Royal Australasian College of Surgeons
Large adrenal tumours mimicking hepatic malignancy: the role of concurrent adrenalectomy and anterior approach hepatectomy
Nitin Agarwal, t Nihar Mohapatra ®, Nilesh S. Patil , Piyush K. Sinha, Gattu Tharun and Viniyendra Pamecha İD
Department of Liver Transplantation and Hepato-Pancreato-Biliary Surgery, Institute of Liver and Biliary Sciences, New Delhi, India
Key words
adrenal tumour, adrenocortical carcinoma, anterior approach, hanging manoeuvre, hepatectomy, phaeochromocytoma.
Correspondence
Dr Viniyendra Pamecha, Department of Liver Transplantation and Hepato-Pancreato-Biliary Surgery, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, India. Email: viniyendra@yahoo.co.uk
N. Agarwal MS, FIAGES; N. Mohapatra MS, MCh; N. S. Patil MS, MCh; P. K. Sinha MS, MCh; G. Tharun MS; V. Pamecha MS, FRCS.
tUndergoing training in liver transplant.
Accepted for publication 16 November 2020.
doi: 10.1111/ans.16511
Abstract
Background: Large right adrenal tumours may invade the liver and mimic primary hepatic malignancy, presenting diagnostic and management challenges. The ‘anterior approach hepatectomy’ (AAH) using hanging manoeuvre may be safe for resection of such tumours. Methods: Prospectively maintained database was analysed to identify the large tumours of adreno-hepatic region subjected to hepatectomy. Clinico-biochemical, radiological and sur- gical details were reviewed. AAH and hanging manoeuvre was adopted for the patients requiring right hepatectomy.
Results: Out of the eight patients with adreno-hepatic tumour, four had indeterminate ori- gin of the mass, two had complex cysts, and two were diagnosed as hepatocellular carci- noma on the pre-operative imaging. The median age was 38 years (23-66); male:female ratio was 5:3. AAH was performed in four patients, while others underwent non-anatomical resection of involved segment(s). This accounted for 1.01% of 789 hepatectomies per- formed during the study period. The median intra-operative blood loss was 525 mL (150-2200 mL) and the median tumour size was 9 cm (6-17 cm). All patients underwent R0 resection. The final histopathological diagnoses were: three phaeochromocytomas (two malignant and one benign), two adrenocortical carcinomas, one malignant para- ganglionomas and two adrenal cysts. Major complication (grade IVa) was experienced in one patient. On median follow-up of 67.5 months (7-84), all were alive and one patient had tumour recurrence.
Conclusion: Adrenal tumours mimicking primary hepatic malignancy are rare but pose diagnostic challenges. The AAH is a safe approach with acceptable R0 resection, blood loss, surgical complications and overall outcome for the patients requiring right hepatectomy.
Introduction
The primary malignant adrenal tumours are rare tumours and include adrenocortical carcinoma, phaeochromocytoma, para- ganglioma and other uncommon tumours. These tumours are asso- ciated with poor prognosis. Among these, phaeochromocytoma has a risk of malignancy in about 10% cases (5-26%).1,2 Occasionally, adrenal masses mimic space-occupying lesions (SOL) of the liver, particularly when they are large and infiltrate into the adjacent liver parenchyma.3 In addition, adrenal rest tumours arising from the intra-hepatic adrenal ectopic tissue (adrenohepatic plate) may also masquerade as primary hepatic malignancy like hepatocellular car- cinoma (HCC).4 Not surprisingly, even benign liver tumours like focal nodular hyperplasia and hepatic adenoma have also been
considered as differential diagnosis on imaging for such intriguing lesions.3 Hence, it is not uncommon for these adrenal tumours with large component of hepatic involvement to be primarily diagnosed as liver tumours.
These atypically behaving tumours may require liver resection of the involved segments or sections. One multi-institutional retro- spective study on adrenocortical carcinoma described 68 patients requiring multi-visceral resection.5 Among these, approximately 28% (n = 19) patients required liver resection. However, the type and extent of liver resection was not described in this study. Another study including 77 patients described liver resection in liver metastasis from adrenocortical carcinoma. Of these, 13 patients had synchronous metastasis and underwent various forms of liver resection including two right hepatectomies.6 Several other isolated
cases or case series have discussed curative liver resection for locally advanced recurrent or metastatic adrenal tumour.3,7,8 In these reports, the authors described right hepatectomy along with adrenalectomy with or without nephrectomy for malignant phaeochromocytoma. They have recommended that a differential diagnosis of primary hypervascular hepatic tumour must be consid- ered in such scenarios, and that multivisceral resection of locally advanced or low-volume metastatic tumours can be curative.
