PRIMARY MUCINOUS ADENOCARCINOMA OF RENAL PELVIS

INTRODUCTION

Squamous cell carcinoma and transitional cell carcinoma are the commonest epithelial malignancies arising from the renal pelvis, accounting for 10–15% and 85–90% of cases, respectively (1). Adenocarcinomas of the renal pelvis account for <1% of cases with the subclassification of mucinous (21.5%), tubulovillous (71.5%) and papillary non-intestinal (7.0%) (1,2). The first report of primary mucinous adenocarcinoma of the renal pelvis was in 1960 by Hasebe et al. (3). It remains a rare disease with fewer than 100 cases reported (4). Without the characteristic symptoms or radiological features, it is difficult to diagnose preoperatively. Also, because of its’ rarity, no standard treatment has been proposed (1). Herein, we present a case of mucinous adenocarcinoma arising from the renal pelvis following the CARE Guidelines and conduct a literature review including all of the cases reported since 2000.

Case presentation 

A 52 year-old man presented with a 6-month history of right flank swelling and pain in addition to episodes of fever. He has a history of right renal stones, for which the patient did not receive any treatment. He was admitted to Prince Hussein Urology Center. A computed tomography scan showed a 17 mm pelvi_uretric stone causing massive right hydronephrosis with the right kidney crossing the midline and the pelvic junction seen in the left side of the abdomen, along with a 17 mm lower group calyceal stone and cortical thinning. A non-functioning right kidney was found on the DMSA scan. He was diagnosed with a non-functioning right kidney and was given the plan of a right simple nephrectomy. He underwent right percutaneous nephrostomy insertion to relieve the hydronephrosis, with an output of 700 ccs 5 days before the surgery. Four days later, on follow-up computed tomography, the hydronephrosis was unchanged along with recurrent blockage of the nephrostomy tube. Laboratory tests showed normal liver and kidney function tests. We diagnosed the patient with calculous non-functioning kidney with the possibility of malignant disease to be excluded. We performed a midline simple nephrectomy.

His kidney was markedly enlarged with a thin cortex.

There was an unintentional spillage of gelatinous material because of the PCN procedures. Therefore, we only performed a nephrectomy without a total ureterectomy. On sectioning the kidney, there was thick gelatinous material. The cut surface showed cystic dilation of the pelvicalyceal system with extensive atrophy and thinning of the renal cortex. The histological diagnosis of 

primary mucinous adenocarcinoma of the renal pelvis along with intestinal metaplasia was made.

This patient was advised to undergo adjuvant chemotherapy because of the spillage of gelatinous material during surgery. He initially refused and was referred to the oncology team. Later on, the patient received 4 cycles of adjuvant chemotherapy of gemcitabine–cisplatin. On follow-up bone and CT scans, he was found to have bone metastasis in the coccyx and left proximal femur. He is now under the care of the oncology team.

DISCUSSION

Mucinous adenocarcinoma, generally seen in organs other than the urinary system, is characterised by abundant mucous secretion comprising more than half of the tumour volume (5, 6). To date, fewer than 100 cases of the rare primary mucinous adenocarcinoma of the renal pelvis have been reported. It can be misdiagnosed as calculus pyonephrosis because it is poorly recognised. As in the present case, PCN can

result in iatrogenic tumour-cell spillage and seeding; PCN can also increase the difficulty of any subsequent

treatment. These factors can contribute to its poor prognosis. Although we are not the first group to report

this disease, our case should remind surgeons about the possibility of malignancy before conducting PCN on patients with pyonephrosis, especially those with elevated serum tumour markers and longstanding obstructive calculus.

We conducted a literature search of PubMed and Embase using the MeSH terms ‘mucinous, adenocarcinoma’ and ‘kidney pelvis’. We included all of the cases reported since 2000 (Table 1), with most being from Asia (83.3%), especially India (33.3%). More men were reported than women (63.33% vs. 33.33%). The only clinical risk factor in mucinous ovarian adenocarcinoma is smoking (6). The higher prevalence of smoking in men may cause a higher rate of adenocarcinoma. In previous cases, no typical symptoms were reported. As in this case, the patient

presented with right waist pain and intermittent fever. Our literature review found that 7/30 patients presented with abdominal mass, 19/30 presented with flank

pain or discomfort and 5/30 presented with gross haematuria. These were consistent with the classical renal cancer triad, indicating late-stage or advanced disease. It is reported .

