Ocular tuberculosis presenting with a conjunctival granuloma.

An 18-year-old female patient was referred with complaints of tearing and redness in the left eye for 3 months after a mild ocular trauma with a turkey feather. She was treated with topical antibiotics and corticosteroids with no improvement. Slit-lamp examination of the left eye showed a vascularized lesion with a polypoidal appearance due to multiple contiguous micronodules on the temporal and inferior bulbar conjunctiva. Results of the anterior and posterior segment examination were unremarkable in both eyes. A biopsy specimen of the conjunctival mass showed multiple tuberculoid granulomas composed of epithelioid histiocytes with associated Langhan's type multinucleate giant cells and a necrotic nodule surrounded by histiocytes and giant cells. The Mantoux test was positive with induration of 15mm. The patient was prescribed antituberculosis therapy. Three months after treatment initiation, the conjunctival lesions had resolved. Mycobacterium tuberculosis should be considered in cases of unilateral chronic recalcitrant conjunctivitis. Biopsy of a conjunctival mass is of utmost importance to establish a definite diagnosis.

Epstein-Barr virus infection in gliomas.

PURPOSE OF THE STUDY: Epstein-Barr virus (EBV) has been involved in the development of some tumors, including Burkitt's lymphoma and Hodgkin's lymphoma. However, its potential role in glioma tumorigenesis remains debated. In this study, we investigated the EBV infection in gliomas from Tunisian patients. PATIENTS AND METHODS: We conducted a retrospective study of 112 gliomas on archival material. The EBV DNA sequence was analyzed by polymerase chain reaction (PCR). Latent membrane protein 1 (LMP1) was detected by immunohistochemistry. In situ hybridization was used to detect EBV encoded small RNA (EBER). Clinicopathological features were recorded. Survival analysis was carried out using the Kaplan-Meier method and the Log-Rank test to compare EBV-positive and EBV-negative patients. RESULTS: Overall, there were twenty-four EBV-positive gliomas (21.4%). EBV DNA was identified in 24 cases. LMP1 and EBER were detected in four EBV DNA-positive cases. All EBV-positive cases were glioblastomas multiforme (GBM). Median overall survival and recurrence-free survival of EBV-negative patients were better than those of EBV-positive patients (Log Rank p = 0.006). CONCLUSION: Altogether, these findings support the occurrence of EBV infection in Tunisian GBM. Furthermore, when compared to EBV-negative tumors, EBV infection seems to be associated with the worst patient prognosis. Advanced molecular studies are recommended to confirm these results and to shed further light on the potential role of EBV in these devastating tumors.

Intracranial angiomatous meningiomas: A 15-year, multicenter study.

BACKGROUND: Angiomatous meningiomas (AMs) represent a rare subtype of meningiomas in which the vascular component prevail. They represent less than 1% of all intracranial tumors and approximately 2.1% of all meningeal tumors (Hasselblatt et al., 2004). The purpose of this study was to determine the clinical characteristics, radiological features and prognosis of AMs based on a Tunisian multicenter experience in the management of 58 successive cases of intracranial AMs. To the best of our knowledge, this is the largest series reported to date. METHODS: We retrospectively reviewed 58 patients of AM treated in the departments of Neurosurgery of The Tunisian National Institute of Neurology, Sahloul University Hospital and Fattouma Bourguiba University Hospital from January 2001 to December 2015. Clinical characteristics, radiographic features and treatment modality, in the form of radical surgery, were noted. Statistical analysis was done with regards to recurrence free survival (RFS) and overall survival (OS) using Kaplan-Meier survival analysis. RESULTS: The median age of the patients on admission was 53.0 years (8-78). Twenty-two patients were males and thirty-six were females. The median duration of symptoms prior to presentation was six months. Signs of increased intracranial pressure were the most common presenting symptom followed by seizures, motor weakness and cranial nerves paresis. Cystic changes were observed in 50% of cases, moderate to severe peritumoral edema in 81% of cases and multiple signal voids suggestive of blood vessels in 86.2% of cases. Forty-two patients underwent Simpson I excision (72.4%) while fourteen had Simpson II excision (24.1%). A Simpson III resection was realized in two patients (3.5%). The surgical resection was hemorrhagic in 63.8% of cases and in 13.8% of cases, several blood transfusions were necessary to maintain hemodynamic stability. No adjunctive treatment was administered. Six out of the fifty-six patients of the Simpson I/II groups recurred while one of the two patients with Simpson III resection had tumor regrowth. Median duration of RFS was 103 months. Second surgery was realized in all cases with no subsequent recurrence. The extent of tumor resection and the location were found to correlate with the risk of developing recurrences (P=0.001). CONCLUSIONS: AMs represent a rare subtype of meningioma characterized by variable cystic components, large peritumoral edema and multiple areas of vascular signal voids. The mainstay of the treatment is gross total resection, ideally following a preoperative embolization. The fate of the tumor remnant after incomplete tumor resection still needs to be evaluated and we do not recommend the systematic use of post-operative adjuvant RT in all cases. As local recurrence can develop many years after initial treatment, Long-term follow-up is mandatory.

