Chondroid syringoma: case report and review of the literature
- Author(s): Sivamani, Raja
- Wadhera, Akhil
- Craig, Errol
- et al.
Published Web Locationhttps://doi.org/10.5070/D32kg558r6
Chondroid syringoma: Case report and review of the literatureUniversity of California, Davis Department of Dermatology
Raja Sivamani, Akhil Wadhera MD, Errol Craig MD
Dermatology Online Journal 12 (5): 8
An 84-year-old man presented with an enlarging bluish, painless subcutaneous nodule on the glabella. The lesion had been excised 4 years prior and was diagnosed as chondroid syringoma, but had gradually regrown. The recurrent lesion was treated by surgical re-excision. Histopathological examination was again consistent with chondroid syringoma, and showed the following: 1) a chondroid matrix, 2) tubuloalveolar structures lined by a double epithelium, 3) ductal structures lined by a single epithelium, 4) nests of polygonal cells, and 5) the presence of keratinous cysts. Chondroid syringoma is a rare mixed tumor of the skin that was first described by Hirsch and Helwig. Characteristically, it is composed of a proliferation of epithelial cells set in a myxoid and chondroid matrix. Although chondroid syringomas are predominantly benign, malignant forms have been reported.
An 84-year-old man presented with a bluish, painless subcutaneous nodule on the glabella that had enlarged and changed color over the past year (Fig. 1). The lesion had been excised 4 years prior and was diagnosed as chondroid syringoma. Recurrent chondroid syringoma was clinically suspected, and the lesion was surgically re-excised.
Histopathological examination revealed a well-circumscribed proliferation of epithelial cells interspersed with foci of myxoid stroma. There were numerous nests of polygonal cells and interconnecting tubuloalveolar structures lined by two layers of cuboidal epithelial cells (Figs. 2-4). The stroma consisted of cells in a bluish chondroid matrix (Figs. 3, 4), areas of fibrosis (Figs. 2, 3, 4), and a lace-like pattern of interconnecting tubuloalveolar epithelial structures (Figs. 3, 4). Ductal structures lined by a single layer of epithelial cells were also apparent (Figs. 2, 3). Keratinous cysts were visible in some of the histological sections (Fig. 4). Cellular pleomorphism or dysplasia was not apparent. The constellation of findings lead to a diagnosis of chondroid syringoma.
|Figure 1||Figure 2|
|Figure 1. Bluish, subcutaneous nodule on the glabella|
Figure 2. Interconnecting tubuloalveolar structures lined by a double epithelium are visible along with nests of polygonal cells. Hematoxylin and eosin stain; Mag: 400X
Chondroid syringomas, also known as mixed tumors of the skin, are composed of both epithelial and mesenchymal components. Hirsch and Helwig gave them the appellation chondroid syringoma because of the presence of sweat gland elements set in a cartilaginous stroma . They proposed the following five histological criteria for diagnosis: 1) nests of cuboidal or polygonal cells; 2) intercommunicating tubuloalveolar structures lined with two or more rows of cuboidal cells; 3) ductal structures composed of one or two rows of cuboidal cells; 4) occasional keratinous cysts; 5) a matrix of varying composition. Chondroid syringomas may have all five characteristics or manifest only some .
The incidence of chondroid syringoma is low, and has been reported at 0.01-0.098 percent . Lesions are typically located on the head and neck, and are non-ulcerating, slow-growing, subcutaneous, or dermal nodules [1, 2, 3]. However, Sungur et al. reported a benign case where rapid growth, ulceration, and necrosis was evident at the tumor site . Chondroid syringomas affect middle-aged men more than women [2, 3, 5]. Although most are benign, malignant forms have been reported [5, 6, 7, 8, 9, 10, 11]. Unlike its benign counterpart, the malignant form occurs predominantly in females, has no age-related predilection, and is observed more commonly on the extremities [5, 6, 11, 12]. Of the malignant type, 48 percent are reported to have regional lymph node metastasis and 45 percent result in distant metastases . Malignant forms do not arise from a preexisting benign mixed tumor [6, 14] and can display bland features, lacking mitoses or marked nuclear pleomorphism . Excessive amounts of mucoid matrix and poorly differentiated chondroid components serve as important indicators of the tumor's malignancy and metastatic potential . Bates and Baithun discuss mixed tumors that have architectural features suggestive of malignancy but do not conclusively fall into the malignant category. For these they suggested the term atypical mixed tumors .
Chondroid syringomas share similarities with pleomorphic adenomas, which are mixed tumors that arise from the salivary glands . In contrast to pleomorphic adenomas, chondroid syringomas are thought to arise from sweat glands. Headington  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 [18, 19].
The tubuloalveolar components of chondroid syringomas are composed of two layers of cells with different immunophenotypes. The inner layer expresses cytokeratin (CK), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA), that is characteristic of cells with an epithelial lineage [5, 20]. The outer layers express vimentin, S-100 protein, neuron-specific enolase (NSE), and in a few cases, glial fibrillary acidic protein (GFAP) [5, 20] that is characteristic of both mesenchymal and epithelial differentiation. Varela-Duran et al. determined that the chondroid area of the tumor shows ultrastructural features identical to myoepithelial cells, a component of the outer layer of cells. They suggest that the chondroid regions are pseudocartilaginous areas that reflect the pluripotentiality of the neoplastic myoepithelial cell . However, subsequent immunohistochemical evaluation of the stroma suggested that myoepithelial cells may not play a major role in the formation of stromal cells . Other studies concluded that the cartilaginous matrix is true cartilage produced by ultrastructurally typical chondrocytes and not pseudocartilage produced by myoepithelial cells [22, 23]. Mills suggests that mixed tumors are monoclonal neoplasms with replicating cells that have the ability to differentiate toward epithelium or mesenchyme, and may account for the histologic variability of mixed tumors of the skin .
