|Year : 2003 | Volume
| Issue : 3 | Page : 257-259
Dermal Cylindroma Presenting as Mass Lesion in Superomedial Orbit.
R Gupta, R Jain, S Sood, H Mohan
Department of Ophthalmology, Government Medical College Hospital, Chandigarh, India
Department of Ophthalmology, Government Medical College Hospital, Chandigarh
Source of Support: None, Conflict of Interest: None
A superomedial orbital dermal cylindroma in an adult female is described.
Keywords: Dermal, cylindroma, orbit, adnexa
|How to cite this article:|
Gupta R, Jain R, Sood S, Mohan H. Dermal Cylindroma Presenting as Mass Lesion in Superomedial Orbit. Indian J Ophthalmol 2003;51:257-9
Dermal cylindromas are rare benign adnexal tumours most commonly occurring on the head, neck and scalp as solitary or multiple mass lesions. Similar lesions have not been described in the periocular area (Medline search). We present herein a case of 55-year-old female patient with a mass lesion in superomedial orbital region that posed a diagnostic dilemma clinically.
| Case report|| |
A 55-year-old Indian female reported to us in January 2001 with a swelling near the inner corner of the left eye of one year duration. The swelling was painless and had gradually increased in size. There were no other associated ocular symptoms such as watering, discharge, redness in the eye, reduction of vision or diplopia. There was no history of any antibiotic intake or previous surgery for the same. There was no history of trauma.
On examination, the best corrected visual acuity was 6/18 in both eyes. Intraocular pressures were 14 and 16 mmHg in the right and left eye respectively. There was no strabismus. Nasolacrimal duct was patent and there was no change in the size of the swelling on syringing. A round skin-coloured mass of about 1.5 cm diameter was present near the medial canthus around 1 cm clear of the lid margin [Figure - 1]. It was firm in consistency, and non-tender. It had a well defined margin extending to around 9 mm above the medial canthus and 6 mm downwards and was mobile in its vertical and horizontal extent. The overlying skin was attached to the underlying mass and the surface was smooth. There was no parotid or submandibular lymphadenopathy. The rest of the ocular examination was within normal limits other than presence of immature senile cataract in both eyes.
On investigation the haemogram was within normal limits. The X-ray orbit did not show any density in the lesion area. There was no nasal extension of the mass and it did not seem to originate from the orbital structures. Our preoperative differential diagnoses were dermoid cyst and lacrimal gland cyst. Excision biopsy under local anaesthesia was carried out. A vertical incision was made in the overlying skin. The underlying mass could be completely dissected around from the surrounding tissues. The mass was a cream coloured, round lesion 1.3cm in diameter with a thick, tense wall and the cut section of the mass was yellowish with areas of haemorrhages. The histopathology of the mass showed a dermal tumour consisting of numerous oval and polygonal nests molded into a jigsaw-like pattern. Masses of epithelial cells were surrounded and penetrated by hyaline sheaths [Figure - 2]. Tumour islands showed two cell types - peripheral small and uniform with dark nuclei, and central larger with more pale-staining nuclei. The cells in the inner layer enclosed luminal secretion at places. The hyaline material comprising the sheath was PAS positive and diastase resistant. A diagnosis of dermal cylindroma, a benign adnexal tumour, was made on the basis of these histopathological findings. The patient is on follow-up and has not shown any recurrence till now.
| Discussion|| |
Cylindromas are rare benign adnexal tumours that classically arise on the scalp, head and neck, thus earning the name of turban tumour. Exact incidence is not known. Female to male ratios of 6:1 and 9:1 have been reported. They are usually solitary but may be multiple and recurrent. Solitary cylindromas affect the face and scalp of older adults and do not seem to be inherited. Multiple tumours are inherited in an autosomal dominant mode and implicated on Cyld 1 gene on chromosome 16 which is a suppressor gene. They may occur on the trunk and extremities as well as head and neck. They usually begin in early adulthood and progress through the time. Guzzo and Johnson reported an unusual abdominal location of a solitary dermal cylindroma in a 78-year-old woman. In our case, the location was in the superomedial orbital area which has not been described so far. Differential diagnosis of dermoid, cysticercus and lacrimal sac tumour were considered, though clinically, the diagnosis did not fit into any of these. Since this one-year-old mass lesion did not have any bony attachment we ruled out dermoid cyst, and the mobility of the mass ruled out lacrimal sac tumour or cysticercus cyst.
Cylindromas are firm, rubbery, pinkish or bluish nodules ranging in size from a few millimeters to several centimeters. Histopathologically, a cylindroma is a very primitive sweat gland tumour and a relationship to the apocrine gland has been suggested because of lysozyme and alpha-1 antichymotrypsin expression. The tumour consists of oval and polygonal nests molded into a jigsaw like pattern. Tumour islands are composed of 2 cell types - peripheral and central. The peripheral cells are small, basophilic and arranged in a palisading pattern. Larger, more pale-staining cells are seen centrally. Masses of tumour cells are surrounded and penetrated by a hyaline sheath. Electron microscopy reveals cells differentiating towards secretory or ductal cells. Thus, ultrastructural features fit differentiation towards eccrine sweat glands.
Treatment for solitary tumours is excision. For smaller tumours the carbon dioxide laser may be used. Multiple tumours require extensive plastic surgery.
Malignant transformation is known in cylindromas though the vast majority remain benign., At least 14 reports exist of malignant transformation. They may develop within solitary cylindromas or more commonly complicate the multiple variant. Clinically malignant change can manifest as ulceration. Histologically, it shows marked anaplasia and pleomorphism of nuclei. Mitoses are increased and there is loss of delicate hyaline sheath. The prognosis is not good with malignancy since visceral metastasis may occur. In our case, the tumour was solitary and did not show any malignant change. The en-masse surgical excision was carried out successfully. This case highlights the fact that rare adnexal tumours should be kept in mind while encountering a mass lesion in the periocular region. These tumours carry a risk of malignant transformation and hence need to be diagnosed and treated early and followed up adequately.
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[Figure - 1], [Figure - 2]
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