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Vol. 110. Num. 1.January - February 2019
Pages e1-e6Pages 1-86
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Vol. 110. Num. 1.January - February 2019
Pages e1-e6Pages 1-86
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DOI: 10.1016/j.adengl.2018.11.002
Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment
Leiomiosarcoma y sarcoma pleomórfico dérmico: directrices para el diagnóstico y tratamiento
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B. Llombarta,
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beatriz.llombart@uv.es

Corresponding author.
, C. Serra-Guilléna, C. Requenaa, M. Alsinab, D. Morgado-Carrascob, I. Machadoc, O. Sanmartína
a Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España
b Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, España
c Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, España
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Abstract

There are 3 types of leiomyosarcoma of the skin: dermal, subcutaneous, and metastatic cutaneous. Dermal leiomyosarcoma arises from smooth muscle fibers in arrector pili muscles, genital dartos muscles, and the nipple-areola complex. It is an intermediate-grade tumor associated with a tendency for local recurrence (24%) and low metastatic potential (4%). Subcutaneous leiomyosarcoma originates from smooth muscle in blood vessel walls and has higher rates of local recurrence (37%) and metastasis (43%).

Plemorphic dermal sarcoma typically affects elderly patients and arises in sun-exposed areas (e.g., the scalp). Its histologic and immunohistochemical characteristics are similar to those of atypical fibroxanthoma, but it is more aggressive (metastasis rate of 10%-20%). Histologically, it can be distinguished from atypical fibroxanthoma by the observation of subcutaneous tissue invasion, perineural invasion, and foci of necrosis.

Keywords:
Sarcoma
Cutaneous
Leiomyosarcoma
Pleomorphic sarcoma
Atypical fibroxanthoma
Diagnosis
Treatment
Resumen

El leiomiosarcoma de la piel se clasifica en tres grupos: dérmico, hipodérmico y cutáneo metastásico. El dérmico se origina de las fibras musculares lisas del músculo erector del pelo, dartos genital o de la areola mamaria. Se considera un tumor de malignidad intermedia, con tendencia a la recidiva local (24%) y un bajo riesgo de metástasis (4%). El leiomiosarcoma hipodérmico se origina de las paredes musculares de los vasos, y se caracteriza por presentar una mayor tasa de recidiva local (37%) y metástasis (43%).

El sarcoma pelomórfico dérmico aparece habitualmente en pacientes ancianos y se localiza característicamente en zonas de piel fotoexpuesta (cuero cabelludo). Comparte características histológicas e inmunohistoquímicas con el fibroxantoma atípico, pero con un comportamiento más agresivo (metástasis en el 10-20%). Los criterios histológicos que lo diferencian son la infiltración del tejido celular subcutáneo, la infiltración perineural y la presencia de focos de necrosis.

Palabras clave:
Sarcoma
Cutáneo
Leiomiosarcoma
Sarcoma pleomórfico
Fibroxantoma atípico
Diagnóstico
Tratamiento

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