Skip to main content
eScholarship
Open Access Publications from the University of California

Chondroid syringoma: Report of two cases in young patients

  • Author(s): Solanki, Lakhan Singh
  • Dhingra, Mandeep
  • Bhalla, Mala
  • Thami, Gurvinder Pal
  • Punia, RPS
  • Batra, Shilpy
  • et al.
Main Content

Chondroid syringoma: Report of two cases in young patients
Lakhan Singh Solanki MD1, Mandeep Dhingra1, Mala Bhalla MD1, Gurvinder Pal Thami MD1, RPS Punia MD2, Shilpy Batra MD2
Dermatology Online Journal 17 (12): 7

1. Department of Dermatology
2. Department of Pathology
Government Medical College and Hospital, Chandigarh, India


Abstract

Chondroid syringoma (CS) is a relatively rare cutaneous tumor arising from sweat glands. It usually presents in the head and neck area as an asymptomatic nodule. Although usually it presents in middle aged or older patients, we here present two much younger patients with CS, located over the nose and cheek areas, respectively.



Introduction

Chondroid syringoma (CS), an uncommon skin appendageal tumor, is also referred to as cutaneous mixed tumor because it consists of both epithelial and myoepithelial cell types. The incidence of CS among primary skin tumors is low and it is usually seen in middle aged or older male patients as a small, single dermal or subcutaneous nodule in the head and neck area. They are rarely multiple or large in size and malignant transformation is very uncommon. No characteristic clinical features are seen, so diagnosis is by histopathological examination. Treatment of benign CS is surgical excision or destruction. There is a rare risk of malignancy, so regular follow up is recommended [1, 2]. We herein present two cases of CS in young individuals located over the nose and cheek areas respectively.


Case report


Figure 1Figure 2
Figure 1. Single well defined nodule, with slight violaceous discoloration in its center

Figure 2. Photomicrograph showing epithelial components in the form of ducts and lobules; background showed homogeneous basophilic abundant fibromyxoid stroma (H&E, x100).

Case 1. A 16-year-old male presented with an 8 month history of a single, asymptomatic, raised lesion on the nose. It initially started as small lesion beneath the skin, which gradually increased in diameter and depth. On examination, he had a single well defined nodule of 1.5 cm x 1.5 cm with slight violaceous discoloration in its center. It was soft to firm in consistency, non-tender, and fixed to overlying skin, but mobile over deeper structures (Figure 1). Cervical lymphadenopathy was absent. An excisional biopsy was done and routine hematoxylin-eosin staining revealed a well-circumscribed tumor composed of epithelial and stromal components. Epithelial components were in the form of ducts and lobules lined by a luminal layer of cuboidal cells and a peripheral layer of flattened cells; the background showed abundant fibromyxoid stroma. Cellular pleomorphism or dysplasia was not apparent (Figures 2 and 3). These histopathological findings are diagnostic of CS. At the 1 year follow up there was no recurrence.


Figure 3Figure 4
Figure 3. Proliferation in small aggregates and duct structures lined by a luminal layer of cuboidal cells and a peripheral layer of flattened cells located in a basophilic fibromyxoid stroma containing myoepithelial cells (H&E, x200)

Figure 4. Single well-defined nodule with atrophy and scarring on its surface

Case 2. A 21-year-old female presented with a single, asymptomatic, slowly growing, raised lesion on the left cheek of three years duration. There was a history of application of some irritant over the lesion a few weeks prior to presentation. On examination, she had a single well-defined nodule of 4 cm x 3 cm, firm to hard in consistency, non-tender, that was fixed to the overlying skin, but mobile over deeper structures. The overlying skin was atrophic and appeared scarred (Figure 4). Cervical lymphadenopathy was absent. The histopathological findings of the biopsy specimen were similar to that of the first case except for the presence of an abundant basophilic myxoid stroma (Figures 5 and 6). The tumor was completely excised and there was no recurrence at a 2-year follow up.


Figure 5Figure 6
Figure 5. Photomicrograph showing tubular lumina with marked variation in size and shape; background showed abundant myxoid stroma (H&E, x100).

Figure 6. Tubular lumina are lined by two layers of epithelial cells: luminal layer of cuboidal cells and peripheral layer of flattened cells, and abundant basophilic myxoid stroma (H&E, x400).

Discussion

Chondroid syringoma (CS) are mixed tumors of the skin. The term, chondroid syringoma, was introduced by Hirsch and Helwig to describe these tumors because of the presence of sweat gland elements set in a cartilaginous stroma [1]. The term, mixed tumors, term was coined by Virchow and Minssen for neoplasms with microscopic features that indicate both epithelial and mesenchymal origin [2].

Chondroid syringoma (CS) or mixed tumor is a relatively rare, benign, skin appendageal tumor. This term has been used to describe cutaneous tumors as well as tumors unrelated to skin, i.e., of salivary glands and of kidney [3]. The incidence of CS among primary skin tumors is low and has been reported at 0.01 percent to 0.098 percent [2].

Chondroid syringoma usually presents as a single, asymptomatic, slow-growing, firm, circumscribed, non-ulcerated, lobulated nodule within the deep dermis or subcutaneous fat in middle aged or older male patients, but both of our cases were of a younger age group. Its usual size is 0.5 cm to 3 cm [3, 4, 5], although cases of large and even multiple tumors have been reported [6-10]. The common sites for CS are on the head and neck area, particularly cheek, nose, or skin above the lip, though it may present anywhere over the body [1, 2].