Optimal treatment of benign chondroid syringomas is surgical excision. Fine needle aspiration cytology has been used for diagnostic purposes and may prove useful to determine pathology before excision, however examination of excised tissue is most reliable in establishing a definitive diagnosis [18, 24]. Because of the lobulated nature of the tumor, it is important to include a margin of normal tissue with the excision to ensure complete removal of the tumor. Otherwise, local recurrence may occur, as was the case in our patient.
Chondroid syringomas are rare, usually benign, tumors occurring predominantly in the head and neck area. They should be included in the clinical differential diagnosis of cutaneous head and neck tumors, especially in middle-aged men. The malignant variant of chondroid syringoma differs from its benign counterpart in that it is more likely to occur on the extremities and is seen more frequently in women.Abbreviations CK: cytokeratin EMA: epithelial membrane antigen CEA: carcinoembryonic antigen NSA: neuron-specific enolase GFAP: glial fibrillary acidic protein
References1. Hirsch P, Helwig EB. Chondroid syringoma. Mixed tumor of skin, salivary gland type. Arch Dermatol 1961 Nov;84:835-47. PubMed
2. Yavuzer R, Basterzi Y, Sari A, Bir F, Sezer C. Chondroid syringoma: a diagnosis more frequent than expected. Dermatol Surg 2003 Feb;29(2):179-81. PubMed
3. STOUT AP, GORMAN JG. Mixed tumors of the skin of the salivary gland type. Cancer 1959 May;12(3):537-43. PubMed
4. Sungur N, Uysal A, Gumus M, Kocer U. An unusual chondroid syringoma. Dermatol Surg 2003 Sep;29(9):977-9. PubMed
5. 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
6. Harrist TJ, Aretz TH, Mihm MC, Jr., Evans GW, Rodriquez FL. Cutaneous malignant mixed tumor. Arch Dermatol 1981 Nov;117(11):719-24. PubMed
7. Ishimura E, Iwamoto H, Kobashi Y, Yamabe H, Ichijima K. Malignant chondroid syringoma. Report of a case with widespread metastasis and review of pertinent literature. Cancer 1983 Nov;52(10):1966-73. PubMed
8. Metzler G, Schaumburg-Lever G, Hornstein O, Rassner G. Malignant chondroid syringoma: immunohistopathology. Am J Dermatopathol 1996 Feb;18(1):83-9. PubMed
9. Steinmetz JC, Russo BA, Ginsburg RE. Malignant chondroid syringoma with widespread metastasis. J Am Acad Dermatol 1990 May;22(5 Pt 1):845-7. No abstract available. PubMed
10. Takahashi H, Ishiko A, Kobayashi M, Tanikawa A, Takasu H, Md MT. Malignant chondroid syringoma with bone invasion: a case report and review of the literature. Am J Dermatopathol 2004 Oct;26(5):403-6. PubMed
11. Mathiasen RA, Rasgon BM, Rumore G. Malignant chondroid syringoma of the face: a first reported case. Otolaryngol Head Neck Surg 2005 Aug;133(2):305-7. PubMed
12. Gupta S, Kumar A, Padmanabhan A, Khanna S. Malignant chondroid syringoma: a clinicopathological study and a collective review. J Surg Oncol 1982 Jul;20(3):139-44. PubMed
13. Barnett MD, Wallack MK, Zuretti A, Mesia L, Emery RS, Berson AM. 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
14. Kiely JL, Dunne B, McCabe M, McNicholas WT. Malignant chondroid syringoma presenting as multiple pulmonary nodules. Thorax 1997 Apr;52(4):395-6. PubMed
15. Bates AW, Baithun SI. Atypical mixed tumor of the skin: histologic, immunohistochemical, and ultrastructural features in three cases and a review of the criteria for malignancy. Am J Dermatopathol 1998 Feb;20(1):35-40. PubMed
16. Satter EK, Graham BS. Chondroid syringoma. Cutis 2003 Jan;71(1):49-52, 55. PubMed
17. HEADINGTON JT. Mixed tumors of skin: eccrine and apocrine types. Arch Dermatol 1961 Dec;84:989-96. No abstract available. PubMed
18. 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):104-8. PubMed
19. 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 Mar;20(2):110-6. PubMed
20. Argenyi ZB, Balogh K, Goeken JA. Immunohistochemical characterization of chondroid syringomas. Am J Clin Pathol 1988 Dec;90(6):662-9. PubMed
21. Varela-Duran J, Diaz-Flores L, Varela-Nunez R. Ultrastructure of chondroid syringoma: role of the myoepithelial cell in the development of the mixed tumor of the skin and soft tissues. Cancer 1979 Jul;44(1):148-56. PubMed
22. Mills SE. Mixed tumor of the skin: a model of divergent differentiation. J Cutan Pathol 1984 Oct;11(5):382-6. No abstract available. PubMed
23. Mills SE, Cooper PH. An ultrastructural study of cartilaginous zones and surrounding epithelium in mixed tumors of salivary glands and skin. Lab Invest 1981 Jan;44(1):6-12. PubMed
24. Agrawal V, Gupta RL, Kumar S, Mishra K, Agarwal S. Malignant chondroid syringoma. J Dermatol 1998 Aug;25(8):547-9. PubMed
© 2006 Dermatology Online Journal