Intramedullary Metastasis of Medulloblastoma: A Case Report

Medulloblastoma (MB) is a malignant brain tumor with a usual potential for leptomeningeal spread. Intramedullary metastases of MB are rare and there are very few cases reported in the literature. Here, we report the case of an 18-year-old man with intramedullary spinal cord metastasis of MB occurring 9 years after the first diagnosis. The patient presented a 2-month history of progressive weakness in both lower limbs associated with urinary incontinence. Magnetic resonance imaging (MRI) demonstrated a large intramedullary spinal cord tumor extending from T10 to L1. The patient underwent surgical decompression and adjuvant therapy. Histological examination confirmed the diagnosis of classic MB metastasis. Postoperatively, the neurological status was stationary. Intramedullary metastasis of medulloblastoma is rare and difficult to manage with a poor prognosis. Comprehensive studies on the medulloblastoma dissemination mechanisms and clinical trials are needed to assess combined therapeutic approaches on metastases of MB.

AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?

Background: The association of primary Sjögren syndrome (PSS) and AA amyloidosis is a rare occurrence. Objective: To describe the phenotype of patients with this association through our two cases and a literature review. Materials and methods: A report of two cases of AA amyloidosis complicating primary Sjögren syndrome with a literature review. Results: Eight patients of Primary Sjögren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. Conclusion: The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.

Primary Testicular Lymphoma: A Case Report and Review of the Literature

Background and Aim: Primary testicular lymphoma (PTL) is a rare form of extranodal non-Hodgkin’s lymphoma. It represents for 1% - 2% of non-Hodgkin’s lymphoma, and mostly affects the elderly. We describe an interesting case of PTL managed by a combined multimodal approach with a review of the literature. Case Presentation: Patient aged 56 years, consulted for an increase in the volume of the right testicle without associated pain, all evolving in the context of a slight decline in general condition. Clinical examination revealed a large painless mass in the right scrotal bursa. A scrotal ultrasound showed a right intra-testicular mass. The patient had undergone inguinal orchiectomy. Pathological analysis showed diffuse large B-cell lymphoma of the testis. Whole-body 18-fluorodeoxyglucose positron emission tomography (18-FDG-PET-CT) showed no suspicious hypermetabolism. Lumbar puncture did not reveal malignant cells in the cerebrospinal fluid (CSF). The patient then received 6 cycles of chemotherapy according to the R-CHOP protocol (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) and 2 cycles of intrathecal methotrexate. After chemotherapy, scrotal radiotherapy at a dose of 30 Gy was delivered. The evolution was marked by the death of the patient six months after the end of the scrotal radiotherapy following a diffuse lymph node relapse with a profound alteration of the general state. Conclusion: The treatment depends imperatively on the stage of the disease. The therapeutic approach is multimodal and combined based on orchiectomy, systemic and intrathecal treatment and scrotal radiotherapy. PTL is an aggressive malignant with a poor prognosis. Randomized trials are needed to define a better therapeutic strategy.

Polymorphisms in oxidative pathway related genes and susceptibility to inflammatory bowel disease

AIM To investigate whether common variants in the oxidative pathway genes influence inflammatory bowel disease(IBD) risk among Moroccan patients. METHODS The distribution of(TAAA)n_rs12720460 and(CCTTT)n_rs3833912 NOS2 A microsatellite repeats, HIF-1 A_rs11549467 and NFKB1-94 ins/delA TTG_rs28362491 was analyzed in 507 subjects grouped in 199 IBD and 308 healthy controls. Genotyping was performed withpolymerase chain reaction-fluorescent method and the TaqMan~? allelic discrimination technology.RESULTS The allele and genotype frequencies of HIF1 A_ rs11549467, NFKB1_rs28362491 and NOS2 A_(TAAA)n did not differ significantly between patients and controls. Analysis of NOS2 A_(CCTTT)n markers evidenced differences between patients and healthy controls. A preferential presence of the(CCTTT)8(P = 0.02; OR = 1.71, 95%CI: 1.07-2.74),(CCTTT)14(P = 0.02; OR = 1.71, 95%CI: 1.06-2.76) alleles in IBD,(CCTTT)8(P = 0.008; OR = 1.95, 95%CI: 1.17-3.23) in CD and(CCTTT)7(P = 0.009; OR = 7.61, 95%CI: 1.25-46.08),(CCTTT)11(P = 0.05; OR = 0.51, 95%CI: 0.25-1.01),(CCTTT)14(P = 0.02; OR = 2.05, 95%CI: 1.07-3.94),(CCTTT)15(P = 0.01; OR = 2.25, 95%CI: 1.16-4.35) repeats in UC patients indicated its possible association with higher disease risk which need to be confirmed in a larger sample size. CONCLUSION Our results suggest that the NOS2 A_(CCTTT)n gene variations may influence IBD susceptibility in the Moroccan population.