|Ahead of print
Chondroid syringoma of an upper eyelid tumor: Unusual case report
Chinmay K Prajapati, Maitrik J Mehta, U Suryanarayan Kunikullaya
Department of Radiation Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
|Date of Submission||14-Feb-2021|
|Date of Decision||21-Feb-2021|
|Date of Acceptance||28-Aug-2021|
|Date of Web Publication||12-Oct-2022|
Chinmay K Prajapati,
Department of Radiation Oncology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat
Source of Support: None, Conflict of Interest: None
We present the case report of a 58-year-old man with recurrent chondroid syringoma, which was histopathologically confirmed, who underwent exenteration surgery of the right eye. Furthermore, the patient was receiving postoperative radiation therapy, and presently there is no local and/or distant evidence of disease in the patient.
Keywords: Chondroid syringoma, mixed tumor, radiotherapy
| » Introduction|| |
Chondroid syringoma (CS) is a rare cutaneous neoplasm that arises from the eccrine or apocrine gland, and it originates most frequently in the head and neck areas and may involve the face and eyelids, accounting for 0.48% of lid tumors.
CSs are uncommon sweat-gland-associated tumors that Billroth first mentioned in 1859, both benign and malignant in form. In 1961, Hirsch and Helwig gave the name “chondroid syringoma” because of the existence of sweat gland elements in a cartilaginous stroma.
CS of the orbit is relatively rare. Therefore, we report an unusual case of a patient who underwent complete resection of an intraorbital CS.
| » Case report|| |
A 58-year-old man presented in the right upper eyelid with a slow-growing painful mass since the past 3 months. The mass was 2 cm × 1 cm firm, tender, and fixed to the tissues at the base. At the same site, he had a similar lesion, which was excised locally 3 years ago.
The patient developed gradually increasing orbital pain and restricted movement of adduction and upward with partial visual field defect. On orbital computed tomography scan, a 49 × 29 mm soft tissue lesion was noted along the roof of the right orbit, which involves superior rectus and superior oblique muscle with right upper eyelid thickening without bone erosion [Figure 1].
|Figure 1: Computed tomography scan showing soft tissue mass involving roof of right orbit and upper eyelid|
Click here to view
He underwent exenteration of his right eye. A histopathological review confirmed the diagnosis of CS. Gross pathological analysis revealed that well-encapsulated tan tissue measuring 3 × 0.7 × 0.6 cm with gray-white smooth cut surface. Histologically, salivary gland tumor involving the upper eyelid, but not the optic nerve or the orbit, with a medial surgical margin. The tumor consisted of glandular structures lined by a two-layered epithelium, with varied cystic dilatation. These glands, surrounded by abundant basophilic chondroid-like stroma, were suggestive of CS. Furthermore, immunohistochemistry (IHC) was also performed to understand the nature and characterization of the tumor, which showed tumor cells that were positive for Anion Exchanger 1 (AE1), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA), and negative for p63, CK5/6 (cytokeratin 5/6), and C-kit (CD117). These cells showed immunostaining for EMA and CEA [Figure 2].
|Figure 2: Hematoxylin and eosin staining of the upper eye lid, cystic dilation with basophilic chondroid stroma|
Click here to view
He was then referred for postoperative radiation treatment to our department. We had scheduled postoperative radiotherapy in five fractions per week on COBALT-60 at 50 Gy dose with a total of 25 fractions. He completed the 25 fractions of radiation therapy without any side effects, and now he has been monitored for 2 years without complaining of any signs of distant or local disease, and the latest follow-up was in January 2021.
| » Discussion|| |
CS is a rare, benign, skin-appendageal tumor that Billroth first described in 1859 in a group of salivary gland tumors containing variable amounts of mucoid and cartilaginous material. Furthermore, Yun YM et al., 2016 referred to them as mixed tumors with both epithelial and mesenchymal origins. In 1961, Hirsch and Helwigintroduced the term “chondroid syringoma” to describe a form of skin tumor originating in apocrine sweat glands, consisting of glandular elements and cartilage-like stroma (mixed skin tumor). They also proposed five histopathological criteria for its 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, and (5) a matrix of varying composition. CSs can have all five features or have only some manifestations. The incidence of CS is between 0.01% and 0.1% of all skin lesions. CS usually affects middle-aged or older men.
Clinically, CS is typically presented as a slow-growing, painless, firm, subcutaneous, or intracutaneous nodule that is not ulcerated. The diameter of the lesion usually measures 0.5 to 3 cm long, and it is locally invasive. The sites of predilection for CS are on the head and neck regions, particularly the cheek, nose, or the skin above the lip., Less commonly, this tumor can develop on the scalp, eyelid, hand, foot, forehead, axillary region, abdomen, penis, or vulva, and scrotum orbital location is exceptional.
