Challenges in the Management of Wilms Tumor in a Developing Country: A Twenty Years' Experience From a Single Center in Pakistan.

BACKGROUND: Wilms Tumor (WT) is one of the most curable childhood cancers. High cure rates seen in the developed countries are not reproduced in developing countries. Lack of access to cancer treatment facilities, financial constraints, late presentation, and abandonment have previously been described. We reviewed our data over the last 20 years to highlight some of these challenges. METHODS: This is a retrospective chart review of children with WT at our center up to the age of 18 years between 1 November 1997 and 30 November 2017. Demographic details, presentation characteristics and treatment details were recorded. Factors associated with poor outcome were analyzed. RESULTS: Two hundred eleven children were registered; 117(55.5%) were males. Median age at presentation was 3 (range 0 to 18) years. Presentation data were available for 184/211 patients, staging details for 159/211 and metastatic status for 178/211. Of the available dataset, 60% presented without prior treatment, whereas 40% presented atleast after primary surgical excision. High-stage (stage III or above) disease was present in 79 (49.7%) patients; 61 (34.3%) was presented with metastases or recurrence; 63 (29.8%) abandoned or refused treatment; 99/172 (57.6%) patients finished treatment, 23 (13.4%) died during treatment, and 6 died before treatment. Of the 99 patients who finished treatment 83 (83.8%) are well off therapy; 15(15.2%) relapsed; 6 (40%) are alive after salvage therapy, while 9 (60%) died. CONCLUSIONS: Our data highlights the challenges of managing WT in resource poor environments. Prior surgery, incomplete staging work-up and abandonment are some of the most frequently encountered barriers. A multipronged approach is required to overcome these challenges.

Primary Hodgkin's Lymphoma of bone in 7-year-old- an exceptional case report of youngest child in literature.

Hodgkin lymphoma (HL) is mainly considered a nodal disease but extra nodal involvement can also be seen with variable frequency. Solitary bone involvement in HL known as Primary osseous hodgkin lymphoma (POHL) is very rare. It is defined as a lymphoma that is restricted to the bone without any simultaneous organ or lymph node involvement at the time of initial diagnosis. Primary Hodgkin lymphoma of the bone can be very challenging to diagnose because of its rarity especially in children and its variable presentation. Here we report the youngest case of POHL in a girl 7 years of age. She presented with the left leg pain and B-symptoms of fever and weight loss. Initial workup and imaging were suggestive of infection or a bone tumor. Bone biopsy of left hip joint after the multiple courses of antibiotics revealed Reed Sternberg cells in the mixed inflammatory background with CD30 and PAX-5 immunohistochemical positivity confirming classical hodgkin lymphoma, mixed cellularity type. CD99 and CD1a were negative excluding ewing sarcoma and langerhan cell Histiocytosis respectively. Absence of significant lymphadenopathy or visceromegaly on staging computerized tomography (CT) scan confirmed a very rare POHL. She received standard conventional chemotherapy with radiation. Our patient is in remission for five years after treatment.

Morbidity and Mortality Associated With Pediatric Critical Mediastinal Mass Syndrome.

Objective The critical mediastinal mass syndrome (CMMS) is a life-threatening condition and is challenging for physicians. We analyse the clinicopathological profile and outcome of CMMS from a large tertiary-care pediatric oncology center in Pakistan. Methods We retrospectively reviewed the medical record of a tertiary-care hospital in Pakistan from April 2017 to September 2019 for all children (1 month-16 years) who presented with an anterior mediastinal mass (AMM). A CMMS case is defined as a child with an AMM presenting with cardiorespiratory compromise and needing intensive care support. Demographic data, clinical profile, pathological diagnosis, and outcome of all such children were recorded. Descriptive statistics were applied using the Statistical Package for the Social Sciences (SPSS), version 22 (IBM Corp., Armonk, NY). Results Of the total 221 mediastinal masses, 61 children were diagnosed as CMMS and enrolled in the study. The mean age was 9 ± 3.3 years, and 68.9%% were male; 65.6% of patients had a weight for age less than the fifth percentile. A total of 49.2% of patients had a duration of illness of more than one month before diagnosis. Fever (97.6%) and lymphadenopathy (82%) were the most common findings, along with respiratory and cardiovascular signs and symptoms; 9.8% had superior vena cava syndrome. The pericardial effusion was present in 54.6% and 27.9% had pleural effusion. Peripheral blood flow cytometry made the diagnosis in 59%, peripheral lymph node biopsy in 13%, mediastinal core biopsy in 5%, and pleural fluid flow cytometry in one case; 62.3% had a white blood cell count of >100,000/mm3. A total of 72.1% (n=44) cases were diagnosed as T-cell acute lymphoblastic leukemia in our cohort. Clinical and laboratory tumor lysis syndrome developed in 10% and 73% of cases, respectively. Mechanical ventilation was required in 9.8% of the cohort. Mortality was reported in 10 (16.4%) patients. Conclusion We found that the 100% fatality rate with controlled positive pressure ventilation and spontaneous breathing is ideal. Tumour lysis syndrome was the most common morbidity in our cohort.

