Tumour lysis syndrome as a rare complication of chemotherapy in paediatric Wilms' Tumour: a case report.

Wilms tumour is the most common renal malignancy in children, with two-thirds of cases diagnosed before five years and 95 percent before 10 years of age. Over the last decade, the five-year survival rate has improved dramatically and now approaches 90 %. Tumour lysis syndrome, commonly seen in association with haematological malignancies, is rarely seen in Wilms tumour. We present two cases of Wilms tumour developing tumour lysis syndrome in the first week of initiation of chemotherapy. Both patients presented with huge abdominal masses causing mass effect on surrounding structures. Chemotherapy was administered as per International Society of Pediatric Oncology guidelines (SIOP). Both patients developed laboratory and clinical tumour lysis syndrome (TLS) after the first cycle of chemotherapy requiring continuous renal replacement therapy (CRRT). However, both died because of multiorgan failure.

Presentation of Acute Lymphoblastic Lymphoma and Colorectal Carcinoma in the Context of Constitutional Mismatch Repair Deficiency Syndrome: A Case Report with Literature Review.

Introduction: Constitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessive disease carrying an increased risk of cancers (paediatric tumours of central nervous system, haematolymphoid malignancies along with gastrointestinal (GI) cancer(s), which are usually seen in the second and third decades), leading to syndromic presentation. Causal mutations are detected in DNA mismatch repair (MMR) genes, including MLH1, PMS2, MSH2 and MSH6 that are also known for their established role in Lynch syndrome. We describe a case of CMMRD with an earlier (first decade of life) presentation of mediastinal acute lymphoblastic lymphoma and colorectal malignancy. Case Presentation: A 5-year-old boy presented with respiratory complaints, bilateral cervical lymphadenopathy, multiple café-au-lait macules (CALMs) on the lower back and history of parental consanguinity with the death of three sisters due to brain tumour within 6 months of diagnosis. Computerised tomographic scan chest revealed a huge mediastinal mass. The patient underwent a trucut biopsy of the mass. The results were significant for a pre-T-cell acute lymphoblastic lymphoma. Suspicion of CMMRD was raised based on a combination of factors described above. A panel of MMR proteins was applied on the biopsy tissue that revealed loss of nuclear expression of MLH1 and PMS2 immunostaining in tumour cells with positive external controls. While on maintenance therapy for lymphoma, about a year later, the patient developed subacute intestinal obstruction due to a stenosing polypoidal circumferential tumour in the mid-sigmoid colon found on flexible sigmoidoscopy that was followed by endoscopic biopsies and insertion of a fully covered self-expanding metallic adult biliary stent with a diameter of 10 mm and length of 6 cm leading to immediate relief of obstruction. Biopsies revealed adenocarcinoma with neuroendocrine differentiation. Metastatic tumour deposits were seen in the omentum, anterior abdominal wall and the left peritoneal wall. Practical Implications: Earlier (first decade) presentation of GI malignancy warrants that an earlier screening through radiological scans for any possible tumours and MMR protein expression analysis (loss in tumour plus normal non-tumour cells) are essential in patients having CALMs and family history of paediatric tumours.

Multifocal myxopapillary ependymoma and iatrogenic spinal cord herniation: Management options and lessons learned.

Although myxopapillary ependymoma is a fairly common tumour of the lumbosacral spine, primary multi-focal myxopapillary ependymoma is a rare variant. Drop metastasis and leptomeningeal spread in the craniospinal axis is seen more frequently in the paediatric population, although it is unusual in adults. Surgical resection of the primary lesion remains the standard treatment. As per the authors' knowledge, to-date there is only one prior case in literature reporting iatrogenic spinal cord herniation with indentation after surgery for thoracolumbar spinal tumour. Here, we are discussing an unusual case of primary multi-focal ependymoma in a 16-year-old Asian boy, with drop metastasis and lepto-meningeal disease, who developed iatrogenic spinal cord herniation after the first surgery for the primary tumour. He presented to the Shaukat Khanum Memorial Cancer Hospital & Research Centre (SKMCH & RC), Lahore, after his first surgery. He underwent the definitive corrective surgery at SKMCH & RC where he was managed further. We discuss the management options for this patient and the lessons learned along the way.

Impact of COVID-19 Pandemic on Treatment of Pediatric Oncology Patients: Report from Resource-limited Setting.

The aim of this study was to determine how the COVID-19 pandemic impacted on the effective management; and the outcome of pediatric oncology patients in Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore. Data was retrospectively reviewed from 15 March to 15 June 2020 after the approval of Institutional Review Board Committee. A total of 258 patients on active oncology treatment between the study period were included. The total number of patients whose treatment was affected were 118 (45.7%), while 140 (54.3%) patients received treatment in time. There was total 34 (13.2%) patients relapsed, 23 (67.6%) patients in which treatment delayed, and 11 (32.4%) patients in which their treatment not delayed; while, n=218 (84.5%) were in remission, and 6 (2.3%) patients absconded. COVID-19 pandemic caused a sudden impediment in the treatment of pediatric oncology patients, and is likely to affect the long-term survival outcome of pediatric oncology patients. Key Words: Chemotherapy, Radiotherapy, Surgery, Long term outcome.

Choriocarcinoma syndrome: A rare presentation of testicular germ cell tumour.

