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Background: There is rise in incidence of pediatric tumors all over the globe. In developing country like India childhood morbidity & mortality is mainly due to malnutrition & infections but there is significant rise in incidence of pediatric neoplasm which is now becoming a distinct & important entity to be dealt upon. Objectives: To study the incidence of solid pediatric malignant neoplasms, the age and sex distribution in cases of solid pediatric malignant neoplasms and various histopathological appearance and patterns of solid pediatric malignant neoplasms. Methods: An observational study of solid pediatric malignant neoplasms in childhood was done over a period of 2 years, from November 2016 to October 2018 in the age group of 0-14 years. The institutional ethical committee Government Medical College & Hospital, Aurangabad- Maharashtra India has given approval for this study. Result: Total 180 cases of solid pediatric neoplasm were studied. The total sample received during study period was 180 out of which 55 were malignant. Out of 55 malignant tumors 30 were observed among males and 25 were observed among females. Most of the malignant tumors were observed among 11-14 yrs age group, followed by those observed among 0-3 years. Conclusion: It can be concluded from our study that common solid malignancies in pediatric age group excluding hemato-lymphoid malignancies include nephroblastoma, retinoblastoma, neoplasms of gonads & bone tumors. Male predominance is a salient feature of childhood tumors. Most common solid tumors of childhood originate from embryonal tissue and tumors of epithelial origin are rare. A larger pediatric population based study would provide us with greater insight details about solid pediatric neoplasms.

Simple Summary Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare neoplasm that most commonly develops within maxilla in infants. It usually has a benign clinical course and is treated with only surgery. However, patients with large, inoperable, metastatic or multiply recurring MNTI may require systemic treatment. The role of pre- and post-surgery chemotherapy (CHT) in the management of MNTI is unclear. Here, we have presented the disease courses and outcomes of four infants treated with multidrug CHT due to inoperable/recurrent MNTI. Additionally, a systematic literature review was performed which revealed 38 similar cases in the last 42 years. Most children with primarily inoperable MNTI responded to CHT, which allowed physicians to perform complete, non-mutilating delayed surgery. However, it is still uncertain whether CHT administered after incomplete resection of MNTI prevents recurrence. This study aimed to contribute to the establishment of standards of management in patients with inoperable, metastatic or persistently recurring MNTIs, which are currently lacking. Abstract Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.

Aggressive enlargements of maxilla in pediatric patients are uncommon and present with diagnostic and therapeutic dilemma. The decision on therapeutic modality is based on an early and accurate diagnosis, minimizing disease-associated morbidity which is of utmost importance considering the young age and thereby resulting in better prognosis. Odontogenic myxoma is a locally aggressive lesion which is primarily seen in relation to odontogenic apparatus in mandibular posterior region in association with an impacted tooth. This presentation describes a unique case of odontogenic myxoma of anterior maxilla in an 8-year-old girl with emphasis on its diagnosis and treatment planning.

Medulloblastoma is the most common posterior fossa tumor of childhood typically within the fourth ventricle. However, extra-axial medulloblastoma in posterior fossa is an uncommon diagnosis. We report a case in a 33-month-old male who presented with repeated complaints of abdominal pain, intermittent emesis, and diarrhea, and diagnosed with right cerebellar extra-axial medulloblastoma, which was surgically resected. Majority of the reported extra-axial medulloblastoma in posterior fossa in the United States are located in the cerebellopontine angle. However, to the best of our knowledge, our case is the first to document medulloblastoma occurring exclusively in the cerebellar hemispheric extra-axial space rather than the cerebellopontine angle. Although the diagnosis can present as a radiological dilemma, a systematic multimodality imaging approach can aid in narrowing the differential diagnosis and timely management. In this case report, we will discuss the imaging characteristics, differential diagnosis, and management strategies, alongside a brief review of the world literature of extra-axial medulloblastoma. Keywords: Medulloblastoma, Pediatric neoplasm, Posterior fossa tumors, Extra-axial