A rare case of constrictive pericarditis as initial manifestation of paediatric anaplastic large cell lymphoma requiring urgent pericardiectomy.

Constrictive pericarditis (CP) is a rare end stage inflammatory disorder affecting both parietal and visceral pericardium leading to a right heart failure. Malignancy is the least common cause of CP. Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of all Non-Hodgkin lymphomas in children. Very few case reports have reported ALCL that is involving the heart and only two have been published involving pericardium but all were managed medically. We present an interesting case of an 11 year old child who presented with an effusive CP that required urgent Pericardiectomy for managing right heart failure. His histopathology was positive for ALK + ALCL.

Retinoblastoma Presentation and Survival: A four-year analysis from a tertiary care hospital.

OBJECTIVE: To study the clinical presentation, treatment, and outcome of Retinoblastoma (Rb) in a tertiary care hospital of Pakistan. METHODS: A retrospective study was conducted in the Department of Pediatric Hematology Oncology, The Indus Hospital (TIH), Karachi from 1st June 2013 to 30th June 2017. Data including patients' demography, clinical symptoms and duration, laterality, extent of the tumor, type of treatment, relapse, and final outcome were extracted and evaluated with respect to progression and survival. RESULTS: A total of 93 patients were included; 34.4% were boys. The median age at presentation was 30 months. Leukocoria was the commonest symptom (61.3%), followed by proptosis (37.6%). Unilateral disease was seen in 59.1%, extraocular tumors in 43.5% and metastasis in 28.1%. Enucleation was performed on 46.2%, chemotherapy given to 80.6% and external beam radiation therapy to 29.3% patients. CONCLUSION: Delayed presentation, recurrent disease, extraocular disease and metastasis on presentation were factors affecting outcome in our cohort. Awareness about the early warning signs and symptoms in both public and health professionals for early recognition and timely management are mandatory to decrease morbidity and mortality.

Expression of PAX-5 in B Cell Hodgkin and Non Hodgkin Lymphoma

Background: B-cell malignancies including Precursor B-cell lymphoblastic lymphoma/leukemia and Hodgkin Lymphoma show a wide spectrum of B-cell differentiation from early stage B-cell precursors to mature B-cells ending in terminal differentiation to plasma cells. Pan-B-cell antigens routinely used for the diagnosis of B-cell lymphoma, include CD19, CD20, CD22 and CD79a.PAX-5 protein, also known as B-cell-specific activation protein is a B-cell-specific transcription factor; essential for commitment and functional maintenance used in the diagnosis of B cell Hodgkin and non-Hodgkin lymphoma. PAX-5 show nuclear positivity in B cell lymphomas and moderate (dim) positivity in Hodgkinlymphoma Reed Sternberg cells make this marker ideal for diagnosing B cell malignances. Objective: To determine the expression of PAX-5 in B cell Hodgkin and non-Hodgkin Lymphoma in order to improve the diagnosis of B-cell lymphomas. Methods: In this Prospective study, all the cases of B cell lymphoma diagnosed at The Aga Khan University Hospital, Karachi from July 2010 to July 2011were included. A panel of Immunohistochemical stain was performed in all cases along with additional PAX- 5 stain with appropriate controls. Results: Total 125 cases were included. Hodgkin Lymphoma (Mixed cellularity) was the commonest B-cell lymphoma subtype, 32 (25%) cases. Other common subtypes included Hodgkin lymphoma (Nodular sclerosis subtype), diffuse large B-cell lymphoma and B lymphoblastic lymphoma. Conclusion: This study demonstrates that PAX-5 is the most sensitive and reliable immuhohistochemicalmarker in the diagnosis of B cell Hodgkin and non-Hodgkin lymphoma.