CD99 and NKX2.2 positive neuroblastoma diagnosed on cytology: A potential diagnostic pitfall and necessity of pathological evaluation of the primary site.

Immunohistochemistry plays a vital role in the diagnosis of small round cell tumors. CD99 immunonegativity is one of the features, which helps in distinguishing neuroblastoma from other small round cell tumors. NKX2.2 is a specific marker of Ewing sarcoma, which is a differential for poorly differentiated neuroblastoma. Here, we present a case of metastatic neuroblastoma showing immunoreactivity for both CD99 and NKX2.2 on cytology of the metastatic site causing diagnostic dilemma. Biopsy study of the adrenal lesion revealed presence of differentiating cells and neuropil, highlighting the importance of evaluation of the primary site and limitation of cytology.

Fixed-Dose Recombinant Urate Oxidase in the Treatment of Paediatric Tumour Lysis Syndrome: A Regional Cancer Centre Experience.

BACKGROUND: Tumor lysis syndrome (TLS) is an oncologic emergency commonly seen in children with hemato-lymphoid malignancies. Recombinant urate oxidase (RUO) is used in both the prophylaxis and treatment of TLS. However, in resource-constrained countries, its role is mostly limited to the treatment of established TLS and data regarding the use of RUO and its outcome is sparse. OBJECTIVE: To describe the outcome of Pediatric TLS following the use of a fixed - dose of RUO. METHODS: A retrospective chart review of all children <15 years of age admitted in the Department of Paediatric Oncology, Kidwai Cancer Institute from April 2017 to July 2018 with TLS and treated with a single, fixed - dose (1.5 mg) RUO was undertaken. RESULTS: During the study period, 255 children with hemato-lymphoid malignancies were diagnosed to be at risk of developing TLS. Of these, only 22 (8.6%) children developed TLS and received RUO. Among those with TLS, 15 (68.2%) had Acute Lymphoblastic Leukemia (ALL) while 7 (31.8%) had Non - Hodgkin lymphoma (NHL). 91% (20/22) children had spontaneous TLS and the remainder developed therapy-related TLS. Median age at presentation was 8 years (IQR 5.25,1.75) with 4.5:1 male: female ratio. The mean urate level at admission was 19.12 mg/dl (+/- 8mg/dl) (Range: 10.7-34.5). 91% (20/22) children received RUO at less than 0.15 mg/kg and the median dose of RUO was 0.05 mg/kg (IQR 0.038-0.08). Of the 22 children with TLS, 2 children failed to achieve normal serum urate levels at 24 hours in response to a single fixed-dose of RUO and hence received an extra dose of RUO. Serum urate levels remarkably declined following RUO administration from 19.12 mg/dl (+/-8) to 8.2 mg/dl (+/-3.9), 3.99 mg/dl (+/-1.6) and 2.84 mg/dl (+/-1.3) at 12h, 24h and 48h respectively. AKI was present in 15 (68.2%) children. The median eGFR of the group at diagnosis was 49 ml/min/1.73m2 (IQR 26.3, 70). None of the children required hemodialysis. No significant adverse events occurred. CONCLUSION: Fixed-dose RUO can achieve rapid, adequate and sustained drop in serum urate levels in Paediatric TLS. It is a useful strategy for managing TLS in resource-constrained settings.

Evaluation of serum procalcitonin, serum interleukin-6, and interleukin-8 as predictors of serious infection in children with febrile neutropenia and cancer.

BACKGROUND: Early diagnosis of sepsis in children with febrile neutropenia remains difficult owing to non-specific clinical and laboratory signs of infection. There is a need to assess the utility of inflammatory markers in clinical risk assessment for their ability to discriminate between low-risk and high-risk neutropenic patients since presently there is insufficient data to recommend their routine use. METHODS: This is a prospective study of children on therapy admitted with febrile neutropenia and sampled for serum procalcitonin (PCT), interleukin-6 (IL-6), and interleukin-8 (IL-8) at admission. The febrile neutropenia episodes were categorized into two groups - Group I: no focus of infection and Group II: clinically/microbiologically documented infection. Statistical analyses for comparison were performed using Z-test and receiver operating curves at various cut-off levels. RESULTS: A total of 46 episodes of febrile neutropenia in 33 children were analyzed. In total, 76% were categorized as group I and 24% as group II. The mean value of PCT in group II was higher (28.07 ng/mL) than group I (1.03 ng/mL) though there was no significant statistical difference. At a cut-off level of 2 ng/mL for PCT, sensitivity of 63%, specificity of 91%, positive predictive values (PPV) of 70%, and negative predictive value (NPV) of 88% were observed. There was no significant difference in the IL-6 and IL-8 levels between both the groups. However, at an optimal cut-off value of 50 pg/mL, IL-6 had an NPV of 80% and at a cut-off level of 130 pg/mL, IL-8 had an NPV of 73%, however, with low sensitivity and specificity. CONCLUSION: IL-6, IL-8, and PCT can be utilized to define a group of patients with a low risk of sepsis in view of their favorable NPV. The use of these biomarkers together can facilitate early discharge from the hospital, and the use of oral antimicrobial therapy, in turn, reducing the cost of supportive therapy in a developing country.