Malignant ectomesenchymoma is a rare pediatric neoplasm with dual mesenchymal and neuroectodermal elements. Mesenchymal component is usually rhabdomyosarcoma, particularly embryonal subtype, whereas neuroectodermal derivatives are frequently a neuroblastic tumor. Ectomesenchymoma manifests in various sites given the wide migration of neural crest cells during development, though the pelvis/perineum is most often involved. Moreover, no unique unifying molecular abnormality has been determined.We conducted a retrospective study to analyze the spectrum of ectomesenchymal tumors encountered in our pediatric population. Six patients were identified and data pertaining to patients' demographic, tumor size and site, histologic components with immunophenotypic profile, molecular alterations, treatment, and outcome were collected.Mesenchymal elements, represented by rhabdomyosarcoma in all instances, were the dominant component in the majority of cases (5/6). Embryonal and alveolar morphology had similar distribution (3/6) and all patients with alveolar subtype harbored the characteristic translocations of this entity. The neuroectodermal component was most often a neuroblastic-like neoplasm (4/6); however, 2/6 cases demonstrated primitive neuroectodermal tumor-like morphology. No unifying alterations were found on molecular studies.Our analysis extends the histologic and molecular spectrum of these tumors and highlights their heterogeneity. The percentage of cases with alveolar rhabdomyosarcoma or primitive neuroectodermal-like tumor components suggests that these types of elements might be underreported. This study is also the first to demonstrate FOXO1 gene rearrangements in malignant ectomesenchymoma with alveolar rhabdomyosarcoma subtype.

International audience; Hepatoblastoma (HB) is the most common liver cancer in children. We aimed to characterize HB related to APC (Adenomatous Polyposis Coli) germline mutation (APC-HB). This French multicentric retrospective study included 12 APC-HB patients under 5 at diagnosis. Clinical features of APC-HB were compared to the French SIOPEL2-3 cohort of HB patients. Molecular and histopathological analyses of APC-HB were compared to 15 consecutive sporadic HB treated at Bicêtre hospital from 2013 to 2015 (non-APC-HB). APC-HB patients have a peculiar spectrum of germline APC mutations, with no events in the main hotspot of classical APC mutations at codon 1309 (P < .05). Compared to sporadic HB, they have similar clinical features including good prognosis since all patients are alive in complete remission at last follow-up. APC-HB are mostly well-limited tumors with fetal predominance and few mesenchymal components. All APC-HB have an activated Wnt/β-catenin pathway without CTNNB1 mutation, confirming that germline APC and somatic CTNNB1 mutations are mutually exclusive (P < .001). Pathological reviewing identified massive intratumor tertiary lymphoid structures (TLS) containing both lymphocytes and antigen-presenting cells in all 11 APC-HB cases who received cisplatin-based neoadjuvant chemotherapy but not in five pre-chemotherapy samples (four paired biopsies and one patient resected without chemotherapy), indicating that these TLS are induced by chemotherapy (P < .001). Conclusion: APC-HB show a good prognosis, they are all infiltrated by cisplatin-induced TLS, a feature only retrieved in a minority of non-APC-HB. This suggests that APC inactivation can synergize with cisplatin to induce an immunogenic cell death that initiates an anti-tumor immune response.

Congenital mesoblastic nephroma (CMN) is a rare tumor of infancy. CMNs can be histologically divided into classic, cellular, and mixed subtypes. Cellular CMNs are difficult to differentiate from Wilms tumors. Herein, a neonate with cellular CMN accompanied by macroscopic hematuria, is described. The clinical, pathological, and imaging features of the disease are discussed.

Granular cell tumors (GCTs) are uncommon benign neoplasms thought to originate from Schwann cells. They most commonly present in adults as papules or nodules on mucosal sites. The clinical spectrum of GCT in children is not well delineated, although mucosal and cutaneous presentations have been reported. We present three children with cutaneous GCTs and review the literature in an attempt to further characterize this rare pediatric neoplasm.