MGMT methylation assessment in glioblastoma: MS-MLPA versus human methylation 450K beadchip array and immunohistochemistry

Purpose: The MGMT gene encodes a DNA repair enzyme that counteracts with chemotherapy efficiency, specifically with alkylating agents such as temozolomide (TMZ). It is well established that MGMT methylation should be screened as a predictive marker for TMZ in glioblastoma, and we thus aimed to determine a reliable and practical diagnostic method of MGMT methylation detection. Patients and methods: 55 glioblastomas were investigated for MGMT methylation status using methylation-specific multiplexed ligation probe amplification (MS-MLPA), illumina human methylation 450K BeadChip array (HM450 K) analysis, and compared to MGMT protein expression by immunohistochemistry (IHC) staining. The methylation status of promoter, intron and all MGMT CpG targeted sites were separately correlated to patient’s survival. Results: In addition to MS-MLPA and 450 K concordance, our results showed significantly higher overall survival (OS) of patients receiving TMZ and presenting MGMT methylated promoter (mean OS = 21.5 months, p = 0.046). Including all glioblastoma cases and regardless of chemotherapy, MS-MLPA showed significant survival difference between MGMT methylated and unmethylated cases (mean OS = 13, p = 0.021). Conclusion: We concluded that in glioblastoma, MGMT promoter methylation predicts TMZ sensitivity. This current comparative analysis leads to consider that MS-MLPA is a valuable as HM450 K array for MGMT methylation status screening (AU)

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MGMT methylation assessment in glioblastoma: MS-MLPA versus human methylation 450K beadchip array and immunohistochemistry.

PURPOSE: The MGMT gene encodes a DNA repair enzyme that counteracts with chemotherapy efficiency, specifically with alkylating agents such as temozolomide (TMZ). It is well established that MGMT methylation should be screened as a predictive marker for TMZ in glioblastoma, and we thus aimed to determine a reliable and practical diagnostic method of MGMT methylation detection. PATIENTS AND METHODS: 55 glioblastomas were investigated for MGMT methylation status using methylation-specific multiplexed ligation probe amplification (MS-MLPA), illumina human methylation 450K BeadChip array (HM450 K) analysis, and compared to MGMT protein expression by immunohistochemistry (IHC) staining. The methylation status of promoter, intron and all MGMT CpG targeted sites were separately correlated to patient's survival. RESULTS: In addition to MS-MLPA and 450 K concordance, our results showed significantly higher overall survival (OS) of patients receiving TMZ and presenting MGMT methylated promoter (mean OS = 21.5 months, p = 0.046). Including all glioblastoma cases and regardless of chemotherapy, MS-MLPA showed significant survival difference between MGMT methylated and unmethylated cases (mean OS = 13, p = 0.021). CONCLUSION: We concluded that in glioblastoma, MGMT promoter methylation predicts TMZ sensitivity. This current comparative analysis leads to consider that MS-MLPA is a valuable as HM450 K array for MGMT methylation status screening.

Adult recurrent pilocytic astrocytoma: Clinical, histopathological and molecular study.

BACKGROUND: PA is a grade I glial tumor that mostly occurs in children. However, although apparently similar to paediatric PA, adult PA presents a different clinical follow-up that could arise from specific molecular alterations. A variety of genetic alterations have been identified as diagnostic or prognostic glioma molecular markers. MATERIAL AND METHODS: We describe a right infratentorial tumor that occurred in a 58-year-old man. Neuroimaging and neuropathological examination suggested PA as an initial diagnosis. The tumor was completely resected. Unexpectedly, two years later, a rapidly growing tumor on the operative site was observed with a second location in the pineal region. Immunohistochemical reactions (IHC), Multiplex ligation probe amplification (MLPA) and fluorescence in situ hybridization (FISH) was performed in both primary and relapse tumor. RESULTS: Neuroimaging and neuropathological examinations suggested an unusual diagnosis for adult patients: a recurrent PA. Both MLPA and FISH analysis contribute to diagnostic confirmation by KIAA1549: BRAF fusion detection. Additional genetic results revealed interesting findings that justified the tumor aggressivity. CONCLUSION: Molecular analysis of adult PA cases should be routinely combined with histopathological and neuroimaging examination to further refine prognostic diagnoses.

