Simultaneous robotic resection of pheochromocytoma and gastrointestinal stromal tumour in an adult neurofibromatosis type 1 patient: A single-docking procedure using da Vinci XI platform.

The risk of tumours, including pheochromocytoma and gastrointestinal stromal tumour is higher in patients with neurofibromatosis Type 1 (NF1). The co-occurrence of these two tumours is rare, and most patients are symptomatic. In this case report we describe the case of an asymptomatic 42-year-old female patient with left adrenal mass and concurrent lesion in the jejunum, just distal to the ligament of Treitz. Both tumours were successfully simultaneously removed by totally robotic transperitoneal technique using da Vinci Robotic Surgical System Xi.

INTRODUCTION

Neurofibromatosis (NF) is an autosomal dominant disease with three known types, NF1, NF Type 2 and schwannomatosis. NF1, also known as von Recklinghausen disease, is a genetic disorder caused by mutation of a gene neurofibromin and is the most common of the three types, comprising of 90% of all cases. The incidence of NF1 is 1 in 2699 births.[1] NF1 affects multiple organs, most commonly skin, bones and nervous system. The disease is also known to predispose to various tumours.[2]

CASE REPORT

We present a case of a 42-year-old female patient with NF1 and coincidentally discovered jejunal gastrointestinal stromal tumour (GIST) and pheochromocytoma [Figure 1a and d], respectively]. This is a rare combination with only 23 cases described in the literature.[3] Further laboratory investigation established gland hypercatecholaminemia, while thorough physical examination revealed orthostatic hypertension as well.

Figure 1

(a) Computed tomography scan of the abdomen showing jejunal gastrointestinal stromal tumour (white arrow). (b) Starting segmental intestinal resection. (c) End-to-end anastomosis. (d) Computed tomography scan of the abdomen showing left adrenal mass (yellow arrow). (e) Adrenalectomy- dissected/clipped adrenal vein (blue arrow). (f) Adrenalectomy- finishing stage

Surgery was performed by a single-docking, totally robotic transperitoneal technique using da Vinci Robotic Surgical System Xi (Intuitive Surgical System, Sunnyvale, CA, USA). The patient was secured on a special no-slip foam and placed in the right lateral decubitus position. The robotic cart was docked over the left shoulder. After establishing pneumoperitoneum by Veress needle, four 8 mm robotic ports (RP) and additional 12 mm port (AP) were inserted. Configuration of two-right handed instruments was employed, with fenestrated bipolar forceps, monopolar curved scissors and tip-up fenestrated grasper being placed into RP 1, 3 and 4, respectively. The endoscope (30° optics) was placed in RP number 2. Valve-less trocar design of 12 mm AP (AirsealR, Applied Medical, USA) provides for high flow insufflation and facilitates smoke evacuation. Moreover, it allows the assistant unimpeded introduction of laparoscopic instruments. At first, segmental resection of jejunal GIST, just distal to the ligament of Treitz, was performed [Figure 1b]. Resected small bowel was safely placed in an endo bag and intestinal continuity was restored by end-to-end anastomosis with interrupted, PDS 4.0 sutures [Figure 1c]. Thereafter, a medial colonic mobilisation was performed for perfect gland exposure. Splenorenal ligaments and gerotasfascia were divided with dissection continued into periadrenal fat to identify adrenal vein (AV) at its junction with left renal vein. The AV was carefully dissected and clipped with standard laparoscopic clips by the bedside assistant [Figure 1e]. Vessel sealer was used for further dissection, avoiding manipulation of tumour [Figure 1f]. Removed mass was placed in the second bag and, after haemostasis was confirmed, both retrieval bags were delivered through auxiliary port site.[4] The patient was perioperatively monitored, and there was no significant fluctuation of blood pressure or other adverse events. Post-operative course was uneventful, and the patient was discharged on postoperative day three.

Histopathological evaluation revealed two GISTs in jejunum measuring 5 mm and 24 mm in diameter and pheochromocytoma of adrenal gland measuring 37 mm. The latter showed diffuse chromogranin positivity, and its Pheochromocytoma of the Adrenal gland Scaled Score was <4, which indicates benign behaviour. GISTs showed spindle cell morphology with skenoid fibres and diffuse immunohistochemical positivity to CD117 and DOG1 and did not show any poor prognostic features such as necrosis, mitoses, size >5 cm or nuclear atypia.

DISCUSSION

GISTs occur in patients with NF1 with a prevalence from 5% to 30%, while the risk of pheochromocytoma in these patients is even smaller as it is diagnosed in approximately 0.1% to 6%. However, albeit rarely, these two tumours can concomitantly occur in NFB I patients, and pheochromocytoma should always be excluded in before these patients undergo surgery for GIST as undiagnosed pheochromocytoma carries a high risk of life-threatening complications during surgery. Our experience, as well as results in the literature, confirm robotic approach as a feasible and well-tolerated modality for the management of adrenal masses.[5] To the best of our knowledge, this is the first report in the literature describing the simultaneous robotic operation of the aforementioned tumours in NF1 patients. The versatility of the da Vinci Xi with integrated accessories allows such a complex surgical procedure to be executed in a safe, precise and effective way.

Consent

Written informed consent was obtained from the patient for publication of this article and any accompanying images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.