Two cases of recurrent pilocytic astrocytoma with leptomeningeal dissemination (LMD) are described. A 6-year-old boy presented with a cerebellar tumor, which was subtotally removed. Tumor recurrence with LMD occurred 4 years later. Reoperation for tumor removal followed by craniospinal irradiation stabilized the LMD over 5 years. A 4-year-old girl presented with a chiasmatic-hypothalamic tumor. Partial removal of the tumor was followed by radiation therapy. Tumor regrowth with LMD occurred 4 years later and was managed by reoperation, chemotherapy and radiotherapy. Tumor recurrence with LMD can be stabilized by multimodal treatment without tumor progression.
Hinge technique is a new method for cerebral decompression that allows the bone flap to move outward in response to brain swelling and essentially allows reconstruction of the cranial vault as a minor procedure under local anesthesia. This retrospective study assessed outcomes following the use of this new decompressive technique. During an approximately 7-year period (June 2004 to March 2011), 58 patients who had suffered head trauma or stroke underwent cerebral decompression using the hinge technique or conventional decompressive craniectomy. Patients were assessed with the Glasgow Coma Scale (GCS), the Glasgow Outcome Scale (GOS), and the modified Rankin scale (mRS). Twenty-one patients (16 males, 5 females; age range, 21-78 yrs; mean age, 57.4 ± 15.5 yrs) underwent cerebral decompression using the hinge technique, and 37 patients (18 males, 19 females; age range, 5-83 yrs; mean age, 54.1 ± 20.9 yrs) underwent conventional decompressive craniectomy. There was no significant difference in preoperative GCS or postoperative GOS or mRS between the two groups. Six patients in the decompressive craniectomy group and none of the patients in the hinge technique group developed bone flap infection (p = 0.02). The bone flap was removed in two cases in the hinge technique group due to low cerebral perfusion pressure as well as elevated intracranial pressure (ICP). The hinge technique with ICP monitoring was effective and safe for management for head trauma or stroke and was not associated with bone flap infection.
A 64-year-old female presented with rapid onset of left ophthalmoplegia and truncal ataxia, after experiencing diplopia due to left abducens nerve palsy for a year. She had undergone surgery twice for left trigeminal neuralgia caused by a large intracranial epidermoid cyst at the age of 48 and 52 years. The intracranial epidermoid cyst grew and became predominantly enhanced by contrast medium on computed tomography (CT) and T 1 -weighted magnetic resonance (MR) imaging, which had not been observed earlier. The tumor was partially removed and the histological diagnosis was squamous cell carcinoma (SCC). Radiation therapy was administered, but she presented with paraplegia of the bilateral lower extremities and anesthesia due to spinal multiple metastases of SCC one year later. Radiation therapy was administered for the spinal lesions, but she died of multiple metastases to the cerebellum and medulla oblongata with hydrocephalus 2 years after the third surgery. Transformation of intracranial epidermoid cysts to SCC appears as predominant enhancement on CT or T 1 -weighted MR imaging with rapid deterioration of neurological features. All reported cases of malignant transformation of intracranial epithelial cysts to SCC with leptomeningeal carcinomatosis have occurred in intracranial epidermoid cysts.
A 22-year-old man presented with tenderness and swelling of the left lateral part of the orbit. Computed tomography revealed a left intraorbital mass measuring 3 cm x 3 cm involving the left lateral wall of the orbit and the greater wing of the left sphenoid bone. Magnetic resonance imaging revealed that the intraorbital mass was extraneuroaxial. During surgery, the tumor was seen to arise from the lateral wall of the orbit and infiltrate into the left temporal muscle. Following the surgery, the patient was administered radiation therapy for the whole cranium and chemotherapy for the residual tumors. However, the tumor recurred, and the patient died about 2 years following the first surgery because the tumor had metastasized to the lung. On light microscopy, the tumor cells were closely packed with uniform, small, and round cells. Immunohistochemical studies showed that the tumor cell membrane stained positive for MIC2. Furthermore, the MIB-1 labeling index was 36.2%. On electron microscopy, small quantities of cytoplasm containing glycogen accumulations without neurosecretory granules and neurofilaments were observed. Based on these results, the tumor was diagnosed to be primary Ewing's sarcoma. Primary orbital Ewing's sarcoma of the skull has been considered to be extremely rare, and a review of the literature was performed.
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