Eccrine porocarcinoma presenting with scrotal lymphedema: a case report and review of systemic treatment
Authors: J. Perez-Garcia, R. Morales, C.M. Valverde, J. Rodon, C. Suarez, M.E. Semidey, V. Garcia-Patos, R. Bartralot, M. Serra, J. Carles
Journal: Annals of Oncology (2010)

Eccrine porocarcinoma is an unusual neoplasm of the intraepidermal sweat gland duct, which represents 0.005%–0.01% of all skin tumors, with a significant risk of local recurrence rate, regional lymph node spread and distant metastasis [1.Wick M.R. Goellner J.R. Wolfe 3rd, J.T. et al.Adnexal carcinomas of the skin. I. Eccrine carcinomas.Cancer. 1985; 56: 1147-1162Crossref PubMed Scopus (152) Google Scholar].Here, we report a case of a 69-year-old man with a 4-week long history of scrotal lymphedema with enlarged inguinal lymph nodes and a hyperkeratotic lesion on the pretibial area. Cytologic analysis of lymph node was positive for malignant cells and an initial punch biopsy specimen of the pretibial plaque was interpreted as a seborrheic keratosis. An incisional biopsy was carried out and the definitive diagnosis was eccrine porocarcinoma with extensive lymphovascular invasion. Positron emission tomography/computed tomography scan revealed increased 2-[fluorine-18]fluoro-2-deoxy-D-glucose uptake in the pretibial area and inguinal lymph nodes.The tumor was considered nonresectable, so the patient was treated with five cycles of cisplatin and docetaxel as induction chemotherapy. Chemotherapy treatment resulted in a sustained abatement of symptoms and the tumor presented partial response (partial response was defined as a metabolic response based on PET scans, and a 30% reduction in the the longest diameter of the eccrine porocarcinoma), allowing a complete resection with clear margins without response of the metastatic ganglionar disease. The patient remains with no evidence of disease at 1-year follow-up.Currently, the recommended treatment of localized porocarcinoma is wide surgical excision with clear margins and in the case of positive metastatic lymph nodes, radical regional lymph node dissection. The use of adjuvant radiotherapy or chemotherapy and the role of sentinel lymph node sampling remain unclear.Eccrine porocarcinoma is considered relatively chemoresistant but anecdotal responses to single-agent chemotherapy and combination chemotherapy have been reported. Single-agent treatments such as docetaxel, paclitaxel and 5-fluorouracil (5-FU) have demonstrated some antitumor activity in metastatic disease. A partial response to docetaxel in a patient previously treated with epirubicin [2.Plunkett T.A. Hanby A.M. Miles D.W. et al.Metastatic eccrine porocarcinoma: response to docetaxel (Taxotere) chemotherapy.Ann Oncol. 2001; 12: 411-414Abstract Full Text PDF PubMed Scopus (51) Google Scholar] and long-term stable disease (stable disease was defined as less than a 30% of reduction and less than a 20% increase in the sum of the products of two perpendicular diameters of all measured lesions and the appearance of no new lesions) in a patient treated with a combination of interferon-α and weekly paclitaxel have been reported [3.Gutermuth J. Audring H. Voit C. et al.Antitumour activity of paclitaxel and interferon-alpha in a case of metastatic eccrine porocarcinoma.J Eur Acad Dermatol Venereol. 2004; 18: 477-479Crossref PubMed Scopus (37) Google Scholar]. Antitumor activity has also been reported using interferon-α alone or in combination with isotretinoin and 5-FU i.v. administered or by intra-arterial infusion in combination with melphalan and regional hyperthermia [4.Swanson Jr, J.D. Pazdur R. Sykes E. Metastatic sweat gland carcinoma: response to 5-fluorouracil infusion.J Surg Oncol. 1989; 42: 69-72Crossref PubMed Scopus (21) Google Scholar].Anthracycline-based combination chemotherapy regimens have also resulted in complete (complete response was defined as the complete disappearance of all measurable disease) and partial responses. A complete response lasting for 16 months and prolonged survival were obtained with the combination of doxorubicin, mitomycin C, vincristine and cisplatin in a patient with an eccrine porocarcinoma with bone and visceral metastasis [5.Piedbois P. Breau J.L. Morere J.F. et al.Sweat gland carcinoma with bone and visceral metastases. Prolonged complete remission lasting 16 months as a result of chemotherapy.Cancer. 