Unusual tumor in a rare location – Chondroid syringoma of orbit

A 75-year-old male presented with 2-year history of painless, progressive swelling in the left upper eyelid. Examination revealed a firm, mobile 2 × 1 cm mass in the superonasal orbit with moderate ptosis. Fig. 1a) Fullness in medial aspect of the left upper eyelid in comparison to the other upper eyelid hollow and narrowing of vertical palpebral fissure of the left eye. Fig. 1b) Sagittal CT scan of left orbit revealed an extraconal intraorbital homogeneous mass just superior to insertion of superior rectus and isointense to soft tissue. Fig. 1c) Axial CT scan indicated mass in the extraconal compartment of orbit. Surgical excision via anterior orbitotomy was performed, Fig. 1d) (40x) and Fig. 1e) (100x) Histopathological examination (100x) demonstrated well-circumscribed multilobulated dermis-based mass with prominent chondro-myxoid stroma enveloping benign bland appearing epithelial component arranged in glands, ducts, cysts, reticular lace-like networks, and nests surrounded by the outer myoepithelial layer, confirming the diagnosis of being chondroid syringoma. The patient is under biannual clinical follow-up.Figure 1: (a) Fullness in medial aspect of the left upper eyelid in comparison to the other upper eyelid hollow and narrowing of vertical palpebral fissure of the left eye. (b) Sagittal CT scan of left orbit revealed an extraconal intraorbital homogeneous mass just superior to insertion of superior rectus and isointense to soft tissue. (c) Axial CT scan indicated mass in the extraconal compartment of orbit. Surgical excision via anterior orbitotomy was performed, (d) (40x) and (e) (100x) Histopathological examination.Chondroid syringoma (CS) is rare, benign mixed-type skin appendageal tumor, with an incidence of 0.01%–0.098%.1 The term reflects its dual origin: “syringoma” from ectodermal sweat glands and “chondroid” from mesodermal cartilage-like stroma. It commonly occurs in males aged 20–60, often on the head and neck, rarely in the orbit as painless, slow-growing, firm subcutaneous lump.2,3 Histologically, it is divided into eccrine and apocrine variants.4 Diagnosis relies on histopathology as imaging is nonspecific and fine-needle aspiration may be insufficient.1 Immunohistochemistry assists in identifying markers like EMA, S-100, and vimentin.1 Differential diagnoses include pleomorphic adenoma, neurofibroma, and cavernous hemangioma. Surgical excision with clear margin is the treatment of choice. Only 51 malignant cases have been documented that tend to affect younger females and appear on extremities or torso.5 Malignant CS is rare, larger than 3 cm, may metastasize, showing features like necrosis and infiltrative margins, and may require radiotherapy.1,5 Authors' contributions Nikunj Gupta: Contribution-Preparation of the manuscript. Correspondence with the pathology lab for the histology images. Literature search and finding the appropriate references. Correspondence with the journal. Rwituja Thomas Grover: Contribution- Reviewing and editing the manuscript. Performing the diagnostic procedure in Operation Theatre. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.