PINAL meningiomas account for approximately 25% of the primary spinal cord tumors and are mainly located in the thoracic region. 1 Usually these are slow growing and histologically benign masses, for which clinical outcome is favorable after complete surgical re- moval. 18 Here, we present the unusual case of a spinal Grade II (atypical) meningioma. The tumor was located in the upper cervical spine, infiltrating the craniocervical junction. Dur- ing the follow-up period two associated metastases devel- oped at the C-6 and T1–3 segments, respectively. Although the meningioma was histologically classified as atypical, it exhibited unusual aggressive behavior and clinical charac- teristics of malignancy. This resulted in the patient’s sur- vival duration of only 21 months, despite multiple re- sections, adjuvant radiotherapy, and hydroxyurea-based chemotherapy. To our knowledge, this is the first reported case of an atypical spinal meningioma associated with CSF metastases and with such fatal progression. Case Report This 23-year-old man suffered an incomplete sensory cross-section syndrome below the C-4 dermatome during a 4-day period in August 2001. Magnetic resonance imag- ing revealed an intra- and paraspinal extramedullary tu- mor located spanning the C1–3 VBs. The paraspinal part of the tumor surrounded the left VA (Fig. 1). Due to the extension of the tumor and his clinical condition, the exci- sion was planned in two steps. At first the intraspinal part of the tumor was resected via a posterior approach with a laminectomy of C2–3 to decompress the spinal cord. Microscopic examination of the specimen showed tis- sue with increased cellularity (Fig. 2A). No cellular whorl, psammoma body, or necrosis was observed. The tumor cells were medium sized with relatively large, round nu- clei and prominent nucleoli. The mitotic index was in- creased up to five mitoses within 10 hpf. Immunoreac- tivity was diffuse for vimentin (data not shown) and focal for EMA (Fig. 2B). Its MIB1 labeling index exceeded J. Neurosurg: Spine / Volume 3 / August, 2005 J Neurosurg: Spine 3:153–158, 2005 An atypical spinal meningioma with CSF metastasis: fatal progression despite aggressive treatment Case report P ATRICK CRAMER, M.D., ULRICH-WILHELM THOMALE, M.D., ALI FUAT OKUDUCU, M.D., ARNE J. LEMKE, M.D., PH.D., FLORIAN STOCKHAMMER, M.D., AND CHRISTIAN WOICIECHOWSKY , M.D., PHD. Departments of Neurosurgery, Neuropathology, and Radiology, Charité, Campus Virchow-Medical Center, Medical School of Berlin, Germany  The authors report the case of a 23-year-old man who presented with a C1–3 spinal mass. Following intraspinal decompression the tumor was histologically classified as an atypical meningioma (World Health Organization grade II). Two further surgical interventions resulted in almost total removal of the meningioma. In addition, radiotherapy was performed. During the 1.5-year follow-up period the diagnostic examinations identified a local tumor recurrence, an intraspinal C-6 metastasis, and a segmental instability with anterior C2–3 slippage and C3–4 kyphosis. The tumor was resected and occipitocervical stabilization was performed. Histological examination showed no change in malig- nancy. Despite additional hydroxyurea-based chemotherapy, the patient presented 4 months later with a hemiparesis and a massive recurrence of the tumor mass involving the posterior fossa and the upper thoracic spine. Because there were no further therapeutical options, the patient died. The authors discuss more aggressive therapeutic options in addi- tion to surgery in patients with metastatic atypical meningiomas. The results in the reported case indicate that menin- giomas associated with cerebrospinal fluid metastasis may represent a higher grade of malignancy. KEY WORDS • cervical spine • atypical meningioma • hydroxyurea • spinal instability S 153 Abbreviations used in this paper: CSF = cerebrospinal fluid; EMA = epithelial membrane antigen; MR = magnetic resonance; VA = vertebral artery; VB = vertebral body; WHO = World Health Organization.