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.

Myxoinflammatory fibroblastic sarcoma--report of a rare case at an unusual site with review of the literature.

We report a case of myxoinflammatory fibroblastic sarcoma in a thirteen year old girl who presented with a tender swelling in the left upper back. The tumor consisted of varying proportions of inflammatory, myxoid and hyalinized areas. Large bizarre cells with virocyte like inclusions and lipoblast like cells were present. To the best of our knowledge this is the first reported case of myxoinflammatory fibroblastic sarcoma of the back, the extremities being the commonest site of involvement. Due to its varied histologic appearance, the tumor should be differentiated from various benign and malignant soft tissue lesions.

Cytogenetic study of non-Hodgkin lymphoma from South India. histologic and geographic correlations.

Cytogenetic analysis of fine needle aspiration cultures was performed on 189 patients with non-Hodgkin lymphoma from South India. Successful karyotyping was possible in 97 patients (51.3%). Burkitt lymphoma constituted 56% of the cases studied followed by diffuse type 20%, follicular 8.8%, lymphoblastic 6.6%, and unclassified 6.6%. Characteristic chromosomal translocations were t(8;14)(q24;q32) [32.2%], t(8;22)(q24;q11) [10%], t(2;8)(p12;q24) [2.2%], t(14;18)(q34;q21) [3.3%], and t(11;14)(q23;q32) [2.2%]. Notable geographical variation of some structural abnormalities was the finding in the present study such as, lower frequency of t(14;18) in follicular lymphomas and higher frequency of t(8;14) in Burkitt lymphomas when compared with the Western studies.