
==== Front
Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00979-9
10.1016/j.ijscr.2024.110198
110198
Case Report
Renal clear cell carcinoma undergoing cystic change: A cases report and review of the literature
Sun Yunji ab
Xu Baoqun c
Zhang Heng ab
Li Lihua ab
Gao Yulan ab
Sun Maokun tsgx1@163.com
ab⁎⁎⁎
a Department of Urology, Shandong Provincial Third Hospital, Shandong University, Jinan 250012, China
b Shandong Stone Disease Prevention and Treatment Center, Jinan 250012, China
c Department of Pathology, Shandong Provincial Third Hospital, Shandong University, Jinan 250012, China
⁎ Corresponding author at: Department of Urology, Shandong Provincial Third Hospital, Shandong University, Jinan 250012, China. tsgx1@163.com
⁎⁎ Corresponding author at: Shandong Stone Disease Prevention and Treatment Center, Jinan 250012, China. tsgx1@163.com
22 8 2024
10 2024
22 8 2024
123 1101982 7 2024
14 8 2024
17 8 2024
© 2024 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Limited.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction

We presented a case diagnosed the renal clear cell carcinoma undergoing cystic change (RCCCC) with detailed clinical data. Along with literature review, we aimed to investigate clinical diagnosis and treatment of RCCCC and explore the differential diagnosis of RCCCC and multilocular cystic renal cell carcinoma (MCRCC).

Case presentation

The patient was diagnosed with a right renal cyst after physical examination, which was misdiagnosed as a renal cyst by imaging examination. Intraoperative surgical treatment was performed to remove the roof and decompress the renal cyst. Rapid pathology revealed MCRCC with low malignant potential during laparoscopic right renal cyst decompression. Radical nephrectomy was performed with the family's signature. The postoperative pathological diagnosis was clear cell carcinoma cystic lesion of kidney (RCCCC). No recurrence or metastasis during 1 year follow-up.

Clinical discussion

RCCCC cases were similar to classical clear cell renal carcinoma. Radical nephrectomy should be avoided in patients with MCRCC, and radical nephrectomy should be chosen in patients with RCCCC, with postoperative and close follow-up. Unroofing decompression of renal cyst was performed during the operation, and the risk of tumor implantation and metastasis was worried after the operation. The patient agreed to receive eight cycles of immune checkpoint inhibitor therapy after surgery. Adrenal insufficiency occurred after 8 cycles of immune checkpoint inhibitor therapy(ICIs), then the immunotherapy was discontinued.

Conclusion

RCCCC is a rare and special type of renal clear cell carcinoma, and its prognosis is the same as that of renal clear cell carcinoma. The preoperative diagnosis of RCCCC mainly depends on imaging examination (CT or B-ultrasound). The early differential diagnosis from multilocular cystic renal cell carcinoma is difficult, and the diagnosis usually depends on postoperative pathological diagnosis.

Highlights

• The patient was diagnosed with a right renal cyst after physical examination, which was diagnosed as a renal cyst(Bosinak II grade F) by imaging examination.In the operation,rapid pathology revealed multilocular cystic renal cell carcinoma(MCRCC) with low malignant potential during laparoscopic right renal cyst decompression.

• RCCCC(clear cell carcinoma cystic lesion of kidney) is a rare and special type of renal clear cell carcinoma, and its prognosis is the same as that of renal clear cell carcinoma.

• Deroofing decompression of renal cyst was performed during the operation, and the risk of tumor implantation and metastasis was worried after the operation.The patient agreed to receive immune checkpoint inhibitor therapy(ICIs) after surgery.

• The early differential diagnosis from multilocular cystic renal cell carcinoma (MCRCC) is difficult, and the diagnosis usually depends on postoperative pathological diagnosis.

Keywords

Renal cyst
Multilocular cystic renal neoplasm
Multilocular cystic renal cell carcinoma
Antidiastole
Clinical pathology
Case report
Immune checkpoint inhibitors
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pmc1 Introduction

Cystic renal cell carcinoma (CRCC) is a tumor that grows primarily in a cystic manner, which accounts for 5 % to 10 % of all renal carcinoma. Its clinical symptoms are similar to those of kidney cancer, and it usually occurs in middle-aged and elderly people. Renal clear cell carcinoma undergoing cystic change (RCCCC)is the more common type [1]. Multilocular cystic rena1 cell carcinoma (MCRCC)accounts for 1 % to 2 % of all renal carcinoma [2]. MCRCC is currently classified as a subtype of clear cell carcinoma, and mostly diagnosed by chance. Although the incidence of MCRCC is low and it is difficult to diagnose accurately, it is even rarer to confuse RCCCC with MCRCC. This paper reported a RCCCC case diagnosed intraoperatively as MCRCC. Preoperative imaging and immunohistochemical staining are important for accurate diagnosis.

