Cholesterol granuloma of the orbit: An atypical presentation.

Cholesterol granuloma is a rare, well-defined lesion of the orbit. In the orbit, diploe of the frontal bone is involved almost exclusively. We report an atypical case of cholesterol granuloma involving superomedial quadrant of orbit. A 42-year-old male presented with progressive, painless, proptosis with inferotemporal displacement of left eye. A large mass was felt beneath the bony orbital margin in the superomedial quadrant of the left orbit. Computerized tomography (CT) scan revealed an extraconal superomedial, heterogeneous enhancing mass which was isodense with brain and pushing the globe inferolaterally and anteriorly. Excision biopsy of the tumor revealed the typical features of a cholesterol granuloma without any epithelial elements. Cholesterol granuloma of the orbit is a rare entity, but it can be diagnosed and differentiated from other lesions of the superior orbit by its characteristic clinical, radiological and histopathological features. An appropriate intervention in time carries a good prognosis with almost no recurrence.

A giant upper eyelid ossifying pilomatrixoma.

Pilomatrixoma of the eyelid is extremely rare in middle age and rarely develops into a large tumor. A 45-year-old female developed a painless, progressive swelling (3.0 cm x 2.0 cm) of the left upper eyelid over a period of two years. Overlying skin was normal in color and texture. A differential diagnosis of dermoid, epidermoid cyst, chalazion and basal cell carcinoma was made. An excisional biopsy was performed. A diagnosis of pilomatrixoma was made on histopathological features (dystrophic calcification of matrix with keratin and foreign body granulomatous reaction, basaloid cells and shadow cells/ghost cells). It also comprises ossification apart from the usual calcification. This is a report of an unusually large ossifying pilomatrixoma in left upper eyelid of a middle-aged woman. The patient should be followed up at regular intervals to rule out any recurrence or malignant transformation.

Orbital amelanotic melanoma in xeroderma pigmentosum: a rare association.

Xeroderma pigmentosum (XP) is an autosomal recessive genetic disorder of DNA repair in which the body's normal ability to repair damage caused by ultraviolet light is deficient. This leads to a 1000-fold increased risk of cutaneous and ocular neoplasms. Ocular neoplasms occurring in XP in order of frequency are squamous cell carcinoma, basal cell carcinoma and melanoma. Malignant melanomas occur at an early age in patients with XP. We report a case of XP with massive orbital melanoma in an eight-year-old boy which is unique due to its amelanotic presentation confirmed histopathologically.