Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge

Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only 1.7% of the cases have been reported in children less than five years of age and approximately one-half arise from a benign peripheral nerve sheath tumour especially in background of neurofibromatosis type 1 (NF1). Primary MPNST in children are even rarer. Case Report: A 3-year-old Malay girl presented with painful right axillary swelling for 6 months duration, initially treated as axillary lymphadenitis and she defaulted follow up. She came back 4 months later with enlargement of the swelling. The biopsy was reported as Schwannoma which correlates with MRI findings of benign peripheral nerve sheath tumour. Final diagnosis after debulking surgery was consistent with MPNST. She succumbed to death 20 months after her initial diagnosis of advanced MPNST and lung metastasis. Pathological findings: Grossly, a huge partlycircumscribed soft tissue mass noted arising from a nerve with solid greyish yellowish myxoid cut surface. Spindle shaped cells arranged in herringbone pattern with marked pleomorphism, brisk mitosis and extensive necrosis are seen microscopically. Immunohistochemistry shows patchy S100 protein staining with loss of expression of H3K27 trimethylation. Conclusion: Although MPNST is rare in paediatric age group, diagnosis should be considered in children without NF1 with rapidly evolving and painful mass in the distribution of a peripheral nerve. In this case, the diagnosis was delayed and made after surgery. Due to its morphologic heterogeneity and lack of specific immunohistochemical markers, MPNST remains a diagnostic challenge

Sporadic malignant peripheral nerve sheath tumour in a 3-year-old girl: A diagnostic challenge

Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only 1.7% of the cases have been reported in children less than five years of age and approximately one-half arise from a benign peripheral nerve sheath tumour especially in background of neurofibromatosis type 1 (NF1). Primary MPNST in children are even rarer. Case Report: A 3-year-old Malay girl presented with painful right axillary swelling for 6 months duration, initially treated as axillary lymphadenitis and she defaulted follow up. She came back 4 months later with enlargement of the swelling. The biopsy was reported as Schwannoma which correlates with MRI findings of benign peripheral nerve sheath tumour. Final diagnosis after debulking surgery was consistent with MPNST. She succumbed to death 20 months after her initial diagnosis of advanced MPNST and lung metastasis. Pathological findings: Grossly, a huge partlycircumscribed soft tissue mass noted arising from a nerve with solid greyish yellowish myxoid cut surface. Spindle shaped cells arranged in herringbone pattern with marked pleomorphism, brisk mitosis and extensive necrosis are seen microscopically. Immunohistochemistry shows patchy S100 protein staining with loss of expression of H3K27 trimethylation. Conclusion: Although MPNST is rare in paediatric age group, diagnosis should be considered in children without NF1 with rapidly evolving and painful mass in the distribution of a peripheral nerve. In this case, the diagnosis was delayed and made after surgery. Due to its morphologic heterogeneity and lack of specific immunohistochemical markers, MPNST remains a diagnostic challenge

A critical appraisal of Covid-19 in Malaysia and beyond

When the first report of COVID-19 appeared in December 2019 from Wuhan, China, the world unknowingly perceived this as another flu-like illness. Many were surprised at the extreme steps that China had subsequently taken to seal Wuhan from the rest of the world. However, by February 2020, the SARS-CoV-2 virus, which causes COVID-19, had spread so quickly across the globe that the World Health Organization officially declared COVID-19 a pandemic. COVID-19 is not the first pandemic the world has seen, so what makes it so unique in Malaysia, is discussed to avoid a future coronacoma

Sporadic malignant peripheral nerve sheath tumour (MPNST) in a 3-year-old girl: a diagnostic challenge

