Eccrine porocarcinoma is a rare malignant tumor arising from the intraepidermal ductal portion of the eccrine sweat gland (acrosyringium). It usually affects older persons and is located most commonly on the lower extremities. We describe a 55-year-old man with an aggressive metastasizing eccrine porocarcinoma diagnosed 10 years after the primary lesion. The metastatic lesions were located in the public area and scrotum and were associated with progressive lymphedema. Several biopsy specimens revealed numerous tumor cells in the dermis and especially in the lumina of lymph and blood vessels. Immunohistochemical studies showed staining with cytokeratin KL1, epithelial membrane antigen, Ca 15-3, and Ca 19-9. Vimentin, S-100 protein, neuron specific enolase, and carcino-embryonic antigen were negative. Treatment with carbon dioxide laser failed. The use of interferon alfa-2a for 9 months stopped progression of the tumor.

https://www.jaad.org/article/S0190-9622(96)90105-X/pdf

Metastasizing eccrine porocarcinoma: report of a case and review of the literature

Metastatic eccrine porocarcinoma: Response to docetaxel (Taxotere) chemotherapy

Background Adnexal carcinomas are rare and diverse cutaneous tumours. They are locally aggressive and have the potential for distant metastasis. Metastatic adnexal carcinomas are very resistant to conventional chemotherapies. Sunitinib, an oral tyrosine kinase inhibitor, is reportedly effective for the treatment of various solid cancers. Its use in adnexal carcinomas has never been reported.

Observations  The first patient had metastatic clear cell hidradenocarcinoma and was stabilized over 8 months with sunitinib, before she relapsed. The second patient had a metastatic malignant hair follicle tumour (trichoblastic carcinoma) and achieved a partial remission with sunitinib, and disease stabilized after 10 months. Dynamic contrast-enhanced ultrasound (DCE-US) performed to evaluate tumour vascularization during treatment depicted a dramatic and early decrease in the tumour blood volume.

Conclusions  Sunitinib was effective in controlling the disease in our two patients. DCE-US using linear raw data may have an early predictive value for tumour response to sunitinib. Further studies involving larger cohorts of patients are warranted in order to confirm the efficacy of sunitinib in these rare tumours.

Sunitinib efficacy in the treatment of metastatic skin adnexal carcinomas: report of two patients with hidradenocarcinoma and trichoblastic carcinoma

One of two patients with systemic metastases from a poorly differentiated eccrine adenocarcinoma of the scalp was found to have a tumor positive for estradiol receptors. In the receptor positive patient, after tamoxifen therapy, the lymph node metastasis regressed completely and was associated with full relief of pain from osseous metastases for nearly 3 years. Subsequently, progressive painful osseous metastases in the spine, skull, pelvis, and femur were palliated for shorter periods with sequential systemic therapy with megestrol acetate and fluoxymesterone. Osseous metastases were also palliated with external radiation therapy. In contrast, despite external radiation therapy, brain metastases proved fatal. Tamoxifen was ineffective in the estradiol receptor negative patient. Based on this report, it may be valuable to determine the presence of estradiol receptor protein in eccrine carcinoma as a predictor of response to hormonal therapy.

Response of eccrine adenocarcinoma to tamoxifen

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.

Malignant Eccrine Spiradenoma: Case Report and Review of the Literature

Sweat gland carcinoma (SGC) is a rare malignancy of the skin. though many patients with SGC die of disseminated metastases, little is known regarding the value of systemic chemotherapy for this disease. We reviewed the records of 20 patients with metastatic SGC who were treated with chemotherapy at Memorial Hospital between 1968 and 1983. A large variety of drugs were given. Although only a few patients were treated with any given regimen, metastatic SGC appears to be poorly responsive to a wide variety of chemotherapeutic regimens. Five major responses were observed in 30 chemotherapy trials performed in 17 patients with measurable/evaluable disease. No patient responded to single agent therapy alone. In this small group of patients, SGC appears to be a relatively chemotherapy resistant tumor. Larger, group-wide or inter-group trials are needed to prospectively evaluate the use of chemotherapy in this disease. Doxorubicin and cyclophosphamide, the two drugs used most commonly in those combinations where responses were seen, appear to be reasonable choices for initial treatment of patients with metastatic disease. Our review does not provide data to support the empiric use of chemotherapy in an adjuvant setting.

A G Huvos

Papers

Chemotherapy of metastatic sweat gland carcinoma. A retrospective review