scholarly journals Rare Case of Multilocular Cystic Renal Neoplasm of Low Malignant Potential with Chronic Pyelonephritis

Author(s):  
Mrunal Kesari ◽  
Shweta Agrawal ◽  
Yoganand Patil ◽  
Indranil Bhattacharya ◽  
Monica Dholpure

Multilocular Cystic Renal Neoplasm of Low Malignant Potential (MCRNLMP) represents a rare variant of clear cell (conventional) renal carcinoma. They constitute between 3-6% of clear cell Renal Cell Carcinoma (RCC). The RCC constitutes less than 1% of all renal tumours. The MCRNLMP has an excellent prognosis with no reports of recurrence or metastasis. Authors reported a case of 67-year-old male patient on account of its rarity, co-existing pyelonephritis and incidental detection of MCRNLMP. Differentiation between MCRNLMP, RCC variants and other cystic lesions with clear cells is important as prognosis, treatment differ markedly. Radiological Bosnaik classification of complex cysts in category IIF and III is challenging and requires microscopic examination for correct diagnosis. Histopathology shows cysts separated and fibrous septae having groups of clear cells with low grade nuclei. Immunohistochemistry with Carbonic Anhydrase-IX (CA-IX), Epithelial Membrane Antigen (EMA), Cytokeratin (CK7) and CD10 is confirmatory. Diagnosis requires detailed imaging studies, meticulous grossing of nephrectomy specimens, extensive sampling of cystic tissue to find clear cells and immunohistochemistry (IHC).

2020 ◽  
pp. jclinpath-2020-206681
Author(s):  
Yankun Song ◽  
Xiaoxue Yin ◽  
Qiuyuan Xia ◽  
Linmao Zheng ◽  
Jin Yao ◽  
...  

AimsXp11 translocation renal cell carcinoma (RCC) is a distinctive subtype of RCC with TFE3 (Transcription Factor Binding to IGHM Enhancer 3) gene rearrangement. The gross features in most Xp11 translocation RCCs closely resemble clear cell RCCs. In this study, we report six cases of Xp11 translocation RCCs with a unique multicystic architecture, reminiscent of multilocular cystic renal cell neoplasm of low malignant potential (MCRN-LMP).Methods and resultsMicroscopically, the renal mass was well circumscribed with multilocular cystic architecture. The cyst walls and septa were mostly lined by a single layer of cells with clear cytoplasm and low-grade nuclei, reminiscent of MCRN-LMP. Psammoma bodies were detected in four cases. One particular patient was misdiagnosed with benign cysts in local hospitals and led to second operation. Tumour cells were settled according to the track of the first surgical procedure. TFE3 fluorescence in situ hybridization (FISH) assay confirmed the diagnosis of Xp11 translocation RCCs. FISH and RNA sequencing analyses confirmed MED15-TFE3 gene fusion in all six cases. Respective patients were alive, without any recent evidence of disease recurrence and/or metastasis.ConclusionsHere, we introduce a relatively inertia-variant of Xp11 translocation RCC which mimics MCRN-LMP. The distinctive morphological condition is linked to MED15-TFE3 gene fusion. In fact, renal neoplasms with morphological features of MCRN-LMP, especially those containing psammoma bodies, should be routinely evaluated for evidence of TFE3 gene rearrangements.


2020 ◽  
pp. 22-26
Author(s):  
Harish Challa ◽  
Hariharasudhan Sekar ◽  
Sandhya Sundaram ◽  
Sriram Krishnamoorthy ◽  
Natarajan Kumaresan

Introduction Multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) is a rare subtype of clear cell renal cell carcinoma (ccRCC) accounting for 2-4% of RCC. It is defined as a neoplasm that is composed entirely of numerous cysts surrounded by fibrous capsule and septa containing clear cells without expansile growth or mural nodules (WHO 2016). The purpose of this manuscript is to highlight that it is imperative to identify this entity by strict histological criteria and distinguish this entity from cystic ccRCC due to its low malignant potential, excellent prognosis with no recurrence or metastasis. Case report A 46-year-old male presented with continuous mild loin pain for a month. There were no lower tract urinary symptoms. Ultrasound abdomen showed left lower pole renal mass. CECT-KUB was done as a definitive investigation which showed a solitary left lower pole renal cystic lesion with enhancement of size 3.8x3.6cm (Bosniak IV). As per CT findings, the patient underwent Laparoscopic partial nephrectomy. Histopathological examination showed multiple cysts with thin septal walls possessing clear cells with low-grade nuclei. 2 years of follow-up postoperatively with imaging studies revealed no recurrence or metastasis. Conclusions The purpose of this report is to emphasize the need to identify this entity by strict histological criteria as per WHO guidelines, as imaging studies were more often inconclusive. Urologists should have an adequate understanding such an entity. Almost all cases are amenable to partial nephrectomy irrespective of size and no documented evidence of recurrence and metastasis which mandates less stringent follow up postoperatively as compared to ccRCC.