Epidemiology and clinical features of retinoblastoma: A tertiary care center's experience in India.

INTRODUCTION: Retinoblastoma (RB) is a prototype of heritable cancers. It is more common in the lower socioeconomic strata. Delayed presentation significantly reduces the overall outcome. We have analyzed the epidemiological and clinical data of children who were diagnosed with RB between the years 2009 and 2014. AIM: RB being a disease of the poor, delayed presentation is common due to lack of awareness. We have analyzed the epidemiological profile of our patients and tried to establish the link between delayed presentation and the presence of high-risk features. High-risk features are associated with higher chance of metastasis and poor rates of vision salvage in RB. METHODOLOGY: Data were collected in a retrospective manner from the patient case files retrieved from the Medical Records Department, Kidwai cancer Institute. The data were analyzed using Excel and SPSS software (IBM Corp. released 2016, IBM SPSS statistics software for Mac OS, version 24, IBM Corp., Armonk, NY). RESULTS: A total of 53 patients were diagnosed with RB in the years 2009-2014. There was a male predominance with 1.2:1 incidence. Bilateral RB was present in 21 cases. The mean age of children with bilateral RB was 2.1 years, against 1.5 years in unilateral cases. High-risk features such as optic nerve invasion, choroidal invasion, intracranial extension, and orbital involvement were found in 12, 6, 5, and 5 eyes, respectively. Bone marrow involvement was detected in 5% and lung metastasis in 2%. Intracranial involvement was found in 10.4% and cerebrospinal fluid positivity in 15%. Children with high-risk features had a significant delay in presentation in comparison to those without high-risk features (P = 0.035). CONCLUSION: Incidence of metastatic disease and delayed presentation is still high in developing countries. Routine eye examination during vaccination visits can ensure early diagnosis and appropriate referral in many of these children.

Tuberculosis Coexistence in Pediatric Hodgkin's Lymphoma: A Tropical Country Experience.

Introduction Hodgkin's lymphoma (HL), being one of the common cancers among children, may occasionally masquerade as an infectious illness. Similarly, an underlying infection like tuberculosis (TB) may be missed in cases of HL because of similarity in clinical and radiological features. Here, we present our data of association of HL with histopathologically proven TB lymph node, their clinical presentation, treatment details, and outcome. Materials and Methods A retrospective review of all the cases of HL diagnosed between January 2007 and December 2016 was done. The cases which had an association of TB, based on the histopathology, were reviewed separately. Results A total of 262 children with HL were treated at our institute from January 2007 to December 2016. Of these cases, 42 children had received empirical antitubercular therapy (ATT) (due to suspicion of TB) before presenting to us, and only five cases had histopathologically proven TB lymph node. Ziehl-Neelsen (ZN) stain for acid-fast bacilli (AFB) was positive in the biopsy specimen of all the five cases, proving TB lymph node coexistence with HL. They were treated with six-drug ATT as per the Revised National Tuberculosis Control Program (RNTCP) guidelines along with chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine regimen. All the five patients are healthy and disease free until their last follow-up. Conclusion A high-end suspicion for concomitant TB and HL is needed, especially in our country where TB is still rampant. Biopsy with immunohistochemistry and demonstration of AFB can enable a definite diagnosis of both the entities.

Multifocal Osteosarcoma: Multiple Primaries or Metastases? A Report of Rare Case and Review of Literature.

