Porocarcinoma C44.L

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 02.12.2024

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Synonym(s)

Carcinoma eccrine epidermotropic; Carcinoma epidermotropic eccrine; dysplastic poroma; Epidermotropic eccrine carcinoma; Hidroacanthoma simplex maligna; malignant eccrine poroma; malignant syringoacanthoma; Porom dysplastic; Porom malignes eccrines; syringoacanthoma malignes

History
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Pinkus and Mehregan, 1963

Definition
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Rare, malignant, infiltrating sweat gland tumor originating from the intraepidermal part of the eccrine sweat gland excretory duct. Retrograde ingrowth into the epidermis is characteristic.

Occurrence/Epidemiology
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Incidence: 0.01-0.005%. m:w=1:1 (Salih AM et al. 2017)

Manifestation
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The average age in a meta-analytical search was 67.6 years. The average duration of the disease was 5.57 years and ranged from <1 year to 60 years.

Localization
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The head and neck are most frequently affected (39.9%), followed by the lower extremities (33.9%). The genital area is less frequently affected.

Clinical features
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Moderately fast-growing, squamous or nodular epithelial tumor with or without ulceration. The tumours are highly variable in terms of appearance, size and location. Porocarcinomas often develop on the basis of a poroma that has existed for years. The clinical malignancy criterion is a sudden rapid growth of a pre-existing tumor (see figure). Porocarcinomas can also develop complicatively in a nevus sebaceus. Eczmatoid changes are described less frequently.

In 31% of cases, metastases are already present at the time of initial clinical presentation. The most common organ to which porocarcinoma metastasizes is the regional lymph node (57.7 %). Seeding of epidermotropic metastases is possible. Visceral metastases rarely develop (Salih AM et al. 2017).

Histology
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Often arising at the base of a poroma, asymmetric carcinoma that broadly infiltrates the deeper portions of the dermis (and subcutis). Tumor formations with reticularly branched, ribbon-like structures that make broad-based contact with the surface epithelium are characteristic. As in poroma, the formation of ductal as well as light cell structures is possible. Cell formations of small basaloid cells with chromatin-rich nuclei are seen. Abundant mitoses. Evidence of mass necrosis (see also under poroma).

The intraepithelial form of porocarcinoma is called malignant hidroacanthoma simplex. It is characterized by poroid epithelial clusters with densely packed tumor cells, numerous mitoses, and a markedly increased proliferation rate.

Differential diagnosis
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Therapy
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Procedure as for spinocellular carcinoma. Radical excision with histological edge control. Safety distance of at least 1 cm. In case of lymph node or organ metastases: Procedure as in spinocellular carcinoma.

Progression/forecast
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Moderately rapid growth. In the presence of lymph node metastases, the mortality rate is over 65%.

Literature
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  1. D'Agostino GM et al. (2024) Porcarcinoma of the foot. JDDG 22: 1550-1552

  2. Gartmann H (1985) Eccrine porocarcinoma. Z Hautkr 60: 555-562
  3. Grayson W et al. (2003) Eccrine porocarcinoma of the penis. J Urol 169: 611-612
  4. Gu LH et al. (2003) Aberrant expression of p16 and RB protein in eccrine porocarcinoma. J Cutan Pathol 29: 473-479
  5. Helmer A et al. (2006) Eccrine porocarcinoma among multiple other epithelial skin tumors. Dermatology 57: 1120-1121
  6. Horn T et al (1985) Malignant eccrine poroma. Z Hautkr 60: 992-999
  7. Johr R et al. (2003) Eccrine porocarcinoma arising in a seborrheic keratosis evaluated with dermoscopy and treated with Mohs' technique. Int J Dermatol 42: 653-657
  8. Miyamoto K et al. (2022) Diagnosis and Management of Porocarcinoma. Cancers (Basel) 14:5232.
  9. Murphy LA; Barlow RJ (2003) Eccrine porocarcinoma treated with Moh`s micrographic surgery: a report of three cases. Br J Dermatol 149 (Suppl 64): 103
  10. Pinkus H, Mehregan AH (1963) Epidermotrophic eccrine carcinoma: a case combining features of eccrine poroma and Paget's dermatosis. Arch Dermatol 88: 597-606
  11. Salih AM et al. (2017) Porocarcinoma; presentation and management, a meta-analysis of 453 cases. Ann Med Surg (Lond) 20:74-79.

  12. Shaw M et al. (1982) Malignant eccrine poroma: a study of twenty-seven cases. Br J Dermatol 107: 675-680
  13. Snow SN et al. (1992) Eccrine porocarcinoma of the face. J Am Acad Dermatol 27: 306-311

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Last updated on: 02.12.2024