Ductal eccrine carcinoma arising in the postaural area.

From the Head and Neck Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York City.

Reprint requests: Ashok R. Shaha, MD, Head and Neck Service, Department of Surgery, MSKCC, 1275 York Ave., New York, NY 10021. Phone: [phone]; fax: [phone].

Abstract

We describe the case of a 64-year-old man with a 1-year history of a postaural mass, which was diagnosed as ductal eccrine carcinoma. We also briefly describe the characteristics and treatment of this rare entity.

Introduction

Ductal eccrine carcinoma is a rare tumor that arises in the eccrine sweat glands. It usually occurs in the head and neck, although in the elderly population it often occurs in the extremities. The disease is characterized by an indolent growth that frequently recurs after surgical excision. There is a high incidence of both regional and distant spread. We present a case of ductal eccrine carcinoma of the postaural area.

Case report

A 64-year-old man had had a right postaural mass for the previous year, which was progressively increasing in size. On examination, the patient had a 3 cm by 3 cm red nodule in the postaural area, which was firm and nontender. At an outside institution, the patient underwent a biopsy, which revealed an extensive invasive tumor that deeply infiltrated the subcutaneous fat and skeletal muscle (figure 1). The tumor was made up of cells arranged in cords, nests, and poorly formed sheets, with occasional duct-like formations lined with 1 to 3 cells (figure 2). These duct-like structures contained pink eosinophilic material in their lumen. Neoplastic cells showed mild to moderate nuclear pleomorphism and some mitosis. This tumor was consistent with ductal eccrine carcinoma. The patient underwent a wide local excision and closure with local flaps at our hospital. The patient then received 6,200 cGy of postoperative radiotherapy and is currently free of disease 2 years after his initial diagnosis.

Discussion

Ductal eccrine carcinoma has been described previously under a variety of synonyms, such as carcinoma simplex and anaplastic syringoma. [1] It tends to occur as a nodular lesion in the head and neck area, but it also occurs in the extremities and less often on the trunk, perineum, and external genitalia. [2] About half of all cases involve ulceration. The color of these lesions varies; it may be flesh-toned, red, or blue. The tumor is usually seen in middle-aged and elderly patients.

Ductal eccrine carcinoma has a peculiar tendency toward a long evolution. Patients often report that a cutaneous mass had been present for several years and had grown gradually. Clinically, the differential diagnosis includes basal cell carcinoma, squamous cell carcinoma, Merkel's cell tumor, metastasis from a visceral malignancy, vascular neoplasm, epidermal inclusion cyst, and ganglion cyst. [3]

Approximately 50% of ductal eccrine carcinomas metastasize, usually to regional lymph nodes, but also to the lungs, liver, bones, and brain. Metastasis can manifest 3 to 5 years after the initial presentation, thus necessitating prolonged followup. Local recurrence is also common, seen in 70 to 80% of all cases. [4]

The recommended treatment of ductal eccrine carcinoma is complete surgical removal with wide margins. [5] Lymph node dissection is indicated only for patients who have clinically apparent lymphadenopathy. Radiotherapy is not effective in managing these patients, and data on the efficacy of chemotherapy are scanty. [6] However, postoperative radiation therapy is often administered when the tumor is large or the resection margins are close. The prognostic factors include the location and size of the tumor and the presence or absence of lymph node metastasis.

References

(1.) Berg JW, McDivitt RW. Pathology of sweat gland carcinomas. Pathol Annu 1968;3:123-44.

(2.) Cooper PH. Carcinomas of sweat glands. Pathol Annu 1987;22:83-124.

(3.) Wick MR, Goellner JR, Wolfe JT, Su WP. Adenexal carcinomas of the skin. I. Eccrine carcinomas. Cancer 1985;56:1147-62.

(4.) Smith CCK. Metastasizing carcinoma of the sweat glands. Br J Surg 1955;43:80-8.

(5.) Wertkin MG, Bauer JJ. Sweat gland carcinoma: Current concepts of surgical management. Arch Surg 1976;l11:884-5.

(6.) Coonley CJ, Schauer P, Kelsen DP, et al. Chemotherapy of metastatic sweat gland carcinoma: A retrospective review. Am J Clin Oncol 1985:8:307-11.