PUJO, pelvis ureteric junction obstruction; RN, radical nephrectomy; NU, nephroureterectomy; NC, data not clear; Y, years; M, months; CEA, carcinoembryonic antigen; NA, not available; CA19-9, carbohydrate antigen 19-9.

There were no typical radiological features of primary renal pelvis mucinous adenocarcinoma (18). Our patient presented with severe hydronephrosis and large renal pelvic calculi with cortical thinning, which led to the simple diagnosis of calculus pyonephrosis. In our literature review, there were 9/30 patients presenting with stones, 16/30 presenting with hydronephrosis, 10/30 presenting with pyonephrosis, 9/30 presenting with a tumour or mass and a few patients with stenosis or obstruction of the pelvic-ureteric junction. None of these features indicate malignancy, except for a mass. According to most of the reported cases, the diagnosis of non-functional kidney and hydronephrosis caused by renal calculi was made by imaging. The diagnosis of primary mucinous adenocarcinomas of the renal pelvis could only be made after pathological assessment (12). However, a few cases reported that the diagnosis of primary mucinous adenocarcinomas of the renal pelvis could be associated with elevated serum tumour markers such as CEA or CA19-9 (2). Our literature review found that one-fifth of the patients had elevated serum tumour markers. We hypothesise that those serum tumour markers, accompanied by imaging (CT scan), can increase the rates of accurate diagnosis.

No standard surgical procedures have been proposed for this adenocarcinoma (2). As indicated in this literature review, because there were no preoperative measures to detect this tumour, most patients underwent a nephrectomy without total ureterectomy (14). However, radical nephroureterectomy with bladder cuff excision is the treatment of choice or the standard treatment for pelvis tumours (28). Moreover, adjuvant therapy was received by a few patients. Only Raphael et al. (23) and Lai et al. (2) reported that adjuvant radiation and interleukin-2 were administered after surgery, respectively. The primary adenocarcinoma of the renal pelvis has a poor prognosis generally and most patients die during the first few years of follow-up (21). It is reported that chemoradiation, chemotherapy and radiotherapy should be recommended for mucinous carcinoma of colorectal and ovarian origins (5, 6). To improve the prognosis, we recommend nephroureterectomy with a bladder cuff followed by adjuvant therapy such as chemotherapy.

CONCLUSION

In conclusion, renal pelvis primary mucinous adenocarcinoma is especially rare without characteristic or typical radiological features and standard treatment. Based on our literature review, careful history taking and radiological studies combined with serum markers may improve accurate diagnosis rates. For better survival outcomes, nephroureterectomy is recommended to be followed by adjuvant therapy.

     REFERENCES

1.Han DS, Yuk SM, Youn CS, et al. Primary mucinous cystadenocarcinoma of the renal pelvis misdiagnosed as ureteropelvic junction stenosis with renal pelvis stone: a case report and literature review. World J Surg Oncol 2015; 13:324. 10.1186/s12957-015-0739-7 [PMC free article] [PubMed] [CrossRef] [Google Scholar]

2. Lai C, Teng XD. Primary enteric-type mucinous adenocarcinoma of the renal pelvis masquerading as cystic renal cell carcinoma: A case report and review of the literature. Pathol Res Pract 2016; 212:842-8. 10.1016/j.prp.2016.06.006 [PubMed] [CrossRef] [Google Scholar]

3.Hasebe M, Serizawa S, Chino S. In a case of papillary cystadenocarcinoma following malignant degeneration of a papillary adenoma in the kidney pelvis. Yokohama Med Bull 1960; 11:491-500. [PubMed] [Google Scholar]

