✓ Magnetic resonance imaging (MRI) of the head and cervical spine unexpectedly revealed a cervical meningioma in a patient who had suffered repeated episodes of subarachnoid hemorrhage. The importance of MRI in the diagnosis of tumors in patients with unusual clinical presentation is stressed.
Giuseppe Scotti, Felice Filizzolo, Giuseppe Scialfa, Donatella Tampieri and Pietro Versari
Richard Leblanc, Donatella Tampieri, Yves Robitaille, André Olivier, Frederick Andermann and Alan Sherwin
✓ The authors describe the association between an anterobasal temporal lobe encephalocele and medically intractable temporal lobe epilepsy in three patients treated successfully by surgery. Two men and one woman, aged 26 to 37 years (mean 31 years), had onset of complex automatism and generalized seizures in their second and fourth decades (mean age 22.7 years). They had been epileptic for 6 to 14 years (mean 8.3 years) before surgery. Preoperative electroencephalograms localized ictal epileptic activity to the left mesial temporal lobe in all cases, and neuropsychological testing revealed dominant temporal lobe dysfunction. Magnetic resonance (MR) imaging demonstrated an anteromedial basal temporal encephalocele extending into the pterygopalatine fossa through a bone defect at the base of the greater sphenoid wing in the region of the foramen rotundum and pterygoid process, a discrete center of embryonal chondrification. At surgery, the encephaloceles were found in front of the uncus, and an area of gliosis extended from the encephalocele to the amygdalohippocampal region. All patients have been seizure-free following anterior temporal resection and amygdalohippocampectomy including the encephalocele. These three cases delineate a condition of disordered embryogenesis wherein a developmental anterobasal temporal encephalocele acts as the substrate for temporal lobe epilepsy. This lesion may be diagnosed preoperatively with MR imaging and should be considered in the differential diagnosis of late-onset temporal lobe epilepsy.
Report of three cases
Mark C. Preul, Richard Leblanc, Donatella Tampieri, Yves Robitaille and Ronald Pokrupa
✓ Spinal angiolipomas are distinct, benign lesions composed of mature lipocytes admixed with abnormal blood vessels. Three new cases of spinal angiolipoma are presented and 34 previously reported cases are analyzed. The 37 total cases (23 females and 14 males) ranged in age from 17 to 73 years (mean 43 years; median 45 years). The mean age of the female patients was older than that for the males (45.0 vs. 41.6 years; p < 0.001, Student's t-test) and most were peri- or postmenopausal. Prior to diagnosis, 97% of the patients had weakness of the lower extremities, 94% had sensory dysfunction, 84% had hyperreflexia and spasticity, 51% had sphincter dysfunction, and 41% had back pain lasting from 1 to 180 months (mean 28 months). Five (22%) of the 23 female patients were pregnant and two had exhibited significant weight gain coincident with the onset of symptoms. The angiolipomas were extradural in 35 patients and intramedullary in two; seven of the extradural lesions infiltrated the surrounding bone. The tumors extended from C-6 to L-4 and had a predilection for the midthoracic region (53% of cases). Plain radiographs were abnormal in 11 (39%) of 28 patients and in all patients with bone infiltration. Myelograms were abnormal in 97% of 32 patients and showed a complete block in 63% of patients. Computerized tomography (CT) and magnetic resonance (MR) imaging revealed the fat-density lesions in all cases studied. There was vascular enhancement in three of five cases with contrast-infused CT and in the one case with gadolinium-infused MR imaging. All patients improved following resection of the epidural lesions and internal decompression of the intramedullary lesions.
It is concluded that spinal angiolipomas predominantly affect women. They involve the thoracic (especially the midthoracic) region, and produce symptoms and signs of spinal compression and, in some cases, bone erosion and pathological fractures. Their symptomatology can be exacerbated by pregnancy and weight gain, suggesting that vascular engorgement and the presence of obesity influence their evolution. Their preponderance in older, peri-, or postmenopausal women, and their clinical exacerbation in pregnant women support a role for hormonal influence. Magnetic resonance imaging is the investigation of choice for the diagnosis of these lesions. Surgery is universally successful in relieving symptoms.