Lymphoma is the third most common pediatric neoplasm. Non-Hodgkin lymphoma (NHL) accounts for nearly half of cases and commonly involves extranodal sites. Compared with adults, this histologic spectrum of pediatric NHL is very narrow and consists of aggressive tumors. Patients typically present with widespread disease. Generally, NHL occurring in children includes Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma, and anaplastic large cell lymphoma. Staging and assessment of therapeutic response are usually based on FDG-PET/CT. Due to the increased susceptibility of young patients to the effects of ionizing radiation, alternative methods of imaging are being explored.

Hepatocellular carcinoma (HCC) is a rare pediatric neoplasm exceptionally reported in infants and fibrolamellar hepatocarcinoma (FLC) a HCC variant. Controversy exists whether FLC has a better prognosis than classic HCC, although recent studies of children and young adults with FLC did not report a better outcome. We present a 4-month-old male infant without any related metabolic or infectious disease who developed a metastatic and multifocal FLC. Serum alpha-fetoprotein determinations were always normal. Induction chemotherapy using cisplatin and Adriamycin resulted in a partial response, however, refractory disease developed and regional metastasis precluded surgical resection. The child died from tumoral progression.

Neuroblastoma (NB) is known as a pediatric neoplasm that is associated with variable histopathological features. The use of biomarkers contributes to the monitoring and treatment of various malignancies such as NB. The identification of novel biomarkers such as (epi)genetic biomarkers and microRNAs (miRNAs) in NB has led to better treatments of NB. Among them, miRNAs have emerged as powerful tools in diagnosis, prognosis and therapeutic biomarkers for patients with NB. Circulating biomarkers such as circulating miRNAs present in body fluids (for example, plasma, serum and urine) provided an interesting field of study in NB treatment. The miRNAs have central roles in different pathogenic events in various malignancies such as NB. Hence, these molecules can be a suitable candidate for monitoring and treating NB patients. Here, we summarize some miRNAs as potential prognosis, diagnosis and therapeutic biomarkers in NB.

Neuroblastoma (NB), a common pediatric neoplasm arising from neural crest cells, is characterized by a diversity of clinical behavior ranging from spontaneous remission to rapid tumor progression and death. Biologic and genetic factors have been identified which, to a certain extent, can predict prognosis and guide risk-based therapy. Recent research has focused on the role of angiogenesis in NB tumor biology and in determining clinical phenotype. Preclinical studies have explored the efficacy of antiangiogenic therapies as a novel treatment modality for children with NB. This review discusses the current understanding of angiogenesis in NB, the clinical implications, and future directions for antiangiogenic therapeutic strategies in NB.

For the past 30 years, retinoblastoma, a tumor that occurs in only 3 % of children with cancer, has been the subject of extensive molecular biologic research. However, apart from a short period in the 1970s, retinoblastoma has not been studied by any of the pediatric cooperative groups. The past decade has witnessed significant multidisciplinary prospective clinical and biologic studies of this rare pediatric neoplasm. The Children’s Oncology Group (COG) has successfully opened four clinical trials with a fifth recently approved. This chapter will review these ongoing prospective multicenter trials.