Liver resections done for these infiltrating adrenal tumours have been non-anatomical resections as well as right hepatectomy. Vari- ous factors such as proximity and adhesions to the inferior vena cava (IVC) anticipated troublesome bleeding, and, in the case of phaeochromocytoma, the risks of intra-operative sudden-onset hypertension make the resection of such locally advanced tumours challenging. To mitigate these risks, the ‘anterior approach hepatec- tomy’ (AAH) is a well-established procedure for large HCC involv- ing right posterior lobe,9,10 and it has also been described for the large adrenal tumours with hepatic invasion.11,12 These authors have described the advantage of AAH using the ‘hanging manoeu- vre’ for safely performing this complex surgery. This approach facilitates in creating an avascular dissection plane on the anterior aspect of the IVC, sparing the lateral aspect of the vena cava during the initial part of the surgery, in addition to restricting the direct handling of the tumour. As originally described, the hanging manoeuvre facilitates AAH.13
The aim of this study was to identify the clinical and diagnostic characteristics of the patients with large primary adrenal lesions mimicking a SOL of the liver and mandating concomitant hepatec- tomy, and to assess the feasibility and safety of AAH in the patients requiring right hepatectomy.
Methods
The study was conducted in the Department of Hepato-Pancreato- Biliary Surgery and Liver Transplantation at a tertiary care hospital in India and approved by the Institutional review board (IRB Number-IEC/2020/79/MA06). The study adheres to the principles of the ‘Declaration of Helsinki’ of the World Medical Associa- tion.14 Our prospectively maintained database was analysed retro- spectively from March 2012 to December 2019 to identify patients with locally advanced adrenal tumours who were referred to us with a suspicion of SOL of the liver for further evaluation and manage- ment. Patients with metastatic, unresectable or inoperable disease, who did not receive upfront surgery, were excluded. The medical case records, electronically stored laboratory and imaging findings were scrutinized for clinical details. The demographic parameters, comorbidities, symptoms, examination findings, laboratory and the imaging features (computed tomography (CT)/magnetic resonance imaging/positron emission tomography scan) were documented. In addition, other relevant investigations, the operative plan, details of surgery (blood loss, remnant liver volume and any significant intra- operative event), the length of hospital stay, final histopathological diagnosis and follow-up details were recorded. In view of our initial experience of the difficulties while performing right hepatectomy by the conventional approach for large posterior HCCs, we have already adopted the AAH for these selected tumours. In the present
series of suspected liver tumour/large infiltrating adrenal mass requiring right hemihepatectomy (excluding those amenable to non-anatomic resection), we adopted the AAH.
Pre-operative preparation
On referral to our centre, a complete review of clinical features and the medical record was undertaken. The patients with the symptoms such as hypertension, headache or palpitation were evaluated for functional adrenal tumours. The 24-h urinary catecholamines, serum adreno-corticotrophic hormone and cortisol levels were examined when a tumour of adrenal origin was primarily suspected. The normal values in urine over 24 h for total catecholamines, epi- nephrine, norepinephrine and dopamine were taken as 14-110 µg, 0-20 µg, 15-80 µg and 65-400 µg, respectively. Viral markers for hepatitis B and C and serum alpha fetoprotein levels were checked in all patients. Cross-sectional imaging with either contrast- enhanced CT abdomen or magnetic resonance imaging was per- formed in all the patients. Functional imaging like I131MIBG (metaiodobenzylguanidine) scan or gallium octreotide scan was performed in one patient each with clinical manifestations sugges- tive of phaeochromocytoma or neuro-endocrine tumour, respec- tively. In patients with hypertension, an echocardiography was performed. These patients received anti-hypertensive treatment with a-blockers (prazosin) and blood pressure was brought under control before being considered for surgery. Image-guided biopsy was per- formed in cases of diagnostic uncertainty except when the antici- pated risk was too high. In patients with a possibility of diagnosis of phaeochromocytoma, we planned for minimal handling of the tumour to avoid excess secretion of catecholamines, uncontrolled hypertension and cardiac failure. Liver volumetry from the venous phase of CT images was calculated. From our previous experience in live donor hepatectomies,15 a minimum remnant liver volume of 35% was acceptable for proceeding with right hepatectomy in these cases. The decision regarding the type of hepatic resection was based on the extent of the liver involvement. Limited hepatic resection was planned for involvement of two or single adjacent segment(s) involvement. Any extension to the right anterior segments and/or tumour splaying/involvement of the right portal vein were considered as an indication for right hepatectomy.
