S1‐guideline cutaneous and subcutaneous leiomyosarcoma

Abstract

Summary Superficial leiomyosarcomas (LMS) are rare skin cancers (2–3% of cutaneous sarcomas) that originate from dermally located hair follicle muscles, dartos or areolar muscles (cutaneous/dermal LMS), or from vascular muscle cells of the subcutaneous adipose tissue (subcutaneous LMS). These superficial LMS are distinct from LMS of the deep soft tissues. Leiomyosarcomas are typically localized at the lower extremities, trunk or capillitium, and present as painful, erythematous to brownish nodules. Diagnosis is made by histopathology. The treatment of choice for primary LMS is complete (R0) microscopically controlled excision, with safety margins of 1 cm in dermal LMS, and 2 cm in subcutaneous LMS, if possible. Non‐resectable or metastatic LMS require individual treatment decisions. After R0 resection with 1 cm safety margins, the local recurrence rate of dermal LMS is very low, and metastasis is very rare. Subcutaneous LMS, very large, or incompletely excised LMS recur and metastasize more frequently. For this reason, clinical follow‐up examinations are recommended every six months for cutaneous LMS, and every three months for subcutaneous LMS within the first two years (in subcutaneous LMS including locoregional lymph node sonography). Imaging such as CT/MRI is indicated only in primary tumors with special features, recurrences, or already metastasized tumors.

Advancing Cancer Research: Current Knowledge on Cutaneous Neoplasia

“…Regarding the treatment, complete surgical excision is the preferred treatment option [ 139 ]. However, there is no consensus on the appropriate safety limits.…”

“…Radiotherapy remains the best solution for local palliative control in tumors with metastases. Neoadjuvant chemotherapy is used for disseminated disease and includes various drugs such as doxorubicin, ifosfamide, gemcitabine, docetaxel, taxotere, dacarbazine, and trabectedin [ 139 ]. Future directions in treatment include immunotherapy, gene therapy, and targeted therapies with tyrosine kinase inhibitors.…”

“…Chest X-rays are performed annually during the first 5 years after surgery, along with general clinical and locoregional lymph node assessments. MRI is occasionally used for recurrent or hypodermic lesions [ 139 ].…”