Abstracts

Bruno E. Paredes, 5. DIFFERENTIAL DIAGNOSIS IN DERMATOPATHOLOGY
Epithelioid tumor Dermatopathology: Practical & Conceptual. July - Sept 2006| Vol 12, #3

Cutaneous angiosarcoma occurs mostly in elderly persons in the fifth to sixth decades of life. Lesions are often
situated on the scalp or face, but the leg is said to be more commonly affected by epithelioid angiosarcoma,
whereas postirradiation angiosarcoma or angiosarcoma associated with lymphedema is located more commonly
on the breast. The etiology of cutaneous angiosarcoma is still unclear, but sun damage, chronic lymphedema, and
radiation have been claimed to promote the condition. Idiopathic cases, however, have been recorded, too.
Rarely, (cutaneous) angiosarcoma has been observed in patients with metal or polymer foreign bodies, even
urate crystals, in chronically immunosuppressed organ transplant recipients and in preexisting vascular lesions.
Cardiac epithelioid angiosarcoma sometimes may manifest itself in the skin as a cutaneous metastasis.
Cutaneous angiosarcoma may recur locally, and sometimes it metastasizes to the locoregional lymph nodes or
the lung. The 5-year survival rate is 10–35%.

Tien Anh Tran, Lydia Evans, Michael Mulvaney, J. Andrew Carlson, PITFALL Saved by the re-excision! What is your diagnosis? Dermatopathology: Practical & Conceptual July - Sept 1998 Vol 4, #3

The epithelioid variant of angiosarcoma is mistaken often for carcinoma or melanoma .1-3 The correct diagnosis is
made by identifying vascular lumina that exhibit jagged outlines and by using immunohistochemical stains for both
epithelial and endothelial markers (e.g., cytokeratins and CD31 and CD34). Epithelioid angiosarcomas often
express keratins in addition to endothelial markers.
Eight months after Mohs' micrographic surgery had been performed, the patient remains healthy and without
evidence of persistence of the angiosarcoma locally or metastasis of it widely.

Mobini N. J Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis. Cutan Pathol 2009 Mar;36(3):362-9.

Angiosarcoma (AS) is a rare neoplasm. Cutaneous AS is the most common form of AS. The epithelioid variant of the disease, however, is a rare entity. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. We present five patients with cutaneous epithelioid angiosarcoma (EAS); in none, the clinical diagnosis included a vascular lesion. Three patients had history of breast conservation surgery with/without radiation therapy. Other patients had no previous radiation, and there was no lymphedema in any of the cases. The histopathological examination of the biopsy specimens by hematoxylin and eosin method was not suggestive of a malignant vascular neoplasm initially and the differential diagnoses included carcinoma, malignant melanoma and atypical lymphoid infiltrate. Only after performing immunohistochemical studies that included vascular markers, a definitive diagnosis was possible. Some cases showed unusual histopathological features. Cutaneous EAS is a rare variant of cutaneous AS that can mimic a variety of more common, non-vascular neoplasms, creating a major pitfall in the diagnosis. A careful and thorough histopathological examination and a high index of suspicion, along with appropriate immunohistochemical evaluation, can help reach a correct diagnosis and provide optimal patient care.

Pawlik TM, Paulino AF, McGinn CJ, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer. 2003;98:1716-1726.

Angiosarcoma is a malignant tumor of vascular endothelial cells that arises in the head and neck. It is a rare, difficult to treat, and lethal tumor.

METHODS: Clinical data from patients who were diagnosed with angiosarcoma of the scalp between 1975 and 2002 at the University of Michigan were reviewed. Analysis was performed to assess for factors impacting time to recurrence and survival.

RESULTS: The study was comprised of 29 patients with a median age of 71.0 years. Most patients presented after a delay in diagnosis with either a bruise-like macule (48.3%) or a nonbruise-like nodule (51.7%). Seventy-five percent of patients had pathologic Stage T2 disease, and 76% of patients had high-grade tumors. Virtually all patients underwent surgical excision (96.6%); however, negative surgical margins were achieved in only 21.4% of patients. Multiple lesions on presentation were associated with a shorter time to recurrence (P = 0.02). The median actuarial survival was 28.4 months. Younger patients and patients with Stage T1 disease had improved survival (P = 0.024 and P = 0.013, respectively). Radiation therapy was associated significantly with a decreased chance of death (hazard ratio, 0.16; P = 0.006).

CONCLUSIONS: Although surgery remains the first option for the treatment of patients with angiosarcoma of the scalp, achieving negative margins often is impossible. Patients who are younger and who have less extensive disease fare better. Postoperative radiation therapy should be employed routinely, as it may lead to improved survival

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