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Multilocular cystic nephroma in an adult: a diagnostic quandary

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Abstract

Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological features that causes diagnostic dilemma. MLCN lies in the spectrum of mixed epithelial and stromal tumor (MEST) family of kidney. According to World Health Organization (WHO 2016 classification), MEST encompasses spectrum of tumors ranging from predominantly cystic tumors, adult cystic nephroma (ACN) to tumors that are variably solid (MEST), thus creating diagnostic dilemma. Moreover, it has several benign and malignant differentials due to its several overlapping histomorphological features which when not cautiously dealt with may result in misdiagnosing it as malignant lesion. We hereby present a case of a woman in late twenties who presented with left flank swelling and pain since 6 months which was misdiagnosed as renal cell carcinoma on radiology which turned out to be ACN on histology and further verified on immunohistochemistry.

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All the data regarding the findings are available within the manuscript.

References

  1. Edmunds W. Cystic adenoma of kidney. Trans Pathol Soc Lond. 1892;43:89–90.

    Google Scholar 

  2. Jevremovic D, Lager DJ, Lewin M. Cystic nephroma (multilocular cyst) and mixed epithelial stromal tumor of the kidney: a spectrum or the same entity? Ann Diagn Pathol. 2006;10:77–82.

    Article  PubMed  Google Scholar 

  3. Moch H, Cubilla AL, Humphrey PA, Reuter VE, Ulbright TM. The 2016 WHO classification of tumours of the urinary system and male genital organs—part a: renal, penile, and testicular tumours. Eur Urol. 2016;70:93–105.

    Article  PubMed  Google Scholar 

  4. Michal M, Syrucek M. Benign mixed epithelial and stromal tumor of the kidney. Pathol Res Pract. 1998;194:445–8.

    Article  CAS  PubMed  Google Scholar 

  5. Wang C, Lin Y, Xiang H, Fang D, Jiang P, Shen B. Mixed epithelial and stromal tumour of the kidney: report of eight cases and literature review. World J Surg Oncol. 2013;11:207.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Dell’Atti L. An unusual presentation of cystic nephroma in an adult man. Rare Tumors. 2015;7:5860.

    PubMed  PubMed Central  Google Scholar 

  7. Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, et al. The International Society of Urological Pathology (ISUP) Vancouver classification of renal neoplasia. Am J Surg Pathol. 2013;37:1469–89.

    Article  PubMed  Google Scholar 

  8. Sacher P, Willi UV, Niggli F, Stallmach T. Cystic nephroma: a rare benign renal tumor. Pediatr Surg Int. 1998;13:197–9.

    Article  CAS  PubMed  Google Scholar 

  9. Wilkinson C, Palit V, Bardapure M, Thomas J, Browning AJ, Gill K, Biyani CS. Adult multilocular cystic nephroma: report of six cases with clinical, radio-pathologic correlation and review of literature. Urol Ann. 2013;5(1):13–7. https://doi.org/10.4103/0974-7796.106958.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol. 1951;23(2):142–52. https://doi.org/10.1111/j.1464-410x.1951.tb02576.x.

    Article  CAS  PubMed  Google Scholar 

  11. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer. 1989;64:466–79.

    Article  CAS  PubMed  Google Scholar 

  12. Foteini Karasavvidou and others. Mixed epithelial and stromal tumor—adult cystic nephroma of the kidney: a case report with immunohistochemical analysis. J Surg Case Reports. 2022;2022(9):387.

    Google Scholar 

  13. Sodhi KS, Suri S, Samujh R, Rao KL, Vaiphei K, Saxena AK. Case report: bilateral multilocular cystic nephromas: a rare occurrence. Br J Radiol. 2005;78:450–2.

    Article  CAS  PubMed  Google Scholar 

  14. Granja MF, O’Brien AT, Trujillo S, Mancera J, Aguirre DA. Multilocular cystic nephroma: a systematic literature review of the radiologic and clinical findings. AJR Am J Roentgenol. 2015;205(6):1188–93. https://doi.org/10.2214/AJR.15.14548.

