Ewing\u27s sarcoma/PNET of kidney in 13-year-old girl

Ewing\u27s sarcoma is the second most common primary tumour of bone in childhood. Less frequently it occurs in soft tissues. Ewing\u27s sarcoma/primitive neuroectodermal tumour (ES/PNET) is an extra ordinarily rare primary tumour in the kidney. Only very few cases of primary renal Ewing\u27s sarcoma have been reported in the literature to date. We present a case of primary right renal Ewing\u27s sarcoma in a 13-year-old girl who was diagnosed as a case of stage IV ES/PNET of kidney with metastases to lung and liver. Right nephrectomy was done followed by adjuvant radiotherapy and chemotherapy with complete response to local and distant area

Clinical profile and short-term outcome of pediatric hyperleukocytic acute leukemia from a developing country

This study was conducted to determine the frequency, clinical profile, and short-term outcome of children with hyperleukocytosis at two pediatric oncology centers in Karachi. Of a total 1,045 patients, 13.97% (n=146) patients had hyperleukocytosis. Majority (61.7%, n=90) were under 10 years of age and 76% (n=146) were male. The symptom duration before diagnosis was more than 30 days in 49.3% (n=72). The median WBC count was 181 x109/L (IQR=130.45- 298.3) and extreme hyperleukocytosis (\u3e200 x109/L) was observed in 44.5% (n=65) patients. Majority (94.5%, n=138) of patients were diagnosed with acute lymphoblastic leukemia. One or more complications developed in 78% (n=114) of cases. Clinical and laboratory tumor lysis syndrome (TLS) was observed in 17.1% (n=25) and 39% (n=57) patients, respectively. Pulmonary and neurological complications related to leukostasis were noted in 9.5% (n=14) and 27.3% (n=40) of cases, respectively. Infectious complications occurred in 23.2% (n=34) patients. The case-specific mortality was 20.5% (n=30). No mortality was related to early complications of hyperleukocytosis

Autoclaved tumor bone for skeletal reconstruction in paediatric patients: A low cost alternative in developing countries

We reviewed in this series forty patients of pediatric age who underwent resection for malignant tumors of musculoskeletal system followed by biological reconstruction. Our surgical procedure for reconstruction included (1) wide en bloc resection of the tumor; (2) curettage of tumor from the resected bone; (3) autoclaving for 8 minutes (4) bone grafting from the fibula (both vascularized and nonvascularized fibular grafts used); (5) reimplantation of the autoclaved bone into the host bone defect and fixation with plates. Functional evaluation was done using MSTS scoring system. At final followup of at least 18 months (mean 29.2 months), 31 patients had recovered without any complications. Thirty-eight patients successfully achieved a solid bony union between the graft and recipient bone. Three patients had surgical site infection. They were managed with wound debridement and flap coverage of the defect. Local recurrence and nonunion occurred in two patients each. One patient underwent disarticulation at hip due to extensive local disease and one died of metastasis. For patients with non-union, revision procedure with bone graft and compression plates was successfully used. The use of autoclaved tumor grafts provides a limb salvage option that is inexpensive and independent of external resources and is a viable option for musculoskeletal tumor management in developing countries

Karachi cancer registry (KCR): Age-standardized incidence rate by age-group and gender in a mega city of Pakistan

Objectives: To estimate the cancer incidence by age group and gender for the population of Karachi Division by analyzing the Karachi Cancer Registry data of 2017-19.Settings: The population of Karachi division is 16.1 million according to national census 2017. \u27Karachi Cancer Registry\u27 which is a part of \u27National Cancer Registry\u27 is collecting data from eight major hospitals in Karachi since 2017. For outcome measures, cancer counts and the age standardized incidence rates (ASIR) per 100,000 population were computed for age groups (0-14, 15-19 and ≥20 years), in both genders and all cancer site/type.Methods: The population denominators were based on the population of Karachi division estimated at 16.1 million in the population census, 2017. Counts and age-standardized incidence rates (ASIR) were calculated for each of the three age categories.Results: From Jan 2017 till Dec 2019 a total of 33,309 malignant cases were recorded in KCR database comprising 17,490 (52.5%) females and 15,819 (47.5%) males. ASIRs in age groups 0-14, 15-19 and ≥ 20 years, among female were 11.5, 2.4 and 223.6 and in males were 17.6, 3.2 and 216.7 respectively. The commonest diagnosis in children, adolescent and adults were (1) among females: children; bone (3.12), leukemia (2.09) brain/CNS (1.26); in adolescents: bone (0.78), brain/CNS (0.27), connective and soft tissue (0.11), in adults: breast cancer (76.07), oral cancer (16.68) and ovary (10.89) respectively, and (2) among males: children; bone (4.56), leukemia (2.79) and brain/CNS (1.88); in adolescent; bone (1.19), brain/CNS (0.31) and leukemia (0.21) and in adults: oral cancer (42.83), liver (16.10) and bone (13.37) respectively.Conclusion: Oral Cancer, a largely preventable cancer is the leading cancer in Karachi adult males while in female adults Breast Cancer is the leading cancer followed by Oral Cancer. In children and adolescents Bone, Leukemia and Brain/CNS malignancies are most common.

