Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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On Sep 2018




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Prof. Somashekhar Nimbalkar
Head, Department of Pediatrics, Pramukhswami Medical College, Karamsad
Chairman, Research Group, Charutar Arogya Mandal, Karamsad
National Joint Coordinator - Advanced IAP NNF NRP Program
Ex-Member, Governing Body, National Neonatology Forum, New Delhi
Ex-President - National Neonatology Forum Gujarat State Chapter
Department of Pediatrics, Pramukhswami Medical College, Karamsad, Anand, Gujarat.
On Sep 2018




Dr. Kalyani R

"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."



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Sri Devaraj Urs Medical College
Sri Devaraj Urs Academy of Higher Education and Research , Kolar, Karnataka
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Dr. Saumya Navit

"As a peer-reviewed journal, the Journal of Clinical and Diagnostic Research provides an opportunity to researchers, scientists and budding professionals to explore the developments in the field of medicine and dentistry and their varied specialities, thus extending our view on biological diversities of living species in relation to medicine.
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Dr Saumya Navit
Professor and Head
Department of Pediatric Dentistry
Saraswati Dental College
Lucknow
On Sep 2018




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Their prompt and timely response to review's query and the manner in which they have set the reviewing process helps in extracting the best possible scientific writings for publication.
It's a honour and pride to be a part of the JCDR team. My very best wishes to JCDR and hope it will sparkle up above the sky as a high indexed journal in near future."



Dr. Arunava Biswas
MD, DM (Clinical Pharmacology)
Assistant Professor
Department of Pharmacology
Calcutta National Medical College & Hospital , Kolkata




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Best regards,
C.S. Ramesh Babu,
Associate Professor of Anatomy,
Muzaffarnagar Medical College,
Muzaffarnagar.
On Aug 2018




Dr. Arundhathi. S
"Journal of Clinical and Diagnostic Research (JCDR) is a reputed peer reviewed journal and is constantly involved in publishing high quality research articles related to medicine. Its been a great pleasure to be associated with this esteemed journal as a reviewer and as an author for a couple of years. The editorial board consists of many dedicated and reputed experts as its members and they are doing an appreciable work in guiding budding researchers. JCDR is doing a commendable job in scientific research by promoting excellent quality research & review articles and case reports & series. The reviewers provide appropriate suggestions that improve the quality of articles. I strongly recommend my fraternity to encourage JCDR by contributing their valuable research work in this widely accepted, user friendly journal. I hope my collaboration with JCDR will continue for a long time".



Dr. Arundhathi. S
MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
On Aug 2018




Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
The journal has a monthly publication and the articles are published quite fast. In time compared to other journals. The on-line first publication is also a great advantage and facility to review one's own articles before going to print. The response to any query and permission if required, is quite fast; this is quite commendable. I have a very good experience about seeking quick permission for quoting a photograph (Fig.) from a JCDR article for my chapter authored in an E book. I never thought it would be so easy. No hassles.
Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".



Dr. Mamta Gupta
Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.


Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
Timely publication of journal: Publication of manuscripts and bringing out the issue in time is one of the positive aspects of JCDR and is possible with strong support team in terms of peer reviewers, proof reading, language check, computer operators, etc. This is one of the great reasons for authors to submit their work with JCDR. Another best part of JCDR is "Online first Publications" facilities available for the authors. This facility not only provides the prompt publications of the manuscripts but at the same time also early availability of the manuscripts for the readers.
Indexation and online availability: Indexation transforms the journal in some sense from its local ownership to the worldwide professional community and to the public.JCDR is indexed with Embase & EMbiology, Google Scholar, Index Copernicus, Chemical Abstracts Service, Journal seek Database, Indian Science Abstracts, to name few of them. Manuscriptspublished in JCDR are available on major search engines ie; google, yahoo, msn.
In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
It is well said that "happy beginning is half done" and it fits perfectly with JCDR. It has grown considerably and I feel it has already grown up from its infancy to adolescence, achieving the status of standard online e-journal form Indian continent since its inception in Feb 2007. This had been made possible due to the efforts and the hard work put in it. The way the JCDR is improving with every new volume, with good quality original manuscripts, makes it a quality journal for readers. I must thank and congratulate Dr Hemant Jain, Editor-in-Chief JCDR and his team for their sincere efforts, dedication, and determination for making JCDR a fast growing journal.
Every one of us: authors, reviewers, editors, and publisher are responsible for enhancing the stature of the journal. I wish for a great success for JCDR."



Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011




Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."



Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011
Anuradha

Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.


Dr. Anuradha
E-mail: anuradha2nittur@gmail.com
On Jan 2020

Important Notice

Case Series
Year : 2022 | Month : May | Volume : 16 | Issue : 5 | Page : ER01 - ER05 Full Version

A Quaint Collation of Childhood Renal Neoplasms- Wilms and Beyond: Perspective of a Tertiary Care Hospital of Eastern India


Published: May 1, 2022 | DOI: https://doi.org/10.7860/JCDR/2022/55504.16310
Meghadipa Mandal, Sanghamitra Mukherjee, Tushar Kanti Das

1. Senior Resident, Department of Pathology, R. G. Kar Medical College & Hospital, Kolkata, West Bengal, India. 2. Assistant Professor, Department of Pathology, R. G. Kar Medical College & Hospital, Kolkata, West Bengal, India. 2. Professor and Head of the Department, Department of Pathology, R. G. Kar Medical College & Hospital, Kolkata, West Bengal, India.

Correspondence Address :
Dr. Meghadipa Mandal,
AC1, AA1, Far Sight Coop HSG SOC, New Town, Rajarhat, North 24 PRGNS, Kolkata-700156, West Bengal, India.
E-mail: meghadipa.mandal41@gmail.com

Abstract

Paediatric renal neoplasms are rarely encountered entities. Histopathology is essential in most of the cases, where there is significant degree of clinical and radiological overlap. Present series has 32 cases which aimed to evaluate clinical and pathologic spectrum of renal tumours in children. Nephrectomy specimens of renal neoplasms of children below 12 years of age were included. Haematoxylin and Eosin (H&E) staining and Immunohistochemistry (IHC) were done. Findings were tabulated in a master sheet along with other demographic variables, clinical histories and imaging findings. Abdominal lump was the commonest presentation. Wilms tumour was the major histologic variant (66%), followed by Congenital Mesoblastic Nephroma (13%). Anaplasia and advanced Children's Oncology Group (COG) staging were the adverse prognostic indicators. Clear Cell Sarcoma and Rhabdoid tumours were seen to have adverse outcomes, whereas Congenital Mesoblastic Nephroma, Multicystic Dysplastic Kidney and Paediatric Cystic Nephromas had overall favorable prognosis. Histopathology plays a key role for confirmatory opinion on nature of the neoplasm. Better understanding of these cases will increase the diagnostic accuracy with early implementation of definitive therapy.

Keywords

Clear cell sarcoma, Congenital mesoblastic nephroma, Cystic nephroma, Multicystic dysplasia, Rhabdoid tumours

Pediatric renal neoplasms are rare and often detected incidentally during routine clinical examination (1). They lack specific symptomatology and hence, pose a diagnostic challenge not only to the clinician but also to the reporting pathologists. Seven percent (7%) of all malignant childhood tumours are of renal origin. This entity is a heterogenous group with wide histopathologic spectrum from non-neoplastic, to benign and malignant lesions, each with widely different therapeutic approach (2). A vast majority of 90% of pediatric renal tumours belong to the category of Wilms Tumour, the other rare variants being Clear Cell Sarcoma of the Kidney (CCSK), Malignant Rhabdoid Tumour of the Kidney (MRTK), Congenital Mesoblastic Nephroma (CMN) and others (3). Histopathology is the key to diagnose these neoplasms with very distinct morphologic patterns. However, some of these neoplasms have a considerable degree of histopathological overlap, where ancillary studies like IHC plays an important role to unveil the exact nature of the tumour.

The present article is a case series of 32 cases done in a tertiary care centre in Eastern India, where all cases of paediatric renal neoplasms were studied along with suitable ancillary investigations, where applicable, to arrive at definitive diagnosis and guide the mode of treatment. The study was conducted for a total duration of seven years from March 2014-March 2021. Children below 12 years of age underwent Nephrectomy after obtaining informed consent from the patient relatives. The specimen were grossed, followed by H&E staining. Ancillary studies like IHCs were done for suitable cases and detailed history along with imaging reports were considered before delivering the final diagnosis.

Case Report

The distribution of cases as per their histologic types is shown in (Table/Fig 1).

