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Why has this man in his 50s developed open bleeding wounds and nodules on his forearms and hips? That's what Adela R. Cardones, MD, MHSc, of Kansas University Medical Center in Kansas City, and colleagues had to determine when the patient presented to the hospital's dermatology department, they reported in .

The patient's medical history was lengthy and multifaceted. About 8 years prior, he had been diagnosed with tumor stage mycosis fungoides (MF), and received systemic and radiation therapy, which included 11 courses of extended beam radiation therapy, including three 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.

However, the disease was refractory to treatment. The following year, the patient underwent a reduced-intensity, matched, unrelated donor, allogeneic hematopoietic stem cell transplant (HSCT), after which he developed severe chronic graft-vs-host disease of the skin, eyes, and gut. Clinicians treated this with systemic corticosteroid therapy, tacrolimus (Prograf), methotrexate, and long-term extracorporeal photopheresis.

Three years later, the areas on the patient's hips, back, and arms that had been irradiated became ulcerated. During corticosteroid treatment and extracorporeal photopheresis, numerous friable, hemorrhagic, and bleeding nodules emerged on both his hips and forearms. These required surgical debulking. At that time, the nodules were excised for biopsy; histological analysis of the samples showed granulation tissue.

Unfortunately, several friable nodules recurred on the outside of his left hip and buttock, with additional nodules noted on his right hip and forearm. These were again surgically excised and biopsied. The patient also received a regimen of systemic treatments, including micafungin, amphotericin B, levofloxacin, dapsone, acyclovir, pentamidine, penicillin VK, and posaconazole.

The patient underwent PET and CT scans, which showed no signs of visceral or extracutaneous metastatic disease. However, clinicians observed intensely hypermetabolic soft tissue along the front and sides of both hips, as well as skin thickening along the front wall of his abdomen, which showed mild metabolic activity.

Microscopic Findings and Clinical Course

"Histopathology of the specimen from the left hip, but not other sites, revealed an atypical vascular proliferation that was consistent with angiosarcoma," Cardones and colleagues wrote. Only one of the nodules biopsied was identified as angiosarcoma. While secondary angiosarcoma would be unlikely to occur in more than one site, this possibility was worthy of consideration, in light of this patient's history and disease presentation.

They excluded possible surgical treatment given the tumor's poorly defined margins and the likelihood that the irradiated tissue would not heal well. Instead, they treated the patient with 4 cycles of paclitaxel. Although the presence of admixed granulation tissue interfered with their ability to assess the patient's response to treatment, they discontinued chemotherapy 4 months later after a PET-CT showed "no evidence of disease progression."

Discussion

is a rare disease, which affects two to five individuals per 1,000,000 annually, and accounts for just 2% of soft tissue sarcomas per year. The disease tends to have a poor prognosis, and carries a high risk of local recurrence and metastasis.

"The tumor originates from vascular endothelial cells, which grow along channels and sinusoidal and cavernous spaces; organize into vessels, masses, or nodules; and can occur in most organs," the group explained.

Tumor tissue may resemble histopathology of other vascular tumors, which complicates diagnosis, they noted. A dissecting growth pattern, the presence of atypical cells that protrude into vascular lumen, and atypical cytomorphology findings of the lining can help differentiate angiosarcoma from other vascular tumors.

Men are more commonly affected than women, at a median age of 60 years, and elderly white men are more likely to develop the cutaneous form, Cardones and team wrote. "To our knowledge, it has not been described in association with allogeneic HSCT or MF previously."

The cutaneous form accounts for about half of all angiosarcomas, most commonly involving the head and neck. The authors referenced a , which noted that "the scalp accounts for approximately 50% of all angiosarcoma cases, yet this still only comprises <0.1% of all head and neck malignancies." Other affected sites include deep soft tissues (10% of cases), with the remaining cases located in parenchymal organs such as the breast, bone, spleen, and liver.

The two most common for angiosarcoma are radiation therapy, an independent risk factor that the authors noted contributes to 25% of cases, and chronic lymphedema. Other known contributors include familial syndromes, exposure to environmental chemical toxins, and foreign bodies.

Risk is thought to be increased in the presence of immunosuppression, Cardones and colleagues wrote, "although the effect is unknown, as angiosarcomas have been reported in kidney transplant patients who have subsequent immunosuppression."

Recurrence rates are high, despite a multidisciplinary approach to treatment that may include surgery for localized, resectable cases, as well as cytotoxic therapy, targeted therapy, and immunotherapy. The rarity of the condition has made collection of data beyond case reports challenging, the authors said.

Initially, presentation of these tumors may resemble a bruise or hemangioma; thus, diagnosis of angiosarcoma is often delayed, with a reported of 5.1 months. Growth of the tumor over time tends to be accompanied by further cutaneous signs, such as tissue infiltration, edema, ulceration, and hemorrhage, the authors noted. "Repeated evaluation is key when there is clinical suspicion of malignant transformation," they concluded.

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