Optimal treatment strategy for intracranial germ cell tumors: a single institution analysis

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>This study retrospectively analyzed the long-term outcomes of 108 consecutive patients to establish the classification and optimal treatment strategy for each subgroup of newly diagnosed germ cell tumors (GCTs).</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>A retrospective review of medical records from the authors' department between April 1989 and March 2007 identified 108 patients with newly diagnosed intracranial GCT. The diagnoses were germinoma in 83 patients, and nongerminomatous GCT (NGGCT) in 25 patients.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>In patients with germinoma, the 10-year overall and progression-free survival (PFS) rates at a median follow-up period of 99 months were 86 and 74%, respectively. Recurrences developed during a range of 6 to 153 months (median 26 months) after starting the initial therapy. Patients treated only with chemotherapy demonstrated a shorter PFS rate, and patients treated with chemotherapy followed by reduced-dose radiation therapy to the whole ventricle, whole brain, or craniospinal axis showed significantly better PFS than patients treated with only radiation or reduced-dose radiation therapy to the focal field. Nongerminomatous GCTs were divided into good, intermediate, and poor prognosis groups as proposed by the Japanese Pediatric Brain Tumor Study Group. In the good and intermediate prognosis groups, the 10-year overall and PFS rates were 100 and 93%, respectively. In the poor prognosis group, the 3-year overall and PFS rates were 56 and 29%, respectively. All patients with NGGCTs, in whom the lesions on MR imaging disappeared after combination therapies consisting of resection, radiation therapy, and chemotherapy, remained alive.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Chemotherapy followed by reduced-dose radiation therapy covering the whole ventricle improves the prognosis for patients with germinoma. Combined therapy of radiation therapy, chemotherapy, and radical resection as an initial or salvage treatment achieved excellent tumor control in the intermediate prognosis NGGCT group. The outcomes were still dismal in the poor prognosis NGGCT group, so initial therapy should target complete disappearance of all lesions on MR imaging.</jats:p></jats:sec>

Improvement of Long-term Results with Neoadjuvant Chemotherapy and Radiotherapy for Central Nervous System Germinoma

“…In contrast, chemotherapy combined with reduced-dose radiation therapy has produced promising results for the control of germinoma (2, 10), although progression was observed even with this combined therapy, with reported recurrence rates between 6.3-20.8% (2,4,6,7,11,16,19).…”

“…Two of these were initially treated with chemotherapy-only regimens and recurred at follow-up of 52 months in a previous report (7). Follow up period in Group C was only 104.6 months in median ranging from 65.1 to 143.9 months.…”

Diagnostic imaging of intracranial germ cell tumors. Review.

“…Germinomas respond well to chemoradiotherapy and their prognosis is relatively good. Mixed tumors composed of any type of teratoma and germinoma are also responsive to appropriate forms of combined therapy including surgery, radiation and/or chemotherapy, and thus their prognosis is better than that of other histological subtypes such as embryonal carcinoma, yolk sac tumor, choriocarcinoma or their mixtures (the so-called "poor prognosis" group), which require more intensive forms of chemo-radiotherapy [5,6]. For this reason, accurate differential diagnosis of these histological subtypes is very important in a clinical setting.…”

Intracranial Germinoma Manifesting as Cavernous Sinus Syndrome

“…The 10-year survival rate is 83.6-90% when a total dose of 40-50 Gy is administered in radiotherapy. 7,8,12,16) Neurohypophyseal germinoma accounts for 42-49% of all intracranial germinomas, 6,10) and the most frequent clinical manifestation is diabetes insipidus, 6) followed by hypopituitarism and bitemporal hemianopsia. However, a germinoma invading the cavernous sinus and presenting with cavernous sinus syndrome is rare, with only a few reported cases.…”