Abstract
Introduction: Soft tissue sarcoma (STS) is cancer of connective tissues and comprises a collection of over 50 distinct diagnoses. Approximately 11,000 cases are diagnosed in the US each year, accounting for about 1% of all adult cancers. Each histologic subtype of STS is considered rare and diagnosis can be challenging. STS can arise anywhere in the body, most frequently in the extremities. Initial presentation can mimic benign etiology, i.e. lipoma, as many patients complain of a painless lump. Clinicians ought to consider sarcoma in the differential diagnosis, as proper diagnosis is the cornerstone of effective treatment. Management of STS is complex and often requires a multidisciplinary approach. Depending on histology, NCCN Guidelines dictate that any combination of chemotherapy, radiation, and surgery may be warranted. Purpose: To highlight a rare case of STS and discuss multidisciplinary approach to sarcoma management. Description: A 72 year old male presents with a two-week history of a grape-sized painless mass in his left upper arm. The patient underwent piecemeal excision of the presumed lipoma. Pathology revealed a high grade sarcomatous lesion with positive margins. He was referred to a high-volume cancer center with a designated Sarcoma Center. Review of pathology revealed myxofibrosarcoma. He then underwent radiation followed by re-resection. Pathology revealed an infiltrating superficial spreading pattern without myxoid features, resulting in change of diagnosis to undifferentiated pleomorphic sarcoma (UPS) with superficial spreading features. The patient participated in a limb perfusion clinical trial. Approximately 6 weeks later, he noted a rapidly growing nodule near the surgical scar. He received one cycle of chemotherapy, which was poorly tolerated. The mass progressed rapidly into a multinodular coalescence measuring over 10 cm in aggregate. He is scheduled for shoulder disarticulation with curative intent. Discussion: Superficial spreading UPS is a rare variant of STS. Advanced Practice Providers (APPs) should consider STS when evaluating subcutaneous masses, as proper diagnosis is vital for appropriate management. In this case, initial attempts at resection were made prior to obtaining tissue diagnosis. The diagnosis was complicated by the piecemeal fashion of the initial resection. A multidisciplinary approach was used in the treatment of this patient and involved surgery, radiation, chemotherapy, and experimental therapy. Ultimately, the biology of the disease prevailed and resulted in the necessity of amputation as the only known means for potential cure. There are ample opportunities for APP involvement at every level of care from primary to tertiary care.
