Intracranial germ cell tumors: a single-institution experience.

BACKGROUND AND OBJECTIVES: Intracranial germ cell tumors (GCTs) are not a common disease. We reviewed the experience of a single institution to determine the variables that affect treatment outcome. DESIGN AND SETTING: A retrospective review of patients with the diagnosis of intracranial germ cell tumors treated in a single institution (KFSHRC) during the period from March 1985 to December 2007. PATIENTS AND METHODS: Fifty-seven patients with the diagnosis of intracranial GCT were recorded in the KFSHRC Tumor Registry during the period from 1985 to 2007. Seven patients with a pineal region tumor treated as germinomas in the earlier years without a tissue diagnosis were excluded. This retrospective study was restricted to the remaining 50 patients with a tissue or marker diagnosis: 31 germinomas and 19 non-germinomatous germ cell tumors (NGGCTs). RESULTS: The 10-year overall survival (OS), event-free survival (EFS) and relapse-free survival (RFS) were 87%, 88% and 96% for patients with germinoma, with a median follow-up of 4.5 (range 2-17) years, compared with 26%, 29% and 46% for patients with NGGCT with a median follow-up of 3 (range 1.5-13) years. For NGGCT, variables favorably influencing OS were younger age (< 16 y vs ≥16 y, P=.01), higher radiation dose (>50 Gy vs ≤50 Gy; P=.03) and later year of diagnosis (>1990 vs <1990 P=.002). CONCLUSIONS: Tissue diagnosis of GCTs is mandatory prior to treatment except for patients with elevated markers. In germinoma, localized radiotherapy (RT) for M0 patients may be adequate. Long-term follow-up is needed to define the benefit of adding chemotherapy. For NGGCT, the use of combined modality treatment and RT dose ;gt;50 Gy are important factors that influence the outcome. Second-look surgery and resection of residual/ refractory tumors is always recommended.

Survival and late effects in children with Hodgkin's lymphoma treated with MOPP/ABV and low-dose, extended-field irradiation.

PURPOSE: Reduced-intensity protocols for pediatric Hodgkin's lymphoma are aimed at preserving excellent relapse-free survival while decreasing the incidence of late effects. PATIENTS AND METHODS: We retrospectively reviewed the outcome of 123 children treated consecutively for Hodgkin's lymphoma at a single institution. Patients with stages I-IIIB disease received three cycles of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP)/ doxorubicin, bleomycin, and vinblastine (ABV) followed by 15 Gy of extended-field irradiation, while those with stage IV disease were treated with six to eight cycles of MOPP/ABV chemotherapy with or without radiotherapy. RESULTS: At a median follow-up of 8.5 years (range, 1.4 to 15.5 years), the estimated 10-year overall survival and event-free survival are 94% (SE, 2.2%) and 88% (SE, 3.1%) respectively. There have been 12 treatment failures and six disease-related deaths. A very large mediastinal mass ( 50% of the maximal thoracic diameter) was associated with a 10-year event-free survival of 50% (SE, 14%) compared with 91% (SE, 4.0%) for smaller masses (P < .001). Late cardiopulmonary toxicity is largely absent, and the incidence of hypothyroidism is 14%. There have been no cases of secondary leukemia and four secondary solid malignancies observed to date. CONCLUSION: MOPP/ABV and low-dose, extended-field radiotherapy is an effective treatment for pediatric Hodgkin's lymphoma. With median follow-up of 8.5 years, late cardiopulmonary effects and secondary malignancies from this treatment regimen are infrequent. Continued longitudinal observations, particularly for breast cancer in female patients and gonadotoxicity, will determine whether the goal of decreasing treatment-related complications while maintaining excellent survival has been achieved.

Renal cell carcinoma in children. Prognostic factors.

OBJECTIVE: To determine the outcome in childhood renal cell carcinoma and the role of surgical and radiation treatment. METHODS: The records of 21 children with renal cell carcinoma were reviewed, 15 patients treated in the University of Toronto centers from 1959 through to 1997 and 6 patients treated in King Faisal Specialist Hospital, Riyadh, Kingdom of Saudi Arabia from 1975 through to 1998. The age was 3-17 (median 13) years. Systematic metastases were present at diagnosis in 5 patients. Regional nodal spread was present in 9 patients and 7 patients had localized disease alone. In the 16 M0 patients, the surgical treatment was radical nephrectomy (14 patients) partial nephrectomy (one patient) and wedge resection (one patient). Postoperative radiation treatment was utilized in 8 (50%) of these patients. RESULTS: The 5 year survival rate for all patients was 52%, and for M0 patients was 70%. No patient with systematic metastases at diagnosis survived beyond 26 months. Four of 7 node negative patients and 8 of 9 node positive patients remained in first complete remission, with the duration of follow up 1-30 (Median 5) years. Seven of 8 M0 patients who did not receive adjuvant radiation therapy continued in first remission (3N0, 2NI, 2N2), compared with 5 of 8 patients who received postoperativeradiation treatment (1 N0, 2 NI, 2 N2). CONCLUSION: The prognosis of localized renal cell carcinoma in childhood may be better than in the adult. Gross complete resection is required for long term survival. Elective postoperativeradiation treatment is not indicated.

Optic gliomas: a retrospective analysis of 50 cases.

PURPOSE: Gliomas of the optic pathways are rare childhood central nervous system tumors. The treatment approach is controversial because of its rarity and the slow and unpredictable growth rates of these lesions. METHODS AND MATERIALS: We reviewed 50 patients with the diagnosis of optic pathway low-grade gliomas treated between January 1980 and December 1995 at King Faisal Specialist Hospital and Research Center, Saudi Arabia. Thirty-five patients presented with chiasmatic/hypothalamic (posterior tumors), and 15 with optic nerve gliomas with or without chiasmal involvement (anterior tumors). Evidence of neurofibromatosis was present in 18 patients. Twenty-nine patients underwent surgery (total or partial resection), and 12 of these received postoperative radiotherapy (RT). Sixteen patients were treated with primary RT. The radiation dose varied between 42 and 54 Gy (median dose 50). RESULTS: The overall actuarial survival rate was 87.5% at 5 years and 75% at 10 years, and the corresponding progression-free survival (PFS) rates were 69% and 62%. Patients with anterior tumors fared better than those with posterior tumors, with a 10-year PFS rate of 72% and 58%, respectively; the difference, however, was not statistically significant (p = 0.58). A PFS advantage was found in favor of patients with posterior tumors treated with RT (primary or postoperative) compared with no RT, with 5-year PFS rates of 68% vs. 42% (p = 0.03). This, however, did not translate into a survival advantage because of the success of salvage treatment. CONCLUSION: In multivariate analysis, age (<3 vs. >3 years) emerged as the only significant determinant for PFS with patients <3 years old faring worse (p = 0.03). Neurologic and endocrine dysfunction are significant problems that need to be addressed.