Chondroid syringoma is usually benign but a few cases of malignant transformation have been reported [1, 6, 11]. These malignant types occur more commonly in younger females. They are often larger than 3 cm, are locally invasive, and show a predilection for trunk or extremities [1, 11, 12, 13].

For the diagnosis of CS, Hirsch and Helwig [1] described five histological criteria: (1) nests of cuboidal or polygonal cells, (2) interconnected tubuloalveolar structures lined by 2 or more rows of cuboidal epithelial cells, (3) ductal structures lined by 1 or 2 rows of cuboidal cells, (4) occasional keratinous cysts, and (5) a matrix of mixed chondroid and myxoid material. Some CSs display all 5 characteristics, whereas others only manifest a few. In our case four of these features are seen. Headington [14] divided chondroid syringomas into apocrine and eccrine variants based on histological differences in the luminal morphology, but there remains debate as to their exact origin [15, 16]. Histological features considered as signs of malignant transformation are cytologic atypia, infiltrative margins, satellite tumor nodules, tumor necrosis, and involvement of deep structures [17], not seen in our cases.

Immunohistochemisrty shows that tubuloalveolar components of chondroid syringomas express cytokeratin (CK), epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), vimentin, S-100 protein, neuron-specific enolase (NSE), and in a few cases, glial fibrillary acidic protein (GFAP) [18, 19].

Because there is no particular clinical appearance, the diagnosis of CS is primarily histopathological [2]. Fine needle aspiration cytology (FNAC) may be used to diagnose the pathology before excision [20]. Optimal treatment of benign CS is surgical excision without affecting aesthetic and functional structures. Other treatment options are dermabrasion, electrodessication, and vaporization with argon or CO2 laser [2]. Because of the risk of malignancy, the patient should be followed up to look for recurrence or any feature of malignancy [21].


Conclusion

These cases have been reported to emphasize that chondroid syringoma may be uncommon but they are not as rare as considered previously. Our patients are of interest because of the younger age of presentation. CS patients should be followed up to look for recurrence or any features of malignancy.

References

1. Hirsch P, Helwig EB. Chondroid syringoma. Mixed tumor of skin, salivary gland type. Arch Dermatol. 1961 Nov;84:835-47. [PubMed]

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

3. TSS Lakshmi, A Rao Gnaneshwar. Multiple chondroid syringoma. Indian J Der and Venereol Leprol. 1988 Mar;64(2):83-84. [PubMed]

4. Satter EK, Graham BS. Chondroid syringoma. Cutis. 2003 Jan;71(1):49-52, 55. [PubMed]

5. Agrawal A, Kumar A, Sinha AK, et al. Chondroid syringoma. Singapore Med J. 2008 Feb;49(2):e33-e34. [PubMed]

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

7. Hardisson D, Linares MD, Nistal M. Giant chondroid syringoma of the axilla. J Cutan Med Surg. 1998 Oct;3(2):115-7. [PubMed]

8. Kakuta M, Tsuboi R, Yamazaki M et al. Giant mixed tumor of the face. J Dermatol. 1996 May;23(5):369-71. [PubMed]

9. Kose R, Okur MI. Giant facial chondroid syringoma. Dermatol Surg. 2009 Feb;35(2):294-5. [PubMed]

10. Borman H, Özcan G. Chondroid syringoma at the fingertip: an unusual localization. Eur J Plast Surg. 1998;21:311-13.

11. Barnett MD, Wallack MK, Zuretti A, et al. Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature. Am J Clin Oncol. 2000 Jun;23(3):227-32. [PubMed]

12. Steinmetz JC, Russo BA, Ginsburg RE. Malignant chondroid syringoma with widespread metastasis. J Am Acad Dermatol. 1990 May;25(5 pt 1):845-7. [PubMed]

13. Metzler G, Schaumburg-Lever G, Hornstein O, Rassner G. Malignant Chondroid Syringoma: Immunohistopathology. Am J of Dermatopathol. 1996 Feb;18(1):83-89. [PubMed]

14. Headington JT. Mixed tumors of skin: eccrine and apocrine types. Arch Dermatol. 1961 Dec;84:989-96. [PubMed]

15. Chen AH, Moreano EH, Houston B, Funk GF. Chondroid syringoma of the head and neck: clinical management and literature review. Ear Nose Throat J. 1996 Feb;75(2):104-108. [PubMed]

16. Mandeville JT, Roh JH, Woog JJ, Gonnering RS, Levin PS, Mazzoli RA, et al. Cutaneous benign mixed tumor (chondroid syringoma) of the eyelid: clinical presentation and management. Ophthal Plast Reconstr Surg. 2004; 20:110-16.

17. 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 Feb;20(1):35-40. Review. [PubMed]

18. Sheikh SS, Pennanen M, Montgomery E. Benign chondroid syringoma: report of a case clinically mimicking a malignant neoplasm. J Surg Oncol. 2000 Apr;73(4):228-30. [PubMed]

19. Argenyi ZB, Balogh K, Goeken JA. Immunohistochemical characterization of chondroid syringomas. Am J Clin Pathol. 1988 Dec;90(6):662-69. [PubMed]

20. Skoro M, Ostović KT, Cikara I, Müller D, Novak NP, Virag M. Fine needle aspiration cytology of chondroid syringoma. Coll Antropol. 2010 Jun;34(2):687-90. [PubMed]

21. Mebazaa A, Trabelsi S, Denguezli M, et al. Chondroid syringoma of the arm: an unusual localization. Dermatol Online J. 2006 Jan 27;12(1):14. [PubMed]

© 2011 Dermatology Online Journal