[Tumors of the lacrimal drainage system]. / Tumoren der ableitenden Tränenwege.

BACKGROUND: The prevalence of tumors affecting the lacrimal drainage system is low, thus generating a risk of late diagnosis and treatment in clinical routine. However, these tumors can be potentially life-threatening, which emphasizes the relevance of early diagnosis and treatment. OBJECTIVE: This review focuses on the symptoms, incidence, management, and prognosis of the different tumor entities affecting the lacrimal drainage system. METHODS: The study provides a PubMed-based literature review and presents own clinical results. RESULTS: Alongside detailed medical history taking and comprehensive clinical examination, precise inspection during external dacryocystography is important for diagnosis of tumors affecting the lacrimal drainage system. There is a wide spectrum of tumor entities located in the lacrimal drainage system. The tumors are classified into three groups: primary epithelial, primary nonepithelial, and inflammatory lesions. The most common primary epithelial tumors include papilloma, squamous cell carcinoma, and transitional cell carcinoma. The most common nonepithelial tumors include fibrous histiocytoma, malignant lymphoma, and malignant melanoma; while the most common inflammatory lesions comprise sarcoidosis, Wegener granulomatosis, and pyogenic granuloma. Treatment depends on the entity and stage of the tumor. In the case of malignancy, a multimodal and interdisciplinary approach is usually indicated. CONCLUSION: Differential diagnostic signs in favor of a malignancy include a long medical history, predisposing conditions in the patient's history, a mass above the medial canthal ligament, teleangiectasis above the mass, and serosanguinous secretion.

Imaging features of medulloepithelioma: report of four cases and review of the literature.

BACKGROUND: Intraocular medulloepithelioma is a childhood tumor arising from the nonpigmented primitive ciliary neuroepithelium. Although rarer than retinoblastoma, it remains the second most common primary intraocular neoplasm in children. The rarity of intraocular medulloepithelioma creates the challenge in establishing a clinical diagnosis, and radiologically the tumor is often confused with other intraocular masses. OBJECTIVE: To describe the clinical, imaging and pathological features of intraocular medulloepithelioma with emphasis on the role of imaging to enable its differentiation from more common intraocular pathology. MATERIALS AND METHODS: We retrospectively analyzed the clinical, histopathological and imaging data of four children with intraocular medulloepithelioma. RESULTS: All four children had medulloepithelioma arising from the ciliary body. The children were imaged with US (n = 3), MRI (n = 4), whole-body (99m)Tc-MDP scintigraphy (n = 2) and CT (n = 1). All four children had enucleation of the involved eye. One tumor was a malignant teratoid variant, two tumors were malignant nonteratoid variants and one was a nonteratoid variant of uncertain malignant potential. None of the tumors had extraocular extension on histopathology or imaging. Two children had associated retinal detachment on US and MRI examinations. All tumors were iso/hyperintense to vitreous on T1-weighted and hypointense on T2-weighted MRI and showed marked contrast enhancement of the solid components. No calcifications were identified on US or CT examinations. CONCLUSION: Our findings are consistent with previously reported cases of medulloepithelioma. This series emphasizes the roles of various imaging modalities, with pathological correlation, in differentiating the tumor from other ciliary body masses, in detecting tumor extension and in identifying associated ocular complications. In this series we also describe the results of postsurgical follow-up for tumor recurrence.

The effect of intralesional steroid injections on the volume and blood flow in periocular capillary haemangiomas.

