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LETTER TO EDITOR  
Year : 2016  |  Volume : 59  |  Issue : 4  |  Page : 565-567
Renal cell carcinoma with rhabdoid differentiation: A novel entity with immunohistochemical study


Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

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Date of Web Publication10-Oct-2016
 

How to cite this article:
Divya A A, Siddhi G S, Avinash R J, Pallavi D B. Renal cell carcinoma with rhabdoid differentiation: A novel entity with immunohistochemical study. Indian J Pathol Microbiol 2016;59:565-7

How to cite this URL:
Divya A A, Siddhi G S, Avinash R J, Pallavi D B. Renal cell carcinoma with rhabdoid differentiation: A novel entity with immunohistochemical study. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Nov 22];59:565-7. Available from: http://www.ijpmonline.org/text.asp?2016/59/4/565/191778


Editor,

Renal cell carcinoma (RCC) with rhabdoid differentiation, also known as rhabdoid RCC (RRCC), is a rare neoplasm with an incidence of 3%-5%. [1],[2] It is an aggressive tumor and its rhabdoid morphology accounts as an independent risk factor for increased death rate in patients. [3] Hence, it is important to recognize it and look for in any conventional case of RCC.

Adult RRCC is currently recognized as any histologic type of RCC containing foci of high-grade malignant cells with rhabdoid morphology. [1],[2] Intense periodic acid-Schiff (PAS) positivity and immunohistochemistry (IHC) characteristic of RCC along with positive p53, high Ki-67 index can confirm the diagnosis of RRCC. [1],[4]

We present two cases of 59-year-old and 65-year-old male patients with vague abdominal complaints and loss of appetite. On examination, both the patients had pallor and microscopic hematuria. Imaging revealed large renal mass occupying most of the right kidney in both the patients. Bone scan ruled out distant metastasis. Radical nephrectomy specimens were received for histopathological examination.

Grossly, in case 1, the kidney appeared irregularly enlarged, bosselated with intact capsule. Cut surface revealed a tumor measuring 12 cm × 10 cm × 5 cm [Figure 1]a. In case 2, externally, the kidney was bosselated with one area showing capsular breach [Figure 2]b. Cut surface showed a tumor mass measuring 10 cm × 9.5 cm × 4.5 cm [Figure 2]a. Both the tumors had variegated appearance composed of gray-yellow, hemorrhagic, cystic, and necrotic areas with interspersed solid gray-white areas. Surgical renal vein cut margins were free of tumor.

Microscopy of both the tumors showed similar picture. The tumor cells were arranged in compact trabecular, tubular, and diffuse sheets. Tumor cells in majority of the foci were optically clear and few with granular eosinophilic cytoplasm with large, vesicular, centrally placed nuclei and prominent nucleoli, separated by prominent arborizing vascular septae [Figure 1]b. Focally, the tumor cells were arranged in sheets and had rhabdoid morphology comprising large pleomorphic cells with abundant intensely eosinophilic cytoplasm and eccentrically placed hyperchromatic nuclei and perinuclear eosinophilic inclusions with absent prominent fibrovascular septa [Figure 1]c and [Figure 2]d. PAS showed positive result in clear cell areas and intense positivity in rhabdoid areas [Figure 1]d. On IHC, the tumor cells showed immunoreactivity for CD10 [Figure 2]c, pan-cytokeratin (pan-CK) [Figure 1]e, epithelial membrane antigen (EMA), CD19, CK10, and vimentin in both the areas, but vimentin stained intensely in the rhabdoid areas [Figure 1]f, [Figure 2]e and f. p53 showed nuclear immunoreactivity only in the rhabdoid areas. INI-1 immunoreactivity was preserved in clear cell and rhabdoid areas [Figure 1]d. Desmin, CK7, and CD117 were negative. Ki-67 labeling index (KLI) was 38%. The above findings confirmed the diagnosis of RRCC.
Figure 1: (a) Cut surface of the kidney showing tumor measuring 12 cm × 10 cm × 5 cm, (b) photomicrograph showing tumor cells in alveolar pattern having optically clear cytoplasm (H and E, ×100), (c) tumor cells having rhabdoid morphology (H and E, ×400), inset showing intense periodic acid-Schiff positivity in rhabdoid areas (PAS, ×400), (d) preserved INI-1 immunostaining in rhabdoid areas (×100), inset showing preserved INI-1 in rhabdoid areas (×400), (e) pan-cytokeratin showing membrane immunoreactivity in clear cell (arrow) and rhabdoid areas (arrowhead) (×100), and (f) vimentin showing cytoplasmic immunoreactivity in clear cell (arrow) and rhabdoid areas (arrowhead) (×100)

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Figure 2: (a) Cut surface of the kidney showing tumor measuring 10 cm × 9.5 cm × 4.5 cm, (b) photomicrograph showing tumor cells invading the capsule (H and E, ×100), (c) tumor cells showing membrane immunoreactivity for CD10 (×100), (d) tumor cells having rhabdoid morphology (H and E, ×100), (e) and vimentin showing cytoplasmic immunoreactivity in clear cell (×100) and (f) rhabdoid areas (×100)

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Pediatric rhabdoid tumor of the kidney, first described in 1978, is a rare but well-recognized aggressive malignancy. In adults, it is infrequent. [5] The occurrence of rhabdoid differentiation is known to be associated with carcinomas, sarcomas, and melanoma of various organs in adults. [2],[5] It represents clonal evolution in malignant tumors and aggressive behavior. [1],[3],[4] Adult RRCC is a novel rare entity, the incidence of which is not known clearly. In two different studies, the incidence documented was 7.4% and 5%, respectively. [1],[2]

