Clinicoradiologic response of neurologic tuberculous mass lesions in children treated with thalidomide.

Neurologic tuberculous pseudoabscesses that clinically progress despite conventional antituberculosis therapy may be responsive to adjuvant thalidomide, a potent tumor necrosis factor-α inhibitor. In this study, the addition of thalidomide provided substantial clinical benefit in the majority of patients, and magnetic resonance imaging evolution of lesions from early-stage "T2 bright" with edema to "T2 black" represented a marker of cure.

Pineal parenchymal tumor of intermediate differentiation: imaging spectrum of an unusual tumor in 11 cases.

INTRODUCTION: Pineal parenchymal tumor of intermediate differentiation (PPTID) was recognized in the 2007 World Health Organization (WHO) classification as a new pineal parenchymal neoplasm, intermediate in malignancy (WHO grade II or III) between pineocytoma (grade I) and pineoblastoma (grade IV). The imaging spectrum of this new tumor has not been previously delineated. We describe the imaging spectrum in 11 pathologically proven PPTIDs and identify findings that may suggest the preoperative diagnosis of this newly recognized entity. METHODS: Electronic medical records over the last 9 years and teaching files between the years 1985 and 1995 were searched for atypical pineal lesions. Additional cases were added from the teaching files of contributing authors. RESULTS: Imaging studies in nine patients (9/11) showed bulky, aggressive pineal region masses with local brain invasion; two patients (2/11) demonstrated circumscribed pineal masses. Two patients had spinal metastases at presentation. On computed tomography (CT), five patients had classic "exploded" calcifications characteristic of pineal parenchymal tumors. All tumors were heterogeneously hypointense on T1WIs and heterogeneously hyperintense on T2WIs. Post-contrast scans showed marked heterogeneous (10/11) or uniform (1/11) enhancement. Cystic foci were identified in eight cases. Intratumoral hemorrhage was present in one case. CONCLUSION: While no singular neuroimaging feature is pathognomonic of PPTID, these tumors are usually larger, demonstrate local invasion, and appear much more heterogeneous than pineocytoma. Because PPTIDs have a higher grade and increased potential for recurrence as compared to pineocytomas, it is important to consider this diagnosis as shorter follow-up, and adjuvant therapy may be indicated in selected cases.

A study of meningiomas in South Africa: investigating a correlation between clinical presentation, histopathology and genetic markers.

OBJECTIVE: To determine whether there are certain genetic markers which correlate with particular clinical characteristics of meningiomas including multiplicity, recurrence and calvarial erosion. METHODS: Thirty-eight South African-born patients with meningiomas were recruited for this study. At surgery, blood and tumour specimens were obtained for histopathological, cytogenetic and molecular analysis. Loss of heterozygosity (LOH) on chromosomes 1p and 22q were investigated and the NF2 gene on 22q12.2 was screened for disease-causing mutations. RESULTS: The commonest tumour locations were convexity (25%) and parasagittal (21%). The histology results showed that 86.8% of the patients had Grade I tumours and the remainder had Grade II tumours. A pathogenic nonsense mutation, R341X in the NF2 gene was found in only one patient. LOH on each of chromosomes 1p and 22q was observed in 44.7% of patients, but in different individuals. Significant associations were found between having specific tumour characteristics and both male gender (p-value = 0.0059) and 22q LOH (p-value = 0.0425). We estimated that having 22q LOH makes an individual approximately four times more likely to develop a tumour that exhibits multiplicity, recurrence or calvarial erosion (OR = 4.8; 95% CI: 1.2-23.4). Adjusting for gender strengthened this effect (OR = 6.1; 95% CI: 1.1-48.7). CONCLUSIONS: Our data indicate that male patients and patients with a meningioma that has 22q LOH are more likely to develop tumours exhibiting multiplicity, recurrence or calvarial erosion. We recommend that this subset of patients should be followed up more closely. Further study is needed to determine the benefit of adjuvant radiation therapy in this scenario.

MRI findings in acute idiopathic transverse myelopathy in children.

OBJECTIVE: To describe the clinical and MRI findings in three children with acute idiopathic myelopathy (AIM). MATERIALS AND METHODS: Retrospective review of the clinical presentation, MRI findings and outcome of three patients diagnosed with acute idiopathic transverse myelitis. RESULTS: Of note was the swift onset of symptoms in all patients, without any preceding illness or history of vaccination in two of the patients, and the rapid resolution of symptoms on steroid therapy in all the patients. MRI showed T2-weighted hyperintensity and patchy enhancement with gadolinium, but the extensive cord involvement did not correlate with the severity of presentation or outcome. CONCLUSIONS: Our findings do not support that MRI evidence alone of diffuse myelopathy is a predictor of poor outcome in childhood AIM.

October 2001: 40-year-old Xhosa male with back pain and leg weakness.

A 40-year-old Xhosa male presented with progressive upper lumbar back pain and weakness At examination he was emaciated and had enlarged lymph nodes in the groin and axilla. Both lower limbs were severely atrophic and weak. Sensation to touch and pain was decreased below L3 bilaterally. MR of the spine showed a discrete, contrast-enhancing epidural mass. A T10-T12 laminectomy revealed an soft, vascular extradural tumor dorsal to the cord. The mass was loosely applied to the dura and easy to remove. The operative specimen consisted of a sausage-shaped (3.5 x 2.0 x 1.2 cm), thinly-encapsulated mass of reddish-brown tissue. The cut surface had a mottled, vaguely nodular, yellowish-brown appearance. Microscopic examination revealed sheets of hematopoeitic elements, including myeloid, red cell and megakaryocytic lines, the latter showing Factor 8-related positivity. The final diagnosis was extramedullary hematopoiesis (EMH). A bone marrow biopsy performed as a result of the diagnosis showed a myeloproliferative disease and polycythemia vera. EMH in the spinal epidural space is a rare but treatable cause of progressive paraparesis in patients with a variety of hematological disorders. Since 1956 there have been more than 50 reported cases, most of which occurred in association with thalassaemia. In spinal cord compression secondary to EMH, the lesions are commonly localized to the mid-lower thoracic region.