AIM: To examine the effect of steroid therapy on the volume estimates and blood flow characteristics of childhood periorbital capillary haemangiomas. PATIENTS AND METHODS: Children at risk of amblyopia due to periorbital haemangiomas were treated with intralesional steroid injections (between 1 and 4 courses) and serial assessment of the volume and blood-flow characteristics of the lesions measured using colour Doppler ultrasonography. The characteristics of the haemangiomas in these children were compared with a cohort of untreated cases. RESULTS: Eight of nine treated children were female, this proportion being significantly different from the equal sex distribution of an untreated cohort (p < 0.05). All children in the steroid-treated group presented within 1 month of birth, compared to the untreated children, who presented at an average of 2.1 months of age (range 0-14, median 2.9 months) (p = 0.04) and they required significantly longer follow-up in the Orbital service (mean 65 months, range 26-105), compared with an average of 35 months (range 4-92, median 23) in the untreated group (p = 0.002). The maximum estimated volume of the lesions were significantly larger in the treated group (treated group mean 8.9 ml, untreated group mean 4.1 ml; p = 0.016), with a trend towards higher maximum measured blood velocities in the treated group (treated mean 64 cm compared with untreated mean 52 cm; p = 0.1). Steroid injections appear to reduce the volume and blood flow of haemangiomas, this suppression persisting for several months (between 5 and 20) before the lesion later displays the cyclic fluctuations in volume and flow seen with untreated lesions. All treated haemangiomas had some residual vascular anomaly, detectable on ultrasonography, at last follow-up--this being despite absence of clinical signs in most cases. CONCLUSION: Periorbital capillary haemangiomas requiring steroid therapy for risk of amblyopia were significantly commoner in females, were larger lesions and presented at an earlier age. Intralesional steroids appear to cause a reduction of blood flow, with a transient reduction in volume and a suppression of the natural cyclic variation seen without treatment. The changes after a course of steroid therapy appear to last for between 5 and 20 months, this period of suppression of the lesion probably being particularly useful during infancy and early childhood when the child is at greatest risk of amblyopia.

[Posttraumatic hyphema?]. / Hifema posttraumatica?

The authors present the case of a patient with ocular pain and visual impairment after an ocular trauma. The ophthalmologic exam and the complementary exams show atypical aspects of the traumatic ocular pathology.

Simultaneous appearance of acute myeloid leukemia in a patient with bilateral primary uveal melanoma.

We report a rare case of a patient with bilateral uveal melanoma (the first eye surgically treated 6 years before the occurrence of melanoma in the contralateral eye), who developed an acute myeloid leukaemia.

[Cavernous hemangioma of the optical canal and superior palpebral fissure].

The paper describes a rare case of cavernous hemangioma spreading to the optical canal and superior palpebral fissure. The distinctive feature of the case is the location of cavernous hemangioma in anterior two thirds of the optical canal and superior palpebral fissure. In this connection, despite small sizes of a neoplasm, it caused a significant decrease in visual function during 10 months. The first sign of the disease within 10 months before surgery was periodic diplopia, which served as an indication for magnetic resonance imaging that revealed a pathological process and a correction decision was taken to follow up the patient due to visual preservation. Worse vision served as an indication for surgery as further follow-up might result in irreversible sequels. Undertaken active policy proved its worth despite its risk. The used respective supraorbital access permitted radical removal of a neoplasm to recover visual functions.

Ultrasonographically guided injection of corticosteroids for the treatment of retroseptal capillary hemangiomas in infants.

PURPOSE: Injection of corticosteroids is a well-documented and successful mode of treatment for periorbital capillary hemangiomas. Because of the greater potential risk involved with retrobulbar injections, no prior study has described this treatment for tumors located behind the orbital septum. Although retroseptal intraorbital capillary hemangiomas comprise only 7% of all adnexal capillary hemangiomas, complications such as optic nerve compression or astigmatism may necessitate treatment. METHODS: Three patients with deep orbital hemangiomas that caused vision-threatening complications were treated with intralesional injections of triamcinolone and betamethasone. Orbital injection was performed with use of real-time ultrasonographic guidance of the needle. This technique was valuable in providing continuous, accurate, and safe advancement of the needletip in the orbit to avoid the globe and orbital walls. Ultrasonography also permitted precise placement of the needle tip within the tumor and visualization of the injected material. RESULTS: Significant improvement was demonstrated in all cases on the basis of both ultrasonographic measurements and regression of clinical manifestations such as astigmatism, chemosis, proptosis, and optic nerve pallor. No complications were noted. CONCLUSION: Intralesional injection of corticosteroids to treat retroseptal and retrobulbar capillary hemangiomas was found to be a safe and effective treatment modality in our patients. Positioning of the injecting needle was guided by ultrasonography.

Symptoms and time to presentation and treatment in ocular melanoma: the Western Canada Melanoma Study.