1987; 60: 170-172Crossref PubMed Scopus (31) Google Scholar]. One partial and one complete response have also been reported with the combination of doxorubicin, cyclophosphamide, vincristine and bleomycin.In conclusion, there is no standard systemic treatment of metastatic and locally advanced disease but anthracycline-based chemotherapy or combination chemotherapy with 5-FU, taxanes and cisplatin should be considered in the first-line treatment of this malignancy. Eccrine porocarcinoma is an unusual neoplasm of the intraepidermal sweat gland duct, which represents 0.005%–0.01% of all skin tumors, with a significant risk of local recurrence rate, regional lymph node spread and distant metastasis [1.Wick M.R. Goellner J.R. Wolfe 3rd, J.T. et al.Adnexal carcinomas of the skin. I. Eccrine carcinomas.Cancer. 1985; 56: 1147-1162Crossref PubMed Scopus (152) Google Scholar]. Here, we report a case of a 69-year-old man with a 4-week long history of scrotal lymphedema with enlarged inguinal lymph nodes and a hyperkeratotic lesion on the pretibial area. Cytologic analysis of lymph node was positive for malignant cells and an initial punch biopsy specimen of the pretibial plaque was interpreted as a seborrheic keratosis. An incisional biopsy was carried out and the definitive diagnosis was eccrine porocarcinoma with extensive lymphovascular invasion. Positron emission tomography/computed tomography scan revealed increased 2-[fluorine-18]fluoro-2-deoxy-D-glucose uptake in the pretibial area and inguinal lymph nodes. The tumor was considered nonresectable, so the patient was treated with five cycles of cisplatin and docetaxel as induction chemotherapy. Chemotherapy treatment resulted in a sustained abatement of symptoms and the tumor presented partial response (partial response was defined as a metabolic response based on PET scans, and a 30% reduction in the the longest diameter of the eccrine porocarcinoma), allowing a complete resection with clear margins without response of the metastatic ganglionar disease. The patient remains with no evidence of disease at 1-year follow-up. Currently, the recommended treatment of localized porocarcinoma is wide surgical excision with clear margins and in the case of positive metastatic lymph nodes, radical regional lymph node dissection. The use of adjuvant radiotherapy or chemotherapy and the role of sentinel lymph node sampling remain unclear. Eccrine porocarcinoma is considered relatively chemoresistant but anecdotal responses to single-agent chemotherapy and combination chemotherapy have been reported. Single-agent treatments such as docetaxel, paclitaxel and 5-fluorouracil (5-FU) have demonstrated some antitumor activity in metastatic disease. A partial response to docetaxel in a patient previously treated with epirubicin [2.Plunkett T.A. Hanby A.M. Miles D.W. et al.Metastatic eccrine porocarcinoma: response to docetaxel (Taxotere) chemotherapy.Ann Oncol. 2001; 12: 411-414Abstract Full Text PDF PubMed Scopus (51) Google Scholar] and long-term stable disease (stable disease was defined as less than a 30% of reduction and less than a 20% increase in the sum of the products of two perpendicular diameters of all measured lesions and the appearance of no new lesions) in a patient treated with a combination of interferon-α and weekly paclitaxel have been reported [3.Gutermuth J. Audring H. Voit C. et al.Antitumour activity of paclitaxel and interferon-alpha in a case of metastatic eccrine porocarcinoma.J Eur Acad Dermatol Venereol. 2004; 18: 477-479Crossref PubMed Scopus (37) Google Scholar]. Antitumor activity has also been reported using interferon-α alone or in combination with isotretinoin and 5-FU i.v. administered or by intra-arterial infusion in combination with melphalan and regional hyperthermia [4.Swanson Jr, J.D. Pazdur R. Sykes E. Metastatic sweat gland carcinoma: response to 5-fluorouracil infusion.J Surg Oncol. 1989; 42: 69-72Crossref PubMed Scopus (21) Google Scholar]. Anthracycline-based combination chemotherapy regimens have also resulted in complete (complete response was defined as the complete disappearance of all measurable disease) and partial responses. A complete response lasting for 16 months and prolonged survival were obtained with the combination of doxorubicin, mitomycin C, vincristine and cisplatin in a patient with an eccrine porocarcinoma with bone and visceral metastasis [5.Piedbois P. Breau J.L. Morere J.F. et al.Sweat gland carcinoma with bone and visceral metastases. Prolonged complete remission lasting 16 months as a result of chemotherapy.Cancer. 1987; 60: 170-172Crossref PubMed Scopus (31) Google Scholar]. One partial and one complete response have also been reported with the combination of doxorubicin, cyclophosphamide, vincristine and bleomycin. In conclusion, there is no standard systemic treatment of metastatic and locally advanced disease but anthracycline-based chemotherapy or combination chemotherapy with 5-FU, taxanes and cisplatin should be considered in the first-line treatment of this malignancy.