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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    ABSTRACT: Leiomyosarcomas are uncommon malignant smooth muscle tumors, mainly derived from vessels or viscera. Superficial leiomyosarcomas are a rare soft tissue sarcoma arising from the dermis or subcutaneous tissue in the skin. According to tumor origin and location, they are divided into cutaneous and subcutaneous leiomyosarcoma. They have distinctly different histologic and prognostic features from each other. Superficial leiomyosarcomas show a predilection for the proximal extremities and tend to be slow growing. We report one rare case of superficial cutaneous leiomyosarcoma on the right temporal area of face, which showed an extremely rapid growing mass within 3 months.
    Full-text · Article · May 2013 · Annals of Dermatology
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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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    ABSTRACT: Primary leiomyosarcoma of the nipple-areola complex is extremely rare. Less than ten such cases have been reported in English literature so far. Herein we describe a 52-year-old female presenting with a 1.5 cmx1.1 cmx0.7 cm nodular lesion over her left nipple, and leiomyosarcoma was proved by pathological examination of the excised specimen. Positron emitted tomogram (PET) revealed no abnormal signal other than the primary site. Microscopically, this poorly circumscribed tumor was composed of interlacing bundles of smooth muscle cells with bizarre and pleomorphic nuclei, as well as prominent nucleoli. Its mitotic count was up to 7 mitoses per 10 high power fields (HPF). Immunohistochemical study of tumor cells revealed positive stain for alpha-smooth muscle actin and vimentin; and negative for cytokeratin, CD34 and S-100. Left simple mastectomy was undertaken and no residual mass lesion was noted on the resected specimen. Related literatures about the diagnosis and treatment for breast leiomyosarcoma will be presented here.
    Preview · Article · Mar 2008 · Journal of Zhejiang University SCIENCE B
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    ABSTRACT: Primary cutaneous leiomyosarcoma is an uncommon malignant neoplasm with a predilection for the lower extremities. A retrospective study of 4 cases was undertaken to analyse the clinicopathological characteristics and immunohistochemical profile of these neoplasms with emphasis on prognosis. Two male and 2 female patients aged between 49 and 80 years presented with painless tumours involving the lower lip, the chin, the scrotum and the shoulder. Histological examination of the initial biopsy specimen established a diagnosis of cutaneous leiomyosarcoma. All cases co-expressed smooth muscle actin and vimentin regardless of primary tumour site. Wide surgical excision of the tumour was performed in only 3 cases, and the remaining patient refused further treatment. Of the patients undergoing surgical intervention, local recurrence occurred in one case. No metastases were observed. Long-term follow-up of patients with cutaneous leiomyosarcoma is mandatory to detect local recurrence and distant metastases that can occur even years after the initial excision.
    No preview · Article · Jan 2008 · Pathologica
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