Cutaneous angiosarcoma_0512 Derm Clinic
A 78-year-old woman with Alzheimer disease was brought in for evaluation of a scalp lesion first noticed by her granddaughter one month earlier. Because of her dementia, the patient was unable to provide a reliable history.
Her granddaughter reported no patient or family history of previous skin cancer or other malignancy. Physical examination revealed a 10-mm × 5-mm pink, somewhat pearly, and firm nodule on the vertex of the scalp. Examination of the head and neck was otherwise unremarkable, and no appreciable adenopathy was detected.
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Histopathology findings were consistent with a diagnosis of cutaneous angiosarcoma (AS). ASs are a group of rare malignant sarcomas originating from neoplastic transformation of the vascular endothelial cells. Most ASs are known to be very aggressive, with a high rate of metastases and mortality. Both the intrinsic biologic properties of the tumor and the high rate of misdiagnosis contribute to the poor prognosis.
Although AS may arise in almost any organ, the skin and soft tissue are most commonly found to be the primary tumor site. Cutaneous AS, which originates in the skin, accounts for approximately 60% off all ASs.1
The five-year survival rate for cutaneous AS is dismal and estimated to be approximately 30%.2 Prognosis is poorer in those individuals with tumor external diameter greater than 5 cm, depth of invasion greater than 3 mm, positive surgical margins, metastases, and tumor recurrence.3
Risk factors for AS include a history of radiation therapy; lymphedema; exposure to such toxins as arsenic, Thorotrast (a radioactive contrast dye used in radiology from about 1930 to the mid-1950s), and polyvinyl chloride (used in plastics); and presence of such foreign bodies as bone wax, dialysis shunts made of Dacron, metal bodies, surgical sponges, and plastic graft material. In addition, AS occurs more frequently in individuals with AIDS.
Cutaneous AS is divided into four variants: (1) AS of the head and neck; (2) Stewart-Treves variant, occurring in the context of lymphedema; (3) radiation-induced AS; and (4) epithelioid AS.
AS of the head and neck, also known as Wilson-Jones AS, is the most common form and occurs almost exclusively in the elderly. The male-to-female ratio is approximately 2:1. The tumor initially appears as an ill-defined bluish macule that is frequently mistaken for a bruise or cellulitis.
With time, the tumor grows and evolves into an indurated bluish nodule or plaque that is usually asymmetric, nodular or ulcerated. Spontaneous localized bleeding, satellite nodules, intratumoral hemorrhage and a peripheral erythematous ring are other distinguishing features of AS of the head and neck. Systemic bleeding or altered coagulation are ominous signs and usually indicative of metastases.
The Stewart-Treves variant, or lymphedema-associated AS, develops in an area of the skin affected with chronic lymphedema. This variant is most frequently seen in the upper arm of a post-mastectomy patient, with an estimated 0.07% to 0.45% prevalence rate post-mastectomy.1 Stewart-Treves is seen less commonly in patients with a history of lymph node dissection or chronic idiopathic lymphedema.
Although there is no known cause for the correlation between AS and lymphedema, several theories exist. Some believe that the decreased clearance of the local lymph node causes the surrounding tissue to be chronically exposed to carcinogens. Others propose that the lymph node removal triggers angiogenesis, subsequently allowing for the growth of malignant cells. Lymphedema-associated AS occurs an average of 10 to 12 years postsurgery. This subtype usually appears as a violaceous ulcerating plaque or nodule superimposed on a background of brawny, nonpitting edema.
The third subtype, radiation-induced AS, occurs anywhere from four to 40 years postradiation exposure. Quicker disease onset is seen in those patients who were treated with radiation for a malignant condition rather than for a benign condition. These tumors present initially with ecchymosis or thickening of the skin within a previously radiated area.
The final subtype includes a rare and more recently discovered aggressive variant called epithelioid AS. Because epithelioid AS often mimics common vascular and nonvascular neoplasms histologically, both a high clinical index of suspicion and an expert thorough histopathologic examination is needed.4
Biopsy of the tumor allows for diagnosis. Care should be taken to provide proper hemostasis after the biopsy to avoid blood extravasation, which would allow for dissemination of the tumor cells. Diagnosis is based on the microscopic features of the biopsy specimen and ultrastructural and histochemical markers. On microscopy, AS will present with irregularly shaped vascular spaces lined with atypical endothelial tumor cells. Unlike benign hemangiomas, AS vascular channels disrupt normal tissue planes and form a network of sinusoids.
Tumors are often characterized as well-differentiated (low-grade), moderately differentiated or poorly differentiated (high-grade). Well-differentiated tumors are more easily diagnosed, while the latter may require special staining techniques to aid in identification.
MRI and/or CT imaging studies should be performed as part of the initial workup, as up to 50% of patients will have evidence of metastasis at the time of diagnosis.
Individuals diagnosed with AS should be referred to a multidisciplinary cancer center. For limited disease, complete surgical excision of the macroscopic tumor followed by moderate-dose and wide-field radiation offers the best opportunity for cure.5 Surgical treatment is contraindicated in patients with tumors that extend into vital organs or those that are of very large size.
Systemic therapies are limited, but chemotherapy with doxorubicin (Adriamycin, Rubex) followed by radiotherapy is a reasonable approach.6 Newer therapeutic options may include antiangiogenic, immunotherapy and multimodality treatments. Unfortunately, treatment for extensive disease is mostly palliative.
All patients with angiosarcoma should be closely monitored for life. Recurrences may occur years later or as early as two years post-cure.
The patient in this case was referred to a hematologist/oncologist for treatment. Presurgical workup revealed no sign of metastases, and primary surgical removal was planned. Unfortunately, the patient was lost to follow-up.
Esther Stern, NP-C, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.
2. Fury MG, Antonescu CR, Van Zee KJ, et al. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J. 2005;11:241-247.
3. Morgan MB, Swann M, Somach S, et al. Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol. 2004;50:867-874.
4. Mobini N. Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis. J Cutan Pathol. 2009;36:362-369.
5. Morrison WH, Byers RM, Garden AS, et al. Cutaneous angiosarcoma of the head and neck. A therapeutic dilemma. Cancer. 1995;76:319-327.
6. Wollina U, Füller J, Graefe T, et al. Angiosarcoma of the scalp: treatment with liposomal doxorubicin and radiotherapy. J Cancer Res Clin Oncol. 2001;127:396-369.
All electronic documents accessed May 9, 2012.