Primary cutaneous diffuse leiomyosarcoma with desmoplasia

Skin and Cancer Foundation Australia, Darlinghurst, New South Wales, Australia.
Australasian Journal of Dermatology (Impact Factor: 1.11). 12/2006; 47(4):291-5. DOI: 10.1111/j.1440-0960.2006.00298.x
Source: PubMed


A 65-year-old man presented with an indurated plaque over his left cheek and left neck. An initial punch biopsy of skin showing increased smooth muscle bundles was consistent with a diagnosis of smooth muscle hamartoma. A second incisional skin biopsy revealed a well-differentiated smooth muscle proliferation invading into the dermis and subcutaneous fat in a diffusely infiltrative pattern and with a desmoplastic component, suggesting a diagnosis of desmoplastic leiomyosarcoma. Resection of the tumour confirmed the presence of a cytologically low grade leiomyosarcoma with an insidious infiltrative growth pattern. This rare pattern of diffuse leiomyosarcoma is important to recognize as the histological features are subtle and may potentially constitute a pitfall in histological diagnosis in a small biopsy specimen. In addition, our case illustrates overlapping morphology between diffuse and desmoplastic types of leiomyosarcoma.

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    • "The metastatic potential is 5 to 10 percent for the cutaneous form compared with 30 to 60 percent for the subcutaneous form6. Our case occurred on the face (1~5%)6, and although we could not find the exact cause, trauma was thought to be a possible factor. Furthermore, the lesion increased suddenly in size within 3 months, so we think that our case differs from a typical superficial leiomyosarcoma. "
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    • "However, in contrast to skin of other sites, it also contains lactiferous ducts that connect to the mammary parenchyma. Table 3 summarizes the clinical features, the treatment and outcome of primary leiomyosarcoma at the nipple-areola complex, the skin of other sites and the mammary parenchyma that reported in literature (Choy et al., 2006; Holst et al., 2002; Lonsdale and Widdison, 1992; Markaki et al., 2003; Uğraş et al., 1997). Tumors of these various sites had similar cytological and architectural characteristics. "
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