Clinicopathological analysis of BRAF and non-BRAF MAPK pathway-altered gliomas in paediatric and adult patients: a single-institution study of 40 patients.

AIMS: Mitogen-activated protein kinase (MAPK) pathway alteration is a major oncogenic driver in paediatric low-grade gliomas (LGG) and some adult gliomas, encompassing BRAF (most common) and non-BRAF alterations. The aim was to determine the frequency, molecular spectrum and clinicopathological features of MAPK-altered gliomas in paediatric and adult patients at our neuropathology site in Kuwait. METHODS: We retrospectively searched the data of molecularly sequenced gliomas between 2018 and 2023 for MAPK alterations, revised the pathology in view of the 2021 WHO classification and evaluated the clinicopathological data for possible correlations. RESULTS: Of 272 gliomas, 40 (15%) harboured a MAPK pathway alteration in 19 paediatric (median 9.6 years; 1.2-17.6) and 21 adult patients (median 37 years; 18.9-89.2), comprising 42% and 9% of paediatric and adult cases, respectively. Pilocytic astrocytoma and glioblastoma were the most frequent diagnoses in children (47%) and adults (43%), respectively. BRAF V600E (n=17, 43%) showed a wide distribution across age groups, locations and pathological diagnoses while KIAA1549::BRAF fusion (n=8, 20%) was spatially and histologically restricted to cerebellar paediatric LGGs. Non-V600E variants and BRAF amplifications accompanied other molecular aberrations in high-grade tumours. Non-BRAF MAPK alterations (n=8) included mutations and gene fusions involving FGFR1, NTRK2, NF1, ROS1 and MYB. Fusions included KANK1::NTRK2, GOPC::ROS1 (both infant hemispheric gliomas), FGFR1::TACC1 (diffuse LGG), MYB::QKI (angiocentric glioma) and BCR::NTRK2 (glioblastoma). Paradoxical H3 K27M/MAPK co-mutations were observed in two LGGs. CONCLUSION: The study provided insights into MAPK-altered gliomas in Kuwait highlighting the differences among paediatric and adult patients and providing a framework for planning therapeutic polices.

Outcome of children with Hodgkin's disease. A 10-year experience from a single institution in Kuwait.

OBJECTIVE: To evaluate the outcome of children with Hodgkin's disease over a period of 10-years from a single institution in Kuwait. METHODS: Sixty-three children with previously untreated Hodgkin's disease, who were diagnosed at the Pediatric Oncology Unit of Kuwait Cancer Control Centre, Shuwaikh, Kuwait from January 1998 to December 2007 were included in the study. All cases were proved by histopathology, and staging was carried out according to the Ann Arbor system. RESULTS: Our series included 37 (59%) males and 26 (41%) females with a median age of 10 years (range 3-15 years). B symptoms were present in 20 (32%) children. Bulky disease was noted in 28 (44%) children, with stages III in 8 (13%) and IV in 12 (19%) children. Chemotherapy was administered as a primary treatment in 63 children. The median number of chemotherapy cycles given was 6 (range 2-8). Radiotherapy was used in 40 (63%) children. Grade III hematological toxicity was observed in 23 (37%) and grade IV in 14 (22%) children. Hypothyroidism was observed in 20 (32%) children. Fifty-five children achieved a complete remission (87%) and 2 children achieved a partial remission (3%) with an overall response rate of 90%. Three children achieved a progressive disease (5%) and response could not be evaluated in 3 (5%) children. At a median follow-up of 67 months (5.5 years), the overall survival was 91%. CONCLUSION: With moderate toxicity, combined modality therapy is effective in the treatment of childhood Hodgkin's disease.

Fetus in fetu: a case report.

INTRODUCTION: Fetus in fetu is a rare abnormality secondary to the abnormal embryogenesis in a diamniotic, monochorionic pregnancy. It is a rare pathological condition and fewer than 100 cases have been reported in the literature. CASE PRESENTATION: A 2 month old girl with an abdominal mass since birth, was referred to the Cancer Institute with a suspected diagnosis of a Wilms' tumor. Conventional radiograph of the abdomen revealed a mass containing numerous calcifications. CT scan showed a heterogeneous retroperitoneal mass containing well-defined calcified structures. The decision was made to recommend surgical exploration and the mass was successfully excised. Physical examination of the mass with review of literature confirmed the diagnosis of fetus in fetu. CONCLUSION: Although it is a rare condition imaging may play an important role in the correct prospective diagnosis of fetus in fetu. Surgical excision is the recommended treatment.