Wilms Tumour (WT): A total of 21 (66%) cases were Wilms tumour, with equal sex distribution (male- 11 cases; 52% and female- 10 cases; 48%); mean age of presentation being 24 months (range- 13-44 months). Common presenting features were abdominal lump and pain; haematuria in 4 cases (19%) and hypertension in 5 cases (24%) were seen (Table/Fig 2).

The institute followed the treatment guidelines as per National Wilms Tumour Study Group (NWTSG)/Children’s Oncology Group (COG), where primary resection followed by chemotherapy was the main modality of treatment (4). All specimens received were that of Radical Nephrectomy; common gross findings being unilateral and unifocal tumour masses with solid, multinodular, tan grey appearance. The range of tumour dimension was 6.5-17 cm. All 20 cases were triphasic Wilms tumour, amongst which, two rare variants were seen- one being diffuse blastemal pattern (Table/Fig 3), which grossly was the largest tumour and second being Teratoid Wilms tumour (Table/Fig 4), which was multifocal with areas of cystic degeneration. A single case of monophasic Wilms Tumour with only blastemal component was seen and confirmed by diffuse Wilms Tumour (WT1) nuclear positivity (Table/Fig 5). As per COG staging, 15 cases (71%) were Stage I, 4 (19%) were Stage II and rest 2 (10%) comprising monophasic Wilms tumour and diffuse blastemal pattern were Stage IV. Amongst Stage II, two cases showed focal anaplasia, whereas, one Stage IV case of monophasic Wilms tumour had diffuse anaplasia. On follow-up, both Stage II cases displaying focal anaplastic features and two Stage IV cases died within two years of follow-up. Rest of the cases (17 cases) had been put on chemotherapy and were doing well for 5 years post-surgery till the presentation of this study.

Congenital Mesoblastic Nephroma (CMN): A total of 4 cases (13%) were that of CMN, comprising two males and two females. Three cases were 1 month old and one case aged 6 months. All of them presented with abdominal masses. There were no feeding complaints or other urinary symptoms. On detailed evaluation, one mother gave history of polyhydramnios, other obstetric histories being uneventful. Ultrasonography showed well-defined mass in the unilateral kidneys. There were concentric echogenic and hypoechoic rings in three cases; whereas one case showed cystic changes within the tumour. Nephrectomy in all the cases showed well-defined masses on medial side of the kidneys with whitish, whorled appearance. They were predominantly firm, whereas friable with areas of cystic degeneration (Table/Fig 6) and haemorrhage in one case, which corroborated with the imaging findings of these cases. The gross mean dimension of the tumour was 5 cm. On histopathologic examination, three cases were that of classical CMN and one case with friable consistency was cellular CMN. The classic variant showed dysplastic cartilage in the adjacent renal parenchyma (Table/Fig 7); whereas the cellular variant had brisk mitosis (Table/Fig 8). All the resection margins, including the medial margin were free from tumour and were Stage I.

Follow-up of all the 3 cases of classic CMN showed good prognosis, with no recurrence within three years of follow-up, till the preparation of this manuscript. The cellular CMN presented with metastasis to retroperitoneal lymph nodes within two years of surgery and was put under chemotherapeutic regimen (Table/Fig 9).

Clear Cell Sarcoma of Kidney (CCSK): A total of 2 cases (6%) were of Clear Cell Sarcoma of unilateral Kidney, both seen in male child. Their ages were 12 months and 24 months respectively. They presented with abdominal masses. Computed Tomography (CT) images showed large heterogeneously enhancing masses measuring 11.5 and 14 cm respectively, centered around renal medulla. They underwent nephrectomy with suspicion for Wilms tumour in a peripheral centre followed by H&E and IHC examinations. The cases were reviewed and re-evaluated in our department with all ancillary studies for confirmatory opinion before initiation of chemotherapy.

Grossly, the mass was unifocal, well-circumscribed but unencapsulated, pale-creamy with mucoid, cystic and focal necrotic areas as per the documentation in the histopathology report. Sections showed uniform cells separated by delicate capillary network of vessels, clear to granular cytoplasm, finely dispersed chromatin and inconspicuous nucleoli. Myxoid stroma with entrapped tubules and glomeruli were noted at the infiltrating border of the tumour. There were no tubular or blastemal components in any of the sections. Thus, a diagnosis of classic CCSK was made (Table/Fig 10). This was further substantiated by Cyclin D1 positivity in tumour cells (Table/Fig 11) as per the IHC slides that were reviewed. One case was Stage I and another was Stage II with infiltration of renal capsule and peri-sinus fat.

