Diagnosis and management of tumor-induced osteomalacia: a single center experience.

PURPOSE: The aim of this study is to review the clinical and laboratory characteristics, diagnostic and treatment modalities of tumor-induced osteomalacia (TIO) cases managed in a single center. MATERIAL METHODS: Demographic and clinical features, biochemical findings, diagnostic procedures, treatment modalities, and outcomes of nine patients who had the diagnosis of TIO were reviewed retrospectively. RESULTS: Mean age of the study group (F/M: 4/5) was 45.8 ± 10.8 years, and mean time from the onset of symptoms to diagnosis was 4.7 ± 2.8 years. The clinical manifestations were muscle weakness and difficulty in walking (8/9), hip pain (3/9), multiple fractures (2/9), stress fracture (2/9). Mean plasma phosphorus concentration was 1.28 ± 0.4 mg/dl at presentation. We performed radionuclide imaging modalities (18F-FDG PET/CT, Ga68-DOTATATE PET/CT, octreotide scintigraphy) in seven of nine patients, and tumor was detected in all. Lower extremity (n = 6; %67), head region (n = 2; %22) and thorax (n = 1; %11) were the tumor locations of our cases. Eight patients underwent surgery and remission was achieved postoperatively in all of the operated patients and plasma phosphorus level normalized in 4 ± 2 days. Pathological examination revealed mesenchymal tumors with different subtypes. Recurrence occurred in three patients at 13 ± 10.5 months after the first surgery. Two patients were reoperated and radiotherapy was also performed in one of them. CONCLUSION: Hypophosphatemia necessitates careful evaluation for the etiology. TIO is one of the important causes of adult-onset hypophosphatemic osteomalacia. Diagnosis of TIO is essential because the laboratory and clinical findings resolve after appropriate treatment.

Systemic Rituximab Immunotherapy in the Management of Primary Ocular Adnexal Lymphoma: Single Institution Experience.

PURPOSE: To evaluate the efficacy of systemic rituximab immunotherapy in the management of primary ocular adnexal lymphomas (OAL). MATERIALS AND METHODS: Clinical records of 10 consecutive patients (11 eyes) with biopsy-proven OAL managed with systemic anti-CD20 monoclonal antibody (rituximab; 375 mg/m(2) intravenously once every three weeks for 6-8 cycles) between June 2008-March 2013 were evaluated retrospectively. Orbital magnetic resonance imaging and positron emission tomography were performed to evaluate any orbital and systemic involvement, respectively. Clinical response was classified as complete or partial. RESULTS: The age of patients ranged between 27-85 (median, 55) years. Nine patients (90%) presented with unilateral and one (10%) with bilateral conjunctival involvement. Orbit was affected in 4 patients (40%), one of which had also choroidal involvement (10%). None of the patients had systemic involvement at initial presentation. All patients received an average of 7 cycles (range, 6-8) of systemic immunotherapy. After a median follow-up of 31 months (range, 10-61 months), complete response without recurrence could be achieved in 4 eyes (36%) with rituximab monotherapy. No systemic or ocular side effects were observed in any patient. Additional radiotherapy was required in 6 patients (7 eyes; 64%) with partial response or recurrence. CONCLUSIONS: Complete regression of primary OALs without recurrence was observed in about one-third of eyes after systemic rituximab monotherapy. Adjunctive radiotherapy was required in remaining two-thirds of the cases to achieve complete response. Thus, considering the balance between high rate of local control and potential ocular complications of radiotherapy, systemic rituximab can be considered as a first-line therapeutic option in the management of primary OAL.

Tumor-related lipid exudation after plaque radiotherapy of choroidal melanoma: the role of Bruch's membrane rupture.

PURPOSE: To identify the risk factors predictive of development of tumor-related lipid exudation (TRLE) after plaque radiotherapy of posterior uveal melanoma. DESIGN: Case-control study. PARTICIPANTS: Cases included 294 patients with posterior uveal melanoma who had developed TRLE after plaque radiotherapy. Controls included 294 patients with posterior uveal melanoma who had not developed TRLE after plaque radiotherapy. Controls were matched with cases for age, gender, and initial tumor thickness. METHODS: Data were extracted from medical charts containing demographic, clinical, and treatment information. Detailed fundus drawings and color fundus photographs were reviewed for each patient. MAIN OUTCOME MEASURES: Tumor and ocular features of eyes with posterior uveal melanoma treated with plaque radiotherapy. RESULTS: Multivariate analysis identified Bruch's membrane rupture (P<0.001), serous retinal detachment (RD) before radiation (P< or =0.019), closer proximity to the optic disc and foveola (P = 0.004 and 0.013, respectively), greater tumor base (P = 0.035), failure to receive transpupillary thermotherapy (TTT) after radiation (P<0.001), and initial increase of serous RD after radiation (P<0.001) as significant risk factors predictive of development of TRLE after plaque radiotherapy of posterior uveal melanoma. Radiation dose at the tumor base correlated with maximum extent of TRLE (P = 0.003). The mean interval between plaque radiotherapy and onset of TRLE was 14 months (median, 11 months; range, 2-97 months), with 88% of cases developing TRLE within 2 years of radiation. The interval between the onset of TRLE and the first evidence of its regression was a mean of 33 months (median, 38 months; range, 2-194 months). CONCLUSIONS: Our study identified Bruch's membrane rupture as an important factor predisposing to development of TRLE after plaque radiotherapy of posterior uveal melanoma. Other predictive factors included serous RD before radiation, large tumor basal diameter, posterior tumor location, lack of adjunctive TTT, and early increase of serous RD after plaque radiotherapy.