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Pathology and Epidemiology
Published in John T. Kemshead, Pediatric Tumors: Immunological and Molecular Markers, 2020
Another renal tumor of childhood which reclassification has separated from Wilms’ tumors has been the congenital mesoblastic nephroma, which in the great majority of instances behaves in an entirely benign fashion. The benefit to the patient of this diagnostic separation has been considerable, as previously such tumors were treated aggressively, as though malignant, whereas since the recognition by Bolande9 of their relatively benign nature, it has been found that adequate nephrectomy alone usually achieves a cure. This finding alone has led to the removal of much of the morbidity and even mortality associated with the treatment of these tumors in the past.
Haematology and oncology
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
Wilms' tumour arises from the kidneys, neuroblastoma from the adrenal gland and hepatoblastoma from the liver, all of which can represent as a mass on the right side of the abdomen. Mesoblastic nephroma is a congenital tumour and occurs in children under the age of 2 years. Non-Hodgkin's abdominal lymphomas occur most frequently in the ileocaecal region and present as an abdominal mass, intestinal obstruction or intussusception.
Congenital mesoblastic nephroma and Wilms tumor
Published in Prem Puri, Newborn Surgery, 2017
Philip J. Hammond, Robert Carachi
Postoperative recovery following resection of mesoblastic nephroma is rapid. Nephrectomy of this benign tumor is curative. If on histology, the tumor is found to be Wilms, it should be treated in accordance with the degree of involvement as outlined in the National Wilms Tumor Study programs.
Neurotrophic tropomyosin receptor kinase (NTRK) fusion positive tumors: a historical cohort analysis
Published in Expert Review of Anticancer Therapy, 2023
Lauriane Lemelle, Delphine Guillemot, Anne-Laure Hermann, Arnaud Gauthier, Matthieu Carton, Nadège Corradini, Angélique Rome, Pablo Berlanga, Anne Jourdain, Aude Marie Cardine, Sarah Jannier, Hélène Boutroux, Anne Sophie Defachelles, Isabelle Aerts, Birgit Geoerger, Marie Karanian, François Doz, Hervé J Brisse, Gudrun Schleiermacher, Olivier Delattre, Gaëlle Pierron, Daniel Orbach
Clinical data were collected retrospectively from medical files (supplemental material 1). To better characterize the specific outcome, patients were analyzed in three groups: IFS (from soft tissue or kidney congenital mesoblastic nephroma (CMN)), other mesenchymal tumors (Other-MT), and CNS tumors. Histological diagnosis was based on local pathology reports. The group of ‘alkylating drugs’ effects included ifosfamide, cyclophosphamide, high-dose cyclophosphamide, thiotepa, temozolomide, procarbazine, and CCNU. The group of ‘anthracycline drugs’ included only doxorubicin. A ‘mutilating surgery’ was defined as either a major resection or a total organ amputation with functional impairment or a significant esthetic impairment. Long term side effects were graded according to the Common Terminology Criteria for Adverse Events v5.0.
Pediatric Primary Yolk Sac Tumour of the Kidney: Recommendations for Pretreatment Diagnosis
Published in Fetal and Pediatric Pathology, 2023
Shilpi Thakur, Aanchal Kakkar, Manisha Jana, Prasenjit Das, Sandeep P. Agarwala, Venkateswaran K. Iyer
Nephroblastoma, or Wilms tumor (WT), is the most common primary renal tumor in children [1, 2]. There are two approaches to its management: either upfront surgery followed by adjuvant therapy, as advocated by the National Wilms Tumor Study/Children’s Oncology Group (NWTS/COG), or preoperative chemotherapy followed by surgery and adjuvant therapy followed by the International Society of Pediatric Oncology (SIOP). Neither of these approaches endorses the requirement for a tissue diagnosis prior to initiation of treatment. Clinical settings that warrant an upfront biopsy include features atypical for nephroblastoma such as age >6 years, atypical radiological features viz. very large lymph nodes, intratumoral calcification, almost totally extra-renal tumor, no visible normal renal parenchyma, and extrahepatic/extrapulmonary metastases [2]. Up to 15% of pediatric renal tumors have been found to be non-Wilms tumors, including congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK), clear cell sarcoma of kidney (CCSK), angiomyolipomas and renal cell carcinoma [3, 4]. An upfront tissue diagnosis avoids risking overtreatment of benign tumors or under-treatment of more aggressive malignancies like CCSK and MRTK. The United Kingdom Children’s Cancer Study Group (UKCCSG) has adopted pre-chemotherapy biopsy in their management protocol [3]. In India, as majority of patients present with advanced stage tumors, the Indian Council of Medical Research (ICMR) developed consensus guidelines favoring pre-operative chemotherapy for most patients with WT [5]. These guidelines recommend that a biopsy may be performed as per individual institutional practice; at our institution and many others, it is the practice to obtain a tissue diagnosis prior to starting chemotherapy [1].
Profile of Non-Hematological Pediatric Tumors: A Clinicopathological Study at a Tertiary Health Care Centre
Published in Fetal and Pediatric Pathology, 2018
Kashmi Sharma, Mehar Aziz, Nishat Afroz, Aaliya Ehsan
Childhood cancers show a wide histopathological spectrum, with varying incidence worldwide by age, sex, ethnicity and geography. In our study the major tumor groups observed were bone and cartilage tumors, soft tissue tumors, lymphomas and central nervous system tumors which is quite similar to study by Punia et al. (13), but is discordant with various other studies (Table 4]. This variation may be attributed to different study designs, geographical, ethnic and environmental variations. Osteosarcoma and ES/PNET were the major malignant bone tumors observed, in accordance with Banerjee et al. (20), and Eyre et al. (21), studies. Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents, with head and neck being the favored site (22), this was also seen in our study. Lymphomas comprised of 12.8% of malignant tumors in our study. This accords with study by Singh et al. (23), where 12% occurrence was reported. NHL was seen more commonly in our study than Hodgkin lymphoma, Mondal et al. (24), also reported a similar trend. Brain tumors contributed 11.9% of all the malignancies, quite close to 12.1%, as reported by Fathi et al. (25), while some studies report a higher percentage of 20% (4,26). This variation may be attributed to geographical and ethnic variation. Astrocytoma was the most common histological subtype in our study. The analysis by Sarkar et al. (27), of 3936 pediatric brain tumors in <18 years of age, astrocytomas were the most frequent type. Retinoblastoma is the most common intraocular malignancy in children (28), which was also observed in our study. Germ cell tumors were mostly gonadal with a female preponderance as quoted by other studies (29,30). Wilms tumor was the only renal tumor seen, all of them being triphasic type. Pediatric renal tumors commonly seen are Wilms tumor, mesoblastic nephroma, clear cell sarcoma and malignant rhabdoid tumor (31).