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 Characteristics and Treatment Outcome of Paediatric Rhabdomyosarcoma; A Retrospective Review.

Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. This paper aimed to assess the stage, site and treatment outcome among RMS patients. Materials and Methods: A retrospective chart review was completed from January 2011 to December 2017 of patients that presented to the Department of Paediatric Oncology, Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, for the management of RMS. Data collection included clinical characteristics, staging, grouping, risk stratification, treatment plan, radiotherapy doses and treatment outcome. Results: Among 24 subjects, there were a total of 13 (54.2%) males and 11 (45.8%) females. The median age at the time of diagnosis was 2.5 years (range: 0.75-17 years). The majority of the subjects (91.7%) were <10 years of age. The median follow-up time was 0.6 years. According to the Children's Oncology Group Classification, 4 (16.7%) subjects were classified as low risk, 14 (58.3%) subjects were rated as intermediate risk and 6 (0.25%) subjects were stratified as high risk. The most common primary tumour site was genitourinary (62.5%) and abdomen/retroperitoneal (20.8%) regions. At the time of analysis, nine (37.5%) subjects had died because of the disease, 12 (50%) were alive with no evidence of disease and one subject had a recurrence of disease and was alive. One subject had abandoned the therapy and another was lost to follow-up. Conclusion: Patients with RMS presented at the late stages of the disease and it most frequently affected genitourinary and abdomen or retroperitoneal areas. Overall, RMS was found to have a poor outcome to therapy.

Malnutrition, Sepsis, and Tumor Lysis Syndrome Are Associated with Increased Rate of Acute Mortality in Mature B Cell Non-Hodgkin Lymphoma in a Pediatric Population-Study from Tertiary Care Hospital in Pakistan.

BACKGROUND: Outcomes of pediatric mature B cell non-Hodgkin's lymphoma in resource-challenged countries are negatively affected by an increased rate of early and toxic deaths. Aim of this study is to assess the rate of acute mortality and define significant risk factors present in children with mature B cell non-Hodgkin's lymphoma. METHODS: A retrospective analysis was done of patients with B cell non-Hodgkin's lymphoma from January 2012 till December 2016. Risk factors studied for acute mortality were malnutrition, stage, prior surgery with open laparotomy, lactate dehydrogenase levels, tumor lysis syndrome, sepsis, and fungal infection. RESULTS: A total of 233 patients were enrolled in the study. Eighty-five (36.4%) were below 15th percentile weight for age. Treatment was started in 226 patients. Eighty-eight percent of children showed a 20% response after COP pre-phase. Tumor lysis syndrome was developed in 20.6% (n = 48) children and 42.9% (n = 100) patients had sepsis, 71/100 patients had culture-proven sepsis. 19.7% (n = 46) patients developed fungal infection. There was 19.7% (n = 46) acute mortality. The most common cause of death was sepsis (n = 22, 47.8%) followed by acute renal failure secondary to tumor lysis syndrome. On multivariate analysis, three independent variables found significant for early death are malnutrition, sepsis, and tumor lysis syndrome. CONCLUSION: Rate of acute mortality in mature B cell NHL is high in our set up and significant risk factors are tumor lysis syndrome, sepsis, and malnourishment at the time of presentation.

A Single Institution's Experience with Cytogenetic and MRD Outcomes in Pediatric Acute Lymphoblastic Leukemia.

OBJECTIVE: To determine the frequency of cytogenetic type and its significance in the prognostic outcome of the pediatric patients in acute lymphoblastic leukemia (ALL), aged 1 to 15 years, and also determine the importance of minimal residual disease (MRD) in the management of the condition. STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: Pediatric Oncology Ward, Shaukat Khanum Cancer Hospital, Lahore, from January 2015 to July 2017. METHODOLOGY: Patients aged 1-15 years, diagnosed with ALL, were included. Studied variables were cytogenetic type and MRD outcome in patients with ALL. Patients under one year of age and more than 15 years, or those having comorbidities, were excluded. RESULTS: Total 150 patients' data were retrieved from the Hospital database. One hundred and thirty-three belonged to age 1 to 5 years group (89%) and 17 (11%) were in 5 to 10 years group. The mean age of the patient was 4.3 +3.1 years. One hundred and two (68%) were males; whereas, 48 (32%) were females. Pre B acute lymphoblastic leukemia was diagnosed in 139 (93%) patients and 11(7%) were diagnosed with Pre T acute lymphoblastic leukemia. Standard risk was observed in 120 (80%) patients and 30 (20%) patients were on high risk as per National Cancer Institute (NCI) Guidelines. Regimen A was used in 125 (83.3%), Regimen B in 16 (10.7%), and Regimen C in 9 (6%) patients. BCR-ABL was positive in 2 (1.30%), TEL-AML in 68 (45%), MLL in 5 (3.30%), and normal in 54 (36%). MRD at day 29 was negative in 40 (93%) and positive in 3 (7%). The karyotyping was done in 128 (85%) patients, out of which 68 (53%) were hyperploids, 41 (32%) euploid, and 19 (15%) were hypoploid. Death was observed in 22 (15%) patients. Nineteen (86%) deaths were due to fungal and bacterial sepsis; and disease-related deaths were noted in 3 (14%) patients. CONCLUSION: The role of MRD and cytogenetics in risk assessment has improved in the early prognosis determination.