Technique of right hepatectomy by AAH and hanging manoeuvre
For anticipated difficult right hepatectomy, an upper midline inci- sion with a J-shaped right subcostal extension is made, and the Thompson retractor system is deployed. Next, a generous Kocher’s manoeuvre is performed to expose the adreno-hepatic mass, Gerota’s fascia and infra-hepatic IVC along with bilateral renal veins. The adrenal gland and tumour are carefully dissected away at this stage from the kidney and IVC after securing the tributary veins. The operability assessment is made during this manoeuvre. The right-sided hepatic pedicles (right- portal vein and hepatic artery) are dissected, looped and then divided. The ischaemic line of transection hence created is marked with the diathermy cautery and this extends along the inferior surface at gall bladder fossa
Table 1 Demographic and clinical profile of the patients with adrenal mass invading liver
| No. | Age/sex | Clinical presentation | Adrenal functional activity (24-h urine catecholamines, serum cortisol | Tumour markers (serum AFP, CEA, CA 19-9) | Imaging and enhancement characteristics, remnant liver volume (RLV) | Pre-operative diagnosis | Pre-operative biopsy |
|---|---|---|---|---|---|---|---|
| 1 2 3 4 | 38/F 23/M 38/M 66/F | Lump right abdomen Mild pain right abdomen Pyrexia under investigation Pyrexia under investigation | Not done Normal Normal Not done | Normal Normal Normal Normal | Right liver mass with arterial enhancement, no venous washout and RPV involvement on CECT abdomen (Fig. 1 upper two) (RLV 44%); adrenal gland not visualized separately, calcifications present Heterogenous mass in segment 6 and 7 with capsular enhancement on CEMRI (RLV 40 %); adrenal gland not visualized separately; calcifications present Hypodense cystic lesion posterior section of liver; peripheral calcification Metabolically active and arterial enhancement lesion in segment 6 on PET-CT with venous washout (Fig. 2); adrenal gland not visualized separately | Atypical hepatocellular carcinoma (HCC); large hepatic adenoma with haemorrhage 1. Phaeochromocytoma; 2. Adrenocortical carcinoma, with right lobe infiltration 1. Adrenal tuberculous abscess; 2. Cystic adrenal tumour, liver infiltration; 3 Right posterior lobe hepatic cyst HCC | No GIST (vimentin, SMA, CD- 117 +ve) Inflammatory cells Atypical cells |
| 5 | 33/M | Malignant hypertension | Catecholamines elevated | Normal | Heterogenous mass on CECT and positive uptake on |131. MIBG whole body scan + SPECT-CT, (Fig. 3) (RLV 50 %) | Bilateral phaeochromocytomas with right liver involvement; spinal haemangioblastoma (VHL disease) | No |
| 6 | 44/M | Abdominal lump, hypertension, MEN type 2A in family | Catecholamines elevated, cortisol and ACTH normal | Normal | Heterogenous adrenal lesion with calcification, invading segment 6 on CT abdomen; positive on 68GA- octreotide (somatostatin receptor-labelled) PET | Right phaeochromocytoma with segment-6 liver infiltration (MEN type 2A) | No |
| 7 | 48/F | Palpitations and moderate hypertension | Catecholamines elevated | AFP = 13 ng/ml | Arterial enhancement with no venous washout on CECT abdomen (RLV 42%); adrenal gland not visualized separately | 1. Right phaeochromocytoma; 2. HCC | Small round malignant cells |
| 8 | 33/M | Mild pain, chronic hepatitis B | Not done | Normal | Hyperintense on T2 phase of MRI (cystic lesion adreno-hepatic region | 1. Large adrenal cyst/cystic tumour; 2. Exophytic hepatic cyst; 3. Hydatid cyst liver | Inflammatory cells |
ACTH, adrenocorticotrophic hormone; AFP, alpha-feto protein; CA 19-9, cancer antigen 19-9; CEA, carcinoembryonic antigen; CECT, contrast-enhanced computed tomography; CEMRI, contrast-enhanced magnetic resonance imaging; GIST, gastrointestinal stromal tumour; HCC, hepatocellular carcinoma; MEN, multiple endocrine neoplasia; MIBG, metaiodobenzylguanidine; PET, positron emission tomography; RLV, remnant liver volume; RPV, right portal vein; SMA, smooth muscle actin; SPECT, single photon emission computed tomography; VHL, Von Hippel-Lindau; VMA, vanillyl mandelic acid.
