Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.
Adenoid cystic carcinoma is a rare epithelial malignancy, which tends to grow slowly. It is an intractable neoplasm due to its ability to invade perineural spaces. A 59-year-old female presented with a gradually increasing mass in the right lower eyelid. An excisional biopsy with wide margins revealed a diagnosis of primary adenoid cystic carcinoma of eyelid skin with perineural invasion. Although a rare neoplasm, primary adenoid cystic carcinoma of eyelid skin should be included in the differential diagnosis of eyelid tumors.
Ocular involvement in non-Hodgkin lymphoma occurs either as primary ocular, central nervous system lymphoma or isolated intraocular lymphoma. Recurrence of systemic non-Hodgkin lymphoma in the form of CNS lymphoma has been reported. However, recurrence as an isolated optic nerve lesion without involving CNS has never been reported in the pediatric age group. We report a case of systemic diffuse large B-cell lymphoma in a 2-year-old female, which primarily occurred as suprapubic mass and later recurred in the form of isolated optic nerve infiltration, after remission of the primary disease. Early detection and prompt treatment resulted in complete reversal of the disease.
Traumatic subperiosteal hematoma (SpH) usually presents late, after the initial trauma. It is generally seen in young males. Computed tomography is the best mode of imaging and helps to rule out orbital fracture or associated subdural hematoma. We present the clinical features and management of four patients seen at the orbit clinic with SpH. Management is based on time of presentation, visual acuity and any communicating bleed. The prognosis of traumatic SpH is excellent if treated with an individualized patient approach.
Hyperimmunoglobulinemia E (Job's) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job's syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and Candida endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job's syndrome.
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