Chondroid Syringoma Elizabeth K. Satter, MD, LCDR, MC, USNR; Bradley S. Graham, MD, LCDR, MC, USNR We present the case of a 25-year-old man who of trauma is occasionally elicited, but trauma is presented with a slowly enlarging nodule on the thought to be coincidental rather than causative.1 upper lip. This nodule first appeared after minor Both tumors are benign encapsulated neoplasms, but trauma to the area. Our original differential diag- they can recur if excision is incomplete. Malignant nosis included foreign body granuloma, muco- forms of mixed tumors can arise, but these tumors cele, inclusion cyst, and adnexal tumor. are even rarer than chondroid syringomas. In this Histopathology results showed an encapsulated article, we report a case of chondroid syringoma that tumor consisting of 2 discrete lobules composed is unusual in presentation and location. of numerous aggregates of cuboid epithelial cells with tubuloalveolar structures and kerati- Case Report nous cysts within a chondroid stroma—consistent A 25-year-old white man presented with an asymp- with a chondroid syringoma. We compare and tomatic but slowly enlarging nodule on the right contrast the clinical and histologic characteris- upper lip. The patient stated that 5 years previously tics of chondroid syringomas (arising from sweat he had sustained a laceration to the upper lip after glands) with those of pleomorphic adenomas minor trauma and that sutures had not been (arising from salivary glands). required. Over the next 2 years, he had developed a fibrous cord in the same area on his lip. Because he term mixed tumor refers to a neoplasm that the area was firm and thickened, suggestive of scar has microscopic features of both epithelial and tissue, it was injected with 0.1 cm3 of 20 mg/mL of T mesenchymal differentiation. Tumors with triamcinolone acetonide. Over the next 3 years, this mixed cell types can arise from either salivary glands area had gradually enlarged and become more or sweat glands and most commonly occur in the indurated, until the patient presented to our derma- head-and-neck region of middle-aged men. As their tology clinic and requested surgical excision of the clinical presentations and histologic appearances are mass (Figure 1). similar, these tumors are often difficult to distin- Results of a physical examination revealed a sin- guish.1-7 Tumors that arise from salivary glands are gle, firm, 2-cm dermal nodule causing an asymmetri- more commonly referred to as pleomorphic adenomas, cal protuberance of the patient’s upper lip. The whereas those that arise from sweat glands are nodule was clearly demarcated from the normal sur- referred to as chondroid syringomas.1 Typically, these rounding tissue of the upper lip; however, there were tumors present as firm subcutaneous nodules that no appreciable epidermal changes. The nodule was have been present for several years and that tend to nontender and mobile, and there was no appreciable remain stable in size or grow slowly and cervical lymphadenopathy. gradually. The incidence of these 2 tumors varies. An intraoral approach was used to extirpate the More than 5000 cases have been reported of pleo- lesion. On blunt dissection, 2 encapsulated firm gray morphic adenomas,8 whereas only 400 cases have nodules, each measuring 0.7 cm in diameter, were been reported of chondroid syringomas.3 A history easily shelled out from the normal surrounding tissue. Results of microscopic studies showed 2 well- demarcated and circumscribed submucosal nodules. Accepted for publication August 20, 2002. The first nodule was composed predominately of a From the Department of Dermatology, Naval Medical Center, myxoid stroma with a few ductal and epithelial San Diego, California. structures. The epithelial component consisted of The views expressed in this article are those of the authors and do small scattered ducts lined by 1 to 2 layers of cuboid not reflect the official policy or position of the US Department of cells with some ducts exhibiting decapitation secre- the Navy, the US Department of Defense, or the US government. Reprints: Elizabeth K. Satter, MD, LCDR, MC, USNR, Department tion. The predominant feature of the myxoid of Dermatology, Naval Medical Center, 34800 Bob Wilson Dr, stroma was that it had numerous plasmacytoid cells San Diego, CA 92134 (e-mail: [email protected]). (Figure 2), some of which were entrapped within a VOLUME 71, JANUARY 2003 49 Chondroid Syringoma AB Figure 1. Side view (A) and close-up front view (B) of right upper-lip nodule. Figure 2. Stroma is myxoid in both lobules but is more so in the first lobule, with a predominance of plasma- cytoid cells (H&E, original magnification ϫ400). more condensed basophilic stroma forming lacunae- areas contained sebaceous