    Article  PubMed  Google Scholar 

  15. Cavıldak İK, Çakıcı MÇ, Karakoyunlu N, Ersoy H. Cystic nephroma: a case report in adult patients. Turk J Urol. 2018;44(4):373–6. https://doi.org/10.5152/tud.2017.56957.

    Article  PubMed  Google Scholar 

  16. Sharma A, Andankar M, Pathak H. A rare presentation of cystic nephroma in a young adult. Asian J Urol. 2017;4(2):128–30.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Sawant AS, Savalia AJ, Pawar P, Narwade S, Chaudhari R. A case report of largest documented multilocular cystic nephroma removed by thoracoabdominal approach. J Clin Diagn Res. 2017;11(7):10–2. https://doi.org/10.7860/JCDR/2017/25612.10244. (Epub 2017 Jul 1).

    Article  Google Scholar 

  18. Dell’ Atti L. An unusual presentation of cystic nephroma in an adult man. Rare Tumors. 2015;18:7(2):5860. https://doi.org/10.4081/rt.2015.5860.

    Article  Google Scholar 

  19. Dong B, Wang Y, Zhang J, Fu Y, Wang G. Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: a case report. Can Urol Assoc J. 2014;8(7–8):E545–7. https://doi.org/10.5489/cuaj.2020.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Ozturk H, Karaaslan S. Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant. Int J Clin Exp Pathol. 2014;15:7(8):5271–9.

    Google Scholar 

  21. Qiu M, Lu J, Ma LL, Yan Y, Zhang SD. Retroperitoneal laparoscopic partial nephrectomy for treatment of cystic nephroma: one case report. Beijing Da Xue Xue Bao Yi Xue Ban. 2014;46(4):650–2 (Chinese).

    PubMed  Google Scholar 

  22. Abrol N, Gupta N, Arava S, Ray R. Cystic nephroma masquerading as hydatid cyst of the kidney. Indian J Pathol Microbiol. 2010;53:877–9.

    Article  PubMed  Google Scholar 

  23. Mohanty D, Jain BK, Agrawal V, Gupta A. Cystic nephroma: a diagnostic dilemma. Saudi J Kidney Dis Transpl. 2010;21(3):518–20.

    PubMed  Google Scholar 

  24. Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, Schultz KA, Cathro HP, et al. DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. Mod Pathol. 2014;27:1267–80. https://doi.org/10.1038/modpathol.2013.242.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Antic T, Perry KT, Harrison K, Zaytsev P, Pins M, Campbell SC, et al. Mixed epithelial and stromal tumors of the kidney and cystic nephroma share overlapping features: reappraisal of 15 lesions. Arch Pathol Lab Med. 2006;130:80–5.

    Article  PubMed  Google Scholar 

  26. Castillo OA, Boyle ET Jr, Kramer SA. Multilocular cysts of kidney. A study of 29 patients and review of literature. Urology. 1991;37:156–62.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pathology, SGPGIMS, C-Block, Lucknow, UP, India

    Ritu Verma, Jyoti Verma & Neelima Gupta

  2. Department of Pathology, AIIMS, Mangalagiri, AP, India

    Jyoti Verma

Authors
  1. Ritu Verma
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  2. Jyoti Verma
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  3. Neelima Gupta
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Contributions

RV carried out concepts and design, literature search. JV carried out data acquisition, data analysis, manuscript preparation concepts and designing, literature search and contributed to manuscript preparation and clinical study and will stand as guarantor also. NG helped in data acquisition, data analysis, and clinical study. All the authors have read and approved the final manuscript.

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Correspondence to Jyoti Verma.

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The authors declare that they have no conflicting interests.

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This case report was conducted by the fundamental principles of the Declaration of Helsinki.

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Consent for the publication and use of images and any related information was taken from the patient involved in the study.

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Verma, R., Verma, J. & Gupta, N. Multilocular cystic nephroma in an adult: a diagnostic quandary. CEN Case Rep (2024). https://doi.org/10.1007/s13730-024-00852-8

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  • DOI: https://doi.org/10.1007/s13730-024-00852-8

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