顶Check for updatesReceived:17 September 2022 Accepted:4 December 2022D01:10.1111/ddy.14989张DDGGUIDELINES1-guideline cutaneous and subcutaneous leiomyosarcomaDoris Helbig'I Edgar Dippel2 Michael Erdmann3 Alexander Frisman4Paula Kages I Ulrike Leiters Thomas Mentzel7 I Clemens SeidelsCarsten Weishaupt?Mirjana Ziemer10 Selma Ugurel11Hospital and Clinic for Dermatology andVenereology,University Hospital CologneSummary2Hospital for Dermatologyand Venereology,Superficial leiomyosarcomas(LMS)are rare skin cancers(2-3%of cutaneous sar-Ludwigshafen Hospitalcomas)that originate from dermally located hair follicle muscles,dartos or areolar3Department of Dermatology,Universitymuscles(cutaneous/dermal LMS),or from vascular muscle cells of the subcuta-Hospital Erlangen,CCC Erlangen EMN,Erlangenneous adipose tissue(subcutaneous LMS).These superficial LMS are distinct from4Hospital and Clinic for Radiation Therapy.LMS of the deep soft tissues.Leiomyosarcomas are typically localized at the lowerUniversity Hospital Leipzigextremities,trunk or capillitium,and present as painful,erythematous to brownish5Department of Dermatology,Venereology andnodules.Diagnosis is made by histopathology.The treatment of choice for primaryAllergology,University Hospital LeipzigLMS is complete(RO)microscopically controlled excision,with safety margins ofCenter for Dermatooncology,University1 cm in dermal LMS,and 2 cm in subcutaneous LMS,if possible.Non-resectableHospital for Dermatology,Eberhard-Karlsor metastatic LMS require individual treatment decisions.After RO resection withUniversity Tubingen1 cm safety margins,the local recurrence rate of dermal LMS is very low,and7MVZ Dermatopathologymetastasis is very rare.Subcutaneous LMS,very large,or incompletely excisedFriedrichshafen/Bodensee,FriedrichshafenLMS recur and metastasize more frequently.For this reason,clinical follow-upHospital and Clinic for Radiation TherapyUniversity Hospital Leipzigexaminations are recommended every six months for cutaneous LMS,and everythree months for subcutaneous LMS within the first two years (in subcutaneousHospital for Skin Diseases,University HospitalMunsterLMS including locoregional lymph node sonography).Imaging such as CT/MRIDepartment of Dermatology,Venereologyandis indicated only in primary tumors with special features,recurrences,or alreadyAllergology,University Hospital Leipzigmetastasized tumors.Department of Dermatology,VenereologyandAllergology,University Hospital EssenCorrespondencePriv.-Doz.Dr.med.Doris Helbig,Department ofDermatology and Venereology at the Universityof Cologne,Kerpener StraBe 62,50937 Koln.Email:doris.helbig@uk-koeln.deGuideline coordinator Priv-Doz.Dt med.DorisHelbig KolnAuthorized Guideline Representative at ADO/DKGProf.Dr.med.Selma Ugurel,EssenEPIDEMIOLOGY,ETIOPATHOGENESIS,ANDCLINICAL APPEARANCEPrimary superficial leiomyosarcoma (LMS)is a rareGuideline prepared under the authority of the Working Group for DermatologicalOncology (ADO,Arbeitsgemeinschaft Dermatologis