In 1999, Kitazawa et al. reported the first case of a woman complaining of left exophthalmos and diplopia. A firm mass was palpable in the left lateral canthal region, and computed tomography and magnetic resonance imaging showed an extraconal mass extending to the malar subcutis and associated with globe compression. The tumor was easily resected, and the diagnosis of benign CS was confirmed. Histologically, CS consists of mixed epithelial and mesenchymal elements with epithelial cells arranged in cords and forming myoepithelial tubules placed in a myxoid or chondroid stroma., Two histological variants of this tumor are outlined – the eccrine type with smaller lumens lined by a single row of cuboid epithelial cells and the apocrine variant lined by two rows of epithelial cells with tubular and cystic branching lumina. Furthermore, immunohistochemical study shows focal positivity for keratin, vimentin, desmin, and AE-1 protein in the stroma. Radiological features of CS are not as suggestive as the histological findings.
The differential diagnosis of CS made with other benign orbital lesions include pleomorphic adenoma, dermoids, lipomas, neurofibromas, meningioma, lymphangiomas, cavernous hemangiomas, and histiocytic tumors. We describe our patient to point out that this tumor should be considered a rare possibility in the differential diagnosis of orbital tumors. CS lesions usually are not clinically or radiology distinctive, and the diagnosis is made on histological examination., The choice of treatment is complete excision without esthetic or functional disruption of surrounding structures. Lateral orbitotomy is a safe approach in the surgical management of lesions in the lateral orbital region, particularly if the lesions are extraconal. However, in our case, recurrence was observed at the same site and the extension into the orbital muscles; furthermore, we have performed a complete eye exenteration here.
CS is a benign tumor, but rare cases have been reported of malignant CS. 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. Because of the risk of malignancy and recurrence, close follow-up of these tumors is recommended. As reviewed in the study by Watarai A. et al., 2011, there is a high incidence of local recurrence after initial resection in malignant CS. The recurrent period varies from 4 months to 4 years with an overall rate of recurrence of 50% from the reported cases available. So the overall rationale is combining surgery of both modalities followed by postoperative radiation for residual microscopic disease, and the dose for local control is 45 to 50 Gy. Depending on the ordinary tolerance of the surrounding tissue, the dose should be increased to 60 to 70 Gy in gross residual disease.
| » Conclusions|| |
As per our case report, the 58-year-old man who developed post excision local recurrence and underwent re-excision by complete exenteration of the eye and received adjuvant radiation treatment remained without evidence of disease at 2 years follow-up. Radiation therapy is an excellent adjuvant choice for these recurrent aggressive tumors. Treatment is well-tolerated with minimal morbidity. Radiation treatment can be added to ensure local control, and this may improve the overall disease-free survival.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| » References|| |
Kumar MA, Srikanth K, Vathsalya R. Chondroid syringoma: A rare lid tumor. Indian J Ophthalmol 2013;61:43-4.
] [Full text]
Hirsch P, Helwig EB. Chondroid syringoma. Mixed tumor of skin, salivary gland type. Arch Dermatol 1961;84:835-47.
Billroth T. Beobachtungen uber geschwulste der speichel-drusen. Virchows Arch Pathol Anat 1859;17:357-75.
Yun YM, Shin S, Kyung H, Song SH, Kang N. Recurrent chondroid syringoma of the alar rim. Arch Craniofac Surg 2016;17:35-8.
Yavuzer R, Başterzi Y, Sari A, Bir F, Sezer C. Chondroid syringoma: A diagnosis more frequent than expected. Dermatol Surg 2003;29:179-81.
Belfquih H, El mostarchid B, Oukabli M, Akhaddar A, Boucetta M. Benign chondroid syringoma of the orbit: A rare cause of exophtalmos. Head Face Med 2012;8:8.
Kitazawa T, Hataya Y, Matsuo K. Chondroid syringoma of the orbit. Ann Plast Surg 1999;42:100-2.
Sirivella S, Gielchinsky I. Chondroid syringoma: A rare tumor of the chest wall. Ann Thorac Surg 2010;89:983-5.
Hamed-Azzam S, Verity DH, Rose GE. Lateral canthotomy orbitotomy: A rapid approach to the orbit. Eye (Lond) 2018;32:333-7.
Bedir R, Yurdakul C, Sehitoglu I, Gucer H, Tunc S. Chondroid syringoma with extensive bone formation: A case report and review of the literature. J Clin Diagn Res 2014;8:FD15-7.
Watarai A, Amoh Y, Aki R, Takasu H, Katsuoka K. Malignant chondroid syringoma: Report of a case with lymph node metastasis 12 years after local excision. Dermatol Online J 2011;17:5.
[Figure 1], [Figure 2]