Aortic Resection with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: a Case Report

Background

Involvement of major retroperitoneal vessels due to extension of gastrointestinal (GI) malignancies is considered a contraindication for surgery in majority of the instances with few exceptions. Locally, advanced pancreatic cancer is one such GI malignancy where vascular resection is well established even with multi-visceral resections in select patients [1]. Retroperitoneal sarcomas are the other set of diseases wherein vascular resection is usually undertaken because of the proximity of these tumours to major retroperitoneal vessels. Such a complete compartmental excision including vascular structures gives better local control [2]. These vascular resections are done in the absence of disease anywhere else in the abdomen, i.e. liver, peritoneum and mesentery. Whether a similar approach can be adopted by combining major vascular resections with CRS (cytoreductive surgery) and HIPEC (hyperthermic intraperitoneal chemotherapy) to achieve good outcomes has not been studied. In this report, we present a case of CRS and HIPEC combined with major retroperitoneal vascular resection for metastasis of mucinous adenocarcinoma of GI origin.

Case Report

A 56-year-old lady was under evaluation for chronic complaints of pain in the abdomen and vomiting related to repeated bouts of subacute intestinal obstruction. Contrast-enhanced computed tomography (CECT) of the abdomen and pelvis revealed a mesenteric mass (8.9 × 7.3cm) with extension into the retroperitoneum abutting the aorta, with bilateral ovarian masses. Serum CA 125 was 344 U/ml, and CEA was 7.6 ng/ml. A CT-guided biopsy revealed mucin secreting moderately differentiated adenocarcinoma. She received 3 cycles of platinum-based chemotherapy in an outside centre, with a working diagnosis of ovarian mucinous adenocarcinoma. When she had no response to this treatment, she was referred to our centre. A repeat CT scan showed progression of disease with retroperitoneal extension of mesenteric mass now circumferentially encasing the aorta, with partial encasement of the inferior vena cava and right-sided hydronephrosis. There was an increase in the peritoneal extension with deposits over the pelvic peritoneum, anterolateral parietal peritoneum and diaphragmatic peritoneum and increase in the sizes of ovarian deposits. A biopsy was repeated, which revealed adenocarcinoma with extracellular mucin. The tumour expressed MUC2 and MUC4 diffusely and MUC5AC focally and was negative for MUC1, MUC6 and PAX8 indicating a GI origin.

The patient was taken for exploration with a plan of CRS and HIPEC. Major vascular resection was anticipated and planned if R0 resection was possible. Possibility of inoperability due to vascular involvement was also discussed with the patient.

The patient was explored with midline laparotomy incision from xiphisternum to pubic symphysis. The findings included growth near the distal ileum, which was abutting the mesentery of the small intestine (Fig. 1) involving major branches of superior mesenteric artery (SMA) supplying the distal small intestine. Appendix was seen separately. Ileal mass was infiltrating into the retroperitoneum to involve the aorta starting from just below the inferior mesenteric artery (IMA) including IMA, aortic bifurcation, proximal parts of both common Iliac arteries and the part of circumference of inferior vena cava (IVC). Bilateral ureters were adherent to the lesion with hydronephrosis of the right ureter. Multiple peritoneal deposits were seen in the pelvic peritoneum, uterus, omentum and anterolateral peritoneum. Peritoneal carcinomatosis index (PCI) was calculated to be 19.

Fig. 1.

Showing the distal ileal mass infiltrating the retroperitoneal major vessels a on initial exploration. Mass (black arrow) seen infiltrating the retroperitoneal vessels. Normal elongated appendix can be seen over the mass (white arrow). b After dissection and small bowel division. Blue arrow showing the beginning of aortic infiltration. Prolene tie on the root of inferior mesenteric artery can be seen

Resection performed included en bloc excision of mass involving a long segment of the ileum along the aorta and its bifurcation and partial circumference of the inferior vena cava (IVC), pelvic peritonectomy, omentectomy and cholecystectomy. IVC was repaired using 5-0 Prolene. Aorto-iliac grafting was done (Fig. 2). An experienced vascular surgeon performed the vascular reconstruction. Bilateral ureteric stenting was done with D-J stents. After attaining a complete cytoreduction (CC-1), HIPEC was given by open method given with mitomycin (15 mg/m²) and adriamycin (15 mg/m²) at 42–43 °C for 60 min. Intra-operative period was smooth.

Fig. 2.

Showing vascular reconstruction. Aorto-bifemoral Dacron graft (black arrow) was used for arterial reconstruction, while the inferior vena cava was repaired primarily (white arrow—root of the inferior mesenteric artery is indicated with white arrow head)

Post-operatively, there was a spontaneous perforation of the jejunum, which is a known complication of HIPEC. However, this was successfully managed with resection anastomosis, and the patient had an uneventful recovery thereafter.

