S1‐guideline cutaneous and subcutaneous leiomyosarcoma

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 superf...

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Published in:Journal der Deutschen Dermatologischen Gesellschaft 2023-05, Vol.21 (5), p.555-563
Main Authors: Helbig, Doris, Dippel, Edgar, Erdmann, Michael, Frisman, Alexander, Kage, Paula, Leiter, Ulrike, Mentzel, Thomas, Seidel, Clemens, Weishaupt, Carsten, Ziemer, Mirjana, Ugurel, Selma
Format: Article
Language:English
Subjects:
Adipose tissue
Humans
Leiomyosarcoma - diagnosis
Leiomyosarcoma - pathology
Leiomyosarcoma - surgery
Lymph nodes
Metastases
Muscles
Skin
Skin - pathology
Skin Neoplasms - pathology
Soft tissues
Subcutaneous Fat
Subcutaneous Tissue - pathology
Tumors
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Summary: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.
ISSN:1610-0379
1610-0387
DOI:10.1111/ddg.14989