Antenatally diagnosed kidney tumor: Answers
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CLINICAL QUIZ
Antenatally diagnosed kidney tumor: Answers Wiebke Solass 1 & Hyunkyu Shin 1 & Cristian Urla 2 & Andreas Schmidt 2 Received: 4 October 2020 / Accepted: 4 November 2020 # The Author(s) 2020
Keywords Renal tumor . Prenatal diagnosis . Pediatric neoplasm . Nephrectomy . Congenital mesoblastic nephroma
Answers The tumor developed during pregnancy in the upper pole of the left kidney and displayed spindle cell components with no atypia and islands of metaplastic cartilage, which is typical for a congenital mesoblastic nephroma (CMN), classic variant. Wilms tumor (WT)/nephroblastoma is the most frequent kidney tumor in childhood, and this diagnosis has to be considered [1]. Additional immunohistochemical staining is helpful to distinguish CMN from WT with heterologous differentiation [1, 2].
Discussion Congenital mesoblastic nephroma represents 3% of all pediatric kidney tumors [3]. It is the most common kidney neoplasm diagnosed in the first 3 months of life, and it is frequently detected antenatally, as described in our case [4]. The malignant potential of the tumor is low.
Histopathology The histological classification of CMN includes three subtypes: classic, cellular, and mixed type [5]. Classic CMN is composed of braiding bundles of spindle cells and frequent This refers to the article that can be found at https://doi.org/10.1007/ s00467-020-04848-1. * Andreas Schmidt [email protected] 1
Institute of Pathology and Neuropathology, University Hospital Tuebingen, Eberhard-Karls University Tuebingen, Liebermeisterstraße 8, Tuebingen 72076, Germany
2
Department of Pediatric Surgery and Pediatric Urology, University Children’s Hospital Tuebingen, Eberhard-Karls University Tuebingen, Hoppe-Seyler-Str. 3, 72076 Tuebingen, Germany
metaplastic cartilage with no capsular boundaries. The tumor often infiltrates the surrounding perirenal fat tissue and parenchyma [6]. Cellular CMN also presents bundles of spindle cells but has a stronger hemangiopericytic pattern and a higher mitotic activity than the classic type. In contrast, the cellular type less frequently infiltrates the perirenal fat and/or kidney parenchyma. Mixed CMN shows, as the name indicates, a mixture of both abovementioned types.
Molecular aspects There are recurrent genetic aberrations described in CMN, including somatic trisomy 11 and, occurring in the cellular and mixed type, the translocation t(12;15)(p13;q25), which results in fusion of ETV6 and NTRK3 [7, 8]. Associations with genetic syndromes are only described in rare cases [8, 9].
Therapy Complete nephrectomy is curative for most patients with stage I/ II disease. The removal of perirenal fat during surgery is important, as CMN tumors often show infiltrative growth into the perirenal fat. However, in case of a high risk of operative or anesthetic complications, preoperative chemotherapy may be considered. Stage III tumors of the classic and mixed histologic subtype are also indicated for nephrectomy alone. Stage III tumors of the cellular type treated only surgi
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