Atypical Facial Chondroid Syringoma, A Rare Clinical Entity: Case Report with Review of Literature

Abstract

Chondroid syringoma (CS) is a rare mixed tumor of sweat gland origin with a reported incidence of less than 0.1%. The tumor is mostly benign and usually seen in males. It comprises of both epithelial and mesenchymal elements. The usual presentation and clinical course of this condition makes it liable to be confused with other commoner skin conditions. We hereby present a case of facial CS in a young female patient.

Introduction

The term “Chondroid syringoma” was first used by Hirsch and Helwig [1]. It is a rare tumor of sweat gland origin with reported incidence of less than 0.1% [2, 3]. It can be regarded as a dermal counterpart of pleomorphic adenoma of the salivary glands. The term “chondroid” is used because of the cartilage like component and the term “syringoma” denotes the presence of sweat gland elements. The usual presentation is that of a soft to firm, gradually progressive, subcutaneous swelling. The tumor can easily be confused with other commoner benign conditions like sebaceous cyst, neurofibromas, epidermal cyst, fibrolipoma etc. Though the lesion is usually benign, malignant chondroid syringomas have also been reported in literature.

Case Report

A 19 year old female patient from rural background presented to our OPD with a history of a gradually progressive swelling over her left cheek. The swelling was first noticed by the patient about 1.5 years back and it was pea-sized initially. It was painless throughout it’s course.

The general and systemic examination of the patient was normal. Local examination of the swelling revealed a multi-nodular mass of about 4 cm × 3 cm size with a subcutaneous swelling palpable on left cheek. The mass was firm, non-tender and free from buccal mucosa. It was largely free from skin except at a point where a small separate bulge was seen on the skin surface. The overlying skin was otherwise normal. There was a slight depression of the angle of mouth on the side of lesion due to mass effect of the swelling as seen in Fig. 1. There was no palpable cervical lymphadenopathy.

Fig. 1.

Clinical photograph of the patient showing swelling over left cheek with depression of left angle of mouth due to mass effect of the lesion

Fine needle aspiration cytology showed abundant cellularity with cells in clusters and dispersed singly. The cells showed abundant cytoplasm with background of chondromyxoid and stromal fragments suggestive of chondroid syringoma.

Contrast enhanced CT scan revealed an ill-defined, heterogeneously enhancing multilobulated lesion seen in subcutaneous plane of left cheek. There was no evidence of any bony erosion or intraoral extension as seen in Fig. 2 and Fig. 3.

Fig. 2.

Coronal section of contrast enhanced CT scan of the facial region showing heterogeneously enhancing, multilobulated lesion

Fig. 3.

Axial section of contrast enhanced CT scan showing ill-defined lesion in subcutaneous plane without bony erosion

Total excision of the mass was done which grossly comprised of a firm nodular mass with multiple yellowish-white nodules as seen in Fig. 4. Histopathology revealed epithelial cells forming solid sheets, nests, trabeculae and glandular structures. Many of the cells had features of hyaline cells and were plasmacytoid. Mesenchymal component showed chondroid, myxoid and mucoid areas (as seen in Fig. 5). A few satellite nodules with infiltrative margins were seen. The features were consistent with atypical chondroid syringoma.

Fig. 4.

Photograph of the excised specimen showing a yellowish multinodular mass of size 3.1 cm × 2 cm × 2 cm

Fig. 5.

Photomicrograph of histopathology showing epithelial cells forming solid sheets, nests, trabeculae and glandular structures. Many of the cells had features of hyaline cells and were plasmacytoid. Mesenchymal component showed chondroid, myxoid and mucoid areas

Discussion

Though the term “Chondroid syringoma” was coined by Hirsch and Helwig, it was first described by Theodor Billroth more than a hundred years back in 1859. It is a relatively rare mixed cutaneous tumor. Clinically, chondroid syringomas present as a gradually progressive, painless, firm, cutaneous or subcutaneous nodule. They are usually seen in middle aged patients with a predilection for head and neck region [4]. The tumor is more frequently seen in males with a male to female ratio reported in literature between 5:1 and 2:1 [1, 4]. With head and neck being a common site, the other sites that may be involved are axillae, trunk, limb extremities and genital regions. It may be adherent to the overlying epidermis with no fixation to the underlying fascia, which may mislead for the preoperative diagnosis of a cyst. The presentation of chondroid syringomas can closely mimic various other commoner diagnoses like sebaceous cyst, dermoid cyst, neurofibroma and epidermal cyst. Hence the presentation can be frequently misleading due to its largely silent course [5]. Though mostly benign, rare malignant forms have been reported in literature. The malignant variants of chondroid syringomas differ from their benign counterparts with trunk and extremities being their commonest sites of presentations [6–8]. The histopathological features that distinguish a malignant lesion from a benign one include cytological atypia, increased mitotic activity, vascular and lymphatic invasion and nuclear pleomorphism. Deniz Tural et al. reported a rare case of malignant chondroid syringoma involving left nasolabial region in a 34 year old male. On histology they observed rare mitotic activity and expansive growth pattern with generalized lymphovascular invasion [9].

Two types have been described as apocrine and eccrine by Headington et al. in 1961. The apocrine type demonstrates irregular branching tubules (tubulocystic pattern) lined by at least 2-cell-thick epithelium. Adipocytes are common constituents of the stroma. The eccrine type is characterized by rather uniform, small, round tubules with a single layer of cells that are evenly spaced within a myxoid-chondroid matrix [10]. Apocrine CS is more common than eccrine CS. Salama et al. in a series of twenty-five cases observed 22 cases to be of the apocrine type. They also found that all the three eccrine cases were negative for adipocytes and did not show pilosebaceous differentiation or chondroid stroma [11]. Wong et al. in a series of eight cases highlighted the capability of pluripotential germinal cells towards multidirectional differentiation. They observed lobular proliferation of epithelial cells displaying a pattern of sebaceous, trichogenic, eccrine and apocrine differentiation [12]. Benign, atypical and malignant variants have been described in literature. Presence of satellite nodules and infiltrative margins indicate atypical variety which was the finding in our case. This was similar to the observation made by Gunduz et al. [13] who reported a case of atypical chondroid syringoma of the lower eyelid in a 46 year old male patient. Bates et al. [14] proposed the term ‘atypical mixed tumors’ for such cases with characteristics of local invasion without proven metastases and recommended careful follow up after complete excision.

Complete surgical excision is the treatment of choice with a cuff of normal tissue. Recurrences may occur when only a simple excision is made without the removal of a rim of normal tissue [15].

Conclusion

Chondroid syringomas are rare mixed tumors of sweat gland origin whose presentation can be confused with either a simple epidermal cyst or a tumor of mesenchymal origin. Their possibility should be kept in mind when confronted with a gradually progressive, soft to firm subcutaneous swelling. Atypical chondroid syringomas are tumors with potential malignant characteristics and a close follow up of such cases after excision is recommended. Complete surgical excision along with a rim of normal tissue is the treatment of choice and a detailed histopathological examination is a must for each case.

This report has been prepared in compliance with the ethical standards and with due consent of the patient. No funding was obtained from any source. There is no conflict of interest with anyone.