Bilateral Laparoscopic Nephrectomy in Autosomal Dominant Polycystic Kidney Disease with Bilateral Renal Masses: A Feasible Option

Abstract

Background: Autosomal dominant polycystic kidney disease (ADPKD) is most common potentially lethal cystic disease occurring in ∼1 in 1000 live births. It is an important cause of end-stage renal disease, which occurs in 75% of patients by the age of 70 years. APDPKD is a systemic disease with involvement of multiple extrarenal organs. Incidence of renal cell cancer in ADPKD is no more than in normal population. High index of suspicion is required due to gross distortion of renal architecture.

Case Presentation: We report a 56-year male, known case of ADPKD on maintenance hemodialysis presenting with hematuria. On evaluation, he was diagnosed with bilateral renal masses on contrast imaging. Bilateral laparoscopic nephrectomy was performed and specimen was retrieved from pfannenstiel incision. Histology showed papillary renal cancer in left kidney and oncocytoma in right kidney with negative margins.

Conclusion: Minimally invasive surgery in ADPKD with renal mass is challenging due to space constraints and large size kidneys. However, laparoscopic approach is a feasible option with minimal morbidity, less pain, and speedy recovery, specially in chronic kidney disease patients already immunocompromised status.

Introduction and Background

Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogenous systemic disease. About half of patients afflicted develop end-stage renal disease by the age of 60 years and require renal replacement therapy. Enlarged nonfunctioning kidneys are prone to infection, hemorrhage, and cyst rupture. Incidence of renal mass in ADPKD is no higher than normal population. Bilateral renal masses in ADPKD are unusual. Diagnosis is challenging and warrants bilateral radical nephrectomy in such cases. We report the feasibility of bilateral laparoscopic nephrectomy in ADPKD with bilateral renal masses.

Presentation of Case

A 56-year-old, chronic hypertensive male presented with multiple episodes of gross painless hematuria of 1 week duration. There was no history of pain in abdomen, vomiting, loss of weight, or appetite. Patient is a known case of ADPKD with end-stage renal disease on maintenance hemodialysis for the past 6 months. In the background history, his sister suffered from the same disease and succumbed at an early age of 42 years. Patient was receiving regular hemodialysis twice a week and his urine output was 800 to 1000 mL/day. On examination, patient was conscious, alert, pale, and average built. His vitals were stable and pulse rate was 104/min. Systemic examination was grossly normal. Routine workup, including hemoglobin was 8.1 gm/dL, total leukocyte count was 5400/cmm, serum sodium was 132 mEq, serum potassium was 3.9 mEq, serum creatinine was 6.3 mg/dL, serum calcium was 7.5 mg/dL, INR was 1.2, and his liver functions were within normal limits. Urine analysis showed fully filled red blood cells and culture was sterile. Arterial blood gas analysis showed pH—7.31, sodium bicarbonate—15, and base deficit was (−6). Ultrasound abdomen reported multiple cyst of variable size in bilateral kidneys and solid hypoechoic mass in the upper pole of left kidney. Contrast enhanced CT abdomen revealed numerous cyst (size 2 mm to 2 cm) in bilateral kidneys replacing whole of kidney parenchyma. The right kidney showed 2 heterogeneous exophytic enhancing masses (each 2.2 × 3 cm) in upper and lower pole of the kidney (HU 40–70). On the left side, there was 6 × 4 cm heterogenous mass in the upper pole of the kidney (Fig. 1A, B). Multiple cysts were present in liver suggestive of spectrum of ADPKD. Patient was counseled and planned for bilateral nephrectomy. Nephrology consult was taken and patient was started on hematinics and calcium supplements. Patient was admitted 1 day before surgery and received one session of hemodialysis and one unit of packed red blood cells. Laparoscopic approach was attempted in view of manageable sized kidneys. Under general anesthesia, patient was initially placed in left lateral position. Laparoscopy ports were placed after creating pneumoperitoneum. Standard steps were followed, including descending colon mobilization, lower pole dissection, hilar identification, clipping of renal artery and renal vein, and completion of nephrectomy (Fig. 2A). The important point to highlight in this case is careful dissection outside Gerota's fascia and prevention of injury to the multiple cysts, which replaced the renal parenchyma. Renal cyst being fragile should be handled diligently. The specimen was bagged and placed in the pelvis. Patient was then placed in right lateral decubitus position. In addition to standard laparoscopy ports, liver retraction port was placed just below the xiphisternum. The steps were followed in similar manner and nephrectomy was completed (Fig. 2B). Blood loss was minimal. Specimen was bagged and both the kidneys were retrieved from muscle splitting pfannenstiel incision (Fig. 3A, B). Postoperative hospital stay was uneventful. Patient was discharged after 72 hours of surgery. Patient was advised to continue hemodialysis and supplemented with oral hematinics and calcium. Histopathology revealed Fuhrmann grade 1 papillary type renal cancer in left kidney (T1b, Nx, Mx) with tumor arranged in the form of papillae with fibrovascular core and moderate pleomorphism. Right kidney showed oncocytoma with tumor cells arranged in form of nest and solid clusters and abundant amount of granular eosinophilic cytoplasm (Fig. 4A, B). At 6 months follow-up, patient is doing well on maintenance hemodialysis and imaging shows no evidence of recurrence. Patient is being counseled for renal transplant after 1 year of disease-free recurrence.

FIG. 1.

Contrast enhanced CT images (A, axial cuts) showing heterogenous enhancing mass in upper pole of left kidney and 2 masses in upper pole and lower pole of right kidney (white arrow) and (B, coronal images) showing multiple cyst of variable sizes in bilateral kidneys replacing the whole parenchyma and few liver cyst (white arrow).

