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CHONDROID SYRINGOMA A CASE REPORT.

Byline: RAHAT KALSOOM MAHERA ROOHI AND FAROOQ AZIZ

ABSTRACT

Chondroid syringoma or mixed tumour of the skin is an uncommon benign sweat gland tumour. It is the cutaneous counterpart of pleomorphic adenoma of the salivary glands. The clinical presentation of conodroid syringoma is non-specific and its histological diagnosis can prove difficult. We report a case of chondroid syringoma arising from a swelling on face in a 40 year old male. The diagnosis was made on histopathological examination. Surgical tumour excision remains the best therapeutic option to avoid relapse of this tumour. Close follow-up is recommended because malignant transformation although rare is possible.

INTRODUCTION

Chondroid syringoma mixed tumour of skin most often occurs as solitary slowly growing nodule. Chondroid syringomas are benign nodular non-ulcerated tumours that occur predominantly on the face head and neck but also on the extremities and trunk. Appearance on other parts of the body is unusual. It usually occurs in middle aged and elderly patients.1 It was described by Billorth in 1859 for a group of tumours of the salivary gland that contained varying amounts of mucoid and cartilaginous material.2 Virchow and Minssen at referred to them as mixed tumors with both epithelial and mesenchymal origins.3 We present a rare case of chondroid syringoma on the face of a young male.

CASE REPORT

A 40 year old male presented with a swelling on face over the previous 8 months. There was no history of pain discharge or previous surgery. Gross examination of the excisional biopsy revealed a 1.5 A- 1 cm firm nodular grey white soft tissue mass covered with skin and surrounded with capsule like tissue. Histological examination reveals abundant bluish chondroid stroma intermingled with a fibro-adipose tissue containing epithelial structures arranged in small aggregates and ducts. The epithelial cells are cuboidal with an eosinophilic cytoplasm and regular oval nuclei (Fig. 1). There were numerous nests of polygonal cells and interconnecting tubuloalveolar structures lined by two layers of cuboidal epithelial cells (Fig. 2). The constellation of findings leads to a diagnosis of chondroid syringoma.

DISCUSSION

Chondroid syringoma also known as mixed tumour of the skin benign adnexal tumour with an unknownaetiopathogenesis.3 Some authors suggest the hypothesis of both epithelial and mesenchymal origin.4 CS is also thought to originate from both secretory and ductal segments of the sweat gland and both eccrine and apocrine variants have been described.5Hirsch and Helwig gave them the appellation chondroid syringoma because of the presence of sweat gland elements set in a cartilaginous stroma. They proposed the following five histological criteria for diagnosis: 1) nests of cuboidal or polygonal cells; 2) intercommunicating tubuloalveolar structures lined with two or more rows of cuboidal cells; 3) ductal structures composed of one or two rows of cuboidal cells; 4) occasional keratinous cysts; 5) a matrix of varying composition. Chondroid syringomas may have all five characteristics or manifest only some.6 The reported incidence of CS among primary skin tumours is less than0.01 percent.7 Chondroid syringoma usually affects middle aged or older male patients.3 In our case it occurred in a 41 year old male.Clinically CS presents typically as a slow growing painless firm non-ulcerated subcutaneous or intracutaneous nodule. The lesion commonly measures 0.5 3 cm in diameter.8 However larger forms of CS have been described.9-11 The sites of predilection for CS are on the head and neck region particularly cheek nose or skin above the lip.8 Less commonly this tumor can develop on the scalp eyelid orbit hand foot fore-head axillary region abdomen penis vulva and scrotum.1213 Chondroid syringoma is often overlooked because of rarity of this tumor and unremarkable clinical presentation.3The differential diagnosis of CS is made with other benign tumors of epidermal or mesenchymatous appendages such as dermoid or sebaceous cyst neurofibroma dermatofibroma basal cell carcinoma pilomatricoma histiocytoma and seborrheic keratosis.12 CS lesions usually are not clinically distinctive and the diagnosis is made on microscopic examination.14Histologically CS consists of mixed epithelial and mesenchymal elements with epithelial cells arranged in cords and forming tubules with a myoepithelial layer set in a myxoid or chondroid stroma.15 Immuno-0histochemical study shows focal positivity for keratin vimentin desmin and S100 protein in the stroma.4 In our patient histological examination revealed an abundant chondroid stroma intermingled with some epithelial structures arranged in small aggregates and ducts (Fig. 1).Optimal treatment of benign chondroid syringomas is surgical excision. Fine needle aspiration cyto-logy has been used for diagnostic purposes and may prove useful to determine pathology before excision; however examination of excised tissue is most reliable in establishing a definitive diagnosis.16 Because of the lobulated nature of the tumor it is important to include a margin of normal tissue with the excision to ensure complete removal of the tumor.

REFERENCES

1. Kumar S. Jain P. Sen R. Kataria S.P. Gupta M. Wadhera R. Hyaline matrix rich chondroid syringoma: a rare lesion of the external auditory canal. Rev Esp Patol.2011; 44 (4): 209-212.2. Sungur N Uysal A Gumus M Kocer U. An unusual chondroid syringoma. Dermatol Surg 2003; 29: 977-9.3. Yuvuzer R Basterzi Y Sari A Bir F Sezer C. Chondroid syringoma: a diagnosis more frequent than expected. Dermatol Surg 2003; 29: 179-81.4. Borman H Ozcan G. Chondroid syringoma at the fingertip: an unusual localization. Eur J Plast Surg 1998; 21;311-3.5. Bates AW Baithun SI. Atypical Mixed Tumor of the Skin: histologic immunohistochemical and ultrastructural features in three cases and a review of the criteria for malignancy. Am J of Dermatopathol 1998; 20: 35-40.6. Hirsch P Helwig EB. Chondroid syringoma. Mixed tumor of skin salivary gland type. Arch Dermatol 1961;84: 835-47.7. Watson JA Walker WM Smith NP Hunt DM. Malignant chondroid syringoma a rare cause of secondary bone tumour. Clin Exp Dermatol 1991; 16: 306-7.8. Bekerecioglu M Tercan M Karakok M Atik B. Benign chondroid syringoma: a confusing clinical diagnosis. Eur J Plast Surg 2002; 25: 316-8.9. Hardisson D Linares MD Nistal M. Giant chondroid syringoma of the axilla. J Cutan Med Surg 1998; 3: 115-7.10. Kakuta M Tsuboi R Yamazaki M et al. Giant mixed tumor of the face. J Dermatol 1996; 23: 369-71.11. Sliwa Hahnle K Obers V Lakhoo M Saadia R. Chond-roid syringoma of the abdominal wall. A case report and review of the literature. S Afr J Surg 1996; 34: 46-8.12. Poku JW Sant GR Ucci AA. Chondroid syringoma ofthe scrotum. J Int Med Res 1996; 24: 482-6.13. Nemoto K Kato N Arino H. Chondroid syringoma of the hand. Scand J Plast Reconst Surg Hand Surg. 2002;36: 379-81.14. Miracco C De Santi MM Lalinga A. Lipomatous mixed tumour of the skin: a histological immunohistochemical and ultrastructural study. Br J of Dermatol 2002; 146:899-903.15. Chen AH Moreano EH Houston B Funk GF. Chondroid syringoma of the head and neck: clinical management and literature review. Ear Nose Throat J 1996; 75 (2): 104-8.
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Publication:Biomedica
Date:Mar 31, 2014
Words:1165
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