Pediatric oncology nursing education initiatives at Children Cancer Hospital, Karachi, Pakistan; local problem –local solution

Background: Pediatric oncology is a newer specialty in Pakistan with limited number of centers and expertise. At Children Cancer Hospital we were facing great difficulty in getting nurses who could take care of children. It was nearly impossible to get nurses with experience in Pediatric oncology. We decided to initiate training courses for technicians and nurses at Children Cancer Hospital in 2009 with the help of My Child Matter Grant from UICC. Methods: One year Pediatric Oncology Nurse Technician course with certification from Sindh Technical Board was initiated in 2009.We started with one nurse educator with extensive pediatric oncology experience later we added one more. Curriculum was developed with the help of colleagues from medical education. The candidates with minimum qualification of inter science with biology were selected from all over Sindh. For registered nurses we initially conducted six week comprehensive course on Pediatric oncology, it was later changed to 2 weeks because of study leave issue. The nursing education department is also conducting other courses and seminars on infection control, critical care etc. Results: We have so far run three pediatric oncology nurse technician and six pediatric oncology nursing courses. We have produced 27 pediatric oncology nurse technicians, and enabled 64 nurses from all over country to enhance their oncology nursing skills. Most of them are working in different centers and have adopted oncology nursing as their profession doing further studies in this specialty. Over the years we have established infrastructure of nursing education department establishing a classroom, seminar room, and library and skill lab. Conclusions: We have been able to find local solution of local problem by creating a nursing education department at Children Cancer Hospital. We have inculcated the culture of further education and training among our nurses. Key words: Pediatric Oncology, nursing, educatio

Erdheim Chester disease–An unusual presentation of a rare histiocytic disease in a 3-year old boy

Erdheim Chester disease (ECD) is a rare, multisystem non-Langerhans histiocytosis seen in adults characterized by a clinical triad of bone pains, bilateral painless exophthalmos and diabetes insipidus. Only 10 pediatric ECD cases are reported. We report here the youngest child in the literature, diagnosed at three years of age. He presented with osteolytic lesions in the skull, diabetes insipidus and bilateral proptosis. He was initially misdiagnosed and treated as Langerhan cell histiocytosis(LCH). In ECD, the typical radiological findings are symmetrical osteosclerosis of long bones. Osteolytic lesions are rare and if present are usually seen in the limbs. This is the first pediatric case with osteolytic lesions solely in the skull. A repeat biopsy confirmed ECD with the presence of foamy histiocytes in fibrous background positive for CD68 andCD163 and negative for CD1a and S100, typical histopathological features of ECD. The BRAFgene mutation was also detected. The patient responded well to interferon alpha therapy, which is now considered the first line treatment in ECD

Erdheim Chester diseaseâAn unusual presentation of a rare histiocytic disease in a 3-year old boy

Erdheim Chester disease (ECD) is a rare, multisystem non-Langerhans histiocytosis seen in adults characterized by a clinical triad of bone pains, bilateral painless exophthalmos and diabetes insipidus. Only 10 pediatric ECD cases are reported. We report here the youngest child in the literature, diagnosed at three years of age. He presented with osteolytic lesions in the skull, diabetes insipidus and bilateral proptosis. He was initially misdiagnosed and treated as Langerhan cell histiocytosis(LCH). In ECD, the typical radiological findings are symmetrical osteosclerosis of long bones. Osteolytic lesions are rare and if present are usually seen in the limbs. This is the first pediatric case with osteolytic lesions solely in the skull. A repeat biopsy confirmed ECD with the presence of foamy histiocytes in fibrous background positive for CD68 andCD163 and negative for CD1a and S100, typical histopathological features of ECD. The BRAFgene mutation was also detected. The patient responded well to interferon alpha therapy, which is now considered the first line treatment in ECD

Clinical features and treatment outcomes of children With anaplastic large cell lymphoma in Pakistan: A multicenter study

