Chondroid syringoma with extensive ossification.

Chondroid syringoma is a rare appendagel skin tumor. Due to its uncharacteristic presentation, it is rarely diagnosed clinically. Here we present one such case in a 50-year-old lady. She presented with a hard mobile lesion over her nose which was excised. The histologic picture is characterized by a combination of epithelial and myoepithelial structures within a chondromyxoid and fibrous stroma. For these tumors, excision is the treatment of choice.

INTRODUCTION

Chondroid syringoma is a rare primary skin tumor with a reported incidence of <0.098%. It affects usually middle aged and older male patients. The site of predilection is the head and neck region where it typically presents as a slow-growing, painless, firm intracutaneous nodule.[1] Because of its noncharacteristic features, it usually goes undiagnosed. Here we report a case of chondroid syringoma and review the relevant literature. This case to the best of our knowledge is the first to be reported in the Indian subcontinent.

CASE REPORT

A 50-year-old lady presented with an 8-month history of a painless, slow-growing lesion over the tip of nose [Figure 1]. The lesion was hard, nontender, mobile, and measured 2 × 2 × 1 cm. There was no lymphadenopathy. By an elliptical incision, the tumor was excised completely and sent for histopathological examination [Figure 2]. Gross examination showed a well-circumscribed whitish nodular firm mass gritty to cut. A microscopic examination revealed a circumscribed biphasic tumor composed of lobules of epithelial nests lined by two layers of cells, and outer myoepithelial and inner epithelial with foci of squamous differentiation separated by a chondromyxoidstroma. The stroma shows loose chondromyxoid areas with bone formation [Figure 3]. There was no evidence of malignancy or atypia.

Figure 1

Preoperative clinical photograph

Figure 2

Postexcision photograph

Figure 3

Microscopy showing extensive ossification in myxoidstroma (H and E, 10×)

DISCUSSION

Histologically, these tumors contain a combination of epithelial and myoepithelial structures within a chondromyxoid and fibrous stroma. Sometimes these tumors may show differentiation toward skin adnexal structures (like hair follicle, hair matrix, and sebaceous and apocrine glands).[2] Ossification is a rare feature and when present it is focal and scant.[3] Extensive ossification in a benign mixed tumor as in our case at any anatomical site is exceedingly rare, and so far, only few such tumors have been reported in the skin.[3-5] Akasaka et al. suggested that ossification in the tumors indicates development from pluripotent stem cells. Apart from the rare occurrence of ossification in cutaneous mixed tumours of the skin, secondary (metaplastic) ossification may be seen in a variety of other skin lesions, including nevi, basal cell carcinomas, and pilomatricomas, and less commonly in trichoepitheliomas, hemangiomas, pyogenic granulomas, schwannomas, lipomas, organoidnevi, epidermal and dermoid cysts, dermatofibromas, desmoplastic melanomas, and some cutaneous metastasis.[5] The first-line treatment is the total excision of the tumor while preserving the esthetic and functional structures as in the present case.

Chondroid syringoma (benign mixed tumor of the skin) is an uncommon skin adnexal tumor usually presenting as a slow-growing, painless nodule in the head and neck region.[6-8] The site of predilection for the tumor is the head and neck region though cases in other areas have been reported. Though various treatment options have been proposed, in view of the chances of malignancy, excision seems the treatment of choice. The chances of malignancy increase with the size of the lesion. Adjuvant chemotherapy and radiotherapy have also been tried.[9] In the evaluation of a cutaneous nose nodule, chondroid syringoma should be considered in the preoperative differential diagnosis which may allow the surgeon to plan for complete excision.