Fungal Infections In Paediatric Patients With Acute Lymphoblastic Leukaemia While On Induction Chemotherapy.

BACKGROUND: Acute Lymphoblastic Leukaemia (ALL) is one of the most common haematological malignancies seen in children. Despite steadily improving long-term outcomes, infections remain a major cause of morbidity and mortality in children receiving therapy for leukaemia. The incidence and risk of invasive fungal infections (IFIs) continue to rise. In some settings, IFIs caused by moulds are more frequent than those caused by yeasts, and Aspergillus spp. is the most common pathogens. The aim of the study was to determine the frequency, type of fungal infection seen during induction chemotherapy and outcomes.. METHODS: This observational retrospective study was conducted in paediatric oncology department at Shaukat Khanum Cancer Hospital Lahore from January 2015 to December 2016 after taking International research board (IRB) approval. The study includes all the patients aged 1-15 who were diagnosed with acute lymphoblastic leukaemia while on induction chemotherapy. The data was retrieved of 165 patients from the hospital database after informed written consent.. RESULTS: The mean age of the patients was 4.6±2.80 with range 1-15years. Total 154 (93%) of the children were of age between 1-5 years whereas only 11 (6.7%) were between 5-10 years. Male sex was predominant in 117 (70.9%) and 48 (29.1%) were girls. Pre-B Acute lymphoblastic leukaemia was diagnosed in 93.3% of the patients and rest 11 (6.7%) were diagnosed with Pre-T Acute lymphoblastic leukaemia. NCI Standard risk patients were 132 (80%) and 33 (20%) were stratified as high risk. Fungal infections were documented in 18 (11%) patients out of which 7(39%) were probable infections, and only 11 (61%) were proven fungal infections. Aspergillus was the commonest organism in 5 (28%) patients. Death was observed in 21 (13%) patients and causes were sepsis due to infections in 18 (86%) out of which fungal infections were 11(61%), bacterial 4(22%), combine bacterial and fungal 3(17%). Remaining 3 (14%) patients death causes include, neutropenic colitis was observed in first patient, second patient died of infection without any identifiable focus, and third patient died due to chemotherapy related toxicity.. CONCLUSIONS: Our study concludes that the fungal infection was the most common cause of mortality in induction in our patients. A prospective study in the form of clinical trial is needed to see if use of prophylactic antifungal can improve outcomes in our setting.

Excellent Outcomes of Grey Zone Lymphoma: Case Series of Paediatric Patients Treated at a Single Centre.

Our objective was to review clinical presentation, treatment protocol used and its efficacy and effectiveness in patients of grey zone lymphoma during last 5 years at our centre. A retrospective chart review of children below 18 years of age was done from 2011 to 2016. A proforma was devised for this purpose and the findings of cases detected during the specified period were noted over it. We treated 4 cases, all with a diagnosis of Grey Zone Lymphoma of ages 13, 13, 15 and 7 years at presentation and all were males. Two patients had stage II and the other two had stage III disease. None had a mediastinal mass. All patients were treated according to UKCCSG NHL guidelines. Tumour lysis syndrome was not observed in any child. All tolerated chemotherapy very well and achieved complete remission. No patient died of the disease or any complication and all are well on their latest follow ups. To conclude from excellent outcomes in our case series, we recommend that children with Grey Zone Lymphoma should be treated according to Non-Hodgkin Lymphoma treatment guidelines. However we need to have more prospective studies, so that treatment guidelines can be established.