Juvenile hyaline fibromatosis: a case report.

Juvenile hyaline fibromatosis is a rare, hereditary disease with distinct clinical and histopathological features. Clinically, it presents with gingival hypertrophy, pappulonodular skin lesions and joint contractures. Bone involvement is usually an uncommon finding. We report a case of a 2-year-old patient, daughter of consanguineous parents, who presented since the age of 2 months with impairment of mental development, multiple joint contractures, motion limitation and nodules on the scalp. The calvarian lesions were surgically removed, and histopathological examination concluded to juvenile hyaline fibromatosis.

[Diagnosis of spinal diseases by MRI]. / Irm et rachis.

For a long time the imaging diagnosis of spine disease was based on conventional radiology (plain radiography, myelography and arteriography). The recent employment of MRI had changed the study of spine disease thanks to an excellent contrast and a multiplanar approache. In fact, nowadays MRI is the most sensible technic for a combined study of spine container and contents and medullar tissue.

[Magnetic resonance imaging features of desmoplastic cerebral ganglioglioma of infancy: report of 1 case]. / Imagerie par résonance magnétique du gangliogliome desmoplastique infantile cérébral: à propos d'un cas.

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of infancy, characterized by solid and cystic component, voluminous size and supratentorial location. These tumors are diagnosed usually below the age of 2 years. We report 1 case of desmoplastic ganglioglioma in 13-year-old male. Computed tomography and magnetic resonance imaging diagnosed supratentorial mixed cystic and solid tumor, which presented as a large cystic component with intense contrast enhancement of a mural nodule. The tumor was surgically removed, and histology revealed desmoplastic ganglioglioma. The patient had a good follow up. This observation emphasizes the possibility of desmoplastic ganglioglioma in older infants. It mustn't be considered as a specific entity of very young age infant and must be recognized in older infant because it may be misdiagnosed as malignant glioma. Despite the pseudo malignant appearance, these tumors have a good prognosis after surgery and when excision is complete they don't led to recurrences.

[Cerebral hydatid disease: imaging features]. / Hydatidose cérébrale: aspects en imagerie.

Cerebral hytatid cysts (HC) are extremely rare, forming 2% of all intra cranial space occupying lesions even in counties where the disease is endemic. HC diagnosis is usually based on a pathognomonic computed tomography (CT) pattern. In order to assess the value of MR we reviewed the CT (n=25) and magnetic resonance (MR, n=4 including diffusion and proton magnetic resonance spectroscopy in 1) imaging of 25 patients with pathologically confirmed cerebral hydatid disease. 19 HC were seen in children under 16 years. All were supra tentorial with 22 in the middle cerebral artery territory. HC was solitary in 18 cases, unilocular in 23 and multi-vesicular in 2 with heavily calcified pericyst in 1. 2 cysts were intra ventricular and 1 intra aqueducal. The most typical features were well defined, smooth thin walled spherical or oval cystic lesions of CSF density and/or signal with considerable mass effect (20/25). Surrounding oedema with complete or incomplete rim enhancement was seen in 3 cases which were labelled as complicated and/or infected cysts. Although CT is diagnostic of hydatid disease in almost all cases (22/25), MRI including diffusion and spectroscopy precisely demonstrate location, number, cyst capsule, type of signal and enhancement and allows diagnosis of atypical or complicated HC and appears more helpful in surgical planning.

[Magnetic resonance imaging features of cystic meningiomas. Report of four cases]. / Aspects en imagerie par resonance magnetique des méningiomes kystiques. A propos de quatre cas.

Four cases of cystic meningioma are reported, and the imaging features and diagnostic pitfalls of cystic meningiomas are reviewed. Cystic meningiomas are infrequent tumors and remain difficult to diagnose in spite of advanced imaging techniques. Our patients were between 15 and 58 years of age, and underwent CT and MR imaging. In all the four cases, the meningiomas were supratentorial and included Nauta type I, type II and type III tumors.

[Calcification of the cervical ligamentum flavum. Case report and review of the literature]. / Calcification du ligament jaune du rachis cervical. A propos d'un cas et revue de la littérature.

