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

Ugo Marone,Corrado Caracò,Anna Maria Anniciello,Maria Luisa Di Cecilia,Maria Grazia Chiofalo,Nicola Mozzillo,Gianluca Di Monta

doi:10.1186/1477-7819-9-32

Abstract

Eccrine porocarcinoma (EPC) is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, representing 0.005-0.01% of all cutaneous tumors. About 20% of EPC will recur and about 20% will metastasize to regional lymph nodes. There is a mortality rate of 67% in patients with lymph node metastases. Although rare, the occurrence of distant metastases has been reported.We report a case of patient with EPC of the left arm, with axillary nodal involvement and subsequent local relapse, treated by complete lymph node dissection and electrochemotherapy (ECT).EPC is an unusual tumor to diagnose. Neither chemotherapy nor radiation therapy has been proven to be of clinical benefit in treating metastatic disease. Although in the current case the short follow-up period is a limitation, we consider in the management of EPC a therapeutic approach involving surgery and ECT, because of its aggressive potential for loregional metastatic spread.

Highlights

Eccrine porocarcinoma (EPC) is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, being a primary tumor or, even more common, a malignant transformation of an eccrine poroma (EP), representing 0.005-0.01% of all cutaneous tumors [1]
About 20% of EPC will recur and about 20% will metastasize to regional lymph nodes [9]
We report a case of patient with EPC of the left arm with axillary nodal involvement and subsequent local relapse

Summary

Background

Eccrine porocarcinoma (EPC) is a rare type of skin cancer arising from the intraepidermal portion of eccrine sweat glands or acrosyringium, being a primary tumor or, even more common, a malignant transformation of an eccrine poroma (EP), representing 0.005-0.01% of all cutaneous tumors [1]. Case presentation In February 2010 a 42-year old man presented with palpable left axillary lymphadenopathy Ten months before this time point, he had been admitted to another institution for excision biopsy of an erythematous plaque less than 2 cm in size on the left arm with histological diagnosis of EPC. Preoperative staging included imaging with ultrasounds (US), revealing evidence of several involved nodes in the left axilla, the largest measuring 4.1 × 2.5 cm in diameter (Figure 3), whole body positron emission tomography (PET/CT), which showed uptake of the radiotracer in the left axilla (SUV 10) without evidence of other metastatic disease, and fine needle aspiration cytology (FNAC), which confirmed replacement by EPC. After a follow-up of five months, complete response of the local recurrence was observed on a clinically macroscopic basis (Figure 4-B), without any complications, well tolerated by the patient, which presented at this time no signs of axillary relapse or systemic disease

Findings

Discussion

Conclusions