Rachna Meel

Invasive Aspergillosis of Orbit in Immunocompetent Patients: Treatment and Outcome

OBJECTIVE Invasive sino-orbital aspergillosis in immunocompetent patients is a rare clinical entity and often misdiagnosed. Invasive sino-orbital aspergillosis is potentially fatal; however, mortality may be avoided with timely treatment. Presently, there are no universal guidelines for its treatment. We present the clinical features, treatment, and outcome in 15 cases of invasive sino-orbital/orbital aspergillosis in healthy individuals. DESIGN Retrospective case series. PARTICIPANTS Fifteen cases of invasive sino-orbital/orbital aspergillosis in immunocompetent individuals. METHODS We retrospectively analyzed all patients with sino-orbital/orbital aspergillosis who were treated at the Dr. Rajendra Prasad Center for Ophthalmic Sciences between June 1999 and September 2009. Diagnosis was based on histopathologic documentation of tissue invasion by septate fungal hyphae with acute angle branching and presence of granulomatous inflammation. MAIN OUTCOME MEASURES Treatment outcome in terms of patient survival, resolution of symptoms, and resolution of orbital mass on imaging. RESULTS Fifteen patients (11 male and 4 female) were studied. Mean age of patients was 35 years. One patient declined treatment. Surgery was performed for orbital disease in 6 patients. Medical therapy consisted of intravenous amphotericin B followed by itraconazole in 10 patients, itraconazole alone in 2 patients, and voriconazole followed by itraconazole in 2 patients. Eleven patients are alive without recurrence at a median follow-up of 20 months. Of these patients, 5 had residual mass on imaging at last follow-up. One patient died of progressive disease. CONCLUSIONS This is a large case series of orbital aspergillosis reported in healthy individuals. The role of orbital surgery is not clear in treatment of this entity and requires further evaluation. Newer antifungal agents alone seem to be effective in controlling the infection.

Clinical predictors of high risk histopathology in retinoblastoma.

Previous studies show that clinical features at presentation, in retinoblastoma patients, like glaucoma and neovascularization of iris are associated with a higher incidence of high risk histopathology findings (HRF) in enucleated eyes. Herein, we analyze association between clinical features at time of enucleation and occurrence of HRF including invasion of anterior chamber, iris, ciliary body, choroid (massive), sclera, extrascleral tissue, optic nerve beyond lamina cribrosa, and optic nerve cut end, in a large series of eyes enucleated for retinoblastoma.

A histopathologic analysis of eyes primarily enucleated for advanced intraocular retinoblastoma from a developing country.

CONTEXT In eyes enucleated for retinoblastoma, presence of histopathologic high-risk factors is associated with a higher risk of local recurrence and systemic metastasis. OBJECTIVE To evaluate histopathologic features in children with retinoblastoma in our population and establish relationship between age, tumor differentiation, and high-risk features. DESIGN Retrospective histopathologic analysis of 609 consecutively enucleated eyes for advanced intraocular retinoblastoma during a 10-year period. A nonparametric test was used to establish relationship between age, differentiation, and high-risk features. RESULTS Poorly differentiated retinoblastoma presented in 80.3% and well-differentiated in 19.7% of eyes. Well-differentiated tumors presented earlier (median 1.2 years) than poorly differentiated tumors (median 2.5 years) (P < .001). One hundred fourteen eyes (18.7%) had 1 and 138 (22.7%) had at least 2 high-risk histopathologic factors. Invasion of anterior chamber was found in 10.0%, iris in 10.7%, ciliary body in 6.7%, sclera in 13.7%, massive choroid in 24.6%, postlaminar optic nerve in 16.1%, resected margin of the optic nerve in 7.4%, and extrascleral tissue in 4.1% of eyes. Extensive necrosis was seen in 31.0% of eyes. Poorly differentiated tumors were significantly associated with presence of more than 1 high-risk histopathologic feature (P < .001) and extensive necrosis (P < .001). CONCLUSION Poorly differentiated tumors present at a later age and are associated with presence of multiple high-risk factors and extensive necrosis. In our population, high-risk histopathologic factors are present in a significant number of eyes. Because we have included only primarily enucleated eyes, this could truly represent the distribution of high-risk histopathologic factors in children with retinoblastoma.

Current therapy and recent advances in the management of retinoblastoma.

Retinoblastoma is the most common intraocular malignancy in children. The survival of retinoblastoma patients and visual outcome has improved dramatically in the developed world. This can be attributed to early tumor recognition and advances in the management of retinoblastoma. Chemoreduction followed by adjuvant consolidative treatment has replaced external beam radiotherapy as the primary modality of treatment for intraocular retinoblastoma. Further, histopathological high-risk factors have been identified in enucleated eyes, allowing use of prophylactic chemotherapy to take care of possible micrometastasis. The survival in case of extraocular retinoblastoma is still low, and the reported survival rate ranges between 50% and 70%. In developing countries, the overall survival of retinoblastoma patients remains low, primarily due to a delayed presentation, resulting in larger proportions of extraocular disease compared with the developed world, where majority of the disease is intraocular. Greater efforts need to be directed toward early tumor recognition in order to improve the survival of retinoblastoma patients in the developing world. In this article, we provide an overview of the current clinical management of retinoblastoma.

