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Abstract: Endolymphatic sac tumor (ELST) is a rare lesion of the skull base for which the origin has recently been ascertained. The endolymphatic sac is derived from neuroectoderm and is located subjacent to the posteromedial surface of the temporal bone. Patients characteristically present with hearing loss, tinnitus, and vertigo; facial nerve paralysis occurs less commonly. An indolent clinical course and long-standing symptom history is typical. Endolymphatic sac tumors are known to occur more frequently in patients with von Hippel-Lindau disease, but this is not a prerequisite for diagnosis because sporadic occurrence is common. Morphologically, all of the ELSTs showed a papillary and glandular architecture. The papillary and glandular structures were lined by a single layer of flattened cuboidal-to-columnar cells that were variably ciliated. Surgery is the treatment of choice for small ELST. Remission may last for years, but local recurrence after surgery, likely secondary to incomplete resection, can occur. Radiotherapy has a 50% cure rate with large or residual tumors. Endolymphatic sac tumor is a rare tumor that can easily be confused with other papillary lesions on histopathologic grounds, with significant treatment implications. Precise preoperative anatomic localization and computed tomography and magnetic resonance imaging feature interpretation play a paramount role in achieving an accurate final diagnosis. [Copyright &y& Elsevier]

Background: After brain metastasis resection, whole brain radiotherapy decreases local recurrence, but might cause cognitive decline. We did this study to determine if stereotactic radiosurgery (SRS) to the surgical cavity improved time to local recurrence compared with that for surgical resection alone.Methods: In this randomised, controlled, phase 3 trial, we recruited patients at a single tertiary cancer centre in the USA. Eligible patients were older than 3 years, had a Karnofsky Performance Score of 70 or higher, were able to have an MRI scan, and had a complete resection of one to three brain metastases (with a maximum diameter of the resection cavity ≤4 cm). Patients were randomly assigned (1:1) with a block size of four to either SRS of the resection cavity (within 30 days of surgery) or observation. Patients were stratified by histology of the primary tumour, metastatic tumour size, and number of metastases. The primary endpoint was time to local recurrence in the resection cavity, assessed by blinded central review of brain MRI scans by the study neuroradiologist in the modified intention-to-treat population that analysed patients by randomised allocation but excluded patients found ineligible after randomisation. Participants and other members of the treatment team (excluding the neuroradiologist) were not masked to treatment allocation. The trial is registered with ClinicalTrials.gov, number NCT00950001, and is closed to new participants.Findings: Between Aug 13, 2009, and Feb 16, 2016, 132 patients were randomly assigned to the observation group (n=68) or SRS group (n=64), with 128 patients available for analysis; four patients were ineligible (three from the SRS group and one from the observation group). Median follow-up was 11·1 months (IQR 4·8-20·4). 12-month freedom from local recurrence was 43% (95% CI 31-59) in the observation group and 72% (60-87) in the SRS group (hazard ratio 0·46 [95% CI 0·24-0·88]; p=0·015). There were no adverse events or treatment-related deaths in either group.Interpretation: SRS of the surgical cavity in patients who have had complete resection of one, two, or three brain metastases significantly lowers local recurrence compared with that noted for observation alone. Thus, the use of SRS after brain metastasis resection could be an alternative to whole-brain radiotherapy.Funding: National Institutes of Health. [ABSTRACT FROM AUTHOR]

Background Papillary tumor of the pineal region (PTPR) is a rare neuroectodermal tumor that was first described in 2003 and formally codified by the World Health Organization in 2007. Limited reports suggest surgical resection is the mainstay of treatment; however, the role of multimodality therapy is not well defined. We evaluated our institutional experience in the treatment of PTPR. Methods A retrospective review of 8 patients with pathologically confirmed PTPR diagnosed between 1999 and 2013 was performed. Results The median age at diagnosis was 37 years (range, 25–56 years). After a median follow-up period of 60 months (range, 10–170 months), 7 of 8 patients were still living. All patients underwent maximum safe surgical resection; 5 received adjuvant radiation (63%). Overall and progression-free survival 5 years after diagnosis were 100% and 51%, respectively. Progression-free survival 5 years after completion of adjuvant radiotherapy was 64%. Crude recurrence rates for patients receiving adjuvant radiotherapy ( n = 5) and patients not receiving adjuvant radiotherapy ( n = 3) were 20% and 67%, respectively. Crude recurrence rate after gross total resection (GTR) and no adjuvant radiotherapy ( n = 2) was 100% versus 0% when adjuvant radiotherapy was administered after GTR ( n = 2). After subtotal resection, 3 patients received adjuvant radiotherapy; 1 of these patients had out-of-field recurrence at 46 months (crude recurrence rate 33%). In all cases, salvage with radiation at the time of recurrence was effective. Conclusions Our institutional experience confirms a recent multicenter retrospective series showing excellent survival but high risk of local recurrence for PTPR. Our findings suggest that radiotherapy provides durable local control, particularly when administered in the adjuvant setting after GTR. [ABSTRACT FROM AUTHOR]

Objective To update outcomes and assess prognostic factors in the modern, multimodality treatment of patients with pineoblastoma. Methods The medical records of patients with pineoblastoma evaluated at the M.D. Anderson Cancer Center between 1982 and 2012 were reviewed retrospectively. Results Thirty-one patients with medical records suitable for review were identified. The majority of patients were female (67.7%) with a median age at diagnosis of 18.2 years (range, 0.3–52.8 years). Twenty-one patients underwent surgical resection, recorded as gross total (n = 9) or subtotal (n = 12) resections. Thirty patients received radiation with photon-based therapy (n = 16), proton-based therapy (n = 13), or radiosurgery (n = 1) to a median craniospinal irradiation dose of 36 Gy (range, 23.4–40 Gy) and a median focal dose of 54 Gy (range, 40–58.4 Gy). Twenty-eight patients received chemotherapy before (n = 10), during (n = 10), and after (n = 22) radiation. Median overall survival was 8.7 years for the entire cohort, with 2-, 5-, and 10- year actuarial rates of 89.5%, 69.4%, and 48.6%, respectively. Median disease-free survival was 10 years with 2-, 5-, and 10- year actuarial rates of 84.3%, 62.6%, and 55.7%, respectively. Univariate analysis failed to correlate age, sex, or extent of surgical resection with disease-free or overall survival. Conclusions Modern, multimodality treatment of pineoblastoma yields a high rate of overall survival, with acceptable short- and long-term toxicity. A greater M-stage at presentation and development of disease recurrence correlate with worse overall survival. Patients who received focal radiation initially experienced a greater rate of disease recurrence compared with those treated to the craniospinal axis. [ABSTRACT FROM AUTHOR]