Giant cell glioblastoma is a rare variant within the spectrum of glioblastoma multiforme (GBM) tumors. A giant cell glioblastoma may be associated with a better prognosis than the common type of GBM after combined treatment involving tumor resection and radiochemotherapy. A giant cell glioblastoma may occur at various sites in the brain and spinal cord. To the authors' knowledge, this type of tumor has not been previously reported as arising as an exophytic tumor from the medulla oblongata. The authors report on a 40-year-old man who presented with a large tumor located in the caudal fourth ventricle. The tumor was removed completely and the patient underwent percutaneous radiotherapy with 60 Gy and concomitant chemotherapy with temozolomide. Histopathological examination of the tumor revealed the typical features of a giant cell glioblastoma. At the 2-year follow-up the patient was doing well and showed no signs of tumor recurrence. It is important to identify variants of GBM because they may predict favorable long-term outcome, even when they arise from the caudal brainstem.
Goetz Luetjens, M. Javad Mirzayan, Almuth Brandis, and Joachim K. Krauss
Dirk Rades, Fedor Heidenreich, Marcos Tatagiba, Almuth Brandis, and Johann Hinrich Karstens
✓ Meningeal melanocytomas are uncommon lesions. They are generally considered to be benign tumors that derive from leptomeningeal melanocytes. A rare case of a metastatic spinal meningeal melanocytoma is presented. All relevant cases reported in literature since 1972, when the term “meningeal melanocytoma” was first used, were reviewed. Rates of tumor recurrence from 1 to 5 years were calculated for this rare lesion, based on published data and on additional information obtained from personal contact with most of the authors. Recurrency rates of 47 patients suitable for evaluation were correlated with the different therapeutic approaches. Complete tumor resection alone and incomplete resection alone followed by irradiation appeared to be superior to incomplete resection alone in terms of disease-free survival. Statistical significance was achieved for complete tumor resection at follow up between 1 and 4 years (range p = 0.010–0.050) and for incomplete resection combined with radiotherapy after 2 years (p = 0.034). Complete tumor resection should be considered the best therapeutic option, followed by incomplete resection combined with postoperative radiotherapy.
Guilherme R. Montibeller, Makoto Nakamura, Almuth Brandis, and Joachim K. Krauss
Florian Roser, Makoto Nakamura, Almuth Brandis, Volkmar Hans, Peter Vorkapic, and Madjid Samii
✓ The authors describe the first case of an intracranial transition of a melanocytoma into a primary malignant melanoma within a short time. A 37-year-old woman presented with progressive brainstem syndrome due to a tumor, originally diagnosed and treated 12 years earlier, that extended from the petroclival area to the anterior craniocervical junction. The histological workup following subtotal tumor resection of the initial tumor had revealed the typical features of a fibrous melanocytic meningioma without increased proliferation. Ten years after the patient had completed treatment for the melanocytic meningioma, control neuroimaging demonstrated growth of the residual tumor with compression of the brainstem. Another neurosurgical intervention revealed a dark tumor of hard consistency. At this time immunohistochemical examinations demonstrated melanocytic features (expression of vimentin, S100 protein, and melan A) of the lesion with focally increased proliferation (5% of Ki-67—positive cells) but no higher mitotic activity. Clinical signs of deterioration along with imaging-confirmed tumor progression precipitated another operation within 7 months. A neuropathological examination revealed epithelial and anaplastic changes and indicated that the MIB-1 indices were greater than 25%. Pleomorphic changes and a focal high mitotic activity led to the diagnosis of a primary cerebral malignant melanoma. The patient's later clinical course consisted of a rapid diffuse meningeal spread of the lesion throughout the entire brain and spine. Despite whole-brain and stereotactic radiation therapy as well as chemotherapy, the patient died 4 months after the last neuropathological diagnosis. Although grossly resembling a meningioma, melanocytomas lack the former's histological and immunohistochemical features. The biological behavior of a melanocytoma is variable and recurrence may happen after subtotal resection, but intracranial transition into a malignant melanoma has not been observed previously.
Hans-Holger Capelle, Makoto Nakamura, Thomas Lenarz, Almuth Brandis, Bernd Haubitz, and Joachim K. Krauss
Intracranial extraaxial cavernous angiomas are rare vascular malformations. Their occurrence at the geniculate ganglion of the facial nerve within the temporal bone is exceptional. The authors describe a 35-year-old man who developed a slowly progressing facial palsy. Initial cranial MR imaging showed no pathological findings, but 2 years later another MR examination detected a small tumor located at the geniculate ganglion of the facial nerve. The tumor was removed via a subtemporal approach. Histological examination revealed a cavernous angioma. Even small cavernomas located at the geniculate ganglion of the facial nerve may result in facial palsy. Isolated facial palsy in a young person should be monitored closely using imaging studies even if the initial imaging study is negative. Early decompression of the facial nerve may help to preserve its function.
Jussi Antinheimo, Hannu Haapasalo, Matti Haltia, Marcos Tatagiba, Sebastian Thomas, Almuth Brandis, Markku Sainio, Olli Carpen, Madjid Samii, and Juha Jääskeläinen
✓ The authors compared the histological appearance and proliferation potential of 35 meningiomas in patients with neurofibromatosis 2 (NF2) and 30 sporadic meningiomas in age- and gender-matched patients without NF2. The NF2 meningiomas showed more mitotic figures (p < 0.001) and nuclear pleomorphism (p = 0.003) than the sporadic meningiomas; however, the incidence of meningothelial, fibroblastic, and transitional subtypes occurred equally in both groups. The proliferation potential was significantly higher in the 35 meningiomas removed from 23 patients with NF2 than in the 30 sporadic meningiomas removed in the 30 patients without NF2 (mean MIB-1 labeling indices: 2.5 vs. 1.75, p = 0.0147). The higher proliferation potential of the NF2 meningiomas may reflect differences in molecular biology between sporadic and NF2 meningiomas and may be related to an earlier onset, multiplicity, and more aggressive behavior of NF2 tumors.