Dermatomyofibroma D21.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

dermal myofibroma (adultes); Dermatomyofibroma

Definition
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Rare tumor originating from myofibroblasts with larger amounts of collagen fibers. S.a. leiomyoma. Adult myofibroma is the analogue of infantile myofibromatosis.

Etiopathogenesis
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Unknown, partly as hamartoma or secondary postoperative.

Manifestation
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Occurs in young adults between 20 and 30 years of age. Women and men are affected in the ratio 8:1. The tumour occurs less frequently in children.

Localization
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Mainly shoulder region, more rarely upper extremity or trunk; also palmar and plantar.

Clinical features
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1-2 cm large, roundish to oval, skin-colored or reddish-brown plaque-like or also calotte-shaped raised tumor (possibly with central crust) with peripheral border wall and surrounding telangiectasia; reminiscent of a basal cell carcinoma.

Histology
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In the reticular dermis, tumour clusters which are separated from the epidermis, are blurred and usually biphasic. Areas of bundles of spindle-shaped cells rich in cytoplasm; next to them areas of irregularly arranged, small, low-cytoplasmic cells with hyperchromatic nuclei. Capillary vessels are incised in varying numbers. Histological subtypes:

  • leiomyoma type (predominance of fascicular parts)
  • Cell-rich spindle cell type (fascicular or mat-like intertwined small spindle cells)
  • Hemangiopericytoma or glomus type (predominance of small myoid, pericyte or glomoid cells)
  • Biphasic type (see above)
  • Immunohistology: Tumor cells are vimentin- and alpha-SMA positive and desmin- and FXIIIa negative.

Differential diagnosis
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Therapy
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Excision in the healthy.

Progression/forecast
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Benign.

Note(s)
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Dermal fibroblasts, vascular smooth muscle cells and pericytes are precursor cells of myofibroblasts. Therefore, these cells can overlap in tumours.

Literature
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  1. Guitart J et al (1996) Solitary cutaneous myofibromas in adults: report of six cases and discussion of differential diagnosis. J Cutan Pathol 23: 437-444
  2. Hill H (1993) Plaque-like dermal fibromatosis/dermatomyofibroma. J Cutan catheter 20: 94
  3. Kamino H et al (1992) Dermatomyofibroma. A benign cutaneous, plaque-like proliferation of fibroblasts and myofibroblasts in young adults. J Cutan Pathol 19: 85-93
  4. Laaff H et al (1990) Cutaneous myofibroma - late manifestation. Dermatologist 41: 617-619
  5. Martinez-Mir A et al (2003) Germline fumarate hydratase mutations in families with multiple cutaneous and uterine leiomyomata. J Invest Dermatol 121: 741-744
  6. Sim JH et al (2011) Development of dermatomyofibroma in a male infant. Ann Dermatol 23 Suppl 1: S72-74.
  7. Tardío JC et al (2011) Dermatomyofibromas presenting in pediatric patients: clinicopathologic characteristics and differential diagnosis. J Cutan Pathol 38: 967-972
  8. Trotter MJ et al (1996) Linear dermatomyofibroma. Clin Exp Dermatol 21: 307-30

Disclaimer

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