Adnexal carcinoma microcystic C44.L

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

malignant syringoma; microcystic adnexal carcinoma; Sclerosing sweat gland duct carcinoma; Sweat gland carcinoma with syringoid features; Sweat gland duct carcinoma sclerosing; syringoma malignes

History
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Goldstein, 1982

Definition
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Rare, malignant, usually well differentiated, eccrine sweat gland tumor characterized by slow but locally aggressive growth and a high tendency to recur. Histologically, it can easily be confused with benign anextremity tumors.

Manifestation
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Accumulation in the 6th decade of life. However, occurrence is possible in every adult age (from the 3rd decade of life). No sex preference.

Localization
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Mostly face (75%), especially upper or lower lip, nasolabial folds, forehead, rarely extrafacial.

Clinical features
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Slowly growing (often clinically inconspicuous), painless, skin-coloured, glassy to yellowish-reddish, rarely ulcerated, usually flat, firm, smooth lumps. It is also possible for the nodules to appear as rough red to skin-coloured papules or plaque with edges that are difficult to delimit and superficial telangiectasia. The affected skin appears normal, only occasionally atrophic. Paresthesias or pain are rarer. Local infiltrations of fatty tissue, muscles and bones may occur and complicate the clinical picture.

Histology
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  • Tubular structures, horny cysts, ribbon-shaped tumor cell strands. Deeply infiltrating growth of epithelial tumor nests. Penetration of the entire corium with tumor cell strands and small to medium-sized cysts filled with concentrically layered eosinophilic material. No stratum granulosum in the cyst wall. Perineural infiltration in the form of epithelial bands. The tumor cell bands consist of medium-sized, predominantly cuboid cells with blistery, light-colored nuclei and eosinophilic or clear cytoplasm. Moderate pleomorphism. Distinct fibrotic stroma reaction.
  • Immunohistology: Cytokeratin and vimentin positive, mostly CEA positive (characteristic of sweat glands).

Differential diagnosis
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Clinical:

  • Basal cell carcinoma, sclerodermiformes: Largely at the level of the skin, waxy or ivory-coloured, only vaguely distinguishable from the surrounding area, rough, shiny infiltrate plate with telangiectasia. A clear clinical differentiation is not possible. The histological image is diagnostic.
  • Carcinoma, spinocellular: Mostly painless, exophytic, skin-coloured, often crusty, coarse humpy, mostly eroded or ulcerated nodules of coarse consistency. The histological picture is diagnostic scar: anamnesis! No size growth; the woody consistency of the microcystic adnexal carcinoma is rather atypical for a scar. Keloids are rather rare in UV-exposed areas.
  • Granuloma eosinophilicum faciei: Typically roundish to oval, 0.5-2.0 cm in size, mostly solitary but also several or numerous, slightly raised, firm, symptomless, brown-red, scale-free plaques with dilated follicle orifices. This results in an "orange peel-like" surface aspect; this is missing in microcystic adnexal carcinoma stests.

Histological:

Therapy
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  • Excision with a sufficiently large safety margin (up to 3.0 cm, if possible in the face) and microscopically controlled surgery (MKC) due to the subclinical tumor extensions and the possible deep infiltration. Long follow-up period.
  • Alternative: Irradiation with fast electrons. Caution: High recurrence rate!

Progression/forecast
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So far no metastasis has been described, no multilocular occurrence. However, locally destructive and growing with extensive tumor cones.

Note(s)
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Remember! In the differential diagnostic evaluation there should be agreement between clinical and histological findings. If the histological substrate " syringoma" does not correlate with the clinical findings (coarse plate-like infiltrate or solid nodules instead of multiple small nodules), the diagnosis "syringoma" must be carefully checked.

Literature
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  1. Abbate M et al (2003) Clinical course, risk factors, and treatment of microcystic adnexal carcinoma: a short series report. Dermatol Surgery 29: 1035-1038
  2. Baxi S et al (2010) Microcystic adnexal carcinoma of the skin: the role of adjuvant radiotherapy. J Med Imaging Radiat Oncol 54:477-482
  3. Chaudhari SP et al(2015) Treatments for microcystic adnexal carcinoma - A review. J Dermatologist Treat 11:1-7
  4. Chi J et al (2002) Microcystic adnexal carcinoma of external auditory canal: report of a case. Otolaryngol Head Neck Surgery 127: 241-242
  5. Culhaci N et al (2003) Microcystic adnexal carcinoma: report of a case. J Oral Maxillofac Surgery 61: 723-725
  6. Diamantis SA et al (2011) Mohs micrographic surgery in the treatment of microcystic adnexal carcinoma. Dermatol Clin 29:185-190
  7. Gartmann H et al (1991) Verrucous microcystic adnexal carcinoma of the nose. Z Hautkr 67: 148-154
  8. Goldstein DJ (1982) Micorcystic adnexal carcinoma: a distinct clinopathological entity. Cancer 50: 566-572
  9. Hamsch C et al (2010) Microcystic adnexal carcinoma - sometimes inconspicuous appearance of a locally infiltrating tumour. JDDG 8: 275-278
  10. Hodgson TA et al (2003) Microcystic adnexal carcinoma: an unusual cause of swelling and paraesthesia of the lower lip. Oral Oncol 39: 195-198
  11. McKinley LH et al (2014) Microcystic adnexal carcinoma: review of a potential diagnostic pitfall and management. Cutis 93:162-165
  12. Stein JM et al (2003) The effect of radiation therapy on microcystic adnexal carcinoma: a case report. Head neck 25: 251-254

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Last updated on: 29.10.2020