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CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 4  |  Page : 332-334  

Tubulocystic renal cell carcinoma: An entity related to papillary renal cell carcinoma?


1 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Surgical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Date of Web Publication2-Aug-2018

Correspondence Address:
K R Anila
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJDRDYPU.MJDRDYPU_206_17

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  Abstract 


A 48-year-old man presented to our surgical oncology division with incidentally detected abdominal mass. On examination, a ballotable swelling was present in the right lumbar region. His imaging studies showed a large multicystic mass in the lower pole of the right kidney. With radiological diagnosis of cystic renal cell carcinoma (RCC), the patient was taken up for surgery. Histopathology showed tubulocystic RCC (TC-RCC) with a focus of papillary RCC. Though closely related to papillary RCC, TC-RCCs of kidney are a distinct group of RCC. Analysis of more cases is required to understand the exact tumor biology and to ascertain prognosis.

Keywords: Indolent, Papillary renal cell carcinoma, tubulocystic renal cell carcinoma


How to cite this article:
Anila K R, Mathews A, Augustine P, Krithika E, Jayasree K. Tubulocystic renal cell carcinoma: An entity related to papillary renal cell carcinoma?. Med J DY Patil Vidyapeeth 2018;11:332-4

How to cite this URL:
Anila K R, Mathews A, Augustine P, Krithika E, Jayasree K. Tubulocystic renal cell carcinoma: An entity related to papillary renal cell carcinoma?. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2019 Oct 22];11:332-4. Available from: http://www.mjdrdypv.org/text.asp?2018/11/4/332/238170




  Introduction Top


Tubulocystic renal cell carcinoma (TC-RCC) is an uncommon, cystic renal epithelial malignancy, constituting <1% of all RCCs. It is a relatively new entity, with about seventy cases reported in the literature till date.[1] TC-RCC was initially considered to be a subtype of collecting duct carcinoma that was first described by Masson, who coined the term “Bellinian epithelioma” or “carcinoma of the collecting ducts” because he thought it originated from the collecting ducts of Bellini.[2] MacLennan et al. were of the opinion that TC-RCC belonged to low-grade spectrum of collecting duct carcinomas.[3] Amin et al. introduced the term “tubulocystic carcinoma” for this entity.[4]


  Case Report Top


A 48-year-old man presented to our surgical oncology division with incidentally detected abdominal mass. He had no other significant complaints. There was no history of abdominal pain, dysuria, or hematuria. On examination, a ballotable swelling was present in the right lumbar region. Ultrasound scan showed a mass lesion in the lower pole of the right kidney with multiple cysts. His computed tomography studies showed a large exophytic, multicystic, hypodense, nonenhancing, noncalcified mass measuring 11.4 cm × 11.3 cm × 7.6 cm with extension to renal sinuses. With a radiological impression of Bosniak Type 4 lesion, the patient was taken up for surgery.

We received radical nephrectomy specimen. Cut surface showed a predominantly cystic neoplasm replacing almost the entire renal parenchyma, with normal renal tissue identifiable only at the periphery. The closely packed small cysts gave a spongy/bubble wrap appearance. An occasional cystic locule was dilated and contained papillary excrescences [Figure 1]. Microscopy showed cysts lined by cuboidal cells which were deceptively bland in low power, with the higher power highlighting the nuclear pleomorphism and prominent nucleoli. Hobnailing was a prominent feature [Figure 2]a and [Figure 2]b. A section from papillary area showed papillae lined by cuboidal cells, with the core containing sheets of foamy macrophages [Figure 2]c. Immunohistochemistry showed positivity for CD10 and vimentin [Figure 2]d in both TC and papillary areas and negativity for CK7 and Alpha-methylacyl-CoA reductase (AMACR) in both papillary and TC areas. A diagnosis of TC-RCC with focal papillary RCC was given.
Figure 1: Nephrectomy specimen, cut surface, showing spongy appearance with focal papillary area, highlighted by arrow

