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Eccrine gland carcinoma
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Eccrine gland carcinoma

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Contributors: Gaurav Singh MD, MPH, Susan Burgin MD
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Synopsis

Eccrine gland carcinoma encompasses a rare group of sweat gland neoplasms that can be subdivided into many different types based on a wide histological spectrum.

Two to five million eccrine glands are distributed on almost the entire skin surface, with sparing of the lips, external auditory canal, glans penis, labia minora, and clitoris. Eccrine gland carcinoma can arise de novo in a normal sweat gland structure, or it can represent a transformation of a pre-existing benign eccrine neoplasm. It tends to occur in the fifth through eighth decades of life. It typically presents as a solitary, asymptomatic lesion, most commonly on the head and extremities and less often on the trunk. There may be ulceration or bleeding. There are reports of rapid growth to larger than 1 cm within a few months, as well as slow growth over several years. Some patients report a rapid burst of growth from a previously stable papule, plaque, or nodule.

The pathogenesis of these sweat gland neoplasms is not well understood. Immunosuppressed patients may be more susceptible to developing both benign and malignant eccrine neoplasms. Unlike squamous cell carcinoma and basal cell carcinoma, there is not a firmly established role for ultraviolet (UV) radiation and environmental toxins in eccrine carcinoma. This may be because the cutaneous appendages are relatively protected from these mutagens by their deep dermal location. However, some studies have entertained a causal role for both UV radiation and genetics in microcystic adnexal carcinoma, one type of eccrine carcinoma.

Eccrine gland carcinomas can be locally invasive, but most pose a low risk for metastasis. There are isolated reports of metastatic eccrine carcinomas, but these represent the minority of cases.

The classification of cutaneous sweat gland adnexal lesions is shown below with the corresponding benign precursors:
  • Poroma (benign) – porocarcinoma (malignant)
  • Hidradenoma (benign) – hidradenocarcinoma (malignant)
  • Spiradenoma (benign) – spiradenocarcinoma (malignant)
  • Cylindroma (benign) – cylindrocarcinoma (malignant)
  • Syringometaplasia (benign) – syringoid carcinoma (malignant)
  • Syringofibroadenoma (benign) – mucinous carcinoma, adenoid cystic carcinoma, and digital papillary adenocarcinoma (malignant)
  • Mixed tumor (chondroid syringoma; benign) – malignant mixed tumor
Some of the other adnexal carcinomas with presumed eccrine derivation include adenoid cystic carcinoma, mucinous carcinoma, digital papillary adenocarcinoma, and microcystic adnexal carcinoma (MAC).

Codes

ICD10CM:
D49.2 – Neoplasm of unspecified behavior of bone, soft tissue, and skin

SNOMEDCT:
400173004 – Eccrine carcinoma of skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The differential diagnosis for these lesions is broad and includes a variety of benign and malignant lesions. All of the entities discussed in this section are on the differential diagnosis for one another. Histopathology and immunohistochemical markers can be used to differentiate.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 07/09/2018
Last Updated: 07/16/2018
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Eccrine gland carcinoma
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