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Mol Clin Oncol 2021 Jun 23;14(6):109. Epub 2021 Mar 23.

Department of Urology, Lourdes Clinic, I-80040 Massa di Somma, Italy.

Multilocular cystic nephroma is a rare benign kidney tumor, which is typically characterized by a unilateral, multicystic renal mass without solid elements. Cystic nephroma has a bimodal distribution and two-thirds of tumors involve children aged between 3 months and 2 years, with male predominance; a second peak affects the age group >30 years old, in which females are predominantly affected. The incidence rate for this rare tumor in patients aged 5-30 years is only 5%. Read More

CEN Case Rep 2021 May 3;10(2):230-235. Epub 2020 Nov 3.

Nephrology Center and Department of Rheumatology, Toranomon Hospital Kajigaya, 1C-1, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.

A 60-year-old Japanese woman was admitted because of the polycystic mass with right flank pain localized in the upper portion of the right kidney. Right nephrectomy was performed because the mass lesion had continuously increased in size over the past 10 years. A surgical specimen showed histology consistent with a mixed epithelial and stromal tumor, which is closely related to multilocular cystic nephroma, and was diagnosed by a defined capsule between the cystic mass lesion and normal renal tissue by CT and MRI, and histology. Read More

Turk J Med Sci 2020 02 13;50(1):18-24. Epub 2020 Feb 13.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey

Background/aim: Non-Wilms renal tumors (NWRTs) are rarely encountered in children. The aim of this study is to determine the treatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey.

Materials And Methods: Medical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995–2018) were reviewed retrospectively. Read More

Magn Reson Imaging Clin N Am 2019 May;27(2):279-290

Department of Radiology, Seattle Children's, University of Washington, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA.

Pediatric renal tumors may be malignant or benign. Wilms tumor, the most common malignant pediatric renal tumor, arises sporadically or with various syndromes. Renal cell carcinoma typically presents in older children. Read More

Diagn Pathol 2018 Oct 27;13(1):84. Epub 2018 Oct 27.

Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Ishikawa, 920-0293, Japan.

Background: Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes.

Case Presentation: A six-month-old girl with persistent high fevers was found to have pyuria and bacteriuria. Read More

Clin Radiol 2019 02 9;74(2):83-94. Epub 2018 Oct 9.

Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, 501 Smyth Road, Ottawa, ON K1H 8L6, Canada. Electronic address:

This article presents an updated review of cystic renal mass imaging. Most cystic renal masses encountered incidentally are benign and can be diagnosed confidently on imaging and require no follow-up. Hyperattenuating masses discovered at unenhanced or single-phase enhanced computed tomography (CT) measuring between 20-70 HU are indeterminate and can be further investigated first by using ultrasound and, then with multi-phase CT or magnetic resonance imaging (MRI); as the majority represent haemorrhagic/proteinaceous cysts (HPCs). Read More

J Chin Med Assoc 2018 Dec 13;81(12):1017-1026. Epub 2018 Jul 13.

Department of Radiology, Taipei Veterans General Hospital and National Yang-Ming University, School of Medicine, Taipei, Taiwan, ROC; Yee Zen Hospital, Taoyuan, Taiwan, ROC; Yuanpei University of Medical Technology, Hsinchu, Taiwan, ROC. Electronic address:

Renal cysts are common incidental findings in clinical practice. Most renal cysts detected in medical imaging are benign simple cysts. However, some are complicated by hemorrhage or infection or are associated with calcification. Read More

Abdom Radiol (NY) 2018 07;43(7):1540-1545

Department of Diagnostic Radiology, Institute of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 7340037, Japan.

Tubulocystic renal cell carcinoma (TC-RCC) has been classified as an independent subtype according to the 2016 World Health Organization (WHO) classification. It is a rare subtype that predominantly affects men. Although few in number, radiological imaging reports have suggested that TC-RCC is characterized by multilocular cystic lesions, which are categorized as the Bosniak classification II-IV, with signature pathological characteristics comprising numerous small cysts or a tubular structure. Read More

Abdom Radiol (NY) 2018 11;43(11):3082-3100

Department of Radiology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.