| No. | Surgical procedure | AAH | Major vascular adhesion/ invasion | Histopathological examination | R0 | Blood loss (mL) | Complications (Clavien grade) | Length of stay (days) | Adjuvant therapy | Follow-up (months) | Recurrence | Status | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Final diagnosis | Tumour size (cm) | Grade | ||||||||||||
| 1. | Right hepatectomy, adrenalectomy + excision of part of | Yes | RPV | Adrenocortical carcinoma - inhibin +ve (Fig. 1 lower left) | 8 | High | Yes | 600 | II | 14 | Mitotane | 7 | No | Alive |
| diaphragm | ||||||||||||||
| 2. | Right hepatectomy + adrenalectomy | Yes | IVC | Paraganglioma - vimentin, NSE, CD- 117 +ve | 17 | Moderate | Yes | 800 | II | 25 | None | 84 | Yes (at 7 years) | Alive |
| 3. | Adrenalectomy + Segments 5, 6 | No | IVC | Chronic inflammatory lesion | 7 | - | - | 450 | II | 28 | None | 60 | No | Alive |
| resection | ||||||||||||||
| 4. | Adrenalectomy + | No | Posterior | Adrenocortical | 15 | High | Yes | 2200 | IVA | 52 | Mitotane therapy | 74 | No | Alive |
| segment 6 | branch | carcinoma - inhibin | ||||||||||||
| resection | of RPV | and calretinin | ||||||||||||
| focal +ve | ||||||||||||||
| 5. | Right hepatectomy | Yes | IVC | Malignant bilateral | 10 | High | Yes | 1150 | II | 12 | None | 65 | No | Alive |
| + Bilateral | phaeochromocytoma | |||||||||||||
| adrenalectomy | - chromogranin +ve (Fig. 3 lower two) | |||||||||||||
| 6. | Right adrenalectomy | No | No | Malignant | 7 | High | Yes | 400 | I | 10 | None | 72 | No | Alive |
| + segment 6 | phaeochromocytoma | |||||||||||||
| resection | (MEN 2A) - | |||||||||||||
| chromogranin +ve | ||||||||||||||
| 7. | Right hepatectomy | Yes | RRV, IVC | Phaeochromocytoma- | 10 | Low | Yes | 350 | II | 10 | None | 47 | No | Alive |
| + adrenalectomy | chromogranin +ve | |||||||||||||
| 8. | Right adrenal cyst excision + non- | No | IVC | Right adrenal cyst- endothelial type | 6 | - | - | 150 | I | 7 | None | 70 | No | Alive |
| anatomical | ||||||||||||||
| resection of | ||||||||||||||
| segment 6 | ||||||||||||||
AAH, anterior approach hepatectomy; IVC, inferior vena cava; MEN, multiple endocrine neoplasia; NSE, neuron specific enolase; RPV, right portal vein; RRV, right renal vein.
along the Cantlie’s line. The supra-hepatic dissection follows and the groove between the right hepatic vein (RHV) and middle hepatic vein (MHV) is defined from the cranial end. Next, the part of caudate lobe anterior to infra-hepatic IVC is divided with dia- thermy. Any caudate tributaries to IVC are clipped and divided at this stage. Using blunt dissection by the Robert’s artery forceps and repeated water injection, a plane is created between the anterior sur- face of the IVC and the posterior liver capsule. This plane finally meets the groove between the RHV and MHV already created from the cranial end. A tape is passed through this space and is used for hanging of liver. Transection of liver parenchyma is performed with the Cavitron Ultrasonic Surgical Aspirator (Valleylab, Boulder, CO, USA) and is facilitated by the liver hanging. Segment 5 and 8 veins are clipped and divided with preservation of the MHV along with the remnant. After completion of the parenchymal tran- section, the RHV is divided and sutured with 4-0 prolene. The right hepatic duct is transected at last and the ductal opening of the remnant liver is closed carefully with 6-0 polydiaxone sutures. Subsequently, the en bloc specimen along with the right lobe is dissected off the lateral aspect of the vena cava and then from the diaphragm starting from medial to lateral aspect. This whole manoeuvre facilitates dissection with minimal handling of the tumour and hence minimizes the risk of haemodynamic fluctuations and tumour seeding. The hanging manoeuvre was not necessary for patients requiring limited hepatic resection.
