Chondroid syringoma of the arm: An unusual localization
A Mebazaa 1, S Trabelsi 1, M Denguezli 1, B Sriha 2, C Belajouza 1, R Nouira 1
Dermatology Online Journal 12 (1): 14

Department of Dermatology1 and Department of Pathology2, Chu Farhat Hached 4002 Sousse Tunisia. dr.denguezli@topnet.tn

Abstract

Chondroid syringoma (CS) is an uncommon, benign, sweat-gland tumor. The usual presentation is of an asymptomatic, slowly growing mass, typically located on the head and neck region. We present a case of a CS located in the right arm of a 43-year-old woman. This tumor developed slowly over 8 years. The diagnosis of CS was made by histopathological examination; it showed an abundant chondroid stroma intermingled with a fibro-adipoid tissue. There was no recurrence after total excision of the tumor with a 2-year followup. The unusual localization of chondroid syringoma made an accurate preoperative diagnosis difficult and diagnosis was achieved only by microscopy. Surgical tumor excision remains the best therapeutic option to avoid relapses of this tumor. Close followup is recommended because malignant transformation, although rare, is possible.



Introduction

Chondroid syringoma (CS) is a rare, benign, skin appendageal tumor, first described by Billorth in 1859 for a group of tumors of the salivary gland that contained varying amounts of mucoid and cartilaginous material [1]. Virchow and Minssen [2] referred to them as mixed tumors with both epithelial and mesenchymal origins. This uncommon eccrine sweat gland tumor presents as a slow-growing, painless, subcutaneous, or intracutaneous nodule [1, 2]. It is often located in the head and neck region [2]. Appearance on other parts of the body is unusual [1,v3]. We report a case of benign CS located on the right arm.


Clinical synopsis

A 43-year-old woman was referred for an indurated nodule on her right arm. Her past history included a benign fibroma of the left breast. The arm lesion had grown slowly over the period of about 8 years. Physical examination showed a firm, painless, mobile, 2-cm nodule covered by normal skin. The lesion was located on the right arm (Fig. 1). There was no palpable regional lymphadenopathy. The nodule was excised and sent for histopathology.


Figure 1 Figure 2
Figure 1. A firm, painless, mobile subcutaneous nodule on the right arm
Figure 2. Proliferation of cord and duct structures in the chondromixoid stroma, distinct from the surrounding fat tissue.The stroma characteristically shows a homogeneous basophilic chondroid staining (H&E × 100).

Figure 3 Figure 4
Figures 3 and 4. Proliferation in small aggregates and duct structures lined by two rows of cuboidal epithelial cells located in a basophilic chondromixoid stroma containing myoepithelial cells (H&E × 250).

Gross examination shows a well circumscribed, whitish firm tumor, surrounded with capsule-like tissue. Histological examination reveals abundant chondroid stroma intermingled with a fibro-adipoid tissue containing epithelial structures arranged in small aggregates and ducts. The epithelial cells are cuboidal with an eosinophilic cytoplasm and regular oval nuclei (Figs. 2-4).

A diagnosis of CS was made. No recurrence was seen 2 years following total excision.


Discussion

Chondroid syringoma, also known as mixed tumor of the skin, is a rare, benign adnexal tumor with an unknown etiopathogenesus [1, 2]. Some authors suggest the hypothesis of both epithelial and mesenchymatous origin [3]. 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 [4]. The reported incidence of CS among primary skin tumors is less than 0.01 percent [1, 2, 5]. A greater incidence of 0.098 percent was reported by Yavuzer et al. [2]. Chondroid syringoma usually affects middle-aged or older male patients [2]. In our observation, CS occurred in a 43-year-old female.

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 [1, 2, 6]. However, larger forms of CS have been described [6, 7, 8, 9].

The sites of predilection for CS are on the head and neck region, particularly cheek, nose, or skin above the lip [1, 2, 3, 6]. Less commonly, this tumor can develop on the scalp, eyelid, orbit, hand, foot, forehead, axillary region, abdomen, penis, vulva, and scrotum [2, 6, 7, 9, 10, 11, 12, 13]. In our patient, CS developed on the right arm.

Chondroid syringoma is often overlooked because of rarity of this tumor and unremarkable clinical presentation [2].

The 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 [1, 2]. CS lesions usually are not clinically distinctive and the diagnosis is made on microscopic examination [1, 2].

Histologically, 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 [2, 4, 14, 15]. Osteoid stroma or mature adipocytes can be present in rare cases [15]. Two histological variants of this tumor are described, the eccrine type with smaller lumens lined by a single row of cuboidal epithelial cells and the apocrine variant with tubular and cystic branching lumina lined by two rows of epithelial cells [2]. Immunohistochemical study shows focal positivity for keratin, vimentin, desmin, and S-100 protein in the stroma [2].

