PEDIATRICS AND NEONATAL NURSING ISSN 2377-1569 http://dx.doi.org/10.17140/PNNOJ-2-114 Open Journal Research The Spectrum of Malignant Solid Childhood *Corresponding author Tumors in the Age Group of 0-12 Years Gite Vandana, MD Associate Consultant Department of Lab Services 1* 2 Apollo Hospitals, Bilaspur Gite Vandana and Dhakane Maruti Chhattisgarh, India Tel. 09755040727; 09893542691 E-mail: [email protected] 1Department of Lab Services, Apollo Hospitals, Bilaspur, Chhattisgarh, India 2Seth GS Medical College and KEMH Parel, Mumbai, India Volume 2 : Issue 3 Article Ref. #: 1000PNNOJ2114 ABSTRACT Article History Objective: To document general baseline data on the patterns of childhood malignant tumors in Received: October 18th, 2015 a surgical pathology department. Accepted: October 29th, 2015 Design, Setting and Participants: This is a retrospective analysis of 35 cases of pediatric tumors Published: October 29th, 2015 in surgical pathology department of tertiary care hospital excluding neurosurgery, cardiotho- racic and haemato-lymphoid malignancies. (Age group 0-12 years) encountered over a period Citation of 5 years. Vandana G, Maruti D. The spectrum Results: 35 children were diagnosed with malignant tumors. The commonest tumor was wilms of malignant solid childhood tumors tumor (9 out of 35 cases) followed by neuroblastoma (4 out of 35 cases). The common age of in the age group of 0-12 years. Pedi- presentation was 1-5 years with male predominance. In the renal tumors only wilms tumors atr Neonatal Nurs Open J. 2015; 2(3): (9 cases) was seen, with classical triphasic tumors were more common. The mean age of pre- 85-90. doi: 10.17140/PNNOJ-2-114 sentation was 3 year with commonest age group of presentation (8 cases out of 9) in the age group 1-5 years. Three of them had showed unfavorable histology. In the adrenal gland, adrenal medullary tumors were more common than adrenal cortex with neuroblastoma (4 of 6 cases) as common individual tumor. Immunohistochemistry performed on 10 of 11 round cell tumors revealed five cases of lymphoma, three cases of Rhabdomyosarcoma (RMS) & two cases of Ewing sarcoma-primitive neuroectodermal tumor (EWS/PNET). Conclusion: Histological type is important for understanding etiology and progression of dis- ease. The likelihood of a given type of tumor being present in a particular age or sex group or particular site may heighten the index of suspicion and ultimately influences etiology, biol- ogy, and natural history, relative incidence and distribution frequency, clinical presentation and manifestations, and response to therapy and outcome. KEYWORDS: Neuroblastoma; Rhabdomyosarcoma; EWS/PNET. ABBREVIATIONS: hCG: human Chorionic Gonadotropin; AFP: Alpha fetoprotein; LDH: Lac- tate dehydrogenase; NHL: Non-Hodgkin Lymphoma; RMS: Rhabdomyosarcoma; ACC: Adre- nocortical carcinoma; PHPV: Persistent Hyperplastic Primary Vitrious. INTRODUCTION Incidence of childhood malignant tumors is on the rise all over the globe, though it is a small fraction of the overall global tumor burden. Yet for children and their families it can be deeply distressing. Although childhood malignant tumors occur infrequently, they present a challenging diagnostic and therapeutic problem. Unfamiliarity with these conditions may lead Copyright to the erroneous diagnosis and unnecessarily aggressive therapy. ©2015 Vandana G. This is an open access article distributed under the Malignancy is the second most common cause of childhood mortality in the developed Creative Commons Attribution 4.0 world, accounting for 12.3% of all childhood deaths in USA.1 Although major cause of child- International License (CC BY 4.0), hood mortality in the developing world is still malnutrition and infections, childhood malignant which permits unrestricted use, th distribution, and reproduction in tumors are also rising in number. About 1/650 children develops malignancy before their 15 2 any medium, provided the original birthday. Malignancies accounts for the major cause of death in Indian children next only to work is properly cited. infection and malnutrition. Approximately 35,000 to 40,000 children develop malignancies Pediatr Neonatal Nurs Open J Page 85 PEDIATRICS AND NEONATAL NURSING ISSN 2377-1569 http://dx.doi.org/10.17140/PNNOJ-2-114 Open Journal each year in India.2 or total surgical pathology sample received. The present study comprises of 35 cases (1.11%) of childhood malignant tumors Thus, the appropriate management of pediatric tumors from a total of 3149 pediatric surgical specimens received over requires detailed clinical history, tumor site, and precise histo- a period of 5 years. It is difficult to compare incidence data be- pathological diagnosis, accurate grading & staging wherever cause of the difficulties associated with retrieving demographic possible along with other clinical investigations. Histological data for previously studied pediatric populations. type is important for understanding etiology and progression