Introduction Haemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels. They are the most common pediatric neoplasm. Kasabach-Merritt syndrome is a rare type of vascular lesion with peculiar characteristics. The diagnosis is based upon three basic findings; enlarging haemangioma, thrombocytopenia and consumption coagulopathy. Case presentation A 5 month old boy was admitted to the Pediatrics department for the management of an abdominal wall mass. He was the first child of consanguineous parents, born in a private hospital following uncomplicated pregnancy and delivery. At birth a bluish birth mark 5 cm × 5 cm was noted below the umbilicus. Over the next five months, this birth mark increased in size and evolved into a swelling. As a result, the patient was admitted to Maternal and Child Health (MCH) unit for the management of this swelling. The clinical findings and imagining studies followed by laboratory investigations strongly suggested the diagnosis of Kasabach-Merritt syndrome. Vincristine was initiated after a trial of corticosteroids when the platelet count was 6000/cmm. One week after the start of vincristine the size of the lesion started to decrease. At the end of 6th week the lesion size decreased to half and the platelet count increased to 49,000/cmm. Vincristine was continued for another 2 weeks, no further improvement in lesion size or platelet count was observed. Vincristine was discontinued and the patient was shifted to the paediatric surgery department. A fresh platelet transfusion was given and the haemangioma was excised completely. The histopathological examination of the excised mass revealed a caverno-capillary haemangioma with infiltration into skeletal muscles. Conclusion Six weeks treatment with vincristine in a dose of 0.5 mg/kg/week followed by surgical excision may be the best management in selected cases of Kasabach-Merritt syndrome.

Background: Conventional cytology (CC) is a well-established and effective methodology for the evaluation of pediatric tumors. Liquid-based cytology (LBC) is a better technique of smear preparation and is at present the standard protocol in cervical cytology samples. Aims: In the present study, we analyzed both smear preparatory techniques in fine-needle aspiration (FNA) samples from solid pediatric neoplasms in terms of adequacy and efficacy with objectives to study the changes in background and morphology of tumor cells in LBC smears. Materials and Methods: This was a prospective observational study conducted in a tertiary care teaching hospital, which included 52 pediatric patients with clinical diagnosis of malignancy and an assessable lump. Both conventional aspiration cytology and LBC smears were prepared as per standard protocols (SurePath BDTM) from FNA samples of cases and examined. Results and Conclusion: On comparing the diagnostic efficacy of cytology smears prepared by both techniques, LBC alone was diagnostic in 80.8% of the cases and conventional smear (CS) alone was diagnostic in 71.2% of the cases (agreement was 83.7%, P = 0.3). Cellular morphology was better preserved in LBC and interpretation was easier. There was a lower inadequacy rate in LBC and none of the samples was inadequate due to poor morphology in LBC smears (P = 0.0003). LBC showed an overall 40% improvement in inadequate cases by CS. LBC has been recommended as a complement to CC in nongynecological samples. Improved morphology and lower inadequacy rate make LBC a finer technique compared to CS in pediatric tumors as well.

Neuroblastoma is an extremely rare pediatric neoplasm whose prognosis becomes poor and poor as the age advances. It can be sporadic or nonfamilial in origin. It is primarily a tumor of abdominal origin from where it metastasis to lymph nodes, liver, intracranial and orbital sites, and central nervous system. There is no standard dental treatment protocol for the management of neuroblastoma due to its poor survival rate and rarity. However, dental treatment may follow the protocol of preventive and restorative. Surgicals should be performed under supervision as it may trigger metastasis. We report a rare case of neuroblastoma in a 3-year-old child presenting classical oral manifestations such as bilateral palatal swelling, rolled border ulcer on the posterior part of hard palate adjacent to primary molars, and bilateral proptosis.

Lipofibromatosis is a relatively rare pediatric neoplasm, which usually manifests as an ill-defined soft tissue mass involving the upper and lower distal extremities, the trunk, and, less frequently, the head. To date, no cytogenetic abnormalities have been reported in this uncommon neoplasm. We present a case of lipofibromatosis featuring a three-way t(4;9;6) translocation in a 5-year-old boy.

Pancreatoblastoma is a rare pediatric neoplasm with distinct acinar and squamoid differentiation that generally affects infants and young children. Although the prognosis is relatively favorable over typical pancreatic cancer, the optimal treatment of pancreatoblastoma has not been established. We experienced two cases of pancreatoblastoma in a 2-year-old girl and boy which was found incidentally by the parents due to the detection of abdominal masses.