Postsurgery, all cases were put to chemotherapeutic regimen. One case lost follow-up, whereas the other succumbed to death within two years due to brain metastasis.

Rhabdoid Tumour: There was one (3%) case of Rhabdoid tumour in a male baby of 12 months, who presented with abdominal mass and haematuria. CT scan showed a 16 cm tumour, involving almost the whole of unilateral kidney with metastasis to ulna. Radical nephrectomy was done. This was actually a review case, who had undergone the surgery along with histopathology and IHC from a peripheral centre and was referred to this institute for re-evaluation, further follow-up and treatment. Grossly the tumour involved the resection margins; with pale creamy colour and wide areas of haemorrhage and necrosis as was detailed in the original histopathology report. H&E showed large atypical rhabdoid cells with vesicular chromatin, prominent nucleoli and eosinophilic hyaline cytoplasmic inclusions (Table/Fig 12). Additionally, there was loss of SMARCB1 expression (Table/Fig 13) as was seen in the received IHC slide. A diagnosis of Rhabdoid tumour of kidney (Stage IV) was confirmed for initiation of treatment.

Post-surgery, he was put on chemotherapeutic regimen. However, the patient died within one year of follow-up.

Paediatric cystic nephroma: There were two cases (6%), comprising one male and one female aged 12 and 16 months respectively. They presented with abdominal masses. Ultrasound showed large cystic spaces involving almost the whole of unilateral kidney. Nephrectomy was done. Gross inspection showed well-circumscribed, cystically dilated kidney measuring 9.5 and 12 cm respectively with loss of cortico-medullary differentiation (Table/Fig 14). H&E sections showed multiple cystic spaces lined by hobnail (Table/Fig 15), cuboidal or denuded epithelium. The septas had fibrous tissue with focal cellular condensation and entrapped tubules (Table/Fig 16), without any solid neoplastic nodules or immature nephroblastic elements. Thus, the diagnosis of Pediatric Cystic Nephroma was offered. Both the patients were doing well on long term follow-up for upto three years till the publication of this manuscript.

Multicystic Dysplastic Kidney (MCDK): Total two cases (6%) of MCDKs were seen. One case was diagnosed during pre-natal ultrasound; another presented as newborn flank mass. The malformation was unilateral with no associated anomalies of other organ systems. Ultrasonography showed large spherical cysts with non-delineation of the renal sinus.

Nephrectomy specimen showed cystically dilated spaces in unilateral kidney. Histopathological examination showed metaplastic cartilage, primitive ducts with lobar disorganisation (Table/Fig 17), (Table/Fig 18). Thus, a diagnosis of MCDK was given. Long-term follow-up of the cases has shown good prognosis with normal functioning of contralateral kidney.

Discussion

Paediatric renal neoplasms are challenging to the clinician as they present with abdominal mass with overlapping imaging findings. Histopathological study is often the mainstay for arriving at definitive diagnosis. They create diagnostic dilemmas to the surgical pathologists due to their undifferentiated nature and wide histologic diversity that often mimics various renal and extra-renal neoplasms with potential for significant diagnostic error. Lack of daily experience further complicates the task of the pathologists, who are not familiar with these entities in day-to-day practice (5).

The mean age of the study population of 32 cases was 20 months, youngest being the newborn with Multicystic Dysplastic Kidney (MCDK) and oldest being 44 months in a Wilms tumour case. This is significantly lower than that of Bozlu G and Çitak EÇ, where the mean age of all cases was 53.26±46.64 months (6). This may be due to the widely heterogenous non-Wilms tumour neoplasms that vary widely in their incidence with significant variation in their demographic characteristics. The male paediatric cases were 59% (19 cases out of 32). These statistics are dominated by the Wilms tumour group that formed the majority (66%) of the study population which was at par with the other studies (6),(7),(8). Among non-Wilms tumour group, the most common histologic type was Congenial Mesoblastic Nephroma (13%), followed by Paediatric Cystic Nephroma, CCSK and MCDK (each being 6%). This was similar to the study conducted by Glick et al (9).