Orbital schwannoma with cystic degeneration.

Background:  Schwannoma is a benign, slowly growing, painless, peripheral nerve sheath tumour that is uncommon in orbit. Longstanding tumours may develop degenerative changes. Extensive cystic change in orbital schwannoma is quite rare. We conducted this study to analyse the cases of orbital schwannoma, which were predominantly cystic and describe their clinical, imaging and histopathological features.

Therapy and outcome of orbital primitive neuroectodermal tumor.

Primary orbital primitive neuroectodermal tumor (PNET) is rare with no reported series. We report six cases of orbital PNET treated at a tertiary care oncology center in northern India from 2003 to 2008. None of them had distant metastases. All were treated with neoadjuvant chemotherapy followed by exenteration in two, radiotherapy and adjuvant chemotherapy in five cases. Three out of six achieved complete remission at end of therapy with globe salvage in three and vision in two cases. Chemoradiotherapy may help us to avoid mutilating surgery in large or locally advanced tumors, allowing preservation of vision or the globe. Pediatr Blood Cancer 2009;52:544–547.

Treatment and outcome of nonmetastatic extraocular retinoblastoma with a uniform chemotherapy protocol.

Nonmetastatic extraocular retinoblastoma is a common entity in South-East Asia. We did a retrospective study of patients treated for isolated extraocular retinoblastoma, that is, International retinoblastoma staging system stages II and III, using a uniform chemotherapy protocol at our oncology center, between June 2003 and June 2008. Out of the 25 patients having nonmetastatic extraocular retinoblastoma, 6 were in stage II, and 19 in stage III. The probability of event-free survival was 0.54 at 18 months of follow-up with no significant difference between the 2 stages and the presence of optic nerve involvement; 11 out of 13 relapses were systemic. This is the largest case series of nonmetastatic extraocular retinoblastoma from South-East Asia.

Bone marrow aspirations and lumbar punctures in retinoblastoma at diagnosis: correlation with IRSS staging.

BackgroundFew studies have suggested that routine bone marrow (BM) and cerebrospinal fluid (CSF) evaluation is not needed in all patients with retinoblastoma. Although the International Retinoblastoma Staging System was developed recently, there remain no clear stage-specific guidelines with regard to the need for BM and CSF evaluations as part of the metastatic workup for patients with retinoblastoma. MethodsThis is a retrospective study analyzing results of CSF cytology and BM evaluation at presentation/diagnosis in patients with retinoblastoma registered at our center from June, 2003 to June, 2009. Only cases with both BM biopsy and lumbar puncture were included. ResultsOf the 259 evaluable patients, 18 (7%) were positive for metastasis either in CSF and/or BM. Although 7 of these had overt metastasis at presentation (stage IV), 1 belonged to stage II and 10 to stage IIIa. No stage 0 or I patient tested positive despite presence of histopathologic high-risk factors. ConclusionsThis is the largest study evaluating the role of metastatic workup in patients with retinoblastoma at presentation. We conclude that CSF and BM evaluation is not required in patients with stage 0 and I retinoblastoma at presentation. No definitive recommendation could be made for stage II patients; while stage III and IV patients must certainly be evaluated.

Orbital teratoma: a rare cause of congenital proptosis

be associated with other ocular conditions like retinitis pigmentosa. Orellana et al. hypothesized that cysts arise from displacement and proliferation of the pigment epithelium originating from the pars plana and not from the iris. Asiyo-Vogel et al. postulated that the cysts formed from displaced retinal tissue, after retinal detachment surgery. In our patient the iris transillumination suggested that the iris cyst was formed from tissue originating from the iris root and dislodged into the vitreous cavity probably through the zonules; this was confirmed histopathologically. We believe that the occurrence of the full thickness macular hole was completely coincidental. Dislodged intravitreal iris cysts are a rare entity usually asymptomatic. Because of their rarity they may represent a diagnostic challenge to the ophthalmologist, especially when combined with other ocular disease.

Childhood osteosarcoma of greater wing of sphenoid: case report and review of literature.

Primary osteosarcoma of skull base is extremely rare. We present a case of primary osteosarcoma arising in greater wing of sphenoid in a child. Our patient had an incomplete excision after which he received adjuvant chemotherapy and radiotherapy. There was good response to adjuvant chemoradiotherapy and the patient is disease free at a follow-up of 18 months. Treatment of skull base osteosarcomas is difficult, as complete excision is often not possible. To the best of our knowledge, this is the first case of sphenoid wing osteosarcoma in childhood to be reported in literature.