This article presents methods to improve MR imaging approach of disorders of the renal sinus which are relatively uncommon and can be technically challenging. Multi-planar Single-shot T2-weighted (T2W) Fast Spin-Echo sequences are recommended to optimally assess anatomic relations of disease. Multi-planar 3D-T1W Gradient Recalled Echo imaging before and after Gadolinium administration depicts the presence and type of enhancement and relation to arterial, venous, and collecting system structures. Read More

J Clin Diagn Res 2017 Jul 1;11(7):PD10-PD12. Epub 2017 Jul 1.

Lecturer, Department of Urology, LTMGH and LTMMC, Sion, Mumbai, Maharashtra, India.

Large renal tumours are not uncommon in developing countries. Enhancing renal tumours are considered to be malignant unless proved otherwise and radical surgical resection remains the mainstay of treatment of such tumours. A giant renal tumour, especially on right side, poses a big challenge for the operating team and requires a thoracoabdominal approach for successful excision. Read More

Case Rep Urol 2016 15;2016:5304324. Epub 2016 Dec 15.

Department of Urology, Centro Médico Nacional 20 de Noviembre, Mexico City, Mexico.

The incidence of Multilocular cystic renal cell carcinoma (MCRCC) in literature is very low and confounding MCRCC with cystic nephroma (CN) is even more unusual. The aim of this report is to present a case of MCRCC and emphasize the importance of the preoperative radiologic evaluation and immunohistochemical staining confirmation to obtain an accurate diagnosis. A 73-year-old woman presented with a history of 4-month right flank pain. Read More

Urol Ann 2016 Oct-Dec;8(4):506-508

Department of Pathology, Hassan Institute of Medical Sciences, Hassan, Karnataka, India.

Multilocular cystic renal cell carcinoma (MCRCC) has been identified as a separate subtype of renal cell carcinoma (RCC) in the 2004 World Health Organization classification of adult renal tumors. MCRCC represents a rare variant of clear cell RCC. The common age group for this tumor is between 40 and 60 years. Read More

Arch Esp Urol 2016 Dec;69(10):711-715

Servicio de Anatomía Patológica. Hospital Universitario Vírgen Macarena. Sevilla. España.

Objective: Multicystic nephroma (multilocular cystic nephroma, multilocular cyst) is a relatively rare benign neoplasm of the kidney. Most patients are asymptomatic and tumours are usually discovered incidentally.

Methods: Between 2010 and 2015, 2 patients with cystic nephroma at our institution were diagnosed and treated. Read More

Urol Oncol 2017 01 23;35(1):35.e7-35.e14. Epub 2016 Sep 23.

Department of Urology, State Key Laboratory of Kidney Diseases, Chinese PLA General Hospital, PLA Medical School, Beijing, China. Electronic address:

Purpose: To evaluate the clinical usefulness of neutrophil-lymphocyte ratio (NLR) in differentiating the ultrasound-indeterminate renal multilocular cystic masses (RMCM) in comparison with computed tomography (CT) and whether NLR has additional benefits to CT on sensitivity of detecting the malignant.

Materials And Methods: Overall, 93 patients who underwent normal ultrasound with a conclusion of indeterminate RMCM were examined by NLR and CT within 30 days before surgery or follow-up from March to September 2014 at PLA General Hospital and enrolled in this retrospective study. Logistic regression model was performed to find independent predictors for differentiating true nature of RMCM; differences in the validity parameters and diagnostic power of CT, NLR, and their combination were compared using McNemar tests and AUC model, respectively. Read More

J Indian Assoc Pediatr Surg 2016 Apr-Jun;21(2):61-5

Department of Paediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India.

Aims: Scrutiny over the clinical behaviors, management, and the final outcome of some rare renal neoplasm in order to find out some hidden facts about these tumors which are playing an important role in the disease course and its management.