of disease. No histological diagnosis can be accurate without a Most of the childhood malignant tumors occur below clinico-radio-pathological correlation. the age of eight years, although wide age variability exists in children.2 In the present study, 12 years was considered as the MATERIALS AND METHODS pediatric age with infancy as a separate age group. Neuroblas- toma, Wilms tumor, retinoblastoma and hepatoblastoma were This was a retrospective analysis of 35 cases of pediat- strikingly more in children younger than 5 years of age, similar ric tumors in surgical pathology department excluding neurosur- to that observed by Kusumakumary P, et al.3 However, round cell gery, cardiothoracic and haemato-lymphoid malignancies. (Age tumors, non-neuroblastic adrenal tumors presented commonly group 0-12 years) encountered over a period of 5 years: January in the age group of 5-10 years. Age also has strong prognos- 2004-December 2008. Surgical specimens and biopsy tissues re- tic relevance in certain tumor. It has observed that, infants with ceived were fixed overnight in 10% buffered formalin and sub- neuroblastoma seemed to have better prognosis than older chil- mitted for processing. Paraffin sections were cut at 4-6 microns dren even after minimal therapy.4 However, the age <1 year at thickness and routine H & E staining was performed. All cases diagnosis has been associated with a worse prognosis in rhab- was re-evaluated histologically on sections from routinely pro- domyosarcoma (RMS).5 Male predominance is a salient feature cessed formalin fixed, paraffin embedded blocks. Special stains of many childhood tumors.2 There is higher incidence in males & Immunohistochemistry were studied wherever necessary. The with male to female ratio of 2.2:1. Male preponderance was seen clinical, radiological and therapeutic data was obtained from pa- in wilms tumor, round cell tumor. There were equal incidence of tients case paper records. Pattern of childhood malignancies was Neuroblastic tumors in males & females. studied with a focus on tumor incidence, age and sex distribu- tion, environmental and other etiological factors, demographic Childhood malignant tumors account for no more than pattern, and histological type. 2% of all cancers. The tumors encountered by Sebastian, et al.6 were lymphomas 44.3%, Wilms tumor 20.1%, sarcomas 11.5%, RESULTS neuroblastoma 8.6%, retinoblastoma 8.0%, teratomas 4.6% and hepatoma 2.9%. In the present study, wilms tumor with 9 cases A total of 3149 pediatric surgical specimen presented (25.71%) was the largest group. over a five years. Of this, 35 were diagnosed with malignant tumors. Average incidence of malignant pediatric tumors was All pediatric renal tumors were Wilms tumor i.e. 100% 1.11%. The commonest tumor was wilms tumor (9 out of 35 as compared to the 78.4% by Louisa Paul, et al.7 This difference cases) followed by neuroblastoma (4 out of 35 cases). The com- may be due to small sample size in the present study. All the mon age of presentation was 1-5 years with male predominance. cases in this study presented with abdominal mass. Eight out In the renal tumors only wilms tumors (9 cases) was seen, with of 09 cases were presented in an age group 1-5 years & 01 case classical triphasic tumors were more common. The mean age of was seen in an age group 0-1 year, which was comparable to the presentation was 3 year with commonest age group of presenta- study done by Louisa Paul, et al.7 The median age of presenta- tion (8 cases out of 9) in the age group 1-5 years. Three of them tion was 3 years. Thus, the age distribution was consistent with had showed unfavorable histology. In the adrenal gland, adre- other studies.7-9 Wilms tumor presenting in infancy when treated nal medullary tumors were more common than adrenal cortex appropriately has a good outcome. Age 4 years at first diagnosis with neuroblastoma (4 of 6 cases) as common individual tumor. is clearly an adverse prognostic factor probably that due to ad- Among the gonaldal germ cell tumors, there were noted one im- verse biologic features. There were seven cases in males & two mature teratoma, two yolk sac tumors of ovary & one yolk sac in females giving a ratio of male to female ratio of 3.5:1. This tumor in testis. Immunohistochemistry performed on 10 of 11 was slightly on higher side as compared to study done by Louisa round cell tumor revealed five cases of lymphoma, three cases Paul, et al.7 Patel A. A., et al10 in their study of 11 infantile Wilms of rhabdomyosarcoma & two cases of EWS/PNET. tumor found the male to female ratio was 2.3:1. Husain A. N., et al.8 found Wilms tumor slightly more common in girls in whom DISCUSSION it tends to present at an older age. In literature, differences have been demonstrated in the The mean tumor size in present study was 8 cm with incidence rates of pediatric malignant tumors as they are stud- a range of 4cm to 15 cm. One of the poles of kidney was com- ied by anatomic site, age, race or gender.