Chondroid Syringoma: An Atypical Presentation

Dear Editor, Chondroid Syringoma (CS) is a rare, benign cutaneous appendageal tumor with a reported incidence of less than 0.01%.[1] CS appears as a slow-growing, painless subcutaneous/intracutaneous nodule not exceeding 3 cm in diameter, commonly involving the head and neck region, with a male preponderance.[2,3] Our patient was a 50-year-old lady who presented with a growth over the dorsa of the right hand, involving the region of the thumb and index finger, for the past 2 years. It had started as a slow-growing, painless, mobile, and smooth-surfaced pea-sized nodule that underwent rapid growth over the past 4 months to attain its current status. No history of trauma and constitutional symptoms were forthcoming. Examination revealed a firm, solid, and partially lobulated tumor with overlying crusting, suppuration, hemorrhage, and necrosis of size 7 × 5 × 4 cm [Figure 1a]. There was no regional lymphadenopathy or organomegaly. X-ray of the right hand did not reveal bone involvement.Figure 1: (a) Lobulated nodular growth over the dorsa of the right hand overlying the region of the thumb and index finger of size 7 × 5 × 4 cm with superimposed crusting, hemorrhage, necrosis, and suppuration. (b) Photomicrograph showing basaloid tumor cells arranged in nests with foci of necrosis (H and E × 100). (c) Photomicrograph of tumor cells interspersed within a chondromyxoid matrix. Some of the tumor cells delineate a plasmacytoid appearance (H and E × 200). (d) Photomicrograph showing tumor cells arranged in a glandular pattern intermingled within a chondromyxoid stroma (H and E × 200). (e) CK 7 positivity highlighting the tumor cells (IHC × 200). (f) Epithelial Membrane Antigen positivity in the tumor cells (IHC × 200). (g) S 100 highlighting the myoepithelial cells that surround the cuboidal tumor cells (IHC × 200)Based on the above findings, differentials of malignant tumor, atypical mycobacterial infection, and subcutaneous mycoses were considered. A deep cutaneous biopsy performed from the lesion revealed focal thinning of the epidermis with multilobulated islands and nests of epithelial and myoepithelial cells focally forming a network of glands and ducts interspersed in a chondromyxoid stroma. Tumor cells delineated a cuboidal morphology with scant cytoplasm and vesicular nuclei. No evidence of malignant change was noticed [Figure 1b-d]. A microscopic diagnosis of benign CS was concluded. Positive immunohistochemistry with cytokeratin (CK) 7, epithelial membrane antigen (EMA), and S100 protein further substantiated the diagnosis [Figure 1e-g]. The patient was subsequently lost to follow-up. Giant presentations of benign CS are extremely rare making it a diagnostic challenge, with histopathological evaluation becoming compulsory for confirmation. Further, as only a handful of reports of the giant phenotype of CS exist [Table 1],[4-7] it is very unlikely to consider this entity in the list of differentials for the same. Besides, our case differed considerably from all previous reports.Table 1: Comparison of past reports of benign giant chondroid syringoma with our patientWe report this case owing to: The tendency of giant benign CS to portray a malignant phenotype, The close clinical approximation of subcutaneous mycoses and atypical mycobacterial infection in our patient, and The necessity of a thorough evaluation in all such cases to avoid any diagnostic lapse. Financial support and sponsorship Nil. Conflict of interest There are no conflicts of interest.