Background: different approaches have been adopted in the treatment of anaplastic large cell lymphoma (ALCL); there is a lack of consensus with regard to standard treatment. Because of paucity of data from low and middle-income countries, we reviewed the clinical features and treatment outcomes of children with ALCL. Method: all ALCL patients under 16 years of age diagnosed from 2005 to 2015 at Aga Khan University Hospital and The Indus Hospital were identified. Clinical features and treatment outcomes were analyzed. Result: thirty-two (n=32) patients met the inclusion criteria. Cervical Lymphadenopathy was the most common presentation (34.3%, n=11). Advanced disease was seen in 68.7% (n=22) (stages III and IV). Fourteen (42.4%) were treated on ALCL-99, 30.3% (n=10) on multicenter protocol-842 regimen, 9% (n=3) on adriamycin-prednisolone-oncovin (doxorubicin, prednisone, vincristine) regimen, and 16% (n=5) were treatment abandonments. Five-year overall survival was 70.6% (95% confidence interval: 47.8%-84.9%), and 5-year event-free survival (EFS) considering treatment abandonment and death as an event was 52.3 % (95% confidence interval: 23.5%-74.8%). Conclusion: significant therapy-related mortality (27.7%) was observed. Treatment abandonment and therapy-related toxicity were the major barriers for better outcomes. However, less intensive outpatient regimens, such as adriamycin-prednisolone-oncovin regimen, may decrease the number of hospitalizations, hence reducing treatment abandonment in the low and middle-income country

Predictors of treatment abandonment for patients with pediatric cancer at Indus children cancer hospital, Karachi, Pakistan

Background: Abandonment of treatment is one of the toughest challenges to deal with in pediatric oncology. It leads to unnecessary mortality and morbidity in patients from low- and middle-income countries.Procedure: The objective of our retrospective study was to determine the prevalence and predictors for abandonment among children with cancer at our hospital in Karachi, Pakistan. We analyzed data on patients younger than 18 years, diagnosed with any malignancy between November 2014 and May 2016.Results: From a total of 821 patients, one hundred and eighty-two (22.2%) patients abandoned treatment at various stages, 92 (11.2%) patients did not initiate treatment at all, and the remaining 90 (11.0%) left during treatment. The gender ratio at registration was skewed toward males but not statistically significant for abandonment. Of 295 registered females, 74 (25.1%) abandoned treatment compared to 108 (20.5%) abandonments among 526 males. In multivariable regression analysis, the type of malignancy, guardian\u27s profession, and travelling from outside the city of Karachi (odds ratio [OR]: 1.48; 95% confidence interval [CI] 1.02-2.15; P = 0.039) correlated with increased abandonment. Treatment abandonment was higher among patients with brain tumors (45.7%) and solid tumors (30.8%) and among those whose guardians were associated with a rural profession (24.7%). Monthly income, age, and number of siblings had no impact on the decision to abandon treatment.Conclusion: Despite the provision of free treatment, the prevalence of abandonment was high. More qualitative data need to be collected to identify and target groups of individuals who may be likely to abandon treatment, thus improving outcome of patients

Primary central nervous system lymphoma causing multiple spinal cord compression and carcinomatous meningitis in a 6-year-old: A case report

Primary central nervous system lymphoma (PCNSL) is an uncommon form of non-Hodgkin lymphoma affecting the brain, spinal cord, and leptomeninges. Carcinomatous meningitis (CM) and spinal cord compression in PCNSL are very rare and usually present in advanced stages of the disease. The average survival time of a CM Patient is about 4 to 6 weeks, which may be extended to about 4 to 6 months with treatment. Here we present a case of CM and spinal cord compression by multiple PCNSL in a 6-year-old girl, who has survived 2 years and 9 months posttreatment with no recurrence. To the best of our knowledge this is the very first case reporting survival after CM. The Patient presented with weakness of her right arm, right leg, and left side of the face. Examination revealed mild facial asymmetry with left facial lower motor neuron palsy and lateral gaze restriction of left eye. Magnetic resonance imaging of her spinal cord showed postcontrast enhancement of the intradural structures on the spinal canal at levels C3-C6 and L1-L5 and along with the intracranial leptomeninges. Histopathological examination of the neoplastic tissue from cauda equina revealed B-cell non-Hodgkin lymphoma. After chemotherapy her disease regressed and magnetic resonance imaging showed no evidence of recurrence or residual disease. In our experience the response to chemotherapy was remarkable and recommend that aggressive tumor resection strategies should be reserved for cases with severe signs of spinal compression