P. Nicholas Plowman

Choroidal Hemangiomas: Visual and Anatomic Results of Treatment by Photocoagulation or Radiation Therapy

PURPOSE Choroidal hemangioma is a benign hamartoma that causes accumulation of subretinal fluid and resultant visual loss. Although photocoagulation can result in resolution of subretinal fluid, some have found that recurrence is common and final visual acuity often is poor. The purpose of this study is to evaluate the visual and anatomic results of radiation therapy and photocoagulation in treating patients with visual loss from choroidal hemangiomas. METHODS A retrospective review was performed of patients with circumscribed choroidal hemangiomas (CCH) or diffuse choroidal hemangiomas (DCH) treated for visual loss caused by accumulation of subretinal fluid. Of 23 patients with CCH, 13 were treated by photocoagulation, 8 by plaque brachytherapy, and 2 by lens-sparing external beam radiation therapy (LSRT). All five patients with DCH were treated by LSRT. RESULTS Of patients with CCH treated by brachytherapy, six (75%) of eight had visual acuity of 6/12 or better at 1 year and 8 (100%) of 8 had no subretinal fluid. Of patients with CCH treated by photocoagulation, 5 (38%) of 13 had visual acuity of 6/12 or better at 1 year and 6 (46%) of 13 had no subretinal fluid. Of patients with CCH treated by LSRT, none of two had visual acuity of 6/12 or better at 1 year and one of two had no subretinal fluid. Of the five patients with DCH treated by LSRT, all had complete resolution of subretinal fluid. Two had marked visual improvement and in the other three, vision was stabilized. CONCLUSIONS Plaque brachytherapy is an effective alternative to photocoagulation for treatment of subretinal fluid caused by CCH. Lens-sparing external beam radiation therapy is effective treatment in patients with DCH.

Chemotherapy in metastatic retinoblastoma

Eleven children with metastatic retinoblastoma diagnosed during the period 1970-1984 were treated with chemotherapy. Short-term complete responses were observed in three children treated with a four-drug combination which included cisplatinum, and in one child treated with vincristine and cyclophosphamide. The median duration of survival of the 11 children receiving chemotherapy was nine months, whilst the median survival of 13 children with metastatic retinoblastoma who were not given chemotherapy was only 2.3 months (p = 0.06). This suggests that retinoblastoma is a chemosensitive tumour and therefore adjuvant chemotherapy may have a role in children with retinoblastoma who at diagnosis are thought to be at high risk of developing metastatic disease.

Tamoxifen as adjuvant therapy in breast cancer. Current status.

Tamoxifen retains its place as the most important drug in the management of human breast cancer. As time passes, it becomes clear that this drug is no longer to be regarded as a simple antiestrogen, since it modifies other cellular signalling mechanisms, giving scientific credence to the growth inhibitory effects observed in estrogen receptor-negative tumours. With the role of tamoxifen in breast cancer being well documented, recent interest has concentrated on its now proven efficacy in adjuvant treatment of patients with the disease in its early stages, and on its prophylactic role in women at high risk of developing the disease. For tamoxifen to have any role at all in the latter situation, it must be demonstrably safe, and here again the toxicity profile is now much better understood.SummaryTamoxifen retains its place as the most important drug in the management of human breast cancer. As time passes, it becomes clear that this drug is no longer to be regarded as a simple antiestrogen, since it modifies other cellular signalling mechanisms, giving scientific credence to the growth inhibitory effects observed in estrogen receptor-negative tumours.With the role of tamoxifen in breast cancer being well documented, recent interest has concentrated on its now proven efficacy in adjuvant treatment of patients with the disease in its early stages, and on its prophylactic role in women at high risk of developing the disease. For tamoxifen to have any role at all in the latter situation, it must be demonstrably safe, and here again the toxicity profile is now much better understood.

Treatment of retinoblastoma vitreous base seeding.

