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Year : 2012  |  Volume : 55  |  Issue : 3  |  Page : 420-422
Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma?


Department of Pathology, Lady Hardinge Medical College, New Delhi, India

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Date of Web Publication29-Sep-2012
 

How to cite this article:
Madan NK, Pujani M, Choudhury M, Andley M. Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma?. Indian J Pathol Microbiol 2012;55:420-2

How to cite this URL:
Madan NK, Pujani M, Choudhury M, Andley M. Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma?. Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Sep 20];55:420-2. Available from: http://www.ijpmonline.org/text.asp?2012/55/3/420/101769


Sir,

The spectrum of cystic renal neoplasms includes multilocular cystic nephroma (MLCN), multilocular cystic renal cell carcinoma (MCRCC), and renal cell carcinoma (RCC) with cystic change. [1] These cystic tumors are extremely difficult to distinguish on clinical, radiological, and gross features and are a cause of diagnostic dilemma. Here, we present a very unusual case of MLCN synchronous with MCRCC.

A 50-year-old lady presented with persistent dull aching right flank pain and weight loss for 3 months with a recent increase in size of a lump in the right flank. There was no fever, hematuria, or any obstructive/irritative voiding symptoms. She gave a history of longstanding right flank mass of more than 10 years duration. The patient revealed that she had been evaluated for an abdominal lump a few years ago with preliminary investigations including an abdominal ultrasound and was diagnosed to be having a benign renal mass for which she had refused surgery. Her general physical examination was unremarkable. On abdominal examination, there was a large, non-tender, non-balottable lump, palpable in the right lumbar region. Complete hemogram, renal function tests, and urinalysis were within normal limits. Abdominal ultrasound showed a heterogeneous, predominantly hypoechoic mass in the right renal fossa, measuring approximately 14 × 12 × 9.7 cm with multiple internal echoes. Contrast-enhanced computed tomography (CECT) of the abdomen displayed a well-defined septated multicystic lesion arising from the right kidney, with foci of calcification and peripheral and septal contrast enhancement. There was no lymphadenopathy or ascites. Urine cytology was negative for malignant cells.

Based on the above findings, a preoperative clinical diagnosis of MLCN was made and the patient underwent right-sided nephrectomy with partial ureterectomy.

The gross specimen consisted of a cystic mass measuring 14 × 12 × 10 cm with a small beak of uninvolved renal tissue at one pole. On cut opening, multiple non-communicating cysts, ranging in size from 0.3 to 8 cm in diameter, were seen replacing almost the entire right kidney. Most of the cysts had thin septa and contained clear serous fluid, and some had thick walls with hemorrhagic contents. The surface of the cysts was smooth and no solid masses or papillary projections were identified. No connection was seen between the cysts and the renal pelvis.

Microscopic examination demonstrated a cystic neoplasm with multiple variable-sized cysts lined by flattened to low cuboidal epithelium with regular nuclei and fine chromatin [[Figure 1]a]. These cysts were separated by septae containing fibrocollagenous stroma with scattered lymphocytes and some septae showed ovarian-like cellular stroma. Multiple foci of calcification, scattered slings of smooth muscle fibers, few corpora albicans-like acellular structures, and few small mature appearing tubules filled with inspissated eosinophilic secretions were noted in the septae [[Figure 1]b].
Figure 1: (a) Areas corresponding to multicystic nephroma showing variable-sized cysts lined by flaened to low cuboidal epithelium (Hematoxylin and Eosin, ×40). (b) Multiple foci of calcification, scattered slings of smooth muscle fi bers, and ovarian-like stroma seen in the septae between the cysts (Hematoxylin and Eosin, ×200)

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The sections also showed few cysts, both small and large, lined by cells with distinctly clear cytoplasm, small round hyperchromatic nuclei, and inconspicuous nucleoli. Small groups of clear cells were seen in some of the septae, with numerous thin-walled capillaries in between them [[Figure 2]a]. Some of the cysts were lined by hemosiderin-laden macrophages. Adjacent kidney was free of tumor and showed features of chronic pyelonephritis with glomerulosclerosis and mild arteriosclerosis. Sections from renal vessels and ureter were free of tumor. A diagnosis of MCRCC synchronous with MLCN was considered, and immunohistochemistry was performed for confirmation.
Figure 2: (a) Few cysts were lined by clear cells with small groups of clear cells in some of the septae (Hematoxylin and Eosin, ×200). (b) Clear cells lining the cysts and those present in clusters showing
EMA positivity (EMA, ×400)


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Immunohistochemically, the cyst epithelium showed diffuse positivity for cytokeratin (CK) and epithelial membrane antigen (EMA) and focal positivity for CD10 and vimentin. The clusters of clear cells seen in few of the septae stained positive for all the above markers [[Figure 2]b]. A final diagnosis of MCRCC with multicystic nephroma was rendered.

