Radical laparoscopic nephrectomy for a huge left renal mass in situs inversus totalis: A case report and video presentation

Abstract

This case report describes the successful laparoscopic radical nephrectomy in a 69-year-old woman with situs inversus totalis and a large, hypervascular left renal mass. Despite the presence of metastatic disease, the patient underwent neoadjuvant immunotherapy followed by surgery. The successful laparoscopic approach highlights the feasibility of minimally invasive surgery even in patients with complex anatomical variations. This report provides valuable insights into the surgical management of renal tumors in individuals with situs inversus totalis.

1 Introduction

Situs inversus totalis (SIT) is a congenital abnormality characterized by a mirror-image transposition of both the abdominal and thoracic organs. Various malformations have been associated with SIT, including cardiac, splenic, and gastrointestinal malformations. The incidence of SIT is approximately one in every 5000 to 20,000 live births. SIT is typically an incidental finding and presents asymptomatically. Despite its historical documentation, this anomaly remains underrecognized within the medical community due to its rare occurrence.

While the exact cause of situs inversus totalis is not yet understood, a mutation in the DNAH11 gene has been identified as a contributing factor in at least one form of the condition. Additionally, a potential links between situs inversus and other genetic components, including lefty genes, nodal genes, ZIC3, ACVR2B, and Pitxz. At the time of gastrulation, latality is established at an early stage of development during the formation of the primitive streak. A cascade of genes and signaling molecules is required.

Renal cell carcinoma (RCC) is a prevalent form of malignancy that causes a considerable number of deaths worldwide, with 81,000 new cases and 15,000 fatalities occurring each year; and had increased by more than 30 % in the last two decades. There has been a significant increase in RCC incidence in the sixth decade, with almost twice as many cases among men as among women. Most cases of renal cancer are detected incidentally; the classic triad of symptoms associated with renal cancer, i.e., hematuria, flank pain, and palpable mass, is an uncommon finding, usually associated with patients with advanced disease. In this report, we describe a patient with situs inversus totalis and a large, hypervascular left renal mass, as well as a brief review of the relevant literature, the clinical presentation, radiological findings, laboratory findings, and surgical approach.

2 Case report

A 69-year-old postmenopausal woman with a history of diabetes mellitus and hypothyroidism presented to the emergency department with a two-month history of left flank pain. This pain was not associated with hematuria, fever, back pain, weight loss, or night sweats. Her surgical history was unremarkable, her social history was non-contributory, and her family history was devoid of any relevant conditions. On physical examination, the patient was alert, oriented and afebrile, and her vital signs were within normal limits. A local abdominal examination revealed a soft abdomen with no masses. The remainder of the physical examination was unremarkable.

Laboratory evaluation, including complete blood count, serum chemistry, renal function panel, and liver function tests, yielded normal results. An enhanced computed tomography (CT) scan of the abdomen and pelvis demonstrated features of situs inversus totalis. There is a large hypervascular locally advanced left-sided renal mass containing a central necrotic component and tiny calcifications, currently measures 10.5 x 10.2 × 12.6 cm compared to 11.7 x 10.3 × 18 cm with extension beyond the posterior pararenal fascia and abutting the left side lower intercostal muscle and left hepatic lobe with minimal invasion. The renal hila exhibit typical vascular anatomy, with a single renal artery and vein present. The left gonadal vein drains into the left renal vein, while the right gonadal vein drains into the inferior vena cava. ( Fig. 1 (A and B)). There is a 5.8 cm pelvic mass centered at the upper vagina and cervix, which currently no measurable mass could be identified. Stable endometrial thickening measures 1 cm. Biopsies from the kidney and vagina confirmed metastatic clear cell renal cell carcinoma (CCRCC) in both sites.

The patient underwent nivolumab-ipilimumab therapy followed by a left laparoscopic radical nephrectomy. Positioned in the right lateral decubitus position, four trocars were inserted: two 12mm ports for the camera and primary working instrument and two 5mm ports for assistance. Intraoperative findings revealed distorted anatomy with the liver situated on the left side and partial visualization of the left kidney. Following medial mobilization of the colon, the gonadal vessels and inferior vena cava were identified. A lower window was created after identifying the ureter and exposing the psoas muscle. The ureter was retracted, and dissection proceeded superiorly, identifying and controlling the lower pole renal vessels with Hem-o-lok clips prior to reaching the renal hilum. The upper pole dissection proved challenging due to hepatic displacement into the surgical field. Following the incision of the hepatorenal ligaments, an additional 5mm port was inserted for liver retraction. The upper pole renal vessels were then identified, clipped with Hem-o-lok clips, and dissected. The renal hilum was secured using a stapling device. The kidney was wholly mobilized, placed within a laparoscopic bag, and extracted. Total surgical time was approximately 60 minutes (skin-to-skin). No drain was placed. Fig. 2 shows the gross specimen of the left kidney following laparoscopic left radical nephrectomy. Visual documentation of the radiological imaging findings and surgical technique is available in the supplementary material (Video 1).