2 Case presentation

A 57-year-old male patient who was hospitalized after clinical examination of a right kidney cyst >3 years ago. The patient was diagnosed with a right kidney cyst 3 years ago and received no treatment. Computer-tomography (CT) imaging showed a circular low-density shadow in the right kidney. The size was approximately 6.7 cm × 4.8 cm, protruding outwards, and the CT value is about 5Hu. The patient had no special medical record. Specialist physical examination revealed the following results: There was no swelling, mass, tenderness or percussion pain in the bilateral renal area and bladder area. Biochemical examination showed no abnormality. Color ultrasound of urinary system detected was detected a cystic dark area in the right kidney. The size of the cystic dark area was about 8.5 cm × 7.6 cm. Multiple compartments could be seen in the cystic dark area. No light spot separation was seen in the collecting system of both kidneys (Fig. 1A). Enhanced urinary CT detected a cystic low density shadow in the right kidney (Fig. 1B). The size of low density shadow was about 8.3 cm × 5.6 cm. Most of shadow protruded outside the kidney profile. Enhanced CT of the cyst at arterial phase was about 17 Hu (Fig. 1C). Small compartments were visible in the cyst. Right renal cyst was classified as Bosinak II grade F. No tumor cells were found in the ureter, Perirenal fat sac and renal pelvis.Fig. 1 Preoperative imaging images. A. Right kidney color ultrasound examination of low density cystic shadow of right kidney. B. CT scan of the kidney showed a cystic shadow of the right kidney, most of which protruded from the kidney surface. C. Enhanced CT examination of the kidney revealed cyst compartmentation and weak enhancement of the cyst wall. Right renal cyst was classified as Bosinak II grade F.

Fig. 1

3 Treatment and outcome

Preoperative diagnosis of the patient was right renal cyst, and laparoscopic decompression of the right renal cyst was performed. During the operation, the cystic mass in the upper poles of the right kidney was found to protrusion. The size of cystic mass was about 8 cm × 7 cm (Fig. 2A). The cyst wall was resected for rapid pathological examination. Rapid intraoperative pathology: There was clear cell tumor in the right kidney. The tumor type was considered to be low grade malignant potential multi-locular cystic renal tumor. After communicating with the patient's family members during the operation, radical resection of the right kidney was performed, and the right kidney was removed for examination (Fig. 2B).Fig. 2 Intraoperative cyst pictures and postoperative kidney specimens. A Intraoperative cysts protruded from the contour surface of the kidney, and the cyst wall was thin. B Cystic renal cell carcinoma has clear boundaries, internal compartments are visible, and there are no obvious dilated nodules in the lesion.

Fig. 2

Postoperative pathological report revealed multilocular cystic clear cell carcinoma of right kidney. Mostly fibrous tissue formed the wall of cyst (Fig. 3A,B). The cyst wall was lined with a single layer of transparent cells. Multiple dilatative clear cell nodules were visible in the cyst wall. The maximum diameter of the clear cell lesion was about 2 mm. Immunohistochemistry for tumor was positive for CK, CAIX, CD34, PAX-2 (Fig. 4A), PAX-8 (Fig. 3B), CD10 (Fig. 4C), CK7 (Fig. 4D), P504S, and EMA.Fig. 3 HE standing. The cyst is lined by neoplastic cells with clear cytoplasm and Fuhrman nuclear grade I.A.x100, B.x200;

Fig. 3

Fig. 4 Immumohistochemical staining. A. Positive staining of PAX-2. B Positive staining of PAX-8. C. Positive staining of CD10. D. Positive staining of CK7.

Fig. 4

Adrenal insufficiency occurred after 8 cycles of immune checkpoint inhibitor therapy, then the immunotherapy was discontinued. No recurrence or metastasis during 1 year follow-up. The work has been reported in line with the SCARE criteria [3].

4 Conclusions

It has been reported that cystic renal carcinoma is divided into four types: single-locular cystic renal carcinoma, multilocular cystic renal carcinoma, cystic lesion of renal carcinoma and single-locular renal cyst mural carcinoma nodules [4]. In the 2004 WHO Classification of Renal Tumors, multilocular cystic renal carcinoma was classified as a separate type of renal cell carcinoma, and the concept of cystic renal carcinoma was no longer mentioned [5]. In 2016, the latest pathological classification of WHO/ISUP changed the name of multilocular cystic renal carcinoma to multilocular cystic renal neoplasm of low malignant potential, and the morphological diagnostic criteria were complete multilocular cystic tumor, lined with a single layer of tumor cells (occasionally multiple layers), and WHO/ISUP nucleolar grade 1 or 2. The causes of cystic renal carcinoma are as follows: (1) Simple renal cyst becomes cancer, the tumor originates from the epithelial cells of the cyst and grows irregularly; (2) Rapid growth of renal carcinoma tissues but insufficient blood supply, resulting in necrosis and formation of cystic changes which is cystic changes of renal carcinoma, the cyst wall is thick and uneven; (3) The tumor grew like a cyst, forming multiple but disconnected cystic cavities, showing multilocular masses, and small focal cancer cells could be seen in the cyst wall or interval [6].

