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CASE REPORT
Year : 2022 | Volume
: 55
| Issue : 5 | Page : 193-195
Facial chondroid syringoma: A rare and important differential diagnosis
Saleh S Elbalka1, Amany Hassan2, Mohamed Hamdy1, Islam H Metwally1
1 Surgical Oncology Department, Oncology Center Mansoura University, Mansoura, Egypt
2 Department of Pathology, Faculty of Medicine, Mansoura University, Mansoura, Egypt
Correspondence Address:
Islam H Metwally
Surgical Oncology Department, Oncology Center Mansoura University, Geehan Street, Mansoura, Dakahlia
Egypt
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/fjs.fjs_75_22
Chondroid syringomas (CSs) are benign adnexal skin tumors usually affecting adolescent females in the periorbital region, and may have calcifications. Malignant transformation is very rarely encountered. There is a tendency to either medical treatment or intralesional injection of these lesions when confirmed benign especially if multiple. We herein describe a female with a periorbital benign CS. The presurgery cytology failed to predict the diagnosis, but surgical excision was attempted with good result. Both pathologists and surgeons are encouraged to add this differential in their diagnostic package of facial subcutaneous swellings.
Keywords: Adnexal tumors, chondroid syringoma, facial swelling, mixed skin tumor
Chondroid syringomas (CSs) are rare, benign skin tumors. In 1859, Billroth first described CS as a mixed tumor of the skin due to its histopathological resemblance to the benign mixed tumor originating from salivary gland.[1]
In a previous review, most patients with CSs presented with a round, firm, nodular, or cystic lesion with well-marginated heterogeneity on sonography.[2] A previous report showed a case with extensive ossification in imaging.[3]
The dermatoscopy does not seem to supply any specific trait to this lesion; as such, it is not useful as a diagnostic tool. Some scholars have proposed the term of atypical mixed tumor of the skin for those CSs with histological features of malignancy but without evidence of metastasis.[4]
Case ReportA female patient aged 19 years old presented to the outpatient clinic with painless facial swelling. On examination, an oblong cystic swelling of 3 × 2 cm size on the right cheek extending from lower eyelid to the nasolabial fold was found. The overlying skin was intact, but ecchymotic, and there were no palpable lymph nodes.
Fine-needle aspiration cytology was taken suggestive of pleomorphic adenoma. On revision with a more senior consultant, CS was suggested, with no malignancy.
Magnetic resonance imaging was done revealing a well-defined subcutaneous soft tissue lesion seen in the right cheek measuring 20 × 24 × 30 mm, low signal in T1 and high signal in T2 and STIR with IV contrast mild-to-moderate homogenous enhancement. It showed no fat tissue or calcifications inside. It invaded the skin with loss of fat planes between the mass and the underlying muscle. No underlying bony erosion and no detected intrasinus extension were found. No intracranial extension was observed. It encroached upon related lower eye lid with no intraorbital extension [Figure 1]. Surgical excision was done through a nasolabial incision followed by primary suturing [Figure 2]. Histopathology confirmed the diagnosis of CS [Figure 3].
Figure 1: Magnetic resonance imaging showing an enhanced swelling encroaching on the right orbit. (a and b) Axial view. (c) Sagittal view
Figure 2: (a) Photograph of the swelling before surgery. (b) Ellipse incision and careful dissection around the tumor capsule. (c) The gross picture of the specimen after excision. (d) Skin closure in the nasolabial fold after excision
Figure 3: (a) Biphasic neoplasm with a prominent myxoid stroma surrounding small epithelial strands and ductal structures (H and E, ×40). (b) Chondroid differentiation is evident (H and E, ×100). (c and d) Disorganized epithelial and myoepithelial elements embedded in myxoid stroma with ductal differentiation (H and E, ×200 and ×400)The postoperative course was smooth.
DiscussionThe term “CS” in lieu of mixed tumor of the skin was introduced by Hirsch and Helwig in 1961.[5]
CSs most commonly occur in the head and neck with a size ranging from 2 mm to ≥1 cm and usually present with solitary, solid, painless, nonulcerative, subcutaneous, or intracutaneous nodule. They often affect middle-age to elderly patients with a male-to-female ratio of 2:1.[6] Furthermore, they had been frequently reported in hands.[7],[8] They have also been reported in uncommon sites such as external auditory canal,[9] scrotum,[10] fingertips,[11] and chest wall.[12]
The malignant variant of CS is rare and most cases affect extremities.[7] Probably, the first case reported in the face was in 2005.[13] In most cases of malignant CS (MCS), anaplastic changes are present from the beginning. MCS is a mixed cutaneous tumor, with epithelial and mesenchymal components, which compromises principally the trunk and extremities.[8] Rarely, a CS of many years duration suddenly undergoes malignant changes with widespread metastasis.
The value of medical therapy in the management of syringomas requires validation by large studies. The precise rationale for the use of most current medical therapies for syringoma treatment remains poorly defined. Retinoids target cellular proliferation, differentiation, and keratinization; atropine inhibits sweat production; tranilast may suppress the proliferation of stromal connective tissue in syringoma lesions by inhibiting the release of interleukin-1 beta from eccrine ducts.[14] Isotretinoin therapy using variable dosing regimens had mixed results.[15],[16]
While CO2 therapy has been shown to be moderately effective, nonspecific thermal damage may result in scarring of the adjacent tissue.[17]
The use of intralesional insulated needles for electrocoagulation decreases the size and number of lesions while sparing epidermal damage, such as scarring and hyperpigmentation. However, this time-consuming method requires multiple sessions for the best results, and long-term efficacy has not yet been determined.[18]
ConclusionCSs are rare adnexal skin pathology. Many medical and interventional options are available for their management. In isolated lesion simple surgical excision is a viable option for both definitive diagnosis and treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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