Final histopathology report showed a low-grade mucinous adenocarcinoma of the distal ileum, invading the tunica media of the resected aortic segment and tunica adventitia of the resected IVC wall. Resected ileal margins showed small foci of tumour deposits. The right ovary showed a focus of low-grade mucinous adenocarcinoma. Even the pelvic peritoneum separately sent ureteral deposits, and the omentum were positive for adenocarcinoma. All retroperitoneal nodes were negative. IHC panel was the same as that of the image-guided biopsy specimen.

The patient successfully started adjuvant chemotherapy after 2 months of surgery. At first evaluation after 4 months of surgery, the tumour marker levels were within normal limits, and a CT scan of the abdomen and pelvis did not reveal any evidence of recurrence. She remained disease-free for 13 months after which recurrence was found at the root of the small bowel mesentery and sigmoid mesentery. She is at present alive with the disease.

Discussion

Major arterial vascular resection with reconstruction in patients with gastrointestinal malignancy is rarely performed and infrequently reported. In locally advanced pancreatic cancers, porto-mesenteric resections are common, but arterial resections are infrequent and are to be done only in super selected patients in experienced centres only [1]. In retroperitoneal tumours 18% cases have a major vascular involvement requiring vascular resection wherein again the venous involvement (IVC) is more common than the arterial [3]. Vascular resections have also been reported in germ cell tumours RPLND [4], as well as a part of cytoreduction in ovarian malignancies [5]. In colorectal cancers majority of the reported cases have been for local recurrence of colorectal cancers with vascular involvement in the absence of peritoneal or metastatic disease [6]. However, presence of peritoneal disease along with vascular involvement presents a major therapeutic challenge since mucinous adenocarcinomas of GI origin are often chemotherapy resistant [7].

Small bowel adenocarcinoma is a rare disease with limited data on management. However, it can frequently present with peritoneal deposits [8]. In peritoneal metastases of appendicular mucinous adenocarcinoma origin, complete cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been demonstrated to have a significant survival benefit and are considered standard of care [9]. Similar analogy can be drawn to manage rarer diseases like small intestinal adenocarcinoma to achieve better outcomes. Better outcomes have been shown with CRS+HIPEC for small bowel malignancy compared with conventional treatments [8, 10, 11], since they are known to be relatively chemoresistant.

The incidence of combined vascular and peritoneal involvement is not known. And, to our knowledge, there are no reports till date on the value of performing this surgery in the presence of retroperitoneal vascular involvement. The decision to go ahead with vascular resection in this case was to achieve R0 resection as the rest of the disease was completely excised. Several series have evaluated the value of an aggressive surgical approach towards resection of the aorta, IVC and iliac vessels for retroperitoneal sarcomas. In high volume centres, this strategy has been found to be safe with acceptable morbidity and mortality [12].

Previous studies have shown that when an experienced team of vascular and oncosurgeon perform a synchronous resection of the tumour and major blood vessels along with reconstruction, the morbidity and mortality of the procedure is not increased [13]. An experienced vascular surgeon performed the reconstruction in the present case. There were no intra-operative or post-operative complications with this strategy. HIPEC was decided after confirming uneventful surgery taking into account the blood loss, anastomosis, PCI and inefficacy of systemic therapy.

Graft-related complications can occur in 6–35% of cases [14–17]. There were no graft-related complications in this case. The patient successfully started adjuvant chemotherapy after 2 months of surgery.

Animal studies on anastomotic healing after HIPEC have given mixed results regarding wound strengths after HIPEC [18–20]. There is no data on how HIPEC affects in situ vascular grafts. Effect of hyperthermia and toxic drugs on vascular grafts has never been studied; however, the modern-day stents are known to withstand considerable degrees of physical insults.

Previous large series of CRS and HIPEC for appendiceal mucinous adenocarcinomas and several small case series of CRS+HIPEC for small bowel adenocarcinoma have demonstrated a significant long-term survival benefit if a complete cytoreduction can be achieved. We present this case to demonstrate that so long as a vascular resection and reconstruction can be safely achieved, the presence of vascular involvement should not be a contraindication to considering a radical surgery with HIPEC. In highly selected cases, this can offer a potential survival benefit.

Conclusion

Vascular resection as a part of CRS and HIPEC for peritoneal metastases can be considered in selected cases with acceptable morbidity and mortality and can offer a potential survival benefit. Synergy between the oncosurgeon and vascular surgeon is essential for a successful outcome. This area needs to be explored further to assess the impact of vascular resections on immediate and long-term outcomes. It may broaden the scope of aggressive surgical resections in such diseases.

Abbreviations

CRS

Cytoreductive surgery

HIPEC

Hyperthermic intraperitoneal chemotherapy

CECT

Contrast-enhanced computed tomography

Compliance with Ethical Standards

Declaration of Interest

The authors declare that they have no conflict of interest.