Materials And Methods: Retrospective evaluation of uncommon (non-Wilms') renal neoplasm in the pediatric population in a tertiary care center. Fifteen cases of uncommon renal tumors were treated in our institution over the last 5 years (January 2008 to December 2012). Read More

Curr Probl Diagn Radiol 2018 Nov 28;47(6):417-427. Epub 2016 Jan 28.

Department of Radiology, Allegheny General Hospital, Allegheny Health Network, Pittsburgh, PA.

Diseases of the kidney often have their names shortened, creating an arcane set of acronyms which can be confusing to both radiologists and clinicians. This review of renal pathology aims to explain some of the most commonly used acronyms within the field. For each entity, a summary of the clinical features, pathophysiology, and radiological findings is included to aid in the understanding and differentiation of these entities. Read More

Zhonghua Zhong Liu Za Zhi 2015 Nov;37(11):845-9

Email:

Objective: To study the CT findings of cystic nephroma (CN) and multilocular cystic renal cell carcinoma (MCRCC) and to improve the accuracy of preoperative diagnosis of these two diseases.

Methods: The CT findings of nine CN cases and 19 MCRCC cases confirmed by pathology were blindly reviewed and compared with their pathological results. Fisher's exact test and independent-samples T test were applied to statistically analyze some of the CT features of the CN and MCRCC lesions. Read More

Pediatr Dev Pathol 2015 Nov-Dec;18(6):504-11. Epub 2015 Dec 23.

1 International Pleuropulmonary Blastoma Registry, Children's Hospital and Clinics of Minnesota, Minneapolis, MN 55404, USA.

Pleuropulmonary blastoma (PPB) is the most common primary malignant neoplasm of the lung in children. Like other solid dysontogenic neoplasms, this tumor typically presents before 7 years of age. The earliest manifestation is the presence of a lung cyst(s), which is usually recognized in the first year of life and is difficult to differentiate on the basis of imaging studies from non-neoplastic cysts of early childhood. Read More

AJR Am J Roentgenol 2015 Dec;205(6):1188-93

1 All authors: Department of Radiology and Medical Imaging, Fundación Santa Fe de Bogotá University Hospital, Universidad de Los Andes, Cra. 7b #123-90, Bogotá 220246, Colombia.

Objective: The objective of our study was to systematically summarize the published evidence of demographic, clinical, diagnostic imaging, and therapeutic characteristics of patients with multilocular cystic nephroma (MLCN).

Conclusion: Cross-sectional imaging evaluation is important for suggesting the diagnosis of MLCN but has several limitations. The number of radical nephrectomies reported for MLCN encourages discussion concerning the utility of percutaneous presurgical biopsy and frozen-section intraoperative biopsy as a more conservative diagnostic approach. Read More

J Pediatr Urol 2015 Jun 31;11(3):149.e1-6. Epub 2015 Mar 31.

Department of Urology, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Drive, Room 4230, Indianapolis, IN 46202, USA. Electronic address:

Introduction: Solitary renal cysts are typically incidentally found in children who have undergone renal ultrasound (US). The main concern is a cystic tumor. There is no US-based grading system for children to guide management. Read More

Zhonghua Bing Li Xue Za Zhi 2014 Nov;43(11):723-7

Department of Pathology, Affiliated Hospital of Qingdao University, Qingdao 266003, China. E-mail:

Objective: To investigate the clinicopathological characteristics and the diagnosis of multilocular cystic renal cell carcinoma (MCRCC).

Methods: The clinicopathological data of 19 MCRCC cases were collected and immunohistochemical staining assays were carried out. Forty-six cases of other cystic kidney lesions within the same period were collected as controls, including extensively cystic clear cell RCC (12 cases), clear cell tubulopapillary renal cell carcinoma (6 cases), tubulocystic carcinoma (2 cases), simple cortical cysts (22 cases), multilocular cystic nephroma (1 cases) and multicystic kidney (3 cases). Read More

Cesk Patol 2014 ;50(4):137-41

Kidney tumours form a broad spectrum of distinguished histopathological and molecular genetic entities. The last WHO classification is dated to 2004. Current classification has been published in October 2013 by ISUP (International Society of Urological Pathology). Read More

Am J Clin Exp Urol 2014 2;2(3):266-72. Epub 2014 Oct 2.