Abdominal mass was the common presenting feature in all these cases, whereas few cases of Wilms tumour presented with haematuria and hypertension, as was also observed by Lamb MG et al., (10). Rhabdoid tumour case had features of distant metastasis during its presentation, which itself was a bad prognostic indicator. Similar cases have also been documented in the literature, thereby indicating the very aggressive nature of the neoplasm and its tendency for early metastasis (11).

The typical triphasic pattern is very diagnostic of Wilms tumour where histomorphology is sufficient to arrive at the definitive diagnosis, except for special cases like Monophasic Wilms tumour. WT1 positivity was utilised in that case to differentiate it from non-Wilms neoplasms, as was also demonstrated by Goyal S et al., (12). Of special mention, was a rare histologic variant namely Teratoid Wilms tumour which belongs to the extreme end of the spectrum of Triphasic Wilms tumour with very few reported cases in the literature and high mortality rate (13). However, this case was doing well till 5 years post-surgery with good chemotherapeutic response. A very potent prognostic indicator of Wilms tumour, i.e., anaplasia was present focally in about 9% and diffusely in about 5% cases, which was at par with the observation of 5% by Davidoff AM (14). Cases with focal to diffuse anaplasia and diffuse blastemal pattern were considered as adverse prognostic factors in Wilms tumour, all these cases dying within two years of follow-up, which was at par with the review article authored by Vujanic´ GM et al., (15).

A very significant history of polyhydramnios was evaluated in one of the cases of CMN, which was observed to be commonly reported by Daskas N et al., (16). The gross morphologic appearances of cellular and classical CMN were similar to the findings by Chaudry G et al., (17). Cellular CMN is a bad prognostic indicator (18) as was also seen in present case that presented with metastasis within two years of follow-up.

The CCSK per se is a rare and very aggressive neoplasm of kidney with very poor prognosis (19). This was reflected by death of 1 out of 2 cases even after chemotherapeutic treatment. The confirmation of histomorphologic diagnosis was Cyclin D1 positivity by IHC that has recently been found to be a very useful marker to differentiate it from blastema-rich Wilms tumour (20), which was that main differential of present case.

Rhabdoid tumours of kidney have a dismal prognosis which is further potentiated by presence of metastasis at the time of presentation as was demonstrated by Reinhard H et al., (21). This was observed in this study as well, where the case being Stage IV died within one year of follow-up. Its diagnosis was strengthened by loss of immunohistochemical expression of SMARCB1 that differentiated it from Renal Cell Carcinoma with rhabdoid features, as has been demonstrated in a similar study by Han E et al., (22).

Paediatric cystic nephromas usually have a benign course with good prognosis following nephrectomy (23), as was seen in this study population. Histomorphology is very important to differentiate it from Cystic Partially Differentiated Nephroblastoma (CPDN) which is characterised by presence of immature nephrogenic elements (24).

Prognosis of MCDK is dependent on timely diagnosis with associated foetal anomalies (25). In this study, since both the cases were devoid of any other malformations, they carried a very good overall prognosis.

Thus, it is clear that a combination of careful gross examination followed by detailed histomorphological and immunohistochemical evaluation is required to arrive at a correct diagnosis. It is important as these tumours range from those which do not require chemotherapy and follow-up (e.g., Mesoblastic nephroma (Classic), Pediatric Cystic Nephroma) to those with a prognosis far worse than the usual favorable histology Wilms tumour (e.g., clear cell sarcoma, rhabdoid tumour) and varying chemotherapeutic regimen. Thus, a better understanding of these cases shall increase the diagnostic accuracy of these neoplasms with improved scope of treatment and favorable outcome for the patients.

Conclusion

Wilms tumour was the most common renal neoplasm in paediatric age group followed by CMN. Although the former mostly had a favourable outcome, anaplasia and advanced COG staging were the adverse prognostic variables in this group. CCSK and Rhabdoid tumours were the most aggressive amongst non-Wilms tumour category. Pediatric Cystic Nephromas and MCDKs were seen to have favourable outcomes.

Acknowledgement

All the co-authors have contributed equally to the preparation of the manuscript. This is an original work that has not been produced in any form in any other platforms.

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DOI and Others

DOI: 10.7860/JCDR/2022/55504.16310

Date of Submission: Feb 06, 2022
Date of Peer Review: Mar 18, 2022
Date of Acceptance: Apr 07, 2022
Date of Publishing: May 01, 2022

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Feb 10, 2022
• Manual Googling: Mar 12, 2022
• iThenticate Software: Apr 05, 2022 (5%)

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