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Secondary skull base malignancies in survivors of retinoblastoma: the memorial sloan kettering cancer center experience.

ABSTRACT: We report the pathology and outcome of secondary skull base tumors in patients previously treated with external beam radiation for retinoblastoma (Rb). Rb patients are at increased risk of second head and neck primary malignancies due to early radiation exposure during treatment and loss of RB1 protein in genetic carriers. An institutional database was reviewed for patients with retinoblastoma who had previously received radiation therapy and subsequently developed skull base tumors. Seventeen patients met the selection criteria. The median age of Rb diagnosis was 12 months. Thirteen cases underwent enucleation in addition to radiation therapy as part of initial Rb treatment. A median of 19 years elapsed between the diagnosis of Rb and diagnosis of skull base malignancy. The most common tumors were osteogenic sarcoma (39%) and leiomyosarcoma (22%). Eleven (71%) patients received postoperative chemotherapy, and 7 (41%) received postoperative radiotherapy. Three (24%) patients underwent salvage surgery for recurrent disease. Five-year survival was 68%, and 10-year survival was 51% by Kaplan-Meier analysis. Secondary malignancy in Rb patients is a well-defined event. The use of surgery with appropriate adjuvant therapy was associated with a 51% 10-year survival in this study population.

SUBMITTER: Liu JC

PROVIDER: S-EPMC3312595 | biostudies-other | 2011 Mar

REPOSITORIES: biostudies-other

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Project description:RationalePlacement of an indwelling pleural catheter is an established modality for symptom relief and pleurodesis in the treatment of malignant pleural effusion. Concerns remain regarding possible infectious complications, risk of hemorrhage, and the rate of pleurodesis with the use of pleural catheters in the treatment of hematologic malignancies.ObjectivesThe goals of our study were: (1) to evaluate the safety and cumulative incidence of pleurodesis with indwelling pleural catheters for patients with hematologic malignancies, and (2) to evaluate overall survival of this cohort of patients with pleural effusions.MethodsWe performed a retrospective review of 172 patients with a hematologic malignancy who underwent placement of an indwelling pleural catheter between September 1997 and August 2011 at the University of Texas MD Anderson Cancer Center in Houston, Texas. A competing risk model analysis was used for complications and pleurodesis. Analysis was based on each patient's first intrapleural catheter.ResultsThere were 172 patients with lymphoma (58%), acute (16%) or chronic leukemia (16%), or multiple myeloma (10%). The effusions were characterized as malignant (85.5%), infectious (4.1%), volume overload (4.7%), or therapy-related (4.7%). Chylothorax was found in 20.1%. Pleural biopsies were obtained from 13 patients. The cumulative incidence of all complications was 13.6%, and the cumulative incidence of all significant catheter-related complications was 9.5%. The incidence of empyema was 2.9%, and major bleeding (requiring transfusion or intervention) was 1.7%. Thirty-day procedure-associated mortality was 0.6%. The cumulative incidence of pleurodesis at 180 days was 50%, with a median time to pleurodesis of 81 days for the entire cohort.ConclusionsIndwelling pleural catheters appear to be safe for patients with hematologic malignancies. Complications and the cumulative incidence of pleurodesis are comparable to those reported for patients with solid organ malignancies.

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Secondary skull base malignancies in survivors of retinoblastoma: the memorial sloan kettering cancer center experience.

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