Zhong-Ping Chen, Daniel Yarosh, Yesenia Garcia, Donatella Tampieri, Gérard Mohr, Adrian Langleben and Lawrence C. Panasci
Adjuvant nitrosourea chemotherapy fails to prolong patient survival significantly as many tumors demonstrate resistance to these drugs. It has been documented in cell lines that O6-methylguanine-DNA methyltransferase (MGMT) plays an important role in chloroethylnitrosourea (CENU) drug resistance.
The authors evaluated MGMT expression in 22 glioma specimens by using an immunofluorescence assay and compared the results with clinical response of the patients to CENU-based chemotherapy.
The patients were treated with CENU after evidence of progressive disease following surgery and radiotherapy. Eight tumor samples had no detectable MGMT, whereas other samples had from 9989 to 982,401 molecules/nucleus. In one group (12 patients), the tumor decreased in size or was stable (effective group), whereas in the other group (10 patients), the tumor demonstrated continuous growth during chemotherapy (progressive group). The median time to progression (TTP) was 6.7 months with a median survival of 13 months. The Mer− patients (MGMT < 60,000 molecules/nucleus) appeared to have more chance of stable disease or response to CENU therapy than the Mer+ patients (MGMT > 60,000 molecules/nucleus) (chi-square = 4.791, p = 0.0286). In patients with glioblastomas multiforme (GBMs), the TTP of Mer+ patients was shorter than that of Mer− patients (t = 2.04, p = 0.049). As a corollary, the MGMT levels were significantly higher in GBM tumors from the progressive group than those from the effective group (t = -2.26, p = 0.029). The TTP and survival time in the effective GBM group were also longer than those in the progressive GBM group. However, there was no significant correlation between MGMT levels and either the survival time (r = 0.04, p = 0.8595) or TTP (r = 0.107, p = 0.6444).
Results from this study suggested that MGMT positivity is indicative of more aggressive disease that progresses more rapidly when exposed to CENU therapy. However, MGMT-negative tumors are not always sensitive to CENU agents, suggesting that other factors may also be important.
Burak Sade, Gérard Mohr, Donatella Tampieri and Arthur Rizzo
✓ An aneurysm completely included within a pituitary adenoma that lies inside the sella turcica is rare and challenging from both a diagnostic and treatment viewpoint. A 39-year-old woman presented with symptoms and signs of acromegaly. Magnetic resonance imaging revealed a pituitary macroadenoma, which was associated with an intrasellar aneurysm. Digital subtraction angiography confirmed the presence of the cavernous carotid artery aneurysm. Complete endovascular obliteration of the aneurysm was achieved using Guglielmi Detachable Coils and the patency of the internal carotid artery was maintained. The pituitary adenoma was resected subtotally via a transsphenoidal microsurgical approach 8 months later. Preoperative detection of a coexisting intrasellar aneurysm in a patient with a pituitary adenoma is mandatory to avoid life-threatening hemorrhagic complications. Endovascular coil placement is an effective treatment option when performed before the transsphenoidal removal of the adenoma.
Marlise P. dos Santos, Jingwen Zhang, Diana Ghinda, Rafael Glikstein, Ronit Agid, Georges Rodesch, Donatella Tampieri and Karel G. terBrugge
Intraspinal tumors comprise a large spectrum of neoplasms, including hemangioblastomas, paragangliomas, and meningiomas. These tumors have several common characteristic imaging features, such as highly vascular mass appearance in angiography, hypointense rim and serpentine flow voids in MRI, and intense enhancement after intravenous contrast administration. Due to their rich vascularity, these tumors represent a special challenge for surgical treatment. More recently, the surgical treatment of intraspinal vascular tumors has benefited from the combination of endovascular techniques used to better delineate these lesions and to promote preoperative reduction of volume and tissue blood flow. Endovascular embolization has been proven to be a safe procedure that facilitates the resection of these tumors; hence, it has been proposed as part of the standard of care in their management.