Post-operative complications and follow-up
Complications were graded according to the Clavien-Dindo classifi- cation.16 All the patients were followed up twice monthly for the initial 3 months, then every 3 month till the end of 1 year. After that, half-yearly follow-up was advised.
Results
During the study period, a total of eight patients were referred to us with a diagnosis of large mass of adreno-hepatic origin. The clinical and demographic details of the cohort are shown in Table 1. The median age was 38 years (range: 23-66); there were five males and three females. In four patients, we had suspicion of indeterminate origin of the mass, which could be of either adrenal or hepatic. In two patients, we proceeded for resection with a provisional diagno- sis of hepatic malignancy (HCC). One of these two had received oral sorafenib therapy for suspected HCC before being referred to us. Two patients had adrenal complex cysts and suspicion of malig- nancy on imaging. One of them had an atypical presentation with prolonged pyrexia treated by percutaneous drainage of the suspected tuberculous abscess/infected cyst. The urinary catechol- amine levels were found to be elevated in three of the five patients tested. The location and characteristics of the lesion and enhance- ment pattern is provided in brief in Table 1. Two patients were part of familial endocrine syndromes; one each had Von Hippel-Lindau disease and multiple endocrine neoplasia 2A. In four patients planned for right hepatectomy, pre-operative CT volumetry rev- ealed remnant liver volume of >35% in all. Two of them also
underwent indocyanine green retention test at 15 min for suspected early parenchymal liver disease and both had <10% retention.
The details of the surgical procedure are enumerated in method- ology section and type of surgery is given in Table 2. We per- formed right adrenalectomy with the right hepatectomy using the AAH and hanging manoeuvre in four patients; whereas in the other four, non-anatomical resection of one or more segments was per- formed. This accounts for 1.01% of 789 hepatectomies performed during the study period, for various indications including donor hepatectomy. Partial involvement/adhesion of lateral aspect of IVC was seen in five patients, whereas the right portal vein or its bra- nches were involved in two patients (Table 2). Of the patients with IVC involvement, one patient was amenable to sleeve resection of IVC and another one to resection of the right accessory inferior hepatic vein after applying the side-biting (Satinsky) vascular clamp, without resultant narrowing. None of them required vena caval replacement or venous patch repair. Resection of involved part of the diaphragm was performed in one patient. The median size of the tumour was 9 cm (range 6-17 cm). The median intra-operative blood loss was 525 mL (range 150-2200 mL). No intra-operative adverse event was documented because of the hang- ing manoeuvre. The final histopathological diagnoses were; three phaeochromocytomas (two malignant and one benign), two adreno- cortical carcinomas, one malignant paraganglioma and two adrenal cysts. The median length of hospital stay was 13 days (range 7-52 days).
Complications
Seven patients experienced either grade I or II complication (Clavien-Dindo). One patient had grade IVA complication. This particular patient had type II diabetes mellitus, peripheral vascular disease and right bundle branch block detected pre-operatively. She had respiratory failure and a prolonged dependency on mechanical ventilation. Tracheostomy was performed on post-operative day 12; she was discharged after a prolonged hospital stay of 52 days. No mortality was experienced in our cohort. None of the patients expe- rienced post-hepatectomy liver failure as per the 50:50 criteria,17 and no biliary complications occurred (Table 2).
Follow-up
All patients are alive after a median follow-up of 67.5 months (range: 7-84 months). However, one patient was detected with recurrent paraganglioma after 7 years of follow-up, while the others are disease-free at the time of the last follow-up.
Discussion
To the best of the authors’ knowledge, this is one of the largest series that discusses the clinical details and surgical management of the adrenal tumours presenting either with additional liver infiltra- tion or mimicking hepatic tumours. All these patients were referred to our hepato-biliary centre with a provisional diagnosis of liver SOLs. On extensive investigations and further cross-sectional and functional imaging, we could narrow down to a possible diagnosis
of adrenal tumour with liver infiltration in four of them. Out of these four, two had syndromic manifestations of phaeochromocytoma with elevated urinary catecholamines. In two patients, in view of the characteristic arterial enhancement pattern, we proceeded for surgery with a possible diagnosis of HCC. In the remaining two patients with cystic mass, mixed adreno-hepatic ori- gin was suspected and one of them had chronic hepatitis B infection.