Cystic nephroma is a rare benign renal tumor which has a female predominance in adults with an age range of 40-60 years. Many researchers have suggested that it lies at the benign end of the cystic renal neoplasms which include MLCN, MCRCC, and RCC with cystic change. MLCN has conventionally been considered not to be related to MCRCC. [2] However, Omar et al. in 2006 reported a case of MCRCC arising in a MLCN. [3] This is the second such case of a MCRCC synchronous with MLCN.

The etiology and pathogenesis of MLCN is not clear. The nonspecific clinical findings and the poor contribution of imaging studies make it impossible to make a definitive preoperative diagnosis. [4] The final diagnosis rests on the microscopic findings which have been characterized by Eble and Bonsib in their extensive study on cystic renal neoplasms. [2] The presence of variable-sized cysts lined by flattened to cuboidal cells, foci of calcification, inflammatory cells, ovarian-like stroma, corpora albicans-like structures, slings of smooth muscle fibers, and tubules with eosinophilic secretions have all been described in cystic nephroma as were seen in our case. MLCN may have few clear cells focally lining the septa, but multiple cysts lined by clear cells and clusters of clear cells in the cyst walls are never found. [2]

Multicystic renal cell carcinoma is a rare cystic renal neoplasm which is considered as a subtype of clear cell RCC. These tumors comprise approximately 1-2% of all renal tumors. [5] MCRCC may present with similar findings as other cystic renal neoplasms, and preoperative biopsy with frozen sections may not be diagnostic. MCRCC has cysts lined by clear cells which are of low Fuhrman grade, with septae containing aggregates of cells with clear cytoplasm. Rich vascularity, occasionally with hemosiderin containing cells, may be noticed.

Carcinomatous degeneration is well known in human renal cystic kidney diseases, most often in a setting of von Hippel-Lindau disease. The findings of MLCN and clear cells resembling those of RCC led us to consider a possibility of synchronous presence of both the tumors in the same kidney, and to support our diagnosis, immune markers were applied. Immunohistochemical markers for RCC include CK, EMA, vimentin, and CD10. Mukhopadhyay et al. in their recent study found that the cyst epithelium of MLCN shows aberrant renal tubular differentiation with positivity for all the above markers diffusely or focally. They suggested that immunohistochemistry plays no role in differentiating MLCN from MCRCC and morphological features are crucial. [6] Thus, MLCN should be extensively sampled, especially the thicker and/or hemorrhagic areas and cyst wall containing clear cells should be carefully looked for.

According to the existing criteria, the diagnosis of MCRCC arising in MLCN appeared most appropriate in our case as the patient had history of a longstanding mass with recent increase in size associated with weight loss. Histopathology revealed clusters of clear cells of RCC along with areas of typical MLCN. Considering a similar immunohistochemical profile and overlapping clinical, radiological, and morphological features, can we say that these two tumors share a histogenetic link? The prognosis of both these tumors is excellent and no recurrences or metastases have been reported. However, true clinical significance of MCRCC arising in MLCN is unclear due to lack of reports on such cases.

 
   References Top

1.Stamatiou KN, Sofras F. Multilocular cystic nephroma and multicystic clear cell carcinoma: Two faces of the Roman God Janus? Int J Surg Pathol 2009;17:170-1.  Back to cited text no. 1
[PUBMED]    
2.Eble JN, Bonsib SM. Extensively cystic renal neoplasms: Cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal pelvis. Semin Diagn Pathol 1998;15:2-20.  Back to cited text no. 2
[PUBMED]    
3.Omar AM, Khattak AQ, Lee JA. Cystic renal cell carcinoma arising from multilocular cystic nephroma of the same kidney. Int Braz J Urol 2006;32:187-9.  Back to cited text no. 3
[PUBMED]    
4.Betigeri AM, Subramaniyam B. Multilocular cystic nephroma - A surgical and radiological dilemma. Int J Cur Bio Med Sci 2011;1:44-6.  Back to cited text no. 4
    
5.Sabhiki A, Abrari A, Sachdev R, Chawla A, Vaidya A. Multilocular cystic renal cell carcinoma: A diagnostic rarity. Indian J Pathol Microbiol 2008;51:457-8.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Mukhopadhyay S, Valente AL, Roza G. Cystic nephroma: A histologic and immunohistochemical study of 10 cases. Arch Pathol Lab Med 2004;128:1404-11.  Back to cited text no. 6
[PUBMED]    

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Correspondence Address:
Neha Kawatra Madan
Department of Pathology, Lady Hardinge Medical College, New Delhi 110001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.101769

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