A retrospective analysis of recurrent intracranial ependymoma.

BACKGROUND: Recurrence occurs in almost 50% of patients with intracranial ependymoma, and their outcome following recurrence is poor. METHODS: We retrospectively reviewed the medical records of 22 patients with intracranial ependymoma and subsequent relapse(s) (59 recurrences) treated at Children's Hospital Los Angeles or New York University between January 1997 and December 2012. RESULTS: Median duration of follow-up was 52 months (7-171 months). Median age at initial diagnosis was 4 years (0.3-19 years) with 8 patients younger than 3 years at presentation. Eleven patients had anaplastic and 11 cellular pathologies. Eighteen patients had infratentorial tumors at diagnosis and 3 (all infratentorial) had metastatic spinal cord involvement at presentation. Cerebrospinal fluid involvement was not identified at diagnosis or relapse. Median time to first recurrence was 16 months (1.3 to 115 months). The number of recurrences in each patient ranged from 1 to 9 (median = 2). Thirty-seven recurrences (63%) were detected asymptomatically by surveillance imaging. Fifteen recurrences (26%) arose outside the initial tumor site. Recurrences were treated by surgical resection (45), with irradiation (30), and with various oral chemotherapies (23) with (7) or without (16) conventional chemotherapy. The 5 and 10 year overall survival rates from first recurrence were 0.37 ± 0.14 and 0.25 ± 0.14. CONCLUSION: Prolonged (5-10 year) survival from first relapse was noted in over one-quarter of our patients. It remains unclear whether early radiographic diagnosis, differing treatment modalities beyond radical surgical resection or possibly unrecognized biological differences contributed towards this prolonged survival.

Solid pseudopapillary tumor of the pancreas: a single-institution 20-year series of pediatric patients.

PURPOSE: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. The objective of this study was to review our institution's experience and provide an update on current management in the pediatric population. METHODS: Our pathology database identified all patients with SPT for a 20-year period (1991-2011). Demographics, clinical characteristics, operative details, pathology, and outcomes data were retrospectively reviewed. RESULTS: Eleven patients with SPT were identified. Most were female and Hispanic. Median age at resection was 14 years (9-17 years). Most patients presented with abdominal pain. Diagnostic imaging was most commonly an ultrasound or computed tomography. All tumors were resected en bloc. Median greatest tumor diameter was 5 cm (3.5-12 cm). Median length of stay was 8 days (5-19 days). Complications included pancreatic leak, chyle leak, delayed gastric emptying, fat malabsorption, and incisional keloid. Recurrence developed after 2.5 years in 1 patient with positive surgical margins. There were no metastases or deaths. Median follow-up was 1.4 years (0.6-5.9 years). CONCLUSION: This pediatric series of SPT from a single institution corroborates previous reports in the literature. In our experience, SPT behaves like a low-grade malignancy and has an excellent prognosis. Surgical resection is dictated by tumor location and remains the treatment of choice.

Supratentorial ependymoma in children: to observe or to treat following gross total resection?

BACKGROUND: The standard treatment for ependymoma is surgical resection followed by postoperative irradiation to the local site. The role of radiation therapy in completely resected supratentorial ependymoma has been questioned over the past two decades. PROCEDURE: Retrospective review of the medical records of all consecutively diagnosed supratentorial ependymoma patients at Children's Hospital Los Angeles between January 1999 and December 2009. RESULTS: Ten patients (three females) were included. The median age at presentation was 5.6 years (range 1.8-15.6 years). Reviewed histology was anaplastic ependymoma in seven patients and cellular ependymoma in three patients. Gross total resection was achieved in six patients; five were observed and one received chemotherapy. In the four patients who underwent subtotal resection, one was observed, two received local irradiation and one received irradiation and chemotherapy. The median length of follow up was 43 (range 22-81) months. Four relapses were observed; two patients who underwent initial gross total resection. All patients who underwent gross total resection were alive at the time of preparation of this article. The 5-year progression-free and overall survival rates were 53 ± 19% and 86 ± 13% respectively. CONCLUSIONS: Radiation therapy was avoided in five patients following gross total resection, four of whom had anaplastic histology. In some children with completely resected supratentorial ependymoma, surgery alone may be an acceptable treatment option.