Reports on all ocular melanomas newly diagnosed between Apr. 1, 1979, and Mar. 31, 1981, were obtained from the cancer registries of British Columbia, Alberta, Saskatchewan and Manitoba. Of the 90 cases, 87 were in patients aged 20 to 79 years, 64 of whom were interviewed about their initial symptoms. Three symptoms--loss of part of the visual field (in 33% of patients), photopsia (in 20%) and blurred vision (in 20%)--emerged as the main indicators of disease. In 17% of cases the tumour was discovered as an incidental finding on ocular examination. The length of delay before consulting a physician was generally short in symptomatic patients, with 85% reporting that they saw a physician within 3 months of onset of the first symptom. Of the 30 patients who received definitive treatment 4 months or more after initial presentation, 13 were seen by general practitioners who delayed before referring the patient to an ophthalmologist. Continuing medical education is needed to ensure earlier referral to ophthalmologists by general practitioners of patients with ocular melanoma.

[Hepatic metastases in CUP (cancer of unknown primary) and painful amaurosis]. / Hepatische Filiae bei CUP ("cancer of unknown primary") und schmerzhafter Amaurose.

A 63-year-old woman presented with a 9-month history of painful amaurosis of the left eye caused by hemophthalmos. She was already undergoing chemotherapy for histopathologically proven liver metastases caused by an assumed melanoma. The location of the primary tumor was unknown. Whole-body FDG-PET/CT staging identified a lesion with characteristically malignant uptake in the region of the left eye as the primary tumor. After enucleation, a primary uveal melanoma was verified that expressed the serological marker melanoma inhibitory activity (MIA). FDG-PET/CT was able to successfully align hepatic metastases due to CUP with local ophthalmologic diagnostic findings and led to a correct diagnosis.

Ocular melanoma presenting as a case of panophthalmitis in a 78-year-old Nigerian woman (masquerade syndrome): an uncommon finding in Benin City, Nigeria.

Theodore states that the term 'masquerade syndrome' first appeared in the ophthalmic literature in 1967 to describe a conjunctival carcinoma that presented as chronic conjunctivitis. Since then, the masquerade syndrome label has been applied to a group of disorders that mimic ocular inflammatory disease. Although some benign conditions can be considered masquerade syndromes, most often the term refers to malignant entities. Tsai and O'Brien also report that ocular oncology, the subspecialty that concerns itself with ocular and orbital malignancies, is by nature a field of uncommon disorders. A discussion of the masquerade syndrome therefore becomes a study in uncommon presentations of uncommon diseases. Nevertheless, the subject deserves wide attention because, in many cases, the diseases that masquerade are not only vision-threatening but potentially fatal. Significant morbidity and mortality can be averted by early recognition and diagnosis of the masquerade. In this article, we present a case of a 78-year-old Nigerian woman who had an ocular melanoma that presented as a panophthalmitis, a rare case of masquerade syndrome in our environment.

[Intraocular non-Hodgkin's lymphoma and its therapy-- a case series of ten patients]. / Das intraokulare Non-Hodgkin-Lymphom und seine Therapie -- eine Fallserie mit 10 Patienten.

BACKGROUND: The timely and correct diagnosis of intraocular non-Hodgkin's lymphoma represents a huge challenge to clinicians. Two thirds of intraocular lymphomas are a manifestation of a primary CNS lymphoma (PCNSL) arising outside the lymphatic system and are localized in the brain, the meninges or the spinal chord. Ten to twenty percent commence as vitreous or retinal infiltrates mimicking uveitis. Ninety five percent of PCNSL are B cell lymphomas. PATIENTS: Three exemplary cases from a group of ten patients treated between 1998 and 2002 are presented. A table provides a summary of the relevant details of all ten patients. RESULTS: The mean age at presentation was 63.5 years with a female to male ratio of 6 to 4. Nine patients were diagnosed as having intermediate or posterior uveitis, in one patient choroidal metastases were suspected. Six patients had a concomitant CNS lesion while four patients showed isolated intraocular lymphoma only. The presence of a highly malignant B cell lymphoma was proven by vitreous biopsy in nine cases and by stereotactic biopsy of a CNS lesion in one patient. All patients were treated by intravenous chemotherapy, however, no binding recommendations with regard to treatment exist to date. CONCLUSIONS: We give an overview of all current treatment regimens and their pitfalls. At present it is recommended that all patients with proven PCNSL be entered in a multicenter randomized study under the auspices of the Department of Internal Medicine III of the Benjamin-Franklin-University-Hospital, Berlin and the Department of Neurology of the University Hospital of Tuebingen.