The retroperitoneum is a closed space harbouring vital organs including the great vessels, kidneys and adrenal glands, ureters, and the ascending and descending colon. Surgical management of retroperitoneal pathologies may need multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs should be attempted, especially in case of benign tumors. We present a case of 15-year-old girl with an 11 × 6 × 5 cm retroperitoneal ganglioneuroma displacing the right kidney, renal vein, and ureter and abutting the IVC which was excised in toto preserving the right kidney and ureter with careful dissection around the great vessels. We also attempt to review the various surgical options available while dealing with these benign retroperitoneal tumors which are often detected incidentally and usually surround important retroperitoneal organs and vessels.
The retroperitoneal space harbours various vital structures including the great vessels, kidneys and adrenal glands, duodenum, ascending and descending colon, and the ureters. This calls for extreme caution and expertise while dealing with pathologies arising from this space. Management of tumors arising in the retroperitoneal space often requires multiorgan resection and reconstruction in order to achieve complete resection of the tumor [
We present a case of a 15-year-old girl with a retroperitoneal mass displacing the right renal vein and ureter, compressing the inferior vena cava and infiltrating the right psoas muscle. The tumor measuring 11 × 6 × 5 cm was excised with careful dissection around the IVC and preservation of the right kidney, ureter, and right renal vein. Histopathology revealed retroperitoneal ganglioneuroma with no undifferentiated component.
A 15-year-old female presented with complaints of pain in the right lower limb and back for 1 month. Ultrasound revealed a retroperitoneal mass adjacent to the lower pole of the right kidney. CT scan revealed a large heterogeneous lobulated mass with intralesional cystic areas of necrosis and heterogeneous postcontrast enhancement in the retroperitoneum measuring 11 × 5.5 × 6 cm in size on the right side at the level of mid and lower pole of the kidney. There was no evidence of calcification in the lesion. The mass had extraneous indentation of the inferomedial cortex of the right kidney. The mass displaced the right renal vein anteriorly and the right proximal ureter anterolaterally with compression of the inferior vena cava anteromedially. Medially, the mass was seen to be infiltrating the psoas muscle and abutting the right lateral cortical margin of the L4 vertebra. The right adrenal gland was seen separately and appeared normal (Figure
Axial and coronal sections on CECT abdomen and pelvis showing the extent of the tumor.
Serum cortisol and serum and urine normetanephrines were normal, ruling out a functional tumor. The patient underwent USG-guided core needle biopsy which revealed retroperitoneal ganglioneuroma as presence of mature ganglion cells in a Schwannian stroma with no evidence of mitosis/necrosis.
The patient underwent exploratory laparotomy through anterior subcostal incision. The mass was found to be displacing the right kidney and proximal ureter laterally with anterior displacement of right renal vein. The right ureter was looped using a vascular sling and traced upwards to identify the right kidney and the right renal vein. The mass was carefully dissected from the surrounding structures using electrocautery and the base of the mass infiltrating the psoas muscle was fulgurated.
The mass was encapsulated and measured 11 × 6 × 5 cm in size. It had a bosselated surface. The cut surface was homogeneous and soft to firm in consistency with no haemorrhage or necrosis (Figure
(a) The intraoperative findings: left arrow indicating the retroperitoneal mass; right arrow indicating the ureter. (b) The ureter looped and traced. ((c), (d)) Tumor excised and base fulgurated. (e) The resected tumor. (f) Cut section of the tumor.
The postoperative course of the patient was uneventful. The histopathology revealed a biphasic tumor with ganglion cells and Schwannian stroma. The ganglion cells were mature with eccentric nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. No undifferentiated component was seen in the tumor. The final histopathological diagnosis was retroperitoneal ganglioneuroma.
Postoperatively, the patient has been relieved of her symptoms, and in the two years of follow-up, there is no evidence of local or distant recurrence so far.
Ganglioneuroma is a rare (one per million population [
Patients with ganglioneuroma are usually asymptomatic or present with nonspecific symptoms. Symptoms occur as a result of mass effect and compression of surrounding organs with signs and symptoms predictable when tumor enlargement causes elevation of the diaphragm, interference with portal structures, distortion and subsequent disturbance in functions of the genitourinary or gastrointestinal tract, pressure on the spinal cord, peripheral nerves or nerve plexuses, and distortion and erosion of bony structure [
The imaging modality of choice is contrast enhanced CT scan and MR imaging. They help in identifying the relationship of the tumor with surrounding organs and vital structures. The surgical approach to the tumor is also decided on the basis of the imaging findings. On CT and MR images, ganglioneuromas usually present as an oval, well-defined mass in the adrenal gland or in extra-adrenal location. Ganglioneuromas characteristically have low attenuation on unenhanced CT scans [
The definitive diagnosis is made on histopathology. In our case, an ultrasound guided biopsy was done prior to going in for surgical resection to confirm the diagnosis. The treatment for retroperitoneal ganglioneuromas is surgery and they bear excellent prognosis following complete surgical excision [
The surgical resection of these tumors can be undertaken via laparoscopic or open technique. Laparoscopic surgery is increasingly becoming common for retroperitoneal pathologies with increasing expertise and availability of advanced laparoscopic instruments and energy sources. In our case, open surgery was conducted owing to the close relation of the tumor with the IVC and displacement of the right renal vein and right ureter along with the large size of the tumor.
Alimoglu et al. [
Open surgery has often been preferred while operating on tumor in close proximity of or surrounding important blood vessels in the abdominal cavity where laparoscopy may face limitations. Vasiliadis et al. [
Some authors have also described novel techniques in order to achieve complete resection without compromising the surgical clearance and also preserving surrounding structures. Wan et al. [
Oue et al. [
Major vascular resections and prosthetic replacements have also been performed for retroperitoneal tumors. Fueglistaler et al. [
Certain principles that may be kept in mind while operating on benign tumors in the retroperitoneum are as follows: Thorough knowledge of the retroperitoneal anatomy, along with careful and meticulous dissection, is an absolute must in order to avoid injury. CECT and 3D reconstruction may help the surgeon in identifying the relationship of the tumor with the surrounding structures and plan the approach to the tumor preoperatively. Vascular structures and ureter may be looped using slings; ureter can also be stented using DJ stents or ureteral catheters. Threshold is low for conversion to open surgery when laparoscopic approach was used in favour of avoiding injury to neurovascular structures. Use of energy sources such as harmonic scalpel may be preferred avoiding lateral thermal injury. Adherence to oncologic principles and complete surgical excision as residual tumor may lead to local recurrence.
Complete surgical resection of benign retroperitoneal tumors calls for meticulous and novel surgical techniques in order to preserve surrounding vital organs. With increasing expertise, this may be achieved laparoscopically as well but each case must be individualized on a case-to-case basis without compromising on oncological principles while dealing with these tumors.
An informed consent was taken from the patient and her relative before drafting of this manuscript.
The authors declare that there is no conflict of interests regarding the publication of this paper.