Cutaneous leiomyosarcoma on the trunk: an unusual presentation with an aggressive course � case report and review of literature

Abstract

Primary cutaneous leiomyosarcoma (PCL) are soft-tissue sarcoma, arising in the dermis, with or without extension into the subcutis. They are thought to have an indolent course compared to their subcutaneous counterparts, they may recur but rarely metastases. We report the case of a patient with a PCL arising in the anterior trunk wall who developed pulmonary, bone and retroperitoneal metastases 6 years after wide surgical excision of the primary tumor.

Cutaneous leiomyosarcoma in the abdominal wall, a rare entity for the general surgeon

“…En la Unión Europea se estima que se presenta con una incidencia de 5 casos nuevos al año por cada 100.000 habitantes 2 . Dentro del grupo de sarcomas de tejidos blandos, el leiomiosarcoma es un tumor maligno del músculo liso que puede originarse en prácticamente cualquier parte del organismo, afectando preferentemente a la cara extensora de las extremidades inferiores y al tronco 1,3 . Afecta con más frecuencia a varones en la 5.ª y 6.ª décadas de la vida [4][5][6][7] .…”

“…Afecta con más frecuencia a varones en la 5.ª y 6.ª décadas de la vida [4][5][6][7] . Algunos de los factores de riesgo descritos en su desarrollo son traumatismos previos, VIH, historia de exposición a radiación y antecedente de leiomioma 3 .…”

“…La presentación clínica habitual es un nódulo solitario de crecimiento indolente con un tamaño entre 0.3 cm y 5 cm, y puede asociar prurito, eritema, dolor o ulceración 3,7 . Dada la inespecificidad de la presentación clínica, el diagnóstico es fundamentalmente histológico e inmunohistoquímico 8,9 , habitualmente analizando una biopsia cutánea o directamente la lesión completa extirpada quirúrgicamente.…”

Advanced Cutaneous Leiomyosarcoma of the Forearm

“…Leiomyosarcoma is a malignant smooth muscle neoplasm, which is traditionally divided into superficial and deep tumors. Superficial leiomyosarcomas are quite rare entities, accounting for approximately 7% of soft tissue neoplasms and 0.04% of all cancers [ 1 ]. Based on localization, superficial tumors are further subclassified in dermal and subcutaneous forms; traditionally, it is supposed that the dermal forms have a less aggressive behavior than lesions primitively localized in the subcutaneous [ 1 ].…”

“…Superficial leiomyosarcomas are quite rare entities, accounting for approximately 7% of soft tissue neoplasms and 0.04% of all cancers [ 1 ]. Based on localization, superficial tumors are further subclassified in dermal and subcutaneous forms; traditionally, it is supposed that the dermal forms have a less aggressive behavior than lesions primitively localized in the subcutaneous [ 1 ]. We report a rare case of primary cutaneous leiomyosarcoma (PCL) highlighting the considerable size of the lesion and the correct surgical and oncological management.…”