Calcification of the cervical ligamentum flavum is a rare entity observed exclusively in Japanese people. We report a new case in a 65-year-old man from Tunisia who presented with symptoms of cervical myelopathy with mild tetra paresis, sensory abnormalities and dysuria. Magnetic resonance imaging (MRI) showed a posterior compression of the spinal cord at C3-C4. CT-scan showed a calcification of the ligamentum flavum at level C3-C4, compressing the left postero-lateral aspect of the spinal cord. C3-C4 laminectomy was performed with removal of abnormal ligamentum flavum tissue. The postoperative course was uneventful and all symptoms resolved. Calcification of the cervical ligamentum flavum is a rare entity; the diagnosis is easy but the pathogenesis remains unclear. Literature regarding this pathology is reviewed.

[Intradural and cervical primary malignant melanoma. Case report and review of the literature]. / Le mélanome malin primitif intra-dural et cervical. A propos d'un cas et revue de la littérature.

Primary malignant melanoma of the central nervous system is an uncommon localization, first reported by Hirsberg in 1906. Since then, to our knowledge, only 39 cases have been reported in the literature. We present a case of primary intradural extra-medullary melanoma which developed in a 51-Year-old man who complained of pain in the lower cervical spine, difficulties in micturition and sexual impotence. The diagnosis was suspected at the MRI which showed a lesion with a paramagnetic signal and was confirmed by the histological examination. The resection was complete and the course has been satisfactory after 19 months follow-up.

[Solitary plasmacytoma. Apropos of a case of craniofacial involvement]. / Plasmocytome solitaire. A propos d'un cas de localisation cranio-faciale.

The authors report the case of a 24 years-old man who had a first tumor in the right maxilla diagnosed as Ewing's sarcoma and treated by chemotherapy and radiotherapy. A second tumor appeared in the skull vault twenty seven months later. The histological diagnosis after removal of the lesion was IgG Lambda plasmacytoma. The revision of the histological cut of the maxillary tumor retained the same diagnosis. The patient is without evidence of local recurrence and systemic diffusion of the disease two years after treatment. The authors insist on the necessity of immunochemistry to establish the diagnosis.

[Intramedullary D12-L1 epidermoid tumor. Report of a case and review of the literature]. / Tumeur épidermoïde intramédullaire D12-L1. Rapport d'un cas et revue de la littérature.

The authors report, after a follow-up period of two years, a case of intramedullary congenital epidermoid cyst treated surgically. The patient, a 21 year-old man, presented with a seven year history of motor disturbances of his left lower limb and more recent urinary incontinence. The diagnosis of an intramedullary tumor was provided by a myelogram and a post-myelogram CT. In the absence of a dermal sinus or vertebral dysraphic anomalies, the epidermoid nature of the mass was an operative and histological finding. The main data from the literature are commented: frequency, topography, clinical diagnosis, and radiologic or M.R.I. imaging. Intracapsular removal of the mass seems to result, as in the presently reported case, in a satisfactory outcome.

[Confluent tuberculomas of the vermis cerebelli associated with an occipital localization. Report of a case and review of the literature]. / Tuberculomes confluents du vermis cérébelleux associés à une autre localisation occipitale. Rapport d'un cas et revue de la littérature.

An unusual case of multiple cerebral tuberculomas is reported. The patient, a 19-year-old woman, presented with a 6-month history of raised intracranial pressure and cerebellar symptoms. CT scan disclosed a conglomerate of several ring-enhancing lesions within the cerebellar vermis and another homogeneously enhancing mass against the right occipital horn. Solid and ring-enhancing lesions are the main mode of presentation of cerebral tuberculomas. Although the patient had no history of systemic tuberculosis, this diagnosis was considered highly probable in this geographic area and was confirmed by pathological examination of the cerebellar mass, the excision of which was considered necessary. Antitubercular medications led to complete regression of the associated occipital lesion, as demonstrated by sequential CT scan examinations.

Cerebral candidiasis. Computed tomography appearance.

A three year old child who had been suffering from oral candidiasis since the age of 1 year presented with osteitis of the clavicle, 2 cerebral frontal abscesses and an occipital abscess which extended across the calvaria and was associated with osteolysis. Histological and microbiological studies following surgery confirmed the diagnosis of candidiasis in this girl who was found to have IgA immunodeficiency. The authors report the computed tomographic appearance of the cerebral lesions and review the literature.