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Biomedical subjects

Ilhan Günalp

Publications and source records attributed to Ilhan Günalp.

8 recordsLinked to original sources

Metastatic retinoblastoma clinical features, treatment, and prognosis.

PURPOSE: To evaluate the clinical features, treatment, and prognosis in patients with metastatic retinoblastoma. DESIGN: Retrospective interventional case series. PARTICIPANTS: Eighteen consecutive patients with metastatic retinoblastoma who were diagnosed and managed at the Oncology Service of Ankara University, Turkey, between January 1999 and January 2005. METHODS: All patients underwent magnetic resonance imaging (MRI) of the orbit and brain, lumbar puncture, bone marrow aspiration, and bone scintigraphy for metastatic evaluation. Histopathologic confirmation of retinoblastoma via enucleation, exenteration, or orbital biopsy was obtained in each patient. The status of extraocular spread (optic nerve, extrascleral extension, or both) was assessed based on histopathologic or MRI results. Systemic treatment for metastatic retinoblastoma consisted of chemotherapy and radiotherapy (craniospinal, orbital, or both), if necessary. MAIN OUTCOME MEASURES: Status of extraocular spread, site of metastasis, and survival from metastatic retinoblastoma. RESULTS: At presentation, the mean patient age was 45 months (range, 13-86). Ten patients had unilateral retinoblastoma, 7 had bilateral retinoblastoma, and 1 had trilateral retinoblastoma. All patients with metastatic retinoblastoma had histopathologic or MRI evidence of unilateral extraocular disease characterized by optic nerve involvement, extrascleral extension, or both. Nine of 18 patients experienced central nervous system (CNS) involvement, 5 patients had distant and CNS metastasis, and 4 patients had distant metastasis only. Fourteen patients underwent craniospinal irradiation and 12 had orbital irradiation. At a mean follow-up of 24 months (range, 4-62), all patients with CNS and concurrent distant and CNS metastasis were deceased. Four patients who had distant metastasis only were alive at a follow-up ranging from 9 to 62 months. CONCLUSIONS: The prognosis for metastatic retinoblastoma is dismal and the presence of CNS involvement may portend an even worse outcome.

Antineoplastic Combined Chemotherapy Protocols↗

Causes of chemoreduction failure in retinoblastoma and analysis of associated factors leading to eventual treatment with external beam radiotherapy and enucleation.

PURPOSE: To evaluate the causes of chemoreduction failure in retinoblastoma and to analyze the associated factors for eventual treatment with external beam radiotherapy and enucleation. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Seventy-one patients with 105 eyes with intraocular retinoblastoma that underwent chemoreduction therapy between October 1998 and January 2003. INTERVENTION: A 6-treatment cycle of chemoreduction therapy with vincristine, etoposide, and carboplatin was administered at monthly intervals. Unresponsive disease was defined as persistence of retinal tumors, vitreous seeds, or subretinal seeds after the second treatment cycle, with no appreciable sign of regression. Eyes with unresponsive disease were enucleated after the second treatment. Eyes that responded to chemoreduction therapy received focal treatment, including indirect laser photocoagulation, transpupillary thermotherapy, cryotherapy, and ruthenium 106 episcleral plaque radiotherapy after the second chemoreduction treatment, if necessary, to achieve complete tumor regression. Recurrence was defined as the regrowth of retinal tumors, vitreous or subretinal seeds after an initial favorable response, and regression. Recurrent retinal tumor, vitreous seeds, or subretinal seeds were treated with focal treatments and 2 to 3 additional chemoreduction treatments. When these methods failed or were not applicable, external beam radiotherapy and/or enucleation was administered. MAIN OUTCOME MEASURES: The use of external beam radiotherapy and enucleation for chemoreduction failure, which was defined as unresponsive or recurrent disease. RESULTS: The mean follow-up was 25.7 months (range: 6-49). Ten of 105 eyes (9.5%) with unresponsive disease were enucleated after the second treatment. Of the remaining 95 eyes, 42 (44.2%) developed recurrence after chemoreduction. Recurrent disease failing to be treated successfully by other methods was treated with external beam radiotherapy in 26 of 95 eyes (27.4%) and enucleation in 22 of 95 eyes (23.2%). External beam radiotherapy was successful in preventing enucleation in 20 of 26 eyes (76.9%). Overall, the globe salvage rate was 69.5%, ranging from 36.1% for Reese-Ellsworth group V disease to 87.0% for groups I to IV disease. Histopathologically, 29 of 31 enucleated eyes (93.5%) had poorly differentiated or moderately differentiated retinoblastoma. Using multivariate logistic regression analysis, factors predictive of eventual treatment with external beam radiotherapy were female gender (P = 0.010), presence of subretinal seeds (P = 0.023), and a greater number of chemoreduction treatments (P = 0.027). By multivariate analysis, the factors associated with the need for eventual treatment with enucleation were recurrence of retinal tumors (P = 0.004), presence of vitreous seeds (P = 0.008), greater tumor thickness (P = 0.015), presence of subretinal fluid (P = 0.040), and older patient age (P = 0.042). CONCLUSIONS: Chemoreduction failure in this article was defined as unresponsive or, more commonly, recurrent retinoblastoma. Older patient age, greater tumor thickness, presence of vitreous seeds and subretinal fluid at baseline, and retinal tumor recurrence after chemoreduction were factors associated with the need for enucleation.

