[Malignant medulloepithelioma mimicking retinoblastoma - clinical and morphological case analysis and cell culture experience]. / . Zlokachestvennaya medulloepitelioma, simuliruyushchaya retinoblastomu ­ kliniko-gistologicheskii analiz sluchaya i opyt polucheniya kletochnoi kul'tury.

The paper presents clinical and morphological case analysis of primary intraocular malignant medulloepitelioma (medulloblastoma) of rare localization (central part of the retina with optic nerve involvement) that simulated retinoblastoma in a 2-year-old child. Histological features of the tumor are given in details (tubular and mesh structures of the tumor, rosettes, ribbons, cells with hyperchromic nuclei, and cellular polymorphism). An experience of creating a primary intraocular malignant medulloepitelioma cell culture, as yet exclusive in the Russian Federation, is described. Culture sensitivity for particular drugs (oxaliplatin, irinotecan, ifosfamide, and ascorbic acid at different concentrations) was evaluated by MTT-assay. Of the four products, IC50 (3.3 mg/ml) was obtained only for ascorbic acid. Despite the relative rarity of primary intraocular malignant medulloepitelioma, its differential diagnosis should be carried out, with retinoblastoma in mind in the first place. The obtained data on the effectiveness of ascorbic acid against intraocular malignant medulloepitelioma cells can be used to supplement the existing chemotherapeutic protocols in pediatric ocular and neuro-oncology.

Retrospective comparison of the effectiveness of various treatment modalities of extragastric MALT lymphoma: a single-center analysis.

We have performed a retrospective analysis of all patients with extragastric mucosa-associated lymphoid tissue (MALT) lymphoma treated at our institution to compare the efficacy of first-line therapeutic modalities including surgery, radiation, systemic therapy, and antibiotics. One hundred eighty-five patients with extragastric MALT lymphoma with a median age of 63 (interquartile range (IQR) 50-74) years and a median follow-up time of 49 (IQR 18-103) months were retrospectively analyzed. Time to progression and time to next therapy were used as surrogate endpoints for efficacy. Patients having either surgery (100 %), chemo/immunotherapy (85.5 %), or radiation (80 %) had significantly (p = 0.01) higher response rates than patients treated with antibiotics (33.3 %). Patients who were irradiated had significantly more progressive disease, but also the longest follow-up time. Stage, elevated LDH, anemia, elevated beta-2 microglobulin, plasmacytic differentiation, monoclonal gammopathy, or autoimmune disease did not influence the rate of disease progression nor did complete remission or partial remission from initial therapy influence time to and rate of progression. There was no significant difference in the median time to progression (p = 0.141), but the estimated time to progression (p = 0.023) as well as the estimated time to next therapy (p = 0.021) was significantly different among the various cohorts favoring surgery, chemo/immunotherapy, and radiation. Our results suggest extragastric MALT lymphoma as a potential systemic disease irrespective of initial stage. Radiation, surgery, and chemo/immunotherapy seem to be equally effective in achieving remissions and prolonged progression free survivals, but a curative potential is questionable. Localized MALT lymphomas affecting the thyroid gland or the lungs have excellent long-term progression-free survivals with surgical treatment only.

Survey on the management of orbital and intraocular tumors among oculofacial surgeons in the Asia-Pacific region.

A web-based anonymous survey was performed to assess the common practices of oculofacial surgeons in the management of orbital and intraocular tumors in the Asia-Pacific region. The questionnaire comprised a series of questions covering controversial topics sent via email to 131 oculofacial surgeons across 14 countries in the Asia-Pacific region. A total response rate of 61.7 % was achieved from May to December 2012. The most common benign orbital tumor was cavernous hemangioma (39.6 %) and the most common malignant orbital tumor was lymphoma (85.7 %). 40 % of surgeons recommended orbital radiation, for which the most common indications were thyroid eye disease (70.0 %) and malignancy (30.0 %). The most common orbitotomy approach was lateral (79.2 %). Most surgeons (87.1 %) offered enucleation for retinoblastoma, but there was also a significant proportion that offered chemoreduction with transpupillary thermotherapy or cryotherapy (58.1 %). Fewer surgeons offered brachytherapy (16.1 %) and intra-arterial chemotherapy (6.5 %). When performing enucleation for retinoblastoma, 81.8 % of surgeons performed a primary orbital implant placement. The most preferred type of implant was silicone/acrylic (90.2, 90.2 and 87.8 % for elderly, adults and children, respectively). The majority of surgeons used donor sclera (57.5 %) or no wrapping material at all (32.5 %). Almost all surgeons (95.1 %) did not drill and peg the implant for motility. We report the results of the first survey of oculofacial surgeons in the Asia-Pacific region on the management of intraocular and orbital tumors. In comparison with previous surveys performed in the USA and the UK, we found the practice patterns of the Asia-Pacific surgeons to be comparable.

