Leiomyoma and Leiomyosarcoma (Primary and Metastatic) of the Oral and Maxillofacial Region: A Clinicopathological and Immunohistochemical Study of 27 Cases.

Smooth muscle neoplasms represent an important group of lesions which is rare in the oral cavity. Leiomyoma (LM) is benign smooth muscle/pericytic tumor usually presenting as non-aggressive neoplasm, while leiomyosarcoma (LMS) represents its malignant counterpart. The rarity of these lesions, together with its unspecific clinical presentation and a variable histopathological appearance, lead to a broad list of differential diagnoses, hampering their diagnoses. Therefore, in this study we describe the clinical and microscopic features of a series of oral and maxillofacial LMs and LMSs. A retrospective search from 2000 to 2019 was performed and all cases diagnosed as LM and LMS affecting the oral cavity and gnathic bones were retrieved. Clinical and demographic data were obtained from the patients' pathology records, while microscopic features and immunohistochemistry were reviewed and completed when necessary to confirm the diagnoses. Twenty-two LMs and five LMSs were obtained. In the LM group, males predominated, with a mean age of 45.7 years. The upper lip was the most affected site, and 18 cases were classified as angioleiomyomas and four as solid LM. In the LMS group, females predominated, with a mean age of 47.6 years. The mandible was the most affected site. Diffuse proliferation of spindle cells, with necrosis and mitotic figures, were frequent microscopic findings. LMs and LMSs were positive for α-smooth muscle actin, HHF-35 and h-caldesmon. In conclusion, oral LM/LMS are uncommon neoplasms with the latter usually presenting as metastatic disease. H&E evaluation may be very suggestive of oral LMs, but h-caldesmon staining is strongly recommended to confirm LMS diagnosis.

Leiomiosarcoma cutáneo ulcerado: Reporte de un caso clínico y revisión de la literatura / Ulcerated cutaneous leiomyosarcoma: A case report and literature review

Los leiomiosarcomas cutáneos son neoplasias malignas, originadas en el músculo liso, infrecuentes, tanto en las formas primarias como en el caso de las secundarias o metastásicas. Existen pocos casos publicados en la literatura. Presentamos el caso de un leiomiosarcoma cutáneo primario, en un hombre de 76 años de edad y realizamos una revisión de sus características clínicas y diagnóstico, así como, de las diferencias pronóstico-evolutivas según las distintas localizaciones de esta afección. Por último, debe señalarse que el tratamiento es fundamentalmente quirúrgico, con escisión y márgenes de seguridad, ya que, no existe una respuesta satisfactoria o significativa al tratamiento radiante y/o quimioterápico, siendo imprescindible efectuar un control evolutivo periódico de los pacientes, debido a la tasa de recurrencia local y la posibilidad de ocasionar metástasis, según la localización histológica del tumor.

Leiomyosarcomas located in superficial soft tissue are very rare or uncommon malignant neoplasms, both as primary tumors and as secondary or metastatic lesions. We present the case of a primary cutaneous leiomyosarcoma in a 76-year-old man, reviewing the clinical and diagnostic features, as well as the different prognoses depending on the site at this level. Treatment is basically surgical, involving excision with wide margins since the response to radiotherapy and/or chemotherapy is not satisfactory or significant. Given the high rate of local recurrence and the possibility of metastasis, depending on the histological location of the tumor, periodic follow-up of the patients is essential and indispensable.