INTRODUCTION: Multifocal osteosarcoma (MFOS) is characterized by multicentricity of osseous osteosarcomas, either synchronous or metachronous, without visceral involvement. They account for about 1.5% of all osteosarcomas. Most synchronous MFOS has one dominant lesion with one to four and very rarely five or more secondary lesions. The distal femur followed by the proximal tibia is the most common site of dominant lesions. Its prognosis remains extremely poor even with combined chemotherapy and surgery. CASE REPORT: We describe a rare case of MFOS in a 10-year-old boy who presented with a short history of severe aching pain in the right lower limb following a trivial fall. Initial workup and relevant investigations revealed a synchronous multicentric osteosarcoma with extensive involvement of appendicular and axial skeletal system. The dominant lesion was at the lower end of the right femur with multiple secondary lesions in the right tibia, left femur, bilateral humeri, pelvis, cervical and dorsolumbar spine, ribs, and sternum. The patient received one cycle of doxorubicin and cisplatin-based chemotherapy but unfortunately succumbed to progressive disease, a month after initiation of chemotherapy. CONCLUSION: MFOS is a very rare presentation of osseous osteosarcoma. The non-specific clinical manifestation, despite the presence of generalized skeletal involvement, presents a diagnostic difficulty for both the clinician and the radiologist. Only biopsy and histopathological examination can confirm the diagnosis of this highly malignant disease and help in proper management.

Pediatric Inflammatory Myofibroblastic Tumors of the Airway: Two Case Reports with Varying Clinical Presentation.

INTRODUCTION: An inflammatory myofibroblastic tumor (IMT) is a rare tumor of intermediate malignant potential. It may occur in a wide range of anatomical locations. One-third are found in the respiratory tract. We report two cases of IMT of the airway diagnosed at our institution. CASE REPORT: Case 1: A 6-year-old male child presented with a 1-month history of hoarseness of the voice. On evaluation, a polypoid nodule was noted in the right vocal cord which was excised through the endolaryngeal route. Histopathology was suggestive of anaplastic lymphoma kinase (ALK)-negative IMT. He presented with recurrence after 4 months, for which he underwent endolaryngeal reexcision and tracheostomy for airway protection. A third recurrence after 6 months was managed with laser excision, and the patient was started on oral celecoxib. After 1.5 years of follow up, endoscopic examination showed no recurrence, and celecoxib was continued. Case 2: A 7-year-old male child presented with cough and respiratory distress. Bronchoscopy and high resolution computed tomography showed a polypoidal lesion with calcification arising from the left anterolateral wall of the trachea with significant narrowing of the lumen. The patient underwent biopsy followed by endoscopic excision, and was diagnosed with IMT. Currently the patient is under follow up with no recurrence. CONCLUSION: IMT indicates a proliferative myofibroblastic growth. Surgical resection should be recommended for all lesions if not prohibited by anatomic location or morbidity. Patients should be followed up closely for recurrence. In most cases, complete surgical excision will suffice; however multiple recurrences can be managed with chemotherapy. These two cases highlight the importance of a multidisciplinary approach in rare tumors in difficult anatomical locations.

Undifferentiated thymic carcinoma with intracranial metastasis in a two-year-old.

Thymic carcinoma with central nervous system involvement is very rare in children. A 27-month-old girl presented with a unilateral squint, vomiting, and behavioral changes. Imaging studies showed a silent anterior mediastinal mass and a large metastatic mass at the base of the skull. Biopsy of the anterior mediastinal mass confirmed an undifferentiated tumor consistent with thymic carcinoma. The child died within 3 months of the onset of symptoms, due to progression of the disease. These lethal tumors of unknown histogeneses and etiology are aggressive in nature, resistant to therapy, and have a rapidly fatal course.

Unusual Clinical Presentation and Role of Decompressive Craniectomy in Herpes Simplex Encephalitis.

Decompressive craniectomy in pediatric central nervous infections with refractory intracranial hypertension is less commonly practiced. We describe improved outcome of decompressive craniectomy in a 7-year-old boy with severe herpes simplex encephalitis and medically refractory intracranial hypertension, along with a brief review of the literature. Timely recognition of refractory intracranial hypertension and surgical decompression in children with herpes simplex encephalitis can be life-saving. Additionally, strokelike atypical presentations are being increasingly recognized in children with herpes simplex encephalitis and should not take one away from the underlying herpes simplex encephalitis.