A case of desmoplastic leiomyosarcoma: A rare variant of cutaneous leiomyosarcoma
Department of Pathology, Baylor College of Medicine, Houston, Texas, USA.Dermatology online journal 06/2010; 16(6):4.
Desmoplastic leiomyosarcoma is a rare histologic variant of cutaneous leiomyosarcoma seen more commonly in men in their 50s and 60s. This neoplasm typically presents as a solitary, enlarging red-pink nodule or plaque on the extensor surfaces of lower extremities. Its unusual histology mimics other cutaneous desmoplastic lesions and the knowledge of this entity and use of an appropriate immunohistochemical panel is essential to arrive at the correct diagnosis. We report a rare case of desmoplastic leiomyosarcoma of the left flank in a 66-year-old male who presented with itching and pain in a long-standing skin lesion. Histopathology showed the presence of individual and small aggregates of spindle to pleomorphic cells with numerous mitoses in a densely fibrotic stroma. Immunohistochemically, the cells were positive for smooth muscle actin, heavy chain myosin, and desmin, confirming their smooth muscle origin. A diagnosis of desmoplastic leiomyosarcoma was made. We discuss the case with a short review of the literature.
- [Show abstract] [Hide abstract]
ABSTRACT: A case of primary desmoplastic cutaneous leiomyosarcoma is reported. A flat and elevated tan plaque, measuring 30 mm × 20 mm, was noticed in the left back of a 74-year-old Japanese male 6 months before the resection. The biopsy specimen showed an overgrowth of desmoplastic fibrocollagenous stroma, focally admixed with a less cellular proliferation of spindle cells having mildly pleomorphic nuclei, but no mitotic figures, arranged in small clusters or appearing as individual cells. Based on these features, we interpreted it as a benign keloid-like lesion. A local resection was done, and gross examination revealed a poorly demarcated grayish tumor lesion, replacing the entire dermis and extending into the subcutis. Microscopic findings demonstrated a sparsely cellular proliferation of atypical spindle cells having cigar-shaped or multi-nucleated pleomorphic nuclei and abundant eosinophilic cytoplasm with few mitotic hot spots, arranged in interlacing bundles, alternating with scattered tumor cells within an abundant desmoplastic stroma. Immunohistochemically, these atypical cells were positive for α-smooth muscle actin, HHF-35, desmin, and caldesmon, and MIB-1 labeling index was greater than 10%. Therefore, we finally made a diagnosis of desmoplastic leiomyosarcoma as a very rare variant of cutaneous leiomyosarcoma. We should be aware that owing to its characteristic features, pathologists might misinterpret it as benign when examining only small or inadequate specimens. It is thus suggested that a large panel of antibodies including smooth muscle cell markers and MIB-1 in immunohistochemistry are useful and adjunctive diagnostic aids for recognizing malignancy, especially in diagnostically difficult cases such as ours.Pathology - Research and Practice 11/2011; 207(11):728-32. DOI:10.1016/j.prp.2011.08.008 · 1.40 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Primary superficial leiomyosarcomas (PSL) are rare malignant lesions that are subdivided into cutaneous and subcutaneous tumors. Primary cutaneous and subcutaneous leiomyosarcomas differ not only as to primary site of origins, but also to differences in prognosis. Guidelines for management and follow-up are not clearly defined in the literature. Retrospective review was conducted from the patient's chart between January 2000 and December 2009. Histopathology, immunohistochemistry, and clinical and surgical records were reviewed. The authors found five cases of PSL and divided them into two cases of cutaneous leiomyosarcomas and three cases ofsubcutaneous leiomyosarcomas. Overall, mean age of the patients was 42.4 years, male: female ratio was 4:1. Clinical presentations were painless mass. Wide excisions were performed in three cases with 2 cm margins. No local recurrence was found in the period of follow-up (6 months to 3 years). One case presented with bony metastasis five years after operation. PSL are rare tumors. Surgical resection remains the main option for curative treatment. Wide excision with at least 2 cm peripheral margins and a depth that includes subcutaneous tissue and fascia are recommended. The natural history of these tumors is not clearly defined. All patients should be followed-up for a period of at least five years after treatments. The authors hoped that further study of these tumors would result in better treatments and follow-up guidelines to be a benefit to such patients in the future.Journal of the Medical Association of Thailand = Chotmaihet thangphaet 03/2013; 96(3):294-301.
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.