Age Factors↗

Magnetic resonance imaging of unilateral lacrimal gland lesions.

PURPOSE: To report the findings on magnetic resonance imaging (MRI) of various benign and malignant unilateral lacrimal gland lesions. METHODS: This is a retrospective noncomparative interventional case series. Thirty-one consecutive patients with a unilateral lacrimal gland lesion were analyzed. The preoperative MRI findings were correlated with the pathology results. The main outcome measures were anatomic extent, configuration, margins, angulation, internal features on T1- and T2-weighted images (with respect to extraocular muscles and cerebral gray matter), contrast enhancement of the lesion and adjacent bone change on MRI. RESULTS: Of the 31 patients, 21 had chronic dacryoadenitis, 3 had lymphoid tumors, and 7 had epithelial tumors including pleomorphic adenoma (3), adenoid cystic carcinoma (3), and pleomorphic adenocarcinoma (1). The results of the patients with chronic dacryoadenitis demonstrated involvement of the orbital lobe alone in 13 patients (62%), involvement of both orbital and palpebral lobes in 8 (38%), a molded configuration with ill-defined margins, sharp angles in 13 (62%), round angles in 8 (38%), lack of bone change, an isointense internal signal on T1-weighted images, a hypointense signal on T2-weighted images, and moderate contrast enhancement. The patients with lymphoid tumors demonstrated involvement of the orbital lobe, a molded configuration with ill-defined margins and sharp angles, lack of bone change, an isointense internal signal on T1-weighted images, an isointense signal on T2-weighted images, and moderate contrast enhancement. Those with epithelial tumors showed involvement of the orbital lobe, a well-circumscribed oval configuration, and round angles. Pleomorphic adenoma demonstrated smooth margins, bone expansion in two patients, and no bone change in one. Adenoid cystic carcinoma and pleomorphic adenocarcinoma showed irregular margins and bone destruction. All epithelial tumors demonstrated an isointense internal signal on T1-weighted images, a hyperintense signal on T2-weighted images, and moderate contrast enhancement. CONCLUSIONS: It is difficult to uniformly correlate the MRI features and histopathologic findings in lacrimal gland lesions. However, MRI seems to be useful in determining the etiology of a unilateral lacrimal gland lesion. Internal tissue features on T1- and T2-weighted images of MRI are most helpful in categorizing these lesions. Although the number of patients is small, our findings suggest that there are differences in orbital MRI findings of inflammatory lesions and lymphoid tumors as compared to benign and malignant epithelial tumors in the lacrimal gland fossa.

Adenoma, Pleomorphic↗

Orbital schwannoma: correlation of magnetic resonance imaging and pathologic findings.

PURPOSE: To report the correlation between magnetic resonance imaging (MRI) findings and histopathology of orbital schwannomas. METHODS: This study is a retrospective noncomparative interventional case series of three consecutive patients with orbital schwannoma. After preoperative MRI, all patients underwent orbitotomy and tumor removal. Histopathologic and immunopathologic studies of the excised tumors were performed. The main outcome measures were MRI features, histopathology, and their relationship to the cohesiveness of the tumor. RESULTS: The patients' ages were 56 years (patient 1), 43 years (patient 2), and 18 (patient 3) years. MRI demonstrated that the tumors were well-circumscribed, intraconal in patient 1, superior extraconal in patient 2 and inferotemporal extraconal in patient 3. The tumor in patient 1 had a partly cavitary appearance, while the tumors in patients 2 and 3 had a solid appearance on MRI. The tumor had heterogeneous signal intensity in patients 1 and 2 and homogeneous signal intensity in patient 3 on T2-weighted images. All patients underwent orbitotomy and tumor removal. In patients 1 and 2, the tumor was gelatinous and fragmented during removal. The fragments were completely excised in patient 1 and an incomplete piecemeal tumor excision was made in patient 2. In patient 3, complete excision without fragmentation was performed. Pathologic examination revealed that the tumor was of Antoni B pattern in patient 1, mixed Antoni A and B patterns in patient 2, and marked Antoni A pattern in patient 3. At a mean follow-up of 23.7 months (range: 22-26 months) after surgery, all patients retained preoperative visual acuity. Patient 2 developed recurrence at 21 months follow-up and underwent Krönlein orbitotomy for excision of the recurrent tumor. CONCLUSIONS: MRI is useful in depicting both the anatomic location and internal consistency of orbital tumors. Schwannomas with MRI evidence of cavitary change or heterogeneous signal intensity on T2-weighted images were found to have marked Antoni B pattern on histopathology, imparting a friable structure and fragmented excision.

