Leiomyoma (overview) D21.M4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Congenital smooth muscle hamartoma; Cutaneous smooth muscle hamartoma; Leiomyoma; Leiomyomas; Leiomyomatas; Leiomyomatosis; Pilary leiomyoma; Piloleiomyoma; Smooth muscle hamartoma; smooth muscle tumor; Smooth muscle tumor

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Virchow, 1854; Kloepfer, 1958

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Benign, congenital or acquired cutaneous tumour originating from smooth muscle cells of cutaneous vessels or the arrectores pili muscle (more rarely the tunica dartos and smooth muscle cells of the nipples and vulva), which can occur either singly or multiply.

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It's gonna be 3 guys and different. The association with internal tumors is clinically significant. :

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In hereditary forms (hereditary multiple leiomyomas of the skin) autosomal-dominantly inherited mutations of the FH gene (fumarate hydratase; gene locus: 1q42.3-43) are detected. The FH gene encodes fumarate hydratase, which catalyses the reaction of fumarate to maleate in the citric acid cycle. In addition, the gene is ascribed properties as tumour suppressor genes.

Hereditary leiomyomatosis is associated with multiple leiomyomas of the uterus as well as with papillary renal cell carcinoma(hereditary leiomyomatosis with renal cell carcinoma).

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Occurrence is possible ubiquitously on the skin. Preferably affected are extremities, glutaeen and the scrotal skin.

Clinical features
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Rare solitary papules or knots. Mostly multiple, often grouped, but also arranged in stripes (in the Blaschko lines), up to 0.5 cm in size, skin-coloured to brownish, often pressure-painful (see below the acronym ANGLES for other painful tumours of the skin) or temperature-reactive, slightly raised nodules. S.a. Myomatosis cutis miliaris. A flat, plate-like infection is rarer (see illustration), in which plaques of 8.0-10.0 cm in size can develop. It is not uncommon for smooth muscle hamartomas (mostly piloleiomyomas) to be associated with a Becker nevus.

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Usually blurred tumour with irregularly shaped, interwoven bundles of smooth muscle cells separated from the epidermis by a narrow border zone. Characteristic features include spindly nuclear formations, a differently pronounced nuclear polymorphism. It is not uncommon to encounter single multinuclear giant cells, often with myxoid stroma and perivascular sclerosis (ancient leiomyoma, bizarre leiomyoma) as well as a honeycomb-like loosening of the cytoplasm (perinuclear halo formation) as an important differential diagnostic sign. S.a.u. Angioleiomyoma; see myofibroma below. In contrast to leiomyosarcoma, mitoses are absent.

Differential diagnosis
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Disseminated, hereditary leiomyomas of the skin occur in coincidence with leiomyomas of the uterus and papillary renal cell carcinoma (renal cell carcinoma syndrome).

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In case of increasing size growth and possibly also painfulness, an excision in healthy persons is recommended.

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Classification of leiomyomas according to origin and localization




starting from M. arrector pili

face, trunk extremities, mostly multiple occurrence


starting from the tunica muscularis of the vascular wall

mainly lower extremities, mostly solitary occurrence

Dartoid leiomyomas

starting from the tunica dartos

nipple, large labia, scrotal skin, mostly solitary occurrence

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The tunica dartos (from gr. dartos, "skinned", "like raw meat") is a thin layer of smooth muscle fibers of the scrotum. Among other things, it serves to regulate the temperature of the testicles.

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  1. Albares MP et al (2002) Digital angioleiomyoma. Int J Dermatol 41: 527
  2. Brown-Falco M et al (2002) Scrotal leiomyoma. Dermatologist 53: 258-260
  3. Kanitakis J et al (2000) Cutaneous leiomyomas (piloleiomyomas) in adult patients with human immunodeficiency virus infection. Br J Dermatol 143: 1338-1340
  4. Kloepfer HW et al (1958) Hereditary multiple leiomyoma of the skin. At J Hum Genet 10: 48-52
  5. Konig A, Happle R (2000) Two cases of type 2 segmental manifestation in a family with cutaneous leiomyomatosis. Eur J Dermatol 10: 590-592
  6. Konig A et al (2001) Type 2 segmental cutaneous leiomyomatosis. Acta Derm Venereol 81: 383
  7. Lang K et al (2002) Type 1 segmental cutaneous leiomyomatosis. Clin Exp Dermatol 27: 649-650
  8. Latoni JD et al (2000) Pilar leiomyoma: a case report and review of the literature. Ann Plast Surgery 45: 662-664
  9. Sahoo B et al (2001) Zosteriform pilar leiomyoma. J Dermatol 28: 759-761
  10. Schüürmann M et al (2017) Smooth muscle hamartoma in volar skin. Dermatologist 68:916-918.
  11. Thiers B et al (2009) Cutaneous mainfestations of internal malignancy. Cancer J Clin 59: 73-98
  12. Thyresson HN, Su WPD (1981) Familial cutaneous leiomyomatosis. J Am Acad Dermatol 4: 430-434
  13. Virchow R (1854) On macroglossia and pathological new formation of striated muscle fibers. Virchows Arch (Pathol Anat) 7: 126-138


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Last updated on: 29.10.2020