5.Shah VB, Amonkar GP, Deshpande JR, et al. Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei. Indian J Pathol Microbiol 2008; 51:536-7. 10.4103/0377-4929.43753 [PubMed] [CrossRef] [Google Scholar]

6.Hugen N, Brown G, Glynne-Jones R, et al. Advances in the care of patients with mucinous colorectal cancer. Nat Rev Clin Oncol 2016; 13:361-9. 10.1038/nrclinonc.2015.140 [PubMed] [CrossRef] [Google Scholar]

7.Morice P, Gouy S, Leary A. Mucinous ovarian carcinoma. N Engl J Med 2019; 380:1256-66. 10.1056/NEJMra1813254 [PubMed] [CrossRef] [Google Scholar]

8.Shioya A, Saito K, Kurose N, et al. Primary enteric-type tubulovillous adenocarcinoma arising in the renal pelvis. Pathol Int 2018; 68:388-90. 10.1111/pin.12651 [PubMed] [CrossRef] [Google Scholar]

9.Patel KN, Patel NA, Gandhi S. Presentation of mucinous adenocarcinoma of renal pelvis masquerading as gross hydronephrosis: A histopathological surprise. South Asian J Cancer 2017; 6:78. [PMC free article] [PubMed] [Google Scholar]

10.Geramizadeh B, Khezri A, Giti R. Incidental mucinous adenocarcinoma in situ of renal pelvis presenting as severe hydronephrosis. J Nephropathol 2017; 6:275-7. 10.15171/jnp.2017.45 [CrossRef] [Google Scholar]

11.Joseph LD, Krishnamoorthy S, Swaminathan R, et al. Mucinous cystadenocarcinoma in a horse shoe kidney masquerading as giant hydronephrosis – A case report: Diagnostic challenges, lessons learnt and review of literature. J Clin Diagn Res 2016; 10:PD12-PD14. [PMC free article] [PubMed] [Google Scholar]

12.Patel RD, Vanikar AV, Modi PR. Mucinous cystadenocarcinoma of renal pelvis presenting as pyonephrosis. Saudi J Kidney Dis Transpl 2014; 25:647-50. 10.4103/1319-2442.132224 [PubMed] [CrossRef] [Google Scholar]

13.Lee HY, Jang MY, et al. Primary mucinous adenocarcinoma of the renal pelvis. Urological Science 2014; 25:65-7. 10.1016/j.urols.2012.07.010 [CrossRef] [Google Scholar]

14.Behzatğlu K, Boyaci C, Okçu O, et al. Mucinous urothelial carcinoma of the renal pelvis. Rare Tumors 2014; 6:5485. 10.4081/rt.2014.5485 [PMC free article] [PubMed] [CrossRef] [Google Scholar]

15.Abbas M, Kramer MW, Spieker T, et al. Primary mucinous adenocarcinoma of the renal pelvis with carcinoma in situ in the ureter. J Egypt Natl Canc Inst 2014; 26:51-4. 10.1016/j.jnci.2013.11.002 [PubMed] [CrossRef] [Google Scholar]

16.Yadav R, Kataria K, Balasundaram P, et al. Mucinous cystadenocarcinoma arising in an ectopic kidney simulating a retroperitoneal dermoid cyst: a rare tumour presenting as a diagnostic dilemma. Malays J Pathol 2013; 35:95-8. [PubMed] [Google Scholar]

17.Chang CP, Wang SS, Wen MC, et al. Mucinous adenocarcinoma of the renal pelvis masquerading as xanthogranulomatous pyelonephritis. Urology 2013; 81:e40-1. 10.1016/j.urology.2013.03.005 [PubMed] [CrossRef] [Google Scholar]

18.Chablé-Montero F, Mendoza-Ramírez S, Lavenant-Borja MI, et al. Mucinous cystadenoma of the pyelocaliceal system: a report of 3 examples and an analysis of 17 previously published cases. Ann Diagn Pathol 2013; 17:239-44. 10.1016/j.anndiagpath.2012.10.008 [PubMed] [CrossRef] [Google Scholar]