In our patient, histological examination revealed an abundant chondroid stroma intermingled with a fibroadipoid tissue that contained some epithelial structures arranged in small aggregates and ducts.

Multiple treatment options have been proposed for CS, including electrodesiccation, dermabrasion, and vaporization with argon or CO2 laser. The usual first-line treatment is total excision of tumor without destroying aesthetic and functional structures [2]. In our observation, the tumor was completely excised without recurrence within a 2-year followup.

Chondroid syringoma is a benign tumor and recurrence does not occur if excised completely [1]. However, rare cases of malignant CS have been reported [1, 3, 16]. These malignant forms occur more commonly in younger female patients, and have a predilection for occurring on the trunk or extremities [1, 16, 17, 18]. These tumors often are larger than 3 cm and are locally invasive. Metastasis to bones and viscera are rare [5, 17, 18, 19]. Histological findings such as cytologic atypia, infiltrative margins, satellite tumor nodules, tumor necrosis, and involvement of deep structures are considered as signs of malignant transformation [2, 4]. However, the histopathological features of malignant CS may be very similar to the benign type [3]. There are reported cases of CS with a benign histology that turned out to be malignant [1, 17, 20]. Immunohistology is of no help in distinguishing between benign and malignant forms [18].

Because of malignant potential, complete excision of CS must be done. The patient should be followed carefully for both local recurrence and metastasis.

Regarding the previous literature reports, our patient's presentation is of interest because of the atypical location of CS [6, 21].

In conclusion, CS should be included in the differential diagnosis of cutaneous or subcutaneous slow-growing solid nodules and a close follow-up of these tumors is recommended because of risk of malignancy.

References

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2. R Yavuzer, Y Basterzi, A Sari et al. Chondroid syringoma: a diagnosis more frequent than expected. Dermatol Surg 2003;29:179-81.

3. Borman H, Özcan G. Chondroid syringoma at the fingertip: an unusual localization. Eur J Plast Surg 1998;21:311-3.

4. Bates A W, Baithun S I. 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.

5. Watson JA, Walker MM, Smith NP, Hunt DM. Malignant chondroid syringoma--a rare cause of secondary bone tumour. Clin Exp Dermatol 1991;16:306-7.

6. Bekerecioglu M, Tercan M, Karakok M, Atik B. Benign chondroid syringoma: a confusing clinical diagnosis. Eur J Plast Surg 2002;25:316-8.

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8. Kakuta M, Tsuboi R, Yamazaki M et al. Giant mixed tumor of the face. J Dermatol 1996;23:369-71.

9. Sliwa-Hahnle K, Obers V, Lakhoo M, Saadia R. Chondroid syringoma of the abdominal wall. A case report and review of the literature. S Afr J Surg 1996;34:46-8.

10. Mencia-Gutierrez E, Gonzales-Daimiel JA, Gutierrez-Diaz E et al. Chondroid syringomas of the eyelid: two cases. Eur J Ophthalmol 2001;11:80-2.

11. Kitazawa T, Hataya Y, Matsou K. Chondroid syringoma of the orbit. Ann Plast Surg 1999;42:100-2.

12. Poku JW, Sant GR, Ucci AA. Chondroid syringoma of the scrotum. J Int Med Res 1996;24:482-6.

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14. Ohnishi Takamitsu, Watanabe Shinichi. Histogenesis of mixed tumor of the skin, apocrine type: immunohistochemical study of keratin expression. Am J of Dermatopathol 1997;19:456-61.

15. Miracco C, De Santi MM, Lalinga AV et al. Lipomatous mixed tumour of the skin: a histological, immunohistochemical and ultrastructural study. Br J of Dermatol 2002;146: 899- 903.

16. Barrett MD, Wallack MK, Zuretti A et al. Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature. Am J Oncol 2000;23:227-32.

17. Steinmetz JC, Russo BA, Ginsburg RE. Malignant chondroid syringoma with widespread metastasis. J Am Acad Dermatol 1990;5:845-7.

18. Metzler G, Schaumburg-Lever G, Hornstein O, Rassner G. Malignant Chondroid Syringoma: Immunohistopathology. Am J of Dermatopathol 1996;18:83-89.

19. Kiely JL, Dunne B, McCabe M, Mc Nicholas WT. Malignant chondroid syringoma presenting as multiple pulmonary nodules. Thorax. 1997;52:395-6.

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21. Gupta JC, Gandagule VN, Panda PK, Gupta N. Chondroid syringoma of skin- upper arm (benign mixed tumour of skin). Indian J pathol Microbiol 1977;20:121-3.

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Title:

Chondroid syringoma of the arm: An unusual localization

Journal Issue:

Dermatology Online Journal, 12(1)

Author:

Mebazaa, A;
Trabelsi, S;
Denguezli, M;
Sriha, B;
Belajouza, C;
Nouira, R

Publication Date:

2006

Publication Info:

Dermatology Online Journal, UC Davis

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