Department of Pathology, New York University Langone Medical Center New York, NY, USA.

Background: Tumors of the kidney are uncommon in children and young adults. Accurate classification is crucial for both prognostication and therapeutic intervention. However, majority of the tumors in this age group have unusual morphology that renders classification challenging. Read More

Can Urol Assoc J 2014 Jul;8(7-8):E545-7

Department of Urology, First Hospital of Jilin University, Jilin, China;

Multilocular cystic nephroma is a relatively rare benign tumour of the kidney, which usually presents as a unilateral multicystic renal mass without solid elements. The lesions typically have a bimodal age, with peak incidence in male children under 24 months and another one in women over 40 old. We present an unusual case report of multilocular cystic nephroma in the right kidney in a 30-year-old male. Read More

Abdom Imaging 2015 Mar;40(3):542-51

Radiology Department, Hospital Clinic, Villarroel 170, 08036, Barcelona, Spain,

Objective: The aim of this study was to assess the accuracy of Ultrasound (US) and contrast-enhanced ultrasound (CEUS) in the characterization of renal nodules indeterminate on CT by identifying benign cystic lesions not requiring further examination.

Methods: 72 patients with 83 indeterminate renal nodules on CT underwent baseline US and CEUS that classified lesions as benign (Bosniak I, II or IIF cysts) or potentially malignant (Bosniak III or IV cysts, solid nodules). The accuracy of US and CEUS in the differentiation between benign cysts and potentially malignant nodules was analyzed and compared with the final diagnosis obtained by histology or follow-up of at least 23 months with CEUS ± a conclusive CT/MR study. Read More

Int J Clin Exp Pathol 2014 15;7(8):5271-9. Epub 2014 Jul 15.

Department of Pathology, School of Medicine, Sifa University Izmir, Turkey.

Background: The multilocular cystic nephroma (MLCN) is a unilateral cystic neoplasm of the kidney exhibiting benign biological behavior. The etiology and histopathogenesis of the disease is controversial (dysplastic/hamartomous/neoplastic). MLCNs show bimodal age distribution, with peak incidence occurring at 2-4 years of age and between the fourth and sixth decades. Read More

Arch Esp Urol 2014 May;67(4):337-41

Department of Urology. Servicio Anatomia Patologica. Complejo Hospitalario Universitario de Albacete.Albacete.Spain.

Objective: To describe the clinical presentation, preoperative diagnostic possibilities, and treatment of cystic nephroma.

Methods: We describe a case of cystic nephroma in an adult male and show that, both in our patient and in the literature, a definitive diagnosis can only be obtained postoperatively, even when there is a reasonable clinical suspicion.

Conclusions: Cystic nephroma is rare, and some authors consider it a questionable entity. Read More

J Med Case Rep 2014 Apr 3;8:112. Epub 2014 Apr 3.

Center for Genomic Medicine, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Introduction: Germ-line mutations in the micro-ribonucleic acid processing gene DICER1 have been shown to predispose to a subset of benign tumors susceptible to malignant transformation, including ovarian Sertoli-Leydig cell tumor, nontoxic multinodular goiter, multilocular cystic nephroma and pleuropulmonary blastoma, which can occur in children and young adults. This may be due to reduced Dcr-1 homolog expression in carriers of germline mutations, which causes impairment of micro-ribonucleic acid processing and deregulates the growth and differentiation of target cells, leading to an increased risk of tumorigenesis. Many carriers of germ-line DICER1 mutations remain unaffected, but development of tumors within carriers is associated with varying prognoses. Read More