FIG. 2.

Intraoperative images showing. (A) Left kidney with multiple renal cysts, upper polar renal mass, and visualization of renal hilum (multiple white arrow). (B) Dissection of right renal hilum with clipping of one renal vein. (white arrow).

FIG. 3.

(A) Gross specimen of bilateral kidney showing multiple renal cyst with adjacent Gerota's fascia. (B) Cut specimen of left kidney showing 5 × 4 cm variegated mass in upper pole and 2 solid mass each 2 × 2 cm in upper and lower pole of right kidney.

FIG. 4.

Histology images showing. (A) Tumor cells arranged in the form of papillae with fibrovascular core with moderately pleomorphic have round to oval nuclei, vesicular chromatin prominent nucleoli, and eosinophilic cytoplasm suggestive of papillary renal cell cancer. (B) Well circumscribed tumor arranged in form of nests and solid clusters. Tumor cells are round to polygonal with abundant amount of granular eosinophilic cytoplasm suggestive of oncocytoma.

Discussion

ADPKD is the most common cystic disease occurring in nearly 1 in 1000 live births. Approximately 78% of families with ADPKD have an abnormality on chromosome 16 (PKD1 locus). Most of the remaining families (14%) have a defect that involves a gene on chromosome 4 (the PKD2 locus), while a minority of families have a defect in the GANAB gene. Patients with PKD2 develop fewer cysts and progress more slowly than those with PKD1. The median age of the onset of end-stage kidney disease is 54 and 74 years for PKD1 and PKD2, respectively, in reported studies. Renal cell carcinoma (RCC) is a uncommon entity found in ADPKD. Although risk of developing RCC is not different from general population, distinctive features peculiar to this entity are early age of presentation, and bilateral and multifocal involvement. Chronic renal failure in ADPKD appears to have association with development of future RCC in these patients. In a study by Jilg et al.¹ of 301 surgeries in ADPKD, 21 patients were found to have malignant lesions. All the patients were on long-term dialysis (mean 46 months) with mean creatinine of 4.5 mg/dL.¹

Prevalence of RCC in ADPKD is ∼4% to 10% as described by different authors. Study into genetic mechanism involved in this transformation was done by Huh et al. who found Xp11.2 translocation in one case of RCC in ADPKD. This translocation is also found in RCC in horseshoe kidney and other scenarios as well.² Diagnosis of RCC in ADPKD is always challenging even by advanced imaging modalities due to gross distortion of renal architecture. Majority of case series published showed that cases were detected during pathologic examination done for nephrectomy due to other reasons such as infection, size, or pain. Hematuria should be taken seriously in patients of ADPKD and extensive imaging should be done to rule out malignancy. Pathologic examination in different series revealed different results. Jilg et al.¹ showed increased percentage of papillary tumors in his series (63%) than clear cell carcinoma (31%). Existence of papillary type was explained by Huh et al.,² in his article where he quoted that greater proportion of ADPKD patients have papillary-type RCC, since these phenomena have pathogenetic similarity to hyperplasia of the epithelial cells lining the cyst, which frequently form papillary-like structures in early pathologic studies. In our index case, left kidney revealed papillary tumor and right kidney tumor was oncocytoma. Oncocytoma is rare with few reported cases in current literature.

Nephrectomy for ADPKD is commonly done by open surgery due to the bulky, massive kidneys and need for the procedure on bilateral sides. These surgeries with large incisions in chronic kidney disease patients and poor wound healing increase the hospital stay and morbidity. However, laparoscopic approach is a feasible option in some instances. Elashry et al. reported the first case of laparoscopic nephrectomy for ADPKD in 1996.³ Since then, many authors have reported their experience and results with small incisions, less pain, less hospital stay, and faster recovery.⁴ Although smaller sized kidneys are suitable for the procedure, Bendavid et al. report their series of 22 patients with median kidney size of 22 cm with 18% needing conversion to open. Laparoscopic nephrectomy in ADPKD is challenging due to space constraints, risk of injury to other organs, and high risk of conversion to open surgery (9%–22%).⁵ Placement of initial Veress needle is cumbersome and use of optical trocars or Hasson's cannula has been advocated by many authors for initial access and creation of pneumoperitoneum. Negotiation of the upper pole and posterolateral region in these massive kidneys is challenging and use of hand-assisted devices or additional ports to facilitate the above has been described. Morcellation of the specimen and cyst aspiration has also been described in literature to facilitate laparoscopy in these patients. However, there is risk of peritonitis and ileus due to cyst fluid spillage and risk of spillage of tumor cells in malignancy. Laparoscopy procedures is reported to have longer operative times and is associated with a learning curve, however, in the hands of an experienced surgeon and with adequate preparation and precaution, it is safe and feasible.

Conclusion

ADPKD patients have an increased survival in this era of dialysis and renal transplantation. A high index of suspicion for RCC should be maintained when a complex cystic or solid mass lesion is noted in ADPKD on imaging studies performed for unexplained symptoms or during routine screening. Treatment of these bilateral massive kidneys with malignancy by minimally invasive means can be safely and effectively done.

Abbreviations Used

ADPKD

autosomal dominant polycystic kidney disease

CT

computed tomography

HU

Hounsfield unit

RCC

renal cell carcinoma

Disclosure Statement

Authors have nothing to disclose.

Funding Information

No funding was received for this article.

Cite this article as: Thummala Y, Parmar K, Mathew J, Tyagi S, Kumar S (2020) Bilateral laparoscopic nephrectomy in autosomal dominant polycystic kidney disease with bilateral renal masses: a feasible option, Journal of Endourology Case Reports 6:4, 353–357, DOI: 10.1089/cren.2020.0104.