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A Case of Multiple Hemorrhagic Friable Nodules

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 50s presented with bleeding wounds and nodules on the bilateral hips and forearms. He previously received failed systemic and radiation therapy for tumor stage mycosis fungoides (MF). He had received 11 courses of extended beam radiation therapy, including 3 courses of total skin irradiation of 24 to 30 Gy, and focal irradiation to the left posterior thigh and hip/buttock with 2.5/3 Gy to 12.5/24 Gy, respectively, and the right posterior thigh and hip with 2 Gy to 20 and 26 Gy, respectively. Seven years before this visit, he underwent a reduced-intensity, matched, unrelated donor, allogeneic hematopoietic stem cell transplant (HSCT). His post-transplant course was complicated by severe chronic graft vs host disease of the skin, eyes, and gut, for which he received systemic corticosteroid therapy, tacrolimus, methotrexate, and long-term extracorporeal photopheresis. Three years after the HSCT, he developed chronic ulcerations on the hips, back, and arms at the site of previously irradiated tumors, as well as multiple friable, hemorrhagic, and bleeding nodules on bilateral hips and his forearms while receiving treatment with corticosteroids and extracorporeal photopheresis. He underwent several debulking procedures and excision biopsies of the nodules, which revealed granulation tissue on histology. In this latest visit, physical examination revealed recurrent hemorrhagic and friable nodules on his left lateral hip and left lateral buttock (Figure 1). Similar nodules were noted on his right hip and forearm. The patient underwent repeated debulking and biopsy. He was also treated with micafungin, amphotericin B, levofloxacin, dapsone, acyclovir, pentamidine, penicillin VK, and posaconazole. Positron emission tomography (PET) computed tomography (CT) results did not show evidence of visceral or extracutaneous disease.

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C. Angiosarcoma

A male in his 50s with a history of MF, radiation, allogeneic HSCT, chronic immunosuppression, and chronic graft vs host disease underwent multiple deep excision biopsies of various, recurrent chronic, hemorrhagic nodules at sites of previous radiation. Previous biopsies demonstrated granulation tissue. Histopathology of the specimen from the left hip, but not other sites, revealed an atypical vascular proliferation that was consistent with angiosarcoma (Figure 2). While PET-CT results did not show evidence of visceral metastatic disease, there was evidence of intensely hypermetabolic soft tissue along the anterolateral margins of the hips bilaterally, as well as skin thickening along the anterior abdominal wall with mild metabolic activity. Angiosarcoma was seen on only 1 biopsy specimen, and a multifocal presentation of secondary angiosarcoma would be unusual. However, given his history and presentation, as well as the limitations of coverage of PET imaging, the possibility of multiple site involvement was still considered. Surgical intervention was deemed suboptimal due to poor definition of the true margins of the neoplasm and anticipated poor wound healing of the previously irradiated area. He was treated with 4 cycles of paclitaxel, and evaluation of response was difficult due to the admixed granulation tissue. Results from PET-CT showed no evidence of disease progression 4 months later, so further treatment with chemotherapy was discontinued.

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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Adela R. Cardones, MD, MHSc, Division of Dermatology, Kansas University Medical Center, 3901 Rainbow Blvd, Mailstop 2025, Kansas City, KS 66160 (acardones@kumc.edu).

Published Online: January 5, 2023. doi:10.1001/jamaoncol.2022.6837

Conflict of Interest Disclosures: Dr Al-Rohil reported consulting fees from Foundation Medicine Inc outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

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