Dermatology Online Journal

Chondroid syringoma: Report of two cases in young patients
Lakhan Singh Solanki MD¹, Mandeep Dhingra¹, Mala Bhalla MD¹, Gurvinder Pal Thami MD¹, RPS Punia MD², Shilpy Batra MD²
Dermatology Online Journal 17 (12): 7

1. Department of Dermatology
2. Department of Pathology
Government Medical College and Hospital, Chandigarh, India

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Abstract

Chondroid syringoma (CS) is a relatively rare cutaneous tumor arising from sweat glands. It usually presents in the head and neck area as an asymptomatic nodule. Although usually it presents in middle aged or older patients, we here present two much younger patients with CS, located over the nose and cheek areas, respectively.

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Introduction

Chondroid syringoma (CS), an uncommon skin appendageal tumor, is also referred to as cutaneous mixed tumor because it consists of both epithelial and myoepithelial cell types. The incidence of CS among primary skin tumors is low and it is usually seen in middle aged or older male patients as a small, single dermal or subcutaneous nodule in the head and neck area. They are rarely multiple or large in size and malignant transformation is very uncommon. No characteristic clinical features are seen, so diagnosis is by histopathological examination. Treatment of benign CS is surgical excision or destruction. There is a rare risk of malignancy, so regular follow up is recommended [1, 2]. We herein present two cases of CS in young individuals located over the nose and cheek areas respectively.

Case report

Figure 1	Figure 2
Figure 1. Single well defined nodule, with slight violaceous discoloration in its center Figure 2. Photomicrograph showing epithelial components in the form of ducts and lobules; background showed homogeneous basophilic abundant fibromyxoid stroma (H&E, x100).

Case 1. A 16-year-old male presented with an 8 month history of a single, asymptomatic, raised lesion on the nose. It initially started as small lesion beneath the skin, which gradually increased in diameter and depth. On examination, he had a single well defined nodule of 1.5 cm x 1.5 cm with slight violaceous discoloration in its center. It was soft to firm in consistency, non-tender, and fixed to overlying skin, but mobile over deeper structures (Figure 1). Cervical lymphadenopathy was absent. An excisional biopsy was done and routine hematoxylin-eosin staining revealed a well-circumscribed tumor composed of epithelial and stromal components. Epithelial components were in the form of ducts and lobules lined by a luminal layer of cuboidal cells and a peripheral layer of flattened cells; the background showed abundant fibromyxoid stroma. Cellular pleomorphism or dysplasia was not apparent (Figures 2 and 3). These histopathological findings are diagnostic of CS. At the 1 year follow up there was no recurrence.

Figure 3	Figure 4
Figure 3. Proliferation in small aggregates and duct structures lined by a luminal layer of cuboidal cells and a peripheral layer of flattened cells located in a basophilic fibromyxoid stroma containing myoepithelial cells (H&E, x200) Figure 4. Single well-defined nodule with atrophy and scarring on its surface

Case 2. A 21-year-old female presented with a single, asymptomatic, slowly growing, raised lesion on the left cheek of three years duration. There was a history of application of some irritant over the lesion a few weeks prior to presentation. On examination, she had a single well-defined nodule of 4 cm x 3 cm, firm to hard in consistency, non-tender, that was fixed to the overlying skin, but mobile over deeper structures. The overlying skin was atrophic and appeared scarred (Figure 4). Cervical lymphadenopathy was absent. The histopathological findings of the biopsy specimen were similar to that of the first case except for the presence of an abundant basophilic myxoid stroma (Figures 5 and 6). The tumor was completely excised and there was no recurrence at a 2-year follow up.

Figure 5	Figure 6
Figure 5. Photomicrograph showing tubular lumina with marked variation in size and shape; background showed abundant myxoid stroma (H&E, x100). Figure 6. Tubular lumina are lined by two layers of epithelial cells: luminal layer of cuboidal cells and peripheral layer of flattened cells, and abundant basophilic myxoid stroma (H&E, x400).

Discussion

Chondroid syringoma (CS) are mixed tumors of the skin. The term, chondroid syringoma, was introduced by Hirsch and Helwig to describe these tumors because of the presence of sweat gland elements set in a cartilaginous stroma [1]. The term, mixed tumors, term was coined by Virchow and Minssen for neoplasms with microscopic features that indicate both epithelial and mesenchymal origin [2].

Chondroid syringoma (CS) or mixed tumor is a relatively rare, benign, skin appendageal tumor. This term has been used to describe cutaneous tumors as well as tumors unrelated to skin, i.e., of salivary glands and of kidney [3]. The incidence of CS among primary skin tumors is low and has been reported at 0.01 percent to 0.098 percent [2].

Chondroid syringoma usually presents as a single, asymptomatic, slow-growing, firm, circumscribed, non-ulcerated, lobulated nodule within the deep dermis or subcutaneous fat in middle aged or older male patients, but both of our cases were of a younger age group. Its usual size is 0.5 cm to 3 cm [3, 4, 5], although cases of large and even multiple tumors have been reported [6-10]. The common sites for CS are on the head and neck area, particularly cheek, nose, or skin above the lip, though it may present anywhere over the body [1, 2].

Chondroid syringoma is usually benign but a few cases of malignant transformation have been reported [1, 6, 11]. These malignant types occur more commonly in younger females. They are often larger than 3 cm, are locally invasive, and show a predilection for trunk or extremities [1, 11, 12, 13].

For the diagnosis of CS, Hirsch and Helwig [1] described five histological criteria: (1) nests of cuboidal or polygonal cells, (2) interconnected tubuloalveolar structures lined by 2 or more rows of cuboidal epithelial cells, (3) ductal structures lined by 1 or 2 rows of cuboidal cells, (4) occasional keratinous cysts, and (5) a matrix of mixed chondroid and myxoid material. Some CSs display all 5 characteristics, whereas others only manifest a few. In our case four of these features are seen. Headington [14] divided chondroid syringomas into apocrine and eccrine variants based on histological differences in the luminal morphology, but there remains debate as to their exact origin [15, 16]. Histological features considered as signs of malignant transformation are cytologic atypia, infiltrative margins, satellite tumor nodules, tumor necrosis, and involvement of deep structures [17], not seen in our cases.

Immunohistochemisrty shows that tubuloalveolar components of chondroid syringomas express cytokeratin (CK), epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), vimentin, S-100 protein, neuron-specific enolase (NSE), and in a few cases, glial fibrillary acidic protein (GFAP) [18, 19].

Because there is no particular clinical appearance, the diagnosis of CS is primarily histopathological [2]. Fine needle aspiration cytology (FNAC) may be used to diagnose the pathology before excision [20]. Optimal treatment of benign CS is surgical excision without affecting aesthetic and functional structures. Other treatment options are dermabrasion, electrodessication, and vaporization with argon or CO2 laser [2]. Because of the risk of malignancy, the patient should be followed up to look for recurrence or any feature of malignancy [21].

Conclusion

These cases have been reported to emphasize that chondroid syringoma may be uncommon but they are not as rare as considered previously. Our patients are of interest because of the younger age of presentation. CS patients should be followed up to look for recurrence or any features of malignancy.

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