Anesthesia for ophthalmic procedures in the standing horse.

Effective delivery of local anesthesia is essential for successful standing surgical procedures in the horse. Local anesthesia can be used to facilitate examination of the eye, diagnostic procedures, therapeutic techniques, and surgical procedures. Understanding the relevant clinical anatomy and techniques for performing local anesthesia is critical to delivering successful local and regional anesthesia and analgesia.

Predictors of ocular surface squamous neoplasia recurrence after excisional surgery.

PURPOSE: To identify predictors of ocular surface squamous neoplasm (OSSN) recurrence after operative resection. DESIGN: Retrospective case series. PARTICIPANTS: Three hundred eighty-nine consecutive patients who underwent excisional biopsy for OSSN lesions at the Bascom Palmer Eye Institute from January 1, 2001, to September 20, 2010. METHODS: Review of pathology records and patient charts. MAIN OUTCOME MEASURES: Identification of factors predictive of OSSN recurrence. RESULTS: Of 389 excised OSSN lesions, 44 recurred during follow-up. The 1-year recurrence rate was 10% and the 5-year recurrence rate was 21%, with a mean time to recurrence in those with a recurrence of 2.5 years (standard deviation, 3.4). Using the American Joint Committee on Cancer (AJCC) clinical staging system, T3 and T2 lesions portended a higher risk of recurrence compared with T1 (T2/T1 hazard ratio [HR], 2.05 [P = 0.04]; T3/T1 HR, 2.31 [P = 0.07]). In addition, a location characteristic that increased the risk of tumor recurrence was tarsal involvement (AJCC T3 stage lesion; HR, 4.12; P = 0.007). Nasal location was associated with a decreased risk of tumor recurrence (HR, 0.41; P = 0.008). Pathologic characteristics significantly associated with tumor recurrence were the presence of positive margins (HR, 2.73; P = 0.008) and higher grade lesions (carcinoma in situ and squamous cell carcinoma versus dysplasia; HR, 2.55; P = 0.02). Treatment with adjuvant cryotherapy significantly decreased the risk of tumor recurrence (HR, 0.51; P = 0.03). In those patients with positive margins, the use of postoperative topical interferon therapy lowered the recurrence rate to a level similar to that of patients with negative margins. CONCLUSIONS: Certain patient and tumor factors are associated with a higher risk of OSSN recurrence after operative excision, such as tarsal tumor location and positive surgical margins. Postoperative adjuvant therapy should be considered in patients with high-risk OSSN characteristics.

Adjuvant 5-fluorouracil in the treatment of localised ocular surface squamous neoplasia.

AIM: To report the outcome of treatment of localised non-invasive ocular surface squamous neoplasia (OSSN) where topical 5-fluorouracil (5-FU 1%) is used as an adjuvant to surgical excision. METHODS: The study was a prospective non-comparative interventional case series. 55 cases of primary localised OSSN and 10 cases of recurrent localised OSSN were treated with excision, cryotherapy and adjuvant 5-FU 1%. In all cases, irrespective of the involvement of surgical margins, a single cycle of 5-FU 1% was administered, four times a day for 2 weeks continuously. The main outcome measures were (1) recurrence and (2) complications related to adjuvant topical chemotherapy. Individuals were reviewed for a minimum of 12 months. RESULTS: There was a single case of recurrence (1.5%). 57% of patients had short-term complications secondary to 5-FU 1%, most frequently lid toxicity (49%), followed by superficial keratitis, epiphora and corneal epithelial defects. Four patients were unable to complete the course of 5-FU 1% because of local toxicity. CONCLUSIONS: The management of localised non-invasive OSSN with adjuvant 5-FU 1% was associated with a low rate of local recurrence. Although 5-FU 1% frequently results in short-term complications, most commonly lid toxicity, a full course is usually tolerated. Serious complications appear uncommon in the treatment of localised disease.

[Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old girl]. / Carcinome adénoïde kystique de la glande lacrymale chez une enfant âgée de 14 ans.

