1% of all malignancies[1]. The mass had previously been stable in size for a few years and was thought to be consistent with a neurofibroma On presentation her exam was notable for a very large soft tissue tumor of the right posterior upper leg (>15cm). Thompson et al reviewed 140 patients of all ages who had a diagnosis of a malignant neoplasm of the upper or lower extremity and underwent screening and/or surveillance with a CT C/A/P12 Of those patients, 14 (10%) had evidence of abdominal/pelvic metastasis, with only 4 (2.9%) with evidence of isolated A/P disease. Our case adds a patient to the literature with a peripheral MPNST who was found to have an isolated adrenal metastasis without evidence of concomitant pulmonary disease. Based on our case and the literature, we would suggest adding screening CT A/P for large (>5 cm), deep tumors of any histology
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