The diagnostic confusion in adreno-hepatic tumours arises mainly because of two reasons. First, any large tumour originating from one infiltrates into the other and second, when the tumour arises from intra-hepatically located ectopic adrenal tissue.18,19 The tumours arising from this ectopic tissue are usually adrenal adeno- mas, which are smaller tumours.18,20,21 All the tumours we encoun- tered in our series belong to the first category, that is, larger adrenal tumours with infiltration to the adjacent liver segments in half, and almost the entire right liver in the other half of the patients. Even pre-operative imaging may not always distinguish accurately between large right-sided hepatic tumours with exophytic extra- capsular extension and adrenal tumours infiltrating the adjacent
liver segments. This differentiation is crucial for appropriate surgi- cal management.
Although liver resection has been well-described for adrenal malignancy with adjacent liver infiltration or non-contiguous liver metastasis,5,6 there are only a few case reports or series published highlighting adrenal tumours mimicking hepatic SOLs.3,18,20,22 Yu et al. and Cho et al. described a small adrenal adenoma inside the liver emphasizing that a prior diagnosis could avoid the need of sur- gery.18,20 On the other hand, Ueda et al. described a large hyper- vascular exophytic phaeochromocytoma invading the liver with feeder vessels from the hepatic artery.3 Yu et al.20 also reviewed 10 cases of adrenal adenoma (largest 10 cm), where the tumours are usually present inside the liver and mimic HCC. Though even- tually found to be benign, nine of them were diagnosed as primary liver cancer. Most of these lesions are non-functional, and under- went hepatectomy for both diagnosis and treatment. In our series, we too had a dilemma about the origin of the tumour (adrenal or liver) in a half of the cases even after thorough investigation. How- ever, as most tumours were large and seemingly intra-hepatic in location, there was little debate about the need for or the extent of
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hepatectomy. Moreover, when doubt about the origin of the tumour still remains, clinical features such as palpitation, hypertension and elevated urinary or plasma catecholamines can help in the diagnosis of adrenal tumours such as phaeochromocytoma. None of the patients had any evidence of cirrhosis on imaging and all had undetectable levels of viral markers for hepatitis except for hepatitis B detected in one patient with a large adrenal cyst (Table 1). Hence, any deviation from the typical enhancement patterns of HCC,23 non-visualization of the right adrenal gland,3 presence of calcifications,23 negative viral markers for hepatitis, absence of cir- rhosis and normal serum alpha fetoprotein levels should be consid- ered as points in favour of other rarer diagnoses, which include adrenal lesions with hepatic infiltration (Table 1, Figs 1-4). In such cases, multi-planar reformation views of thin sections of the adreno-hepatic area and pre-operative tissue biopsy become
relevant for better planning. Pre-operative biopsy was performed in the majority of our patients with diagnostic uncertainty, except the first patient and those with typical clinical features and elevated uri- nary catecholamines suggestive of phaeochromocytoma (Table 1). The first patient had a radiological interpretation of either an atypi- cal HCC or a large hepatic adenoma with haemorrhage. As the tumour deemed resectable upfront and there was anticipated risk of rupture by pre-operative biopsy in view of pre-existing haemorrhage, we proceeded for resection in this particular patient. Patient number 7 (Table 1) despite having clinical features and ele- vated urinary catecholamines indicative of phaeochromocytoma, did undergo image-guided percutaneous biopsy, albeit before being referred to us.
As described in Methods section, extension of the tumour to the right anterior segment(s) or abutment of the right portal vein, was
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considered as an indication for right hepatectomy. In view of our previous experience with hepatectomy by the ‘anterior approach’ for large posteriorly located HCCs with or without adjacent organ involvement, we adopted this approach in four patients requiring right hepatectomy (Fig. 1). Other tumours were amenable to re- section of the adjacent segment(s) along with en bloc removal of the specimen. In five of our patients, the IVC was pushed by the tumour with minimal infiltration of the IVC wall and right acces- sory inferior hepatic vein in one case each. These latter two cases were amenable to sleeve resection after applying a side-biting (Satinsky) vascular clamp. None of these patients required either a venous patch or total vascular exclusion.
