Angiomyxoma cutaneous D23.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiomyxoma; cutaneous myxoma; focal dermal mucinosis; Mucinosis focal cutaneous; superficial angiomyxoma

Definition
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Rare, benign, dermal or subcutaneous new formation(s) consisting of mucus tissue, which can occur solitary or multiple times.

Cutaneous angiomyxomas usually occur monotopically as harmless neoplasms in the skin.

Cutaneous myxomas can be partial symptoms of syndromic entities (see below NAME syndrome or LAMB or Carney syndrome - cardiac and cutaneous myxomas, pigment disorders, neuroendocrine tumors).

Etiopathogenesis
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Unclear, discussed are dominant inheritance, excessive production of acidic mucopolysaccharides of fibroblasts of unclear genesis and persistence of embryonic connective tissue.

Manifestation
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Occurs more frequently between the 2nd and 3rd decade of life, with extracutaneous localisation preferably between the 6th and 7th decade of life.

Localization
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extremities, neck and neck, genitals

Clinical features
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Papular or nodular, flat, completely asymptomatic, soft, gentle protrusions of unmodified or slightly reddened skin. From time to time, emptying of a thread-forming mucus is possible. The changes develop very slowly and are seen as cosmetic disorders rather than "disease".

The (systemic) myxomas occurring in the NAME syndrome are located acrally on the skin (see NAME syndrome below).

Histology
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A tumour localised in the dermis and subcutis, usually multilobular, clearly demarcated from the surrounding tissue, but not encapsulated. Spindly or star-shaped, reticularly connected cells are found. Storage of gelatinous substances in the meshwork, ectatic vessels. S.a.u. Myxoma cavernosum. Capillary vessel sections of varying density.

Differential diagnosis
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Fibroma; lipoma. Furthermore, the clinical picture must be distinguished from acral persistent papular mucinosis.

Therapy
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Progression/forecast
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The tendency to malignant degeneration is assessed differently, very recurrent.

Literature
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  1. Alaiti S et al (2000) Solitary cutaneous myxoma. J Am Acad Dermatol 43: 377-379
  2. Allen PW et al (1988) Superficial angiomyxomas with and without epithelial components. Report of 30 tumors in 28 patients. Am J Surg Pathol 12: 519-530
  3. Calikoglu E et al (2002) Hyaluronate accumulation and decreased CD44 expression in perifollicular solitary cutaneous myxoma. Dermatology 205: 122-126
  4. Choi HJ et al (2007) Unusual presentation of solitary cutaneous myxoma. J Eur Acad Dermatol Venereol 21:403-404
  5. Fetsch JF et al (1997) Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol 16: 325-334
  6. Li Y et al (2003) Herpes simplex virus type 1 infection associated with atrial myxoma. At J path 163: 2407-2412
  7. Kahn SL et al (2014) Angiomyxoma of the nasal dorsum treated by Mohs surgery. Australas J Dermatol doi: 10.1111/ajd.12245
  8. Kura MM et al (2014) Solitary superficial acral angiomyxoma: an infrequently reported soft tissue tumor. Indian J Dermatol 59: 529
  9. Nishimoto K et al (2004) Surrounding muscle edema detected by MRI is valuable for diagnosis of intramuscular myxoma. Oncol Rep 11: 143-148
  10. Senff H et al (1988) Cutaneous myxoma (focal dermal mucinosis). Dermatologist 39: 606-610

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020