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Malignant eccrine spiradenoma L74.8

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 20.07.2025

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Definition
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Very rare, undifferentiated, malignant adnexal tumor developing at the base of a spiradenoma.

Localization
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The head, neck, upper trunk area or bending sides of the arms are affected. Infestation of the vulva is described.

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Clinic
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Mostly solitary, suddenly growing node, which has grown on the ground of a pre-existent spiradenoma. The clinical picture is not very characteristic apart from the possible painfulness. The diagnosis is generally a histological accidental finding.

Histology
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In most cases the very distinct histological morphology of the spiradenoma is still detectable with a deeply dermal nodular tumor complex, partly consisting of one or more nodes. Parenchyma consisting of basaloid, poorly differentiated polymorphic cell complexes. Partially also spindle cell differentiation. Numerous mitoses, mass necroses. Often clear lymphocytic infiltrate.

Complication(s)(associated diseases
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Metastasis in 20% of cases.

Due to the rarity of metastatic apocrine carcinoma, there are no standardized therapies. A therapeutic trial with S-1 monotherapy (oral antitumor drug containing tegafur, 5-fluorouracil precursor and 2 enzyme modulators, gimeracil and otaceril (Ishizuki S et al.2025) proved to be successful in individual cases.

Therapy
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Excision according to the criteria of the spinocellular carcinoma, necessarily with marginal incision controls and lymph node mapping ( sentinel lymph node dissection).

Note(s)
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Spiradenocarcinoma is "frequently" associated with cylindromes.

Literature
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Catteau X et al. (2020) Low grade malignant eccrine spiradenoma of the vulva: case report, review of the literature and discussion about the role of p53 and HPV. Diagn Pathol 15: 24.
Granter SR et al. (2000) Malignant eccrine spiradenoma (spiradenocarcinoma): a clinicopathologic study of 12 cases. Am J Dermatopathol 22: 97-103
Ishizuki S et al. (2025) Complete response of a metastatic cutaneous apocrine carcinoma under oral S-1 monotherapy. J Dtsch Dermatol Ges 23:524-526.
Leonard N et al. (2003) Low-grade malignant eccrine spiradenoma with systemic metastases. Am J Dermatopathol 25: 253-255
Mirza I et al. (2002) Malignant eccrine spiradenoma. Arch Pathol Lab Med 126: 591-594
Rutten A (2002) Eccrine sweat gland carcinoma of the skin. Pathologist 23: 79-88