Germ cell tumours (GCT) are the most common malignancy in men between the ages of 15 and 35 years. Pure choriocarcinoma is a very rare entity of non- seminomatous germ cell tumour and comprises only 1% -3% of all the testicular tumours. Choriocarcinoma syndrome is a clinical condition in which haemorrhage occurs from the metastatic sites with elevated level of the Beta-Human Chorionic gonadotropin (Beta -HCG). A 15-year-old adolescent boy presented in with left sided testicular swelling along with dark coloured cutaneous lesions of the scalp, chest and chin for the last one month. He underwent left sided orchiectomy, and the histopathology report showed Pure Choriocarcinoma. Unfortunately, he died after the beginning of chemotherapy due to alveolar haemorrhage.

Clinical Outcome of paediatric ewing sarcoma and significance of pathological necrosis for mortality after neoadjuvant chemotherapy: Single institutional study.

OBJECTIVE: To determine the clinical outcome and significance of pathological necrosis after neoadjuvant chemotherapy. METHODS: The retrospective study was conducted in Shaukat Khanum Memorial Cancer Hospital and Research Center and comprised data from January 2010 to December 2015 related to young with newly diagnosed Ewing sarcoma on histopathology. Data was collected on patients aged <20 years of either gender along with primary tumour site, metastatic status, tumour volume, mode of local therapy, degree of necrosis post-surgery, tumour margins after resection, outcome at the end of treatment and at last follow-up visit. Tumours were categorised as grade I = little or no necrosis, grade II = 50-90% necrosis, grade III = 90-99% necrosis, and grade IV = 100% necrosis. Data was analysed using SPSS 20. RESULTS: Of the 124 patients, 89(72%) were non-metastatic; 35(28%) were metastatic; 37(29.8%) underwent surgery; 58(46%) received radiotherapy; 7(5.6%) received both surgery and radiotherapy; 22(17.7%) received no treatment. Histopathology report post-surgery showed little grade 1 necrosis in 10(8%) patients, grade II in 8(6.5%), grade III in 8(6.5%) and grade IV in 14(11%). Event-free survival in grade IV necrosis was 93% in 14 (11.3%) patients, EFS in grade III necrosis was 71% in 8(6.5%) patients , EFS in grade II necrosis was 22% seen in 9(7.3%) patients and EFS in grade- I necrosis was 35% seen in 14 (11.3%) patients. Overall survival in grade IV necrosis was 93% in 14 (11.3%) patients, OS in grade III 75% seen in 8(6.5%), OS in grade II 25% seen in 9(7.3%) and OS in grade I was 50% in 14(11.3%) patients. Event-free survival was 48 (38%) patients and overall survival of Ewing sarcoma patients was 52 (46.6%) patients respectively. CONCLUSION: Tumour necrosis and histopathological changes post-surgery were found to have great impact on survival outcome in Ewing Sarcoma.

Retrospective analysis of clinical features and treatment outcomes of children with Hodgkin's Lymphoma treated with different chemotherapy protocols at a tertiary care center in Pakistan.

PURPOSE: Hodgkin lymphoma (HL) is one of the most curable paediatric cancers, with long-term survival rates now exceeding 90% after treatment with chemotherapy alone or combined with radiotherapy (RT). Treatment options for Hodgkin's Lymphoma differ among various study groups and there is still no consensus regarding the standard treatment for Hodgkin's lymphoma. Taking into account the impact of treatment-related mortality in low- and middle-income countries we propose to study the the clinical features and treatment outcomes by using different chemotherapy protocols in Hodgk in s' s Lymphoma children's at Shaukat khanam hospital Lahore.. METHODS: Clinical data from a large regional cancer center Pediatrics patients with Hodgkin's Lymphoma from January 2009 till December 2015 was retrospectively collected after Institutional Review Board (IRB) approval. RESULTS: A total of 748 patients were reviewed retrospectively. Mostly (45%) were in 6-10 years age group. Male showed predominance ,male to female ratio was 4:1. B symptoms were present in 51%, bulky disease in 44% and ESR was more than 30mm in 26% of patients. CD 30 was positive in 95%, Bone marrow involved in 13% of patients. Stage I in 8%, stage- II in 27%, stage -III in 39% and stage IV in 26% was seen. COPDAc/ABVD was given in 412 patients, CHLVPP/ABVD in 176 patients, OEPA/COPP in 57 patients, OEPA in 35 patients, OEPA/COPDAC in 33 Patients and remaining 33 received various chemotherapy protocol combination. XRT was given in 17% of patients. Of these 86% of patients were alive ,5% patients died , 3% patients abandoned, 6% patients relapsed ,3% patients progressed while on chemotherapy. Five years Overall survival was 94% and 5 Years Event free survival was 91%. Minimum haematological and other toxicity was seen in patients who had received COPDac/ABVD when compared to other regimen. CONCLUSIONS: Hodgkin's lymphoma patients had good outcome with different chemotherapy regimens, however our experience showed that the COPDac/ABVD regimen wass better tolerated with minimum toxicity.

Primary Extra-renal Wilms' Tumor in Urinary Bladder: Rare Presentation of a Common Pediatric Malignancy.

In pediatric population, the most common solid malignant tumor of the kidney is Wilms' tumor (WT). Incidence of Extra-Renal Wilms' Tumor (ERWT) is immensely unusual. Prognosis of ERWT is comparable to renal WT. Hence, diagnosis, staging workup and start of treatment in a timely manner is very essential. The most common location of ERWT is the retroperitoneum. The staging of ERWT is done according to National Wilms' Tumor Study group (NWTS). ERWT is considered as stage II or higher; because it is outside renal capsule. A 6-year boy presented with complaints of increased frequency of micturition and difficulty in urination for 15 months. Biopsy of bladder mass showed WT by immunohistochemistry stains such as WTI and Desmin. Cross-sectional imaging showed both kidneys clear of any tumor. Hence, the final diagnosis of ERWT was made.