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Figure 2: (a) Low-power view showing cystic spaces lined by deceptively bland cells with hobnailing (H and E, ×100). (b) High-power view showing high-grade nucleus, prominent nucleoli, and hobnailing (H and E, ×400). (c) Papillary areas with core showing foamy macrophages (H and E, ×100). (d) CD10 and vimentin positivity (IHC, ×200)

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  Discussion Top


TC-RCCs of the kidney are considered as low-grade, indolent neoplasms. TC-RCC has a male predominance and occurs in adults with a wide age range. Most patients present in the fifth and sixth decades of life. They are a distinct group of renal carcinoma with specific macroscopic and microscopic findings. The gross appearance – spongy/bubble wrap appearance due to multiple closely packed cysts and microscopic appearance of hobnailing and high-grade nucleus (Fuhrman nuclear Grade 2–3) – is a characteristic feature and hence immunohistochemistry is not essential for reaching a diagnosis. The high-grade nuclear feature and hobnailing help to differentiate this entity from the benign cystic neoplasm such as cystic nephroma and oncocytoma with predominant cystic and tubular patterns. Cystic RCC as another differential can be excluded by the lack of hobnailing and clear cell morphology.

TC-RCC is immunohistochemically reactive for CD10, AMACR (P504-S), CK8, CK18, and CK19. Our case was not a pure case of TC-RCC. In our case, TC-RCC was found to coexist with papillary RCC. Tumor cells in both these areas were found to be immunoreactive for CD10 and vimentin and negative for AMACR and CK7. There are previous reports in the literature, similar to our case wherein TC-RCC was seen coexisting with papillary RCC.[5] These authors are of the opinion that though TC-RCC is considered to have an indolent course, when coexisting with other histological types such as papillary RCC, the outcome may not be as indolent as in a pure case of TC-RCC. Our patient is on follow-up for the past 1 year following surgery and the follow-up period has been uneventful.

Yang et al. through comparative genomic microarray analysis observed that TC-RCC showed gains of chromosome 17, but not chromosome 7, whereas most papillary RCCs showed chromosomal gains in both 7 and 17 (trisomies). Therefore, they concluded that TC-RCC is closely related to papillary RCC as they often coexist in proximity or intermingled with each other.[6] However, a recent study by Tran et al. in pure cases of TC-RCC with fluorescence in situ hybridization has shown TC-RCC to be a distinct entity.[7] Their cases did not show gain of chromosome 17 or 7. A detailed analysis of lot more cases is required to understand the exact tumor biology and ascertain prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rheault MJ. Pierre Masson: The father of histopathology teaching in Canada. Ann Chir 1991;45:833-6.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
    
3.
MacLennan GT, Farrow GM, Bostwick DG. Low-grade collecting duct carcinoma of the kidney: Report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin. Urology 1997;50:679-84.  Back to cited text no. 3
    
4.
Amin MB, MacLennan GT, Gupta R, Grignon D, Paraf F, Vieillefond A, et al. Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. Am J Surg Pathol 2009;33:384-92.  Back to cited text no. 4
    
5.
Deshmukh M, Shet T, Bakshi G, Desai S. Tubulocystic carcinoma of kidney associated with papillary renal cell carcinoma. Indian J Pathol Microbiol 2011;54:127-30.  Back to cited text no. 5
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6.
Yang XJ, Zhou M, Hes O, Shen S, Li R, Lopez J, et al. Tubulocystic carcinoma of the kidney: Clinicopathologic and molecular characterization. Am J Surg Pathol 2008;32:177-87.  Back to cited text no. 6
    
7.
Tran T, Jones CL, Williamson SR, Eble JN, Grignon DJ, Zhang S, et al. Tubulocystic renal cell carcinoma is an entity that is immunohistochemically and genetically distinct from papillary renal cell carcinoma. Histopathology 2016;68:850-7.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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