The descriptive term "rhabdoid" refers to tumor cells that morphologically resemble, but ultrastructurally and IHC differ from rhabdomyoblastic cells. [5] RRCC is defined as any histologic type of RCC containing foci of high-grade malignant cells with rhabdoid morphology, characterized by large cells with large eccentric vesicular nuclei, prominent nucleoli, globular eosinophilic paranuclear inclusion bodies, and abundant eosinophilic cytoplasm. [1],[2] The proportion of the rhabdoid areas in RRCC can vary from 1% to 90% of the tumor mass. [1],[5] Among kidney tumors, it is most common in clear cell RCC (ccRCC). [2],[4] In one study, rhabdoid differentiation was identified most frequently with ccRCC followed by papillary RCC and sarcomatoid RCC. [4]

Grossly, ccRCC is classically golden yellow due to the high lipid content. In contrast, rhabdoid areas appear solid, homogenous gray white as they are glycogen rich, lipid deficient, and less vascular. [1],[2] RRCC tends to be larger tumors with an average size of 7.5-8.8 cm compared to ccRCC, which has an average size of 4-7 cm. [1],[2] In both our cases, the tumors were of large size (12.5 cm and 10 cm) with classical golden yellow areas of clear cell component and solid gray-white rhabdoid areas.

Microscopic Fuhrman nuclear grade of ccRCC is usually low whereas the presence of rhabdoid morphology in RRCC automatically changes the nuclear grade to Fuhrman Grade IV. [1],[3] Rhabdoid cells give intense PAS positivity due to the rich glycogen content. [1],[2] Our cases showed similar findings.

The significance of recognition of rhabdoid differentiation in ccRCC is still undergoing evaluation. [1],[2],[3] Most studies believe that RRCC is twice as likely to undergo extrarenal invasion, distant metastasis, and early death, as compared to conventional ccRCC. [1],[3] A study documents that rhabdoid differentiation is not independently associated with increased death rate, and hence suggest that rhabdoid differentiation should not be grouped together when assessing risk in a patient with Grade 4 RCC. [6] The International Society of Urological Pathology, 2012, has categorized RRCC as a high-grade tumor (Grade 4). [3],[6] It is worth mentioning that in the present study, the first case had no capsular or perinephric fat invasion. The second case had only capsular invasion. None of the two cases had bony metastasis.

RRCC shows identical immunoprofile of the underlying RCC such as CK19, pan-CK, EMA, CD10, and vimentin immunoreactivity in clear cell and rhabdoid areas. [1] Rhabdoid areas exhibit intense vimentin and pan-CK positivity. [1],[4] It stains negatively for desmin, CD7, and CD117. [4] The similar immunophenotype between the rhabdoid and nonrhabdoid tumoral foci supports the origin of the rhabdoid cells from the ccRCC areas. p53 nuclear positivity and KLI are higher in rhabdoid areas compared to clear cell areas. This indicates higher proliferative activity in rhabdoid areas and may also represent progression to aggressive biological behavior. [4] INI-1 gene mutation characteristic of pure rhabdoid tumor is not seen in RRCC, and hence it retains INI-1 immunoreactivity. [2],[7]

Our experience from the present cases highlights the rarity of the lesion and importance of extensive sampling and IHC study in predicting clinical outcome. The knowledge about its origin, risk factors associated, and biological behavior is not yet clearly known. [1],[6] Hence, extensive studies need to be carried out to throw more light on the histogenesis and progression of this tumor.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Gökden N, Nappi O, Swanson PE, Pfeifer JD, Vollmer RT, Wick MR, et al. Renal cell carcinoma with rhabdoid features. Am J Surg Pathol 2000;24:1329-38.  Back to cited text no. 1
    
2.
Shannon B, Stan Wisniewski Z, Bentel J, Cohen RJ. Adult rhabdoid renal cell carcinoma. Arch Pathol Lab Med 2002;126:1506-10.  Back to cited text no. 2
    
3.
Przybycin CG, McKenney JK, Reynolds JP, Campbell S, Zhou M, Karafa MT, et al. Rhabdoid differentiation is associated with aggressive behavior in renal cell carcinoma: A clinicopathologic analysis of 76 cases with clinical follow-up. Am J Surg Pathol 2014;38:1260-5.  Back to cited text no. 3
    
4.
Yang X, Xi C, Jin J, Zhou L, Su J, Liu L, et al. Adult renal cell carcinoma with rhabdoid differentiation: Incidence and clinicopathologic features in Chinese patients. Ann Diagn Pathol 2015;19:57-63.  Back to cited text no. 4
    
5.
Peng HQ, Stanek AE, Teichberg S, Shepard B, Kahn E. Malignant rhabdoid tumor of the kidney in an adult: A case report and review of the literature. Arch Pathol Lab Med 2003;127:e371-3.  Back to cited text no. 5
    
6.
Zhang BY, Cheville JC, Thompson RH, Lohse CM, Boorjian SA, Leibovich BC, et al. Impact of rhabdoid differentiation on prognosis for patients with grade 4 renal cell carcinoma. Eur Urol 2015;68:5-7.  Back to cited text no. 6
    
7.
Podduturi V, Campa-Thompson MM, Zhou XJ, Guileyardo JM. Malignant rhabdoid tumor of the kidney arising in an adult patient. Proc (Bayl Univ Med Cent) 2014;27:239-41.  Back to cited text no. 7
    

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Correspondence Address:
A Ail Divya
Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Narhe, Pune - 411 041, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.191778

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