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Chondroid syringoma of the scalp: case report and discussion of clinical features, histopathology, and treatment.

Abstract

Chondroid syringoma is a generally benign, adnexal tumor most commonly found in the head and neck. It is typically diagnosed retrospectively from histopathology, and careful evaluation must differentiate it from tumors of salivary gland origin. It presents as a slowly growing, subcutaneous or intradermal nodule. Treatment consists of wide local excision with a margin of normal tissue. We report a case involving a 51-year-old man with a chondroid syringoma arising from the scalp. Clinical features, histopathology, and treatment are discussed.

Introduction

Benign mixed tumors are neoplasms that exhibit features of both epithelial and mesenchymal origin. When these tumors originate in the salivary glands, they are referred to as pleomorphic adenomas; when they originate in the sweat glands, they are referred to as chondroid syringomas. Chondroid syringomas may be preceded by trauma and rarely prove to be malignant. Complete excision is the treatment of choice.

Case report

A 51-year-old man presented with an asymptomatic, right parietal subcutaneous nodule that had been slowly growing for 10 years. He had no history of cutaneous malignancy or trauma in the area. The mass had begun to bother him when he combed his hair, and he desired excision. Physical examination revealed a firm, painless, mobile, 1 x 1-cm nodule covered by skin and located in the right parietal area without overlying skin changes. The subcutaneous nodule was excised.

Histopathologic examination revealed a well-circumscribed lesion located in the dermis and extending into the subcutis (figure). It was composed of numerous small, nonbranching tubules set in a myxoid and cartilaginous stoma (figure inset). Based on this pathology, the definitive diagnosis was chondroid syringoma.

Discussion

Chondroid syringoma is a rare, generally benign skin tumor arising from sweat glands and often located in the head and neck region. Billroth identified these lesions as benign mixed tumors of the skin in 1859. (1) Hirsch and Helwig subsequently coined the term chondroid syringoma in 1961, thus providing tumors of sweat gland origin with their own unique identity. (1,2) Hirsch and Helwig proposed that chondroid syringomas originate from apocrine or eccrine sweat glands and are histopathologically distinct from pleomorphic adenomas, which originate from salivary glands. (3)

Because of the morphologic and histopathologic similarities between chondroid syringomas and pleomorphic adenomas, they previously have been considered together. However, these two tumors should not be mistaken for each other and must be differentiated based on their site of origin and their location. Another source of confusion involves nomenclature, since in the past chondroid syringoma also was called pleomorphic adenoma of the skin.

The clinical presentation of chondroid syringomas may vary; however, patients generally present with a slowly growing, asymptomatic, 0.5- to 3-cm mass in the head and neck region involving the nose, cheek, lip, scalp, forehead, or chin. (4,5) These tumors also have been reported in other parts of the body, including the chest, abdomen, and extremities. Rarely, they are located on the scrotum, eyelid, or orbit. (6)

On physical examination, chondroid syringomas are nontender, smooth, firm, and either subcutaneous or intradermal. Their pigmentation may appear identical to that of the surrounding skin, or they may exhibit a purplish or reddish hue. These tumors have a predilection for middle-aged men and a male-to-female ratio of 2:1. (6) These masses represent 0.01% to 0.098% of all primary skin tumors. (4,6)

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The diagnosis of chondroid syringoma is typically ascertained after histopathologic evaluation. This tumor should be considered in the differential diagnosis of head and neck lesions, which includes sebaceous cyst, implantation dermoid, neurofibroma, dermatofibroma, basal cell carcinoma, squamous cell carcinoma, pilomatrixoma, and histiocytoma. The standard for both diagnosis and treatment involves complete excision of the skin lesion and histopathologic examination. During excision, it is important to obtain a specimen with margins free of tumor to prevent its recurrence. (l,4,6,7)

As mentioned earlier, chondroid syringomas may originate either from apocrine or eccrine sweat glands, or they may evolve from entrapped ectopic cell rests of embrylogic origin stimulated to proliferate by an unknown factor. (3,4,7) Two histopathologic subtypes have been described: Chondroid syringomas of apocrine origin exhibit ducts, with lumina lined by two layers of epithelial cells; those of eccrine origin characteristically have ducts lined by a single layer of flattened, cuboidal epithelial cells.