The discovery of unilateral exophthalmia requires multidisciplinary care made all the more difficult in the case of a 14-year-old girl living in a tropical environment. The child, C.A., resident of the town of Djenné in Mali, was examined for nonpulsate, nonretractile, left lateral unilateral exophthalmia, painful upon palpation of a left upper-external mass under the orbital rim. The initial examination highlighted the existence of an intraorbital tumor with a moderate effect on visual function. In view of the slow and progressive development over 2 years, and with the assistance of an association, the child was transferred to a specialized medical structure where the tumorous nature was confirmed after surgical exeresis. The diagnosis was cylindroma, or adenoid cystic carcinoma. Neither chemotherapy nor radiation were used. At 15 months after surgery, the child showed no clinical sign of recurrence. Intraorbital tumors in children present diagnostic and therapeutic problems in countries with limited medical resources. Cylindromas or adenoid cystic carcinomas of the lachrymal gland are the second most common cause of epithelial tumors in this gland, which are characterized by a high degree of malignancy, a very high rate of recurrence, and a low survival rate at 5 years. In a tropical environment, diagnostic and therapeutic difficulties are compounded by limits to comprehensive care for the child.

Orbital reconstruction: bone-anchored implants.

The authors present their experience on reconstruction by extraoral bone-anchored implants technique, showing two patients from a total of 13, treated for orbital reconstruction. They were selected from 66 patients treated for reconstruction of orbit, ear and nose using this technique by the staff of the Department of Plastic and Reconstructive Surgery of the University of Tor Vergata, Rome, Italy. They underwent orbital emptying due to cancer pathology. Surgical technique is described in detail; its final purpose being positioning of titanium fixtures on the orbital borders, necessary to anchor the silicone epithesis. The advantages and potential limits of this technique are exposed, exposing the important role of patient's compliance. The importance of preliminary study on the patients is also emphasized and includes clinical evaluation and instrumental examination for surgical planning, completed by a photographic study in the three standard projections.Satisfactory aesthetic results were obtained by the use of this technique and the authors believe it may represent a valid option; complementary to more traditional ones, but is a first choice in patients who cannot endure major surgery or in case of failure of traditional techniques.

The clinical characteristics and treatment results of ocular adnexal lymphoma.

PURPOSE: To assess the clinical pattern, the histopathological findings, the response to treatments, the recurrence pattern and the prognosis of malignant lymphoma in the ocular adnexa. METHODS: This study was performed on 22 total eyes from 17 patients who were diagnosed with ocular adnexal malignant lymphoma. We retrospectively analyzed the medical records for patient information including the histological classification based on age, the gender of each patient, the symptoms and signs at the initial diagnosis, the presence of binocular invasion, the findings of the surgical biopsy, the clinical stage of each patient's tumor, and the treatment methods used and their effectiveness. The mean follow-up period was 24.8 months. RESULTS: The mean age of patients studied was 46.8 years old. Six females and 11 males were included in the study. Fifteen cases consisting of 20 total eyes represented extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT). Five of seven patients (71.4%) whose lymphoma occurred within the conjunctiva relapsed after irradiation or chemotherapy, and four of the relapsed patients were salvaged with further therapy. CONCLUSIONS: Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) constituted 88.2% of all lymphomas involving the ocular adnexa. Lymphoma in the ocular adnexa responded well to conventional treatment, but the recurrence rate of lymphoma in the conjunctiva was significantly high.

Treatment of primary intraocular melanoma.

The treatment of intraocular melanoma has evolved recently. Enucleation has been superseded largely by brachytherapy, proton beam radiotherapy, stereotactic irradiation, trans-scleral local resection, transretinal resection and diode laser phototherapy. Many patients develop metastatic disease, which usually involves the liver and occurs hematogenously. Disseminated disease rarely responds to therapy, and is usually fatal within 1 year of the onset of symptoms. Uveal melanomas develop characteristic chromosomal abnormalities, such as loss of chromosome 3. This is associated with a reduction in the 5-year survival from approximately 95% to less than 50%.

[The organ-saving treatment of intraocular tumors (development trends)]. / Organosokhrannoe lechenie vnutriglaznykh opukholei (tendentsii razvitiia).

A trend towards an increase of malignant tumors observed during the recent 30-40 years, an unsatisfactory efficiency of enucleation as of a method applicable to treating such tumors and the designing of a modern technical basis stimulated a new trend in ophthalmooncology, i.e. organ-saving treatment. Outlined in the paper is an analysis of achievements of modern radiation treatment techniques and of local surgical extraction of tumors suggested both by Russian and foreign researchers; the methods are shown to be promising for future development.

