| Abstract|| |
Chondroid syringoma (CS) is a rare benign tumor of the sweat gland. It generally presents as a painless, slowly growing, subcutaneous swelling, typically located on the head and neck region. The majority of the cases develop in middle aged and elderly males. We present a case of CS located over the right thumb of a 25-year-old male. The diagnosis was made initially by cytological evaluation and later confirmed by histopathological examination. There was no recurrence on two year follow up after the complete surgical excision of the tumor.
Keywords: Cytology, extremity, histology, syringoma
|How to cite this article:|
Barman DC, Bhowmik A. An unusual presentation of chondroid syringoma. Indian J Pathol Microbiol 2016;59:362-4
| Introduction|| |
Chondroid syringoma (CS) is a relatively uncommon benign, skin appendageal tumor. It is the cutaneous counterpart of mixed tumor of the salivary gland. Billorth, for the first time, reported this tumor in 1859. These tumors had both epithelial and mesenchymal origin as described by Virchow and Minssen. It commonly occurs in the head and neck region as a solitary, painless, subcutaneous, slowly growing nodule in middle-aged and elderly males., Here, we report a case of benign CS located on the dorsum of right thumb which is a very uncommon site of presentation [Figure 1].
| Case Report|| |
A 25-year-old man presented with a firm nodule at the base of the right thumb which gradually developed over a period of 2 years. He had no complaint of pain. On examination, a firm, painless, subcutaneous nodular swelling of 3.5 cm diameter was noted over the dorsal aspect of right thumb. Fine-needle aspiration cytology was performed from the swelling. It was later excised, and histopathological examination was done. Cytological examination revealed loose clusters and sheets of ovoid and spindle cells embedded in a chondromyxoid ground substance. The cells have oval nuclei with bland finely granular chromatin and moderate to the abundant well-defined cytoplasm. The stromal material stained purple in Leishman-Giemsa and pale pink in hematoxylin and eosin (H and E) stain [Figure 2] and [Figure 3].
|Figure 2: Loose clusters of epithelial and myoepithelial cells embedded in a chondromyxoid ground substance (Leishman-Giemsa, ×40)|
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|Figure 3: Loose clusters of epithelial and myoepithelial cells embedded in a chondromyxoid ground substance (H and E, ×40)|
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The histopathological sections revealed a well-circumscribed tumor mass in the dermis and subcutaneous tissue. Tumor showed epithelial and myoepithelial component in nests, islands, ducts, and tubular structures blending into a chondroid, myxoid, and fibrous stroma. The epithelial cells were round, oval, and cuboid with regular nuclei having fine granular chromatin. Few tubules were large, complex, and multilayered. Oncocytic and squamous metaplasia were noted in a few places. Mitotic activity was low [Figure 4].
|Figure 4: Epithelial and myoepithelial cells in nests, islands, ducts, and tubular structures blending into a chondroid, myxoid, and fibrous stroma (H and E, ×10)|
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The patient was doing well following complete surgical excision of the tumor, and no recurrence was noted on 2 years follow-up.
| Discussion|| |
CS, also known as a mixed tumor of the skin, belongs to the group of the benign adnexal tumor., The term “chondroid syringoma” was coined by Hirsch and Helwig. Its etiopathogenesis is unknown, however, some authors suggest the hypothesis of both epithelial and mesenchymal origin. Apocrine and an eccrine variant of this neoplasm were described by various workers. The reported incidence of CS among primary skin tumors is <0.01%., A greater incidence of 0.098 percent was reported by other workers. The middle-aged and elderly males are commonly affected. In our case, it was a 25-year-old male presenting with the tumor. Clinically, CS presents usually in the head and neck region. Less commonly, this tumor can develop on the chest, abdomen, eyelid, orbit, axillary region, penis, vulva, scrotum, and distal extremities., Lesions are painless, slow growing, nonulcerated subcutaneous nodules. Rare cases with a more aggressive and rapid growth have been reported by Sungur et al. The lesion commonly ranges from 0.5 to 3 cm in diameter.,, However, larger forms of CS have been described. In our case, the lesion was 3.5 cm in diameter. Various benign lesions such as lipoma, neurofibroma, and dermoid cysts, pilomatricoma clinically mimicks this lesion., Cytological smears show loose cohesive clusters, sheets of epithelial, and myoepithelial cells embedded in a chondromyxoid stroma. The cells can be round, oval, spindle, or plasmacytoid having round nuclei with bland finely granular chromatin and moderate amount of well-defined cytoplasm. Histologically the tumor represents an admixture of epithelial and mesenchymal components. The epithelial component is arranged in nests, islands, ducts and tubular structures., They may show sebaceous or squamous differentiation in rare instances. The myoepithelial cells in the chondromyxoid stroma may be cuboidal, stellate, or spindle-shaped. The stroma may contain osteoid or adipose tissue. Epithelial cells are a carcinoembryonic antigen and cytokeratin positive; The outer layer of ductal cells (myoepithelial cells) are Vimentin and S100 positive. Although most are benign, rare cases of malignant CS have been reported in young females where distal extremities are the sites of predilection., The lesion may be locally invasive or metastasize to the regional and distant lymph nodes. Immunohistochemistry is of no help in distinguishing between benign and malignant lesions. Because of malignant potential, complete excision of CS with a wide margin along with careful follow is mandatory.
We report a case of CS due to its atypical clinical presentation. Only a few cases of this tumor have been documented till date where the tumor developed in such unusual location. Fine-needle aspiration can provide a valuable diagnostic clue as seen in our case while the histopathological evaluation remains the gold standard for diagnosis. The tumor should be considered in the differential diagnosis of other benign subcutaneous tumors. Prompt excision and close follow-up are essential to avoid the risk of recurrence or malignant transformation.
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Dr. Abhijit Bhowmik
C/O, Sri Bindeswari Prasad Singh, Road - 12, Rajendranagar, Patna, Bihar
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]