Sarcomas cutâneos primários

Abstract

Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review. Keywords: Angiosarcoma; Dermatofibrosarcoma; Leiomyosarcoma; Liposarcoma; Sarcoma/diagnosis; Skin neoplasm Resumo: Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sar-

Leiomiossarcoma cutâneo no tronco

“…Although local recurrence has been described in some cases, its potential for distant metastasis is much smaller. 1,2,3 According to the best treatment referred in the literature, the patient was subjected to radical surgical exeresis of the lesion, without local recurrence up to the date of the last note taken during appointment. 5,7,14,15,16 As the patient did not choose to cooperate, we considered his stage classification incomplete, since it was impossible to carry out an imaging exam to clarify the nature of the liver "nodules" (even though they might be related to comorbidity and not to neoplasia), which may raise doubts regarding their prognostic value.…”

“…The superficial leiomyosarcoma is a rare variant and does not exceed 3% of the total number of sarcomas. [1][2][3][4] This division has an important prognostic value: the dermal variant is a locally aggressive tumor with frequent recurrence (30-50%), but it almost never metastasizes, while the subcutaneous variant is related to greater probability of metastasis and higher recurrence rates (50-70%). [5][6][7][8] The dermal leiomyosarcoma may appear in any location: 50 to 75% of these tumors occur on the lower limbs, mainly on the thigh, 20 to 30% on upper limbs, 10-15% on the trunk and less than 5% on the face.…”

“…[5][6][7][8] The dermal leiomyosarcoma may appear in any location: 50 to 75% of these tumors occur on the lower limbs, mainly on the thigh, 20 to 30% on upper limbs, 10-15% on the trunk and less than 5% on the face. 2,3,7,9 The subcutaneous variant is clinically described as a solitary, although well circumscribed, nodule of soft consistency; the longer axis usually measures more than 2 cm. It is rarely multinodular and the skin that covers the tumor may be pink, brownish or apparently normal.…”

“…Patients may refer pain when the tumors are pressed, pruritus, paresthesia and hemorrhage. 2,7,10 Histologically, such tumors have a good or moderate degree of differentiation and are composed of bundles of fusiform smooth muscle cells, with vesicular nuclei with blunt extremities (the classical "cigar" shape) and eosinophilic cytoplasm. The leiomyosarcomas express desmin, vimentin and frequently actin.…”

“…4,7,12 The leiomyosarcoma, when restricted to superficial tissues, is not an aggressive tumor and is associated with low mortality rate. 2,11 The local recurrence rate is around 30% for the dermal variant and 50% for the subcutaneous variant, and in around 30% of these patients metastases occur.…”