Adolescent↗

Encouraging result of tamoxifen in a retinoblastoma patient with central nervous system metastasis.

Extraocular retinoblastoma occurs more frequently in developing countries as a delayed diagnosis and prognosis of patients with conventional therapy is very poor. Metastatic retinoblastoma, especially in the central nervous system (CNS), is a highly lethal disease. Tamoxifen has been used in some previous studies with variety of responses to therapy in patients with unresectable recurrent brain tumors. A 7-year-old girl with recurrent metastatic retinoblastoma received 60 mg/m2 tamoxifen in addition to chemotherapy and CNS radiotherapy. She was in remission until she has died in a traffic accident at week 114. The authors think that tamoxifen can be added to treatment protocols of metastatic retinoblastoma to provide longer and at least higher quality of life for these patients.

Angiogenesis Inhibitors↗

Corneal topographic changes after retinal detachment surgery.

PURPOSE: To evaluate the changes in corneal topography after scleral buckling surgery for retinal detachment. METHODS: Twenty-one eyes of 21 patients with the diagnosis of retinal detachment were included in this prospective study. Scleral buckling surgery was performed on all patients. The corneal topography of each was measured before surgery and at 1 week and 1 and 6 months after surgery by computer-assisted videokeratoscopy using the EyeSys 2000 Corneal Analysis System. Changes in corneal videokeratographies were analyzed using the Holladay Diagnostic Summary (HDS) software package. RESULTS: A statistically significant central corneal steepening (average, 1.8 diopter) was noted 1 week after surgery. The total and irregular astigmatic components both revealed a significant but transient increase in the first postoperative month. All these topographic changes persisted for as long as 6 months but returned to preoperative values afterward. CONCLUSION: Scleral buckling was found to induce transient changes in corneal topography producing both myopia and corneal astigmatism. Videokeratography is helpful for documenting such corneal changes after scleral buckling surgery for retinal detachment.

Aged↗

Orbital leiomyoma: report of a case and review of the literature.

A 10-year-old female presented with slowly progressive proptosis of 1 year's duration. Orbital MRI revealed an extraconal oval mass occupying the lateral half of the mid and posterior orbit. The tumor was isointense to the extraocular muscle and cerebral gray matter on T1-weighted images and hyperintense on T2-weighted images. The patient underwent superolateral orbitotomy, and it was felt that the tumor was totally excised in a piecemeal fashion. Histopathologic examination showed that the tumor was composed of spindle cells arranged in a fibrous stroma rich in dilated sinusoidal capillaries. The nuclei of tumor cells were oval with blunted ends. There were no mitotic figures. Immunohistochemically, the tumor stained positive with smooth muscle actin, desmin, and vimentin. Based on the histopathologic and immunohistochemical staining results, a diagnosis of orbital leiomyoma was made. At 34 months follow-up, the patient continues to have 20/20 vision and is free of tumor recurrence clinically. Orbital leiomyoma is an uncommon tumor. Search of the English literature showed only 15 previous cases of orbital leiomyoma that have been published since 1963.

Biomarkers, Tumor↗

A rare case of atypical chondroid syringoma of the lower eyelid and review of the literature.

A 46-year-old man presented with a painless firm mass in the eyelid margin of the left lower eyelid, which had been present for 9 years. Biopsy nine years previously had not established a diagnosis. We performed excisional biopsy of the lesion and lateral advancement skin flap for reconstruction of the skin defect. Histopathologic and immunohistochemical findings were consistent with atypical chondroid syringoma with eccrine differentiation. The systemic metastatic work-up was negative, and no recurrence or metastasis was present at 30-month follow-up. Chondroid syringoma is a rare skin tumor that occurs, very rarely, in the eyelids, especially the lower eyelid. Only 26 cases of chondroid syringoma in the periorbital area, including our case, have been reported, with various characteristics. Chondroid syringomas are classified as two types, the apocrine type characterized by tubular and cystic branching lumina lined by two layers of epithelial cells, and the eccrine type, which has small tubular lumina lined by a single layer of epithelial cells. The tumor can have benign, atypical, and malignant variants. Our patient had the atypical variant, which is characterized by benign cytological features, as well as the presence of infiltrative margins and/or satellite nodules. Even benign chondroid syringomas, if incompletely excised, can recur with malignant transformation; thus, complete excision and regular follow-up is recommended for all chondroid syringomas.

Adenoma, Pleomorphic↗