PubMed · 4311553
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M A Storkan, I Cliff. 1969. Solid hidradenoma.. https://doi.org/10.1001/archderm.100.4.504
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BACKGROUND: Eccrine syringofibroadenoma (ESFA) is a rare eccrine tumor that has characteristic histopathologic features and variable clinical findings. OBJECTIVE: Our purpose was to define the clinical and histopathologic features of ESFA, to assess a possible syndromic association and heredity, and to propose a clinicopathologic classification. METHODS: Three solitary and five multiple cases of ESFA were analyzed and the findings compared with those of previously published cases. RESULTS: Clinically, solitary ESFA was typically a nonhereditary verrucous growth. Multiple examples presented as palmoplantar keratoderma and keratotic papules in a mosaic pattern. In the multiple form, four patients had the Schöpf syndrome (hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities); one had an incomplete form of the syndrome. Histologically, ESFA showed a reticulate proliferation of thin strands of acrosyringeal cells with focal lumen formation, and a fibrovascular stroma. Malignant ESFA was present in one multiple case. CONCLUSION: Multiple (palmoplantar) ESFAs are a new cutaneous marker of the Schöpf syndrome.
Although a benign syringofibroadenoma has been recognized as an eccrine tumor exhibiting net-like ductal proliferation embedded in fibroma-like stroma, a malignant counterpart has not been described heretofore. An 82-year-old man with severely sun-damaged skin had a 3 cm plaque in the dorsum of his left hand. The clinical diagnosis was squamous cell carcinoma. Histologically, the lesion was a bowenoid squamous cell carcinoma, both in situ (Bowen's disease-like) and bluntly invasive, with a concurrent phase showing unequivocal and widespread ductal differentiation, local expression of carcinoembryonic antigen, and sclerotic stroma. This syringofibrocarcinoma also impinged laterally on a mature and benign syringofibroadenoma and occurred above a minute deep dermal trichoepithelioma. This report presents a probably unrecognized new malignant tumor, clearly distinct yet related to both squamous cell carcinoma and eccrine carcinoma. Because its organoid architecture and the resemblance to and impingement on syringofibroadenoma, the term syringofibrocarcinoma is proposed.
Malignant eccrine spiradenoma is an exceedingly rare tumor. A case of a 72-year-old women with this highly aggressive malignancy arising from a long-standing lower leg lesion is reported. Management during the course of disease included surgery, radiation therapy (RT), hyperthermic limb perfusion chemotherapy, and chemotherapy. The patient died of her disease, with widespread metastatic disease 20 months after the diagnosis. A review of the literature is presented, and treatment considerations are summarized.