OBJECTIVE The purpose of the study is to describe results of a new treatment for retinoblastoma vitreous base seeding. DESIGN The study design was a retrospective review of patients treated at an ocular oncology referral center. PARTICIPANTS Five eyes of five patients with vitreous base seeding that occurred after previous external beam radiation therapy were treated between October 1987 and December 1994. INTERVENTION A customized plaque made from iridium-192/platinum wire was placed to deliver 4000 cGy to the tumor apex along its entire length and systemic chemotherapy (consisting of carboplatin, vincristine, and etoposide) was given. MAIN OUTCOME MEASURES Eye preservation and tumor control were measured. RESULTS Vitreous base seeding was controlled completely and the eye preserved in four of the five treated eyes with an average follow-up of 26.2 months. CONCLUSIONS The combination of a customized iridium-192 plaque and systemic chemotherapy is an effective means of treating vitreous base seeding of retinoblastoma.

Advances in the Management of Paediatric Cushing’s Disease

Cushing’s disease (CD) is rare in the paediatric age range, but may present a difficult therapeutic challenge. Most paediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. Prior to definitive treatment, a diagnostic protocol for investigation is required which broadly follows the model for adult patients. Treatment strategies for CD are described and critically appraised. The management of paediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life.

Positron emission tomography in the diagnosis and management of intracranial germ cell tumours.

Positron emission tomography (PET) with 18F-fluorodeoxy-glucose indicates metabolically active tissue. When investigating enhancing intracranial tumours, we have suggested that PET positivity might suggest an intracranial germ cell tumour (IGCT). Here, we present a case with dicentric IGCT where PET was initially discordant between the lesions and where PET then became negative despite clearly aggressive clinical behaviour. A cautionary note is introduced with respect to the interpretation of negative 18F-FDG PET when investigating enhancing intracranial lesions.

Adjuvant Therapy in Breast Cancer Optimal Use in the Elderly

SummaryBreast conservation surgery may replace mastectomy in patients with small, localised breast tumours of up to 4cm in diameter. Clear surgical margins (microscopically defined) are desirable. Radiotherapy to the conserved breast is recommended for all patients, although it may be relatively safe to withhold it in some better-risk T1 patients. This decision must frequently be taken in the elderly and infirm. Adjuvant tamoxifen has an established place in the systemic management of all elderly patients with invasive breast cancer. In contrast, adjuvant chemotherapy has no established role in the elderly.

Stereotactic radiosurgery XX: ocular neuromyotonia in association with gamma knife radiosurgery.

Summary We report three patients who developed symptoms and signs of ocular neuromyotonia (ONM) 3–6 months after receiving gamma knife radiosurgery (GKS) for functioning pituitary tumours. All three patients were complex, requiring multi-modality therapy and all had received prior external irradiation to the sellar region. Although direct causality cannot be attributed, the timing of the development of the symptoms would suggest that the GKS played a contributory role in the development of this rare problem, which we suggest clinicians should be aware of as a potential complication. Learning points GKS can cause ONM, presenting as intermittent diplopia. ONM can occur quite rapidly after treatment with GKS. Treatment with carbamazepine is effective and improve patients quality of life.

Systemic Rituximab for Conjunctival Marginal Zone (Malt) Lymphoma is not Protective for Subsequent Disease Development in the Contralateral Eye

Purpose: We report a case of a 35-year old male who presented with unilateral conjunctival extranodal marginal zone (MALT) lymphoma and underwent systemic Rituximab immunotherapy as primary treatment. After an excellent initial response and four years of complete remission he presents with MALT lymphoma to the contralateral conjunctiva. Methods: MALT lymphoma is the most common lymphoid neoplasm of the conjunctiva, and has an indolent clinical course. Rare cases of spontaneous remission, bilateral involvement, systemic dissemination, and common local and contralateral relapses have been reported. Rituximab is a chimeric anti-CD20 antibody, currently used as first-line treatment of CD20 positive non-Hodgkin’s lymphoma. Several mechanisms can be responsible for rituximab resistance. Transformation of CD20 positive indolent to aggressive CD20 negative form is one of them. Results: In this case, the patient did not demonstrate any systemic or orbital involvement. Both histological reports of the incisional biopsies revealed CD20 positive immunohistochemistry. Conclusion: This is a highly unusual case of new primary conjunctival MALT lymphoma to the contralateral eye after systemic Rituximab treatment. This further supports the assumption that rituximab does not suppress MALT lymphoma formation in the long term in the other eye, suggesting that the predisposing immune factors are not annihilated by the course of rituximab. Long term review of these patients is therefore warranted.