Histologic findings are variable and may be related to the location of the chondroid syringoma. The tumors that arise from the head and neck region characteristically exhibit nests of cells with a lace-like stranding pattern of epithelial cells and tubuloalveolar structures in a loose myxochondroid stroma, with a differentiation pattern of sweat glands and ducts. The nests of eosinophilic staining cells vary in shape from cuboidal to polygonal, and they form ducts and trabecular cords. Additionally, keratinous deposits and cysts may be present within these tumors. (6,7)

Histologic staining indicates these tumors to be positive for periodic acid-Schiff, mucicarmine, and Alcian blue. Immunohistochemical studies indicate that the epithelial membrane antigen (EMA) marker may be present; that the epithelial cells may stain positive for keratin markers, including CAM5.2; and that the myoepithelial cells may stain positive for S-100. (6,8)

Most of the reported cases of chondroid syringoma are benign; however, a malignant variation of this tumor has been identified. These lesions are larger, generally extend from the trunk and extremities, and have a female predilection. (8) Histologically, they appear more aggressive, with nuclear pleomorphism, cytologic atypia, increased mitotic activity, focal necrosis, and lymphatic/vascular invasion. Treatment involves wide local excision with adjuvant external beam radiation therapy. (2) No cases of malignant degeneration or transformation of benign tumors have been reported.

In conclusion, chondroid syringoma is an uncommon disease entity that mainly arises in the head and neck region. While it is rarely considered in the differential diagnosis of a head and neck mass, careful histopathologic examination will delineate it from other tumors, including those of salivary gland origin, for which it is commonly mistaken. Diagnosis and treatment involve excision of this mass with a clear margin of normal tissue. Recurrence of these tumors most likely results from either inadequate excision or the existence of satellite tumor islands. (1,4,5)

References

(1.) Ogawa R, Mitsuhashi K, Oki K, Hyakusoku H. A rare case of chondroid syringoma arising from the lower eyelid with ectropion. Plast Reconstr Surg 2006;118(6):137-40e.

(2.) Mathiasen RA, Rasgon BM, Rumore G. Malignant chondroid syringoma of the face: A first reported case. Otolaryngol Head Neck Surg 2005;133(2):305-7.

(3.) Satter EK, Graham BS. Chondroid syringoma. Cuffs 2003;71 (1):4952, 55.

(4.) Schulhof Z, Anastassov GE, Lumerman H, Mashadian D. Giant benign chondroid syringoma of the cheek: Case report and review of the literature. J Oral Maxillofac Surg 2007;65(9):1836-9.

(5.) Chao PZ, Lee FP. Pleomorphic adenoma (chondroid syringoma) on the face. Otolaryngol Head Neck Surg 2004;130(4):499-500.

(6.) Kaushik V, Bhalla RK, Nicholson C, de Carpentier JP. The chondroid syringoma: Report of a case arising from the external auditory canal. Eur Arch Otorhinolaryngol 2005;262(10):868-70.

(7.) Chang CW, Vincek V. Pathology quiz case 1. Chondroid syringoma. Arch Otolaryngol Head Neck Surg 2005;131 (7):642, 644.

(8.) Chen AH, Moreano EH. Chondroid syringoma of the head and neck: Clinical management and literature review. Ear Nose Throat J 1996;75(2):104-8.

From the Department of Otolaryngology-Head and Neck Surgery, University of Missouri-Columbia School of Medicine, Columbia, Mo.

Corresponding Author: Young S. Paik, MD, Department of Otolaryngology-Head and Neck Surgery, University of Missouri-Columbia School of Medicine, One Hospital Dr., Columbia, MO 65212. E-mail: paiky@health.missouri.edu
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Title Annotation:ORIGINAL ARTICLE
Author:Paik, Young S.; Liess, Benjamin D.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Geographic Code:1USA
Date:Apr 1, 2011
Words:1262
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