glands, and a papillary like structures similar to mature chondrocytes. mesenchymal body, indicative of pilosebaceous- The second nodule had more of an epithelial apocrine differentiation, was found (Figures 4 component with less of a myxoid stroma (Figure 3). and 5). Given the definitive apocrine-sebaceous- These ducts also were lined with 1 to 2 layers of follicular differentiation, the diagnosis of a chon- cuboid cells with decapitation secretion, but the droid syringoma was favored over that of a salivary ducts were more extensive and had long inter- gland pleomorphic adenoma. connecting and branching patterns. Other areas were The patient was referred to the otorhinolaryngol- composed of solid epithelial nests without ducts. ogy department to discuss the option of conservative Some areas included focal collections of pale reexcision. The patient and the surgeons in that eosinophilic shadow cells consistent with matrical department felt that observing the area for recur- differentiation and scattered keratinous cysts. Several rence would be best and that, if the tumor recurred, 50 CUTIS® Chondroid Syringoma Figure 3. Overall lobular encapsulated architecture of the second, more epithelioid nodule, composed of ducts, eosinophilic collections of ghost cells, and keratinous cysts in a myxoid stroma (H&E, original magnification ϫ20). the patient would undergo further surgery. Thirteen derived from a series of brachial clefts and pharyn- months after the original surgery, the patient has yet geal pouches, there are multiple opportunities dur- to experience a recurrence. ing embryologic development for the entrapment of ectopic cell rests, which can ultimately, once stimu- Comment lated, have the potential to express various patterns Although chondroid syringomas and pleomorphic of differentiation.8 adenomas share many clinical and histologic fea- Pleomorphic adenomas are the most common tures, their unique cell types and preferential tumors to arise in the salivary glands. They gener- anatomic distributions can readily distinguish these ally involve the major salivary glands, especially the tumors. Both tumors typically present in middle- parotid glands, but they also can arise in the minor aged men as painless, slowly growing small encapsu- salivary glands of the palate, the buccal mucosa, lated subcutaneous nodules. The most common and (least commonly, in Ͻ4% of reported cases) the presumptive diagnoses include epidermoid cysts, upper lip.8,11-13 Chondroid syringomas, on the other mucoceles, adnexal tumors, or a granulomatous pro- hand, are thought to arise either from eccrine or cess.5,6 It is usually not until after the tumor is apocrine glands. They also occur within the head- excised and sent to pathology that the actual diag- and-neck region but are more frequently found on nosis can be ascertained. Neither the origin of the the nose, cheek, upper lip, scalp, forehead, and glandular component nor which cell is responsible chin.1,4 Chondroid syringomas can exhibit 2 sepa- for the production of the unique myxoid stroma is rate histopathologic patterns involving the glandu- known. It is hypothesized that the cells may arise lar structures. Type I chondroid syringomas, the from an abnormal outgrowth of an ectodermal more common variant, have ducts with lumina that analage or cell rest that has maintained its pluripo- vary sizes, that are lined with 2 layers of epithelial tent nature.1,2,7 Results from ultrastructural studies cells, and that exhibit decapitation secretion char- have suggested that the plasmacytoid cell found in acteristic of apocrine glands. Type II chondroid the stroma exhibit myoepithelial differentiation and syringomas have ducts with lumina lined with a therefore may represent the cell responsible for the single flattened layer of cuboid cells, some of which production of the myxochondroid stroma.6,9,10 Other have microvilli, and nonbranching ducts more typ- authors have suggested that the myoepithelial cell ical of eccrine glands.4,14 plays only a minor role in the development of mixed Histologically, the 2 types of mixed tumors share tumors and that it is the filamentous cells, which are many features having both mesenchymal and derived from the ectodermal analage, that produce epithelial components.1,15,16 However, chondroid the myxochondroid stroma.7 In either case, because syringomas can be distinguished from pleomorphic the head-and-neck region is developmentally adenomas because the former more commonly VOLUME 71, JANUARY 2003 51 Chondroid Syringoma Figure 4. Papillary mesenchymal body (H&E, original magnification ϫ400). Figure 5. Keratinous cyst centrally surrounded by small clusters of sebaceous cells and solid islands of basaloid cells (H&E, original magnification ϫ400). exhibit