AAH was initially advocated for large posteriorly located right- sided HCCs. A randomized trial by Liu et al. in 2006 discussed the advantages of AAH for large HCCs.10 The authors found that AAH was associated with a better overall survival and it also led to fewer patients with major intra-operative blood loss (<2 L). Another recent meta-analysis of controlled trials also reported favourable
intra-operative blood loss and better overall survival.24 However, the advantages of AAH for large adrenal tumours with hepatic involvement are barely discussed in literature.11,12 Coppa et al. described advantages of AAH in 10 patients with large retro- hepatic tumours with secondary IVC involvement. They included retroperitoneal sarcoma and other soft tissue tumours, along with four cases of adrenocortical carcinoma.12 However, they have not elaborated the diagnostic difficulties faced and the origin of the tumour in the adreno-hepatic region. They have advocated partial IVC resection with or without venous patch reconstruction or, in cases of major involvement of the IVC, total vascular exclusion and IVC replacement. By adopting AAH, we could also minimize direct handling of the tumour and did not experience any hyperten- sive crisis in patients with phaeochromocytoma. In addition, any adhesion of the tumour to the lateral aspect of the IVC could be dealt with delicately under direct vision. The median blood loss was 525 mL and only two patients had blood loss of more than 1 L.
STUDY 1
2/17/2015
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TUDY 1
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17/2015
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27 IMA 8 / 15
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E 2.5
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W 300.0
BW 300.0
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1
Large right adrenohepatic tumour (CECT/MRI)
Symptoms (Hypertension, headache and palpitation etc.) Elevated urinary/plasma catecholamines
Cirrhosis, positive Viral markers, 1 AFP (19)
Functional imaging (131 MIBG)
Prolonged/slow PVP contrast washout (23) Right adrenal gland not visualized separately No fat plane between adrenal and liver (3), Calcification present (23)
Arterial enhancement PVP contrast washout Right adrenal gland visualized separately
Phaeochromocyto ma
Adrenocortical tumours
Adrenal cyst Atypical tumour Metastasis
HCC
Image guided biopsy and IHC staining (19)
Resectable disease
Adrenalectomy+ Right hepatectomy (AAH (12)/ conventional)
Preoperative preparation
Adrenalectomy+ Limited hepatic resection
As per the follow-up protocol, contrast-enhanced CT abdomen/ positron emission tomography scan were performed 6 months after surgery and yearly thereafter. All the patients are alive with one recurrent paraganglioma detected at 7-year follow-up.
To summarize, this is one of the largest series highlighting the diagnostic difficulties of adreno-hepatic lesions requiring hepatec- tomy. The exact origin of such a posterior mass near the adreno- hepatic region may not be evident until after resection. AAH with hanging of the liver provides specific advantages for these posterior tumours like lower risk of bleeding and minimal tumour handling. Admittedly, we had a limited number of patients precluding a com- parison of this technique with the conventional approach of resection. Nonetheless, we have proposed an algorithmic approach for the diag- nosis and management of these large tumours based on our own experience and the existing literature, which we hope will guide other hepatic surgeons for better management of these complex tumours (Fig. 4). We feel that other surgeons too may benefit from this tech- nique, as they are otherwise not usually conversant with AAH.
Conclusion
Adrenal tumours mimicking primary hepatic malignancy are rare, but pose diagnostic and treatment challenges. A meticulous algo- rithmic approach could help in management of such tumours. The results of AAH for large right adrenal tumours with liver involve- ment are encouraging and safe in terms of R0 resection, blood loss, surgical complications and overall long-term survival.
Author contributions
Nitin Agarwal: Conceptualization; data curation; formal analysis; methodology; validation; writing-original draft; writing-review and editing. Nihar Mohapatra: Conceptualization; data curation; for- mal analysis; project administration; resources; software; writing- review and editing. Nilesh Patil: Formal analysis; methodology; supervision; validation; writing-review and editing. Piyush Sinha: Data curation; formal analysis; investigation; supervision; valida- tion; writing-review and editing. Tharun Gattu: Data curation; for- mal analysis; methodology; writing-original draft. Viniyendra Pamecha: Conceptualization; formal analysis; supervision; valida- tion; writing-review and editing.
Conflicts of interest
None declared.
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