Introduction: Malignant peripheral nerve sheath tumour (MPNST) is an uncommon malignant neoplasm of childhood with unfavourable prognosis. Only a limited number of cases have been reported in children less than 12 years of age, and approximately one-half arise from a benign peripheral nerve sheath tumour, especially in the background of neurofibromatosis type 1 (NF1). Primary MPNST in children is even rarer. Case report: A 3-year-old Malay girl presented with painful right axillary swelling for six months, initially treated as axillary lymphadenitis and she defaulted follow up. She came back four months later with enlargement of the swelling. The previous biopsy was reported as Schwannoma, which correlates with a benign peripheral nerve sheath tumour’s MRI findings. The final diagnosis after debulking surgery was consistent with MPNST. She succumbed to death 20 months after her initial diagnosis of advanced MPNST and lung metastasis. Pathological findings: Grossly, a huge partly circumscribed soft tissue mass was noted arising from a nerve with a solid greyish yellowish myxoid cut surface. Spindle-shaped cells arranged in a herringbone pattern alternated with areas of myxoid hypocellular areas exhibited marked pleomorphism, brisk mitosis, and extensive necrosis are seen microscopically. Immunohistochemistry shows patchy S100 protein staining with loss of expression of H3K27me3. Conclusion: Although MPNST is rare in the paediatric age group, the diagnosis should be considered in children without NF1 with a rapidly evolving and painful mass in the peripheral nerve distribution. In this case, the diagnosis was delayed and made after surgery. Due to its morphologic heterogeneity and lack of specific immunohistochemical markers, MPNST remains a diagnostic challenge

ASEAN Orthopaedic Association Education Committee - Orthopaedic curriculum for residency training 2019

In November 2013, Dr Dohar Tobing, who was the President of the ASEAN Orthopaedic Association at that time, organized a Seminar on Orthopaedic Education in Yogyakarta to discuss residency training in ASEAN. At this initial meeting the training program in each country was presented by representatives from each ASEAN country. Subsequently the ASEAN Orthopaedic Association established the Education Committee under the chairmanship of Professor Lee Eng Hin to develop a common syllabus/curriculum for all ASEAN countries based on minimum standards required to practice competently as a general orthopaedic surgeon. The main objective of having a common curriculum is to facilitate exchanges of residents and fellows amongst the ASEAN countries to increase their exposure to orthopaedic conditions in the region and improve their breadth and depth of knowledge and skills. The first meeting of the Education Committee was held in Cebu in November 2014 and the Committee has since met twice a year in May and November. Training programs in the ASEAN countries were discussed and a consensus was reached on the structure and length of the training program for the AOA curriculum. In May 2016, three workgroups were formed under the leadership of Professor David Choon, Dr Dohar Tobing and Professor Lee Eng Hin to develop the detailed curriculum in the following areas: a) General Orthopaedics/Trauma/Infection/Sports/Pathophysiology/Basic Science Lead: David Choon Members: Arturo C. Canete, Jean Pierre F. Leung, Apipop Kritsaneephaiboon, Maung Mg Htwe, Sabarul A. Mokhtar b) Adult Orthopaedics/Spine/Upper Limb/Foot & Ankle/Biomaterials/Biomechanics Lead: Dohar Tobing Members: Myint Thaung, Peter Lee, Yeo Sing Jin, Kamarul Ariffin Khalid, Khin Mg Myint, Azlina Abbas c) Pediatrics/Oncology Lead: Lee Eng Hin Members: Edward HM Wang, Kanyika Chamniprasas, Pornchai Mulpruek, Istan Irmansyah Irsan, Abdul Razak Sulaiman, Wan Faisham Nu’man Wan Ismail In addition to their contributions as workgroup members, the following individuals made substantial contributions in the following areas: Pathophysiology/Basic Science: Vivek Ajit Singh and David Choon General Ortho: Wan Faisham Nu’man Wan Ismail and Kamarul Ariffin Khalid Trauma: Arturo Cañete and Jean Pierre Leung Sports Medicine: Aung Myo Win Adult-Hip & Knee: Maung Mg Htwe and Myint Thaung Spine: Dohar Tobing Upper limb/Hand: Khin Mg Myint Foot & Ankle: Inderjeet Singh Paediatrics: Lee Eng Hin and Abdul Razak Sulaiman Oncology: Edward Wang and Istan Irmansyah Irsa