19.Sisodia SM, Khan WA, Bhavsar SP. Incidental primary papillary mucinous adenocarcinoma of the renal pelvis in a case of a non-functioning kidney due to chronic pyelonephritis and pelvic calculus. Saudi J Kidney Dis Transpl 2012; 23:592-3. [PubMed] [Google Scholar]

20.Otani M, Tsukamoto T, Serizawa H. Mucinous adenocarcinoma of the renal pelvis: A case report and review of the literature. Virchows Archiv 2012; 461:S295. [Google Scholar]

21.Bharathi K, Anuradha S, Khalique A. Cytodiagnosis of mucinous cystadenocarcinoma of renal pelvis – A rare case report. Int J Pharma Biosci 2012; 3:136-41. [Google Scholar]

22.Ye YL, Bian J, Huang YP, et al. Primary mucinous adenocarcinoma of the renal pelvis with elevated CEA and CA19-9. Urol Int 2011; 87:484-8. 10.1159/000329767 [PubMed] [CrossRef] [Google Scholar]

23.Sidharth1, Maskey P, Chalise PR, et al. Primary mucinous adenocarcinoma of the renal pelvis and ureter. Nepal Med Coll J 2011; 13:229-30. [PubMed]

24.Raphael V, Sailo S, Bhuyan A, et al. Mucinous adenocarcinoma of the renal pelvis with adenocarcinoma in situ of the ureter. Urol Ann 2011; 3:164-6. 10.4103/0974-7796.84951 [PMC free article] [PubMed] [CrossRef] [Google Scholar]

25.Gulwani H, Jain A. Primary papillary mucinous adenocarcinoma of the ureter mimicking genitourinary tuberculosis. Pathology Res Int 2010; 2010:738407. [PMC free article] [PubMed]

26.Rao P, Pinheiro N, Jr, Franco M, et al. Pseudomyxoma peritonei associated with a primary mucinous borderline tumour of the renal pelvicalyceal system. Arch Pathol Lab Med 2009; 133:1472-6. [PubMed] [Google Scholar]

27.Flores MR, Argueta VL, Ruiz MR, et al. Mucinous cystadenocarcinoma of the renal pelvis: a case report and clues to histogenesis. BMJ Case Rep 2009. doi: 10.1136/bcr.06.2008.0307 [PMC free article] [PubMed] [CrossRef] [Google Scholar]

28.Ho CH, Lin WC, Pu YS, et al. Primary mucinous adenocarcinoma of the renal pelvis with carcinoembryonic antigen production. Urology 2008; 71:984.e7-8. 10.1016/j.urology.2007.11.069 [PubMed] [CrossRef] [Google Scholar]

29.Takehara K, Nomata K, Eguchi J, et al. Mucinous adenocarcinoma of the renal pelvis associated with transitional cell carcinoma in the renal pelvis and the bladder. Int J Urol 2004; 11:1016-8. 10.1111/j.1442-2042.2004.00932.x [PubMed] [CrossRef] [Google Scholar]

30.Mathur S, Singh MK, Rao SI, et al. Mucinous metaplasia of the renal pelvic epithelium in a case of recurrent urolithiasis and pyelonephritis. Urol Int 2004; 72:355-7. 10.1159/000077694 [PubMed] [CrossRef] [Google Scholar]

31.Kaur G, Naik VR, Rahman MN. Mucinous adenocarcinoma of the renal pelvis associated with lithiasis and chronic gout. Singapore Med J 2004; 45:125-6. [PubMed] [Google Scholar]

32.Park S, Meng MV, Greenberg MS, et al. Muconephrosis. Urology 2002; 60:344. 10.1016/S0090-4295(02)01707-7 [PubMed] [CrossRef] [Google Scholar]

33.M, Hoshida Y, Hanai J, et al. Catheterised urine cytology of mucinous carcinoma arising in the renal pelvis. A case report. Acta Cytol 2000; 44:442-4. 10.1159/000328495 [PubMed] [CrossRef] [Google Scholar]