Author(s):  
Moumita Maiti ◽  
Ranu Sarkar ◽  
Aritra Bhattacharya ◽  
Prasenjit Sen Ray

Introduction: Renal Cell Carcinoma (RCC) is the most common adult renal malignancy. Histopathologically, clear cell RCC accounts for 65-70% of all RCCs. Carbonic Anhydrase-IX (CA-IX) is a transmembrane protein and takes a role in cancer development and progression. A 75-100% clear cell RCCs show CA-IX expression. But this varies with grade and stage of tumour. Vascular Endothelial Growth Factor (VEGF) is responsible for tumour angiogenesis and expressed variedly in RCCs. Both VEGF and CA-IX expression is mediated by Hypoxia Inducible Factor-1α (HIF-1α). Aim: The present study aimed to evaluate the expression of CA-IX and VEGF in respect to different grades and stages of RCC and assessing their prognostic significance. Materials and Methods: This was a cross-sectional, observational study done on 45 histopathogically diagnosed cases of RCC. It was performed in the Department of Pathology, Nil Ratan Sircar Medical College, Kolkata, West Banglore, India over a period of two years (February 2018 to January 2020). Expression of VEGF and CA-IX were studied by immunohistochemistry. Results were analysed in Statistical Package for Social Sciences (SPSS) software (version 16.0) using Pearson Chi-square test. A p-value of <0.05 was regarded as significant. Results: Out of 45 cases of RCC, 34 tumours (32 clear cell carcinoma and two multilocular cystic renal neoplasm of low malignant potential) were evaluated for CA-IX immuno expression. About 25 cases showed CA-IX positivity which inversely associated with grade and stage of RCC (p-value <0.05). The CA-IX had a diagnostic value in detecting clear cell RCC with sensitivity 73.53%, specificity 100% and accuracy 80%. With 28 (62.2%) cases of RCC showed VEGF positivity among which nine were VEGF 1+ and 19 cases VEGF 2+. The VEGF expression showed a positive association with the grade and Tumour-Node-Metastasis (TNM) stage of tumour (p-value <0.05). Finally, authors found a statistically significant inverse association between CA-IX and VEGF expression in RCCs with clear cell morphology including clear cell RCC and multilocular cystic renal neoplasm of low malignant potential (p-value=0.001). Conclusion: High grade RCCs show low expression of CA-IX and strong positivity with VEGF. Both these markers have a prognostic significance. From the therapeutic point of view, VEGF positive tumours, especially inoperable and metastatic cases, may be benefited by anti-VEGF therapy whereas CA-IX positive tumours respond well by treatment with Interleukin-2.


2020 ◽  
pp. 1-6

Multilocular Cystic Renal Neoplasm of Low Malignant Potential (MCRN-LMP), previously known as multilocular cystic renal cell carcinoma as a rare variant of cystic kidney. It is composed of multiple cysts separated by thin septa covered with clear cells with no expansile growth. The termMCRN-LMPshould be used exclusively to identify a cystic kidney lesion with a small clusters of neoplastic clear cells in the cyst walls. Clinically these cases may present with flank pain or the majority of these tumors are incidentally found on radioimaging. Grossly, the tumor is well demarcated and consists of multiple various-sized cysts. The fibrous septa are generally thin and there is no discernible expansile nodule. The WHO/ISUP nuclear grade is generally low and usually corresponds to grade 1 or 2. In the World Health Organisation (WHO) 2016 classification of kidney tumors recognizes MCRN-LMP as a rare variant of cystic kidney. MCRN-LMP generally exhibits a low stage of TNM classification. These tumors have a favorable clinical course. The clinical course of these lesions suggests that patients may benefit from nephron-sparing surgery. The aim of this review is to bring attention, that MCRN-LPM is a low aggressive neoplasm with no recurrence and no metastatic potential. The updated diagnostic modalities and conservative line of management may be applicable for this rare entity for the better care of patients.


BMC Cancer ◽  
2008 ◽  
Vol 8 (1) ◽  
Author(s):  
Véronique Ouellet ◽  
Tak Hay Ling ◽  
Karine Normandin ◽  
Jason Madore ◽  
Christian Lussier ◽  
...  

2009 ◽  
Vol 16 (04) ◽  
pp. 609-610
Author(s):  
QURATULAIN SOOMRO ◽  
Hafeez Soomro ◽  
KHAN MUHAMMAD ◽  
Sardar Khatoon ◽  
ABDUL SAMAD SHAIKH ◽  
...  

During 05 years period from January 2003 to December 2007,45 cases of Ca. Pancreas were reported to LINAR and only onecase of Pseudo Papillary Tumour of Pancreas is registered. It is a rare, low malignant potential pancreatic tumour. It has unknown pathogenesisand found in young females. We report a case of 24 years old female, who presented with pain in abdomen and low grade fever of 07 monthsduration. Lepratomy and excisional biopsy was done. H/P came as Pseudo Papillary Tumour of Pancreas. At presentation no any L.N. werefound enlarged. Like other malignant pancreatic tumours, this neoplasm does not metastasize. Its definitive treatment is complete surgicalremoval, no any role of chemotherapy because it doesn't metastasized, limited role of radiotherapy in local invasive tumour or in residual mass.


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