The clinical characteristics of MCRCC are low TNM stage and good prognosis. The common symptoms of MCRCC are lack of specificity, most of MCRCC are found by physical examination or asymptomatic abdominal mass, and only a few case have hematuria and lumbar discomfort. RCCCC is caused by rapid tumor growth, bleeding and necrosis. The metastasis and mortality of cystic renal clear cell carcinoma is around 40 % [[7], [8]]. Clear cell renal cell carcinoma is a tumor with solid areas, common bleeding, necrosis, and sometimes cystic formation. Its clear nucleus has a large atypia, and its Fuhrman grade is often II and III. Currently, mural or septal nodules with a diameter of >5 mm are considered to be the main radiographic differentiators between RCCCC and MCRCC. However, in the imaging examination of this patient, there was no obvious enhanced nodule in the capsule wall, which affected the preoperative diagnosis. In RCCCC, the size of the cystic cavity varies, the distribution is uneven, and there is no obvious fibrous tissue separation. The tumor cells are mainly solid clear cell regions, and the nuclear heteromorphism is more significant than that of MCRCC [9]. Immunohistochemical results showed that positive distal nephron markers (CKl9, AEl/AE3, and EMA) were detected in MCRCC, while positive proximal nephron markers (CDl5 and CD10) were found in RCCCC [[10], [11]]. If a substantial area of tumor cells or a swollen nodule of tumor cells is present in the lesion, RCCCC should be considered instead of MCRCC. The difference between RCCCC and MCRCC is to look for neoplastic clear cell nodules in the interval. In the postoperative pathological section of this patient, multifocal dilatative clear cell nodules were observed under the microscope. The maximum diameter of the clear cell lesion was about 2 mm, so it was considered to be RCCCC.

MCRCC can be cured by surgical procedures including radical nephrectomy, simple nephrectomy, and nephron-sparing surgery (NSS). At present, more and more scholars argued that MCRCC should be treated with conservative surgery (such as NSS) [[12], [13]]. MCRCC has a good prognosis with a 5-year survival rate of over 90 %. RCCCC cases are similar to classical clear cell renal carcinoma, and the 5-year survival rate is significantly lower than MCRCC cases. Radical nephrectomy should be avoided in patients with MCRCC, and radical nephrectomy should be chosen in patients with RCCCC, with postoperative and close follow-up. On April 02, 2024, the China National Medical Products Administration approved the new indication of toripalimab (trade name: Tuyi ®, product code: JS001) combined with axitinib for the first-line treatment of patients with intermediate and high-risk unresectable or metastatic renal cell carcinoma. Unroofing decompression of renal cyst was performed during the operation, and the risk of tumor implantation and metastasis was worried after the operation. The patient agreed to receive immune checkpoint inhibitor therapy(ICIs) after surgery. Adrenal insufficiency occurred after 8 cycles of immune checkpoint inhibitor therapy, then the immunotherapy was discontinued.

MCRCC cannot reliably be distinguished from RCCCC neither by physical examination nor by radiologic evaluation. Immunohistochemical staining assay is useful to differentiate between MCRCC and RCCCC. Accurate diagnosis is always important in a cancer context even the low malignant potential of this tumors.

Abbreviations

RCCCC renal clear cell carcinoma undergoing cystic change;

MCRCC multilocular cystic renal cell carcinoma;

CRCC cystic renal cell carcinoma

CT computer-tomography;

ICIs immune checkpoint inhibitor therapy;

Ethical approval

The study is approved by the Ethical Committee of Shandong Provincial Third Hospital.

Funding

Not applicable.

Author contribution

YJS designed the research study and wrote the manuscript; MKS analyzed and interpreted the data; BQX, HZ, LHL, YLG, performed research. All authors reviewed the manuscript. All authors read and approved the final manuscript.

Guarantor

Yunji Sun accepted full responsibility for the work.

Ethics approval and consent to participate

Not applicable.

Patient consent for publication

Signed informed consent was obtained from the patient for publication of this case report and any accompanying images.

Conflict of interest statement

The authors declare that they have no competing interests.

Data availability

All data generated or analyzed during this study are included in this published article.

Acknowledgements

Not applicable.
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