MR imaging in retinoblastoma.

We studied the appearance of retinoblastoma on unenhanced and gadolinium-enhanced images and the accuracy of tumour staging with MR imaging. The MR images were obtained in 18 children with retinoblastoma and compared with histopathological findings after enucleation. The MR imaging included T1-weighted and dual-echo T2-weighted images before, and T1-weighted images after, gadopentetate dimeglumine injection. The contrast between tumour and ipsilateral vitreous strongly increased (57 %) after gadolinium on T1-weighted images (p = 0.004). Tumour was strongly hypointense as compared with ipsilateral vitreous in all patients using heavily T2-weighted (TE = 120 ms) images (p = 0.001). The estimated T2 of tumour (mean 96 + 14 ms) did not correlate with histological grading or degree of calcification. Unenhanced T1-weighted MR images rightfully excluded extrascleral growth in 16 of 16 cases, but its presence was confirmed after enucleation in only one of 2 abnormal MR scans. Invasion of the optic nerve behind the cribriform plate was confirmed in 2 of 3 abnormal gadolinium-enhanced MR scans, but also in 1 of the 15 cases in which MR images were normal. The T2-weighted images were useful in assessing retinal detachment. We conclude that heavily T2-weighted images, unenhanced T1-weighted images and gadolinium-enhanced T1-weighted MR images are complementary in characterizing and staging retinoblastoma.

Topical 5-fluorouracil in treating epithelial neoplasia of the conjunctiva and cornea.

PURPOSES: To evaluate the efficacy of topical 5-fluorouracil (5-FU) in treating conjunctival and corneal epithelial neoplasia. METHODS: Three patients underwent surgical excision of bulky disease followed by topical 1% 5-FU in artificial tear base for 2 to 3 weeks or until epithelial separation occurred. An additional three patients underwent treatment with topical 1% 5-FU alone. RESULTS: Minimum follow-up was 6 months. In one patient with conjunctival in situ carcinoma and no detectable normal conjunctiva, who had normal findings on conjunctival histologic examination after application of topical 5-FU, a focus of invasive disease requiring orbital exenteration. One patient had a favorable response to 5-FU therapy but required a repeat excision for control of bulky disease. Four patients have remained disease-free for 10, 13, 18, and 30 months after topical 5-FU therapy. CONCLUSION: With its potential selective toxicity on dysplastic epithelium, topical 5-FU shows promise as an adjunctive treatment for managing conjunctival and corneal epithelial neoplasia.

Computer model of ultrasonic hyperthermia and ablation for ocular tumors using B-mode data.

Computer simulations have been conducted to examine hyperthermia and ablation for treating ocular tumors. An interactive software package has been implemented that permits relevant tissue dimensions to be determined from B-mode data. This package also permits interactive beam positioning, and it provides image displays depicting computed absorbed doses and temperature rises. Results are presented showing how hyperthermia temperature patterns are influenced by beam position, beam geometry and frequency. Images showing ablative temperature rises at various time intervals are also presented. For hyperthermia, geometric models of beam profiles showed that a non-uniform beam pattern (with a central low-intensity region) can produce more uniform heating of small ocular tumors than a beam with a uniform intensity profile. For a given tumor, the uniformity of hyperthermia temperatures was found to be a function of frequency, with 4.75 MHz providing reasonably uniform results for typical tumor heights (near 7 mm). For ablation, diffraction computations were employed to calculate beam intensity profiles; results show an initially rapid rise in temperature levels with subsequent, slower heating beyond the -3-dB limits of the focal volume. The model is now being refined, and additional phenomena, including nonlinear propagation, will be incorporated.

Ophthalmic procedures and surgery in the standing horse.

Although many ophthalmic procedures can be performed on the standing horse, the decision to perform a procedure under sedation rather than general anesthesia must be made on the basis of the temperament of the horse, severity of the injury, and skill of the veterinarian. For example, veterinarians who are not comfortable with small suture material may find accurate repair of an eyelid laceration easier after general anesthesia rather than attempting to handle this suture in a moving patient. Correct use of nerve blocks and topical anesthesia will greatly facilitate examination, diagnosis, and treatment, and, in addition, will serve to prevent further damage to the eye during these procedures.