Intracranial germ cell tumors: a single-institution experience

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. 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. 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]. 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 1990 vs <1990 P=.002]. 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 >50 Gy are important factors that influence the outcome. Second-look surgery and resection of residual/refractory tumors is always recommended

Improved survival with combined chemo-radiotherapy in primary central nervous system lymphoma

Primary CNS lymphoma [PCNSL] is an aggressive primary brain tumor. Cranial irradiation alone rarely results in long term disease control or prolonged survival. We retrospectively analyzed data on the effect of adding high-dose methotrexate [HDMTX] prior to whole brain irradiation [WBI]. All patients with PCNSL diagnosed and managed during 1991-2004 were identified and demographic characteristics, prognostic factors, treatment and outcome were reviewed. Of 62 patients, 10 were excluded [4 had WBI < 40 Gy and 6 had no treatment]. Radiation alone was considered curative with a dose > 40 Gy. Combined modality therapy included 3-4 cycles of HDMTX [3g/m2] followed by WBI. Of 52 patients analyzed for outcome, 36 had WBI [dose > 40 Gy], 16 received 3-4 cycles of HDMTX followed by WBI [combined modality therapy [CMT]]. Median age was 48.2 years; 42 years in the CMT group, 51 years in WBI. Patient characteristics were comparable between two groups except for higher multifocal tumor in the CMT group [92% vs. x22%, P=.029]. Median follow up was 12.83 +/- 6.4 months. The hazard ration for an event was 0.64 [95% CI, 0.52-0.98] and for death 0.58 [95% CI, 0.48-0.92], both in favor of CMT. Univariate regression analysis using one-way analyses of variance [ANOVA] and multivariate Cox regression analysis for prognostic factors including age [< 60 vs. >60], ECOG PS [0-2 vs. 3-4], extent of surgery [biopsy vs. debulking], solitary vs multifocal tumor and dose of radiation therapy [>50Gy vs. >50 Gy] failed to identify any prognostic factor. This retrospecitive comparison supports phase II trial results that indicate that high-dose methotrexate followed by WBI in PCNSL improves outcome

Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma

While treatment outcomes for patients with Hodgkin lymphoma [HL] have improved remarkably, patients with disseminated disease still have a poorer outcome. Stage IV HL is often repported with other "advanced stage" categories, confusing the specific contribution of disease dissemination to the outcome. This single-institution report looks at characteristics and outcomes of this specific category. The medical records of pediatric HL patients [<14 years] from 1975 through 2003 were retrospectively reviewed and the data analyzed. Stage IV patients [n=67] had more poor -risk characteristics than patients in stages I-III [n=300] [B symptoms 86.6% vs. 19.3%, bulky disease 57.6% vs. 45.5% and mediastinal mass 77.6% vs. 29.7%; P < .001 for all characteristics]. The liver was the most common extralymphatic site [in 51.5% of patients with stage IV diseease. Stage IV patients received chemotherapy [CT] alone [n=55] or combined modality therapy [CMT] [n=12]. Fifty-four patients [80.6%] achieved complete remission, 2 [3%] partial remission, 10 [14.9%] had progressive disease and 1 was lost to follow up. Overall survival was 79.4% and event-free survival [EFS] was 63.9% at 5 years. There was a non-significant benefit for CMT [OS=91.7% v. 77.1%, P=.3; EFS=70.7% v. 62.7%, P=.3]. Ten of 12 relapsed and only 1 of 10 progressive disease patients were salvaged. On multivariate analysis, failure to achieve complete remission with CT was associated with a poorer outcome. Stage IV disease is associated with poor risk features and confers a worse outcome than stage I-III disease. Achievement of complete remission with CT is an important prognostic feature. Slow responders may require novel and/or aggressive therapy to achieve complete remission

Morphologic, immunphenotypic and clinical discriminators between T-cell / histiocyte-rich large B-cell lymphoma and lymphocyte-predominant Hodgkin lymphoma

Features of T-cell/histiocyte rich large B-cell lymphoma [THRLBCL] overlap with those of lymphocyte predominant Hodgkin lymphoma [LPHL]. The two lymphomas may represent a spectrum of the same disease, and differentiation between the two can sometimes be difficult. We looked at histomorphologic, immunophenotypic and clinical information that may help differentiate the two entities. Cases of THRLBCL and LPHL were blindly reviewed and studied for histological pattern [nodular vs. diffuse], nuclear features and pattern of expression of CD20, CD30, CD57, epithelial membrane antigen [EMA] and Epstein-Barr virus [EBV]. A score encompassing diffuse histology, high nuclear grade, CD20 single-cell pattern, CD30+, CD57-, EMA-, and EBV+ was estimated for the diagnosis of TCHRLBCL. There were 58 cases, including 30 cases of TCHRLBL and 28 cases of LPHL. The median age was 36 years for TCHRLBCL and 21 years for LPHL [P=0.0001]. Three types of nuclei were identified [lymphocytic/histocytic, Reed-Sternberg and centroblast-like]. The latter two high-grade nuclei were suggestive of TCHRLBCL. TCHRLBCL and LPHL, respectively, showed diffuse histology, 90% vs. 4% [P=0.001], single CD20+ cells, 93% vs. 3.5% [P=0.00004], CD30+ cells, 30% vs. 0% [P=0.01], CD57+ cells, 41% vs. 93% [P=0.008], EMA+ cells, 27% vs. 60% [P=0.113], EBV+ cells, 24% vs. 0% [P=0.117], high nuclear grade, 70% vs. 0% [P=0.001], total score 2-7 [mean 4.68] vs. 0-2 [mean 0.72] [P=0.001], high stage, 86% vs. 7% [P=0.0001]. Our findings indicate that a combination of multiple parameters can help differentiate between the two diseases. Two cases originally diagnosed as LPHL were re-assigned the diagnosis of THRLBCL

Pineal tumors: Treatment and results in 24 patients at one institution

From 1979-1991, 24 patients with pineal region tumors were treated at King Faisal Specialist Hospital. Nineteen patients had histologically Verified tumors, pineal parenchymal 14, germ cell 3 and gliomas in 2 patients. Two of the 5 patients without biopsy had elevated levels of alpha fetoproteins. Surgery was performed in 20 patients. Tumor excision was complete in one patient and partial in 11 patients. Biopsy alone was done in 7 cases. Seventeen patients required a Shunting procedure, and in one patient, it was the only surgical procedure. Twenty patients received primary radiation treatment, localized radiation to the primary site 5, whole brain irradiation followed by a boost to the primary site 6 and craniospinal radiotherapy in 9 patients. The radiation doses varied between 30-55 Gy for the primary site and from 30.6-36 for the whole neuraxis. Three patients with germ cell tumors received chemotherapy. The overall 5-year actuarial survival was 42%