Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava-a European Society of Endocrine Surgeons (ESES) survey
Radu Mihai . Maurizio Iacobone . Ozer Makay . Pablo Moreno . Andrea Frilling . Jean-Louis Kraimps . Arturo Soriano · Jesús Villar del Moral . Marcin Barczynski . Manuel C. Duran . Gregory P. Sadler . Bruno Niederle . Henning Dralle · Barney Harrison . Bruno Carnaille
Received: 17 August 2011 / Accepted: 9 November 2011 /Published online: 2 December 2011 C Springer-Verlag 2011
Abstract
Background Most patients with adrenocortical cancer (ACC) continue to present with advanced disease. Invasion into the inferior vena cava (IVC) defines stage III disease and the management of such patients raises additional difficulties. Method A multicentre survey was organized by emailing a standardized proforma to members of the European Society
of Endocrine Surgery (ESES). Anonymised retrospective clinical data were collected.
Results Replies were received from 18 centres in nine countries. ACC with IVC invasion was encountered in 38 patients (18F:20M, age 15-84 years, median 54 years). There were 16 nonfunctioning tumours and 22 functioning tumours predominantly right-sided (26R:12L) and measur-
This work was presented at the ESES workshop in Lyon, France, May 2011.
R. Mihai (K) · G. P. Sadler ☒ Endocrine Surgery Unit, Department of Surgery, John Radcliffe Hospital, Oxford OX3 9DU, UK e-mail: radumihai@doctors.org.uk
G. P. Sadler e-mail: Greg.Sadler@orh.nhs.uk
M. Iacobone Endocrine Surgery Unit, Department of Surgical and Gastroenterological Sciences, University of Padova, Padova, Italy e-mail: maurizio.iacobone@unipd.it
O. Makay Department of Surgery, Ege University Hospital, Izmir, Turkey e-mail: ozer.makay@ege.edu.tr
P. Moreno Endocrine Surgery Unit, Department of General Surgery, Hospital Universitario de Bellvitge, C/Feixa Larga s/n, Hospitalet, 08907 Barcelona, Spain e-mail: 25108pml@gmail.com
A. Frilling Department of Surgery and Cancer, Imperial London College, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK e-mail: a.frilling@imperial.ac.uk
J .- L. Kraimps Department of Endocrine Surgery, Jean Bernard Hospital, 86021 Poitiers, France e-mail: j.l.kraimps@chu-poitiers.fr
A. Soriano Hospital U. N. Sra de Candelaria, Tenerife, Spain e-mail: asorben@gmail.com
J. Villar del Moral General Surgery Department, Virgen de las Nieves University Hospital, Granada, Spain e-mail: jevillarmo@yahoo.es
M. Barczynski Department of Endocrine Surgery, Jagiellonian University, Medical College, Krakow, Poland e-mail: marbar@mp.pl
ing 18-255 mm (median 115 mm). Fourteen patients had metastatic disease at presentation. Tumour thrombus extended in the prehepatic IVC (n=21), subdiaphragmatic IVC (n=6) or into the SVC/right atrium (n=3). Open adrenalectomy was associated with resection of surrounding viscera in 24 patients (nephrectomy n=16, liver resection n=14, splenec- tomy n=3, Whipple procedure n=2). IVC was controlled locally (n=27), at suprahepatic levels (n=6) or necessitated cardiac bypass (n=5). Complete resection (R0, n=20) was achieved in the majority of patients, with a minority having microscopic persistent disease (R1, n=7) or macroscopic residual disease (R2, n=4). Perioperative 30-day mortality was 13% (n=5). Postoperative Mitotane was used in 23 patients and chemotherapy in eight patients. Twenty-five patients died 2-61 months after their operation (median 5 months). Currently, 13 patients are alive at 2-58 months (median 16 months) with known metastatic disease (n=7) or with no signs of distant disease (n=6).
Conclusion This dataset is limited by the lack of a denomina- tor as it remains unknown how many other patients with ACC presenting with IVC invasion did not undergo surgery. The relatively low perioperative mortality and the long disease-free survival achieved by some patients should encourage surgeons with adequate experience to offer surgical treatment to patients presenting with advanced adrenocortical cancers.
Keywords Adrenocortical cancer · Inferior vena cava . Vascular invasion
M. C. Durán
Endocrine Surgery Unit, Department of Surgery, University Hospital of Fuenlabrada,
Faculty of Health Sciences, King Juan Carlos University, Madrid, Spain
e-mail: mduran.hflr@salud.madrid.org
B. Niederle Endocrine Surgery, Division General Surgery, Department of Surgery, Medical University,
Vienna, Austria e-mail: bruno.niederle@meduniwien.ac.at
H. Dralle
Department of Endocrine Surgery, Martin Luther University, Halle-Wittenberg, Germany
e-mail: henning.dralle@medizin.uni-halle.de
B. Harrison
Department of Surgery, Royal Hallamashire Hospital, Sheffield, UK e-mail: Barney.Harrison@sth.nhs.uk
B. Carnaille
Chirurgie Endocrinienne, Hopital Huriez, Lille, France
e-mail: bruno.carnaille@chru-lille.fr
Introduction
Adrenocortical cancer (ACC) is a very rare malignancy with a world-wide incidence of ~1/million population and it represents one of the most aggressive human cancers. Despite the encouraging 60% survival reported by highly specialized centres [1], the overall 5-year survival remains only 30-40% [2, 3]. In the last decades, there has been no significant improvement in survival after operations for ACC and the majority of patients continue to present with advanced disease [2, 4].
Because of the rarity of ACC the experience of individual surgeons with the management of these aggressive tumours remains very limited. For example a recent paper described the experience gained in three large University centres in the UK over a decade with 40 patients managed in Newcastle, Sheffield and Cardiff [5]. Outside such recognized UK centres for endocrine surgery the experience remains minimal since most adrenal surgery in the UK is done by surgeons who undertake less than five cases per year [6]. The world-wide situation in most hospitals is likely to be similar.
Several national and pan-European structures have been established to improve the therapeutic options offered to patients with ACC and, in addition, to promote basic and clinical research in this field. Data collected initially in national registries in Germany, France and Italy have provided reports on larger patient cohorts with ACC. Recently, the European Network for the Study of Adrenal Tumours (ENSAT) has taken a lead in promoting common European standards for diagnosis and therapy, data collection and tumour banking for ACC. For the first time, patients with metastatic or unresectable ACC have been considered for entry in a prospective multicentre international randomized controlled trial comparing different cytotoxic chemotherapy regimes (FIRM-ACT) [7] whose results are pending publication.
Tumour invasion into the inferior vena cava (IVC) defines a small subgroup of patients with added therapeutic difficul- ties. The largest series of 15 patients with IVC invasion reported from Cochin Clinic (Paris, France) included a review of a further 106 cases reported in the literature over three decades and concluded that involvement of the IVC should not be considered a contraindication for surgery if complete resection of the primary tumour and its locoregional extension can be achieved [8].
The aim of this study was to collate the experience of members of the European Society of Endocrine Surgeons (ESES) involved in the care of patients with ACC invading the IVC.
Method
A standardized proforma was used to collect anonymised clinical data retrospectively on patients with ACC and IVC
invasion. This proforma was sent to ESES national representatives who forwarded it by them to members of their national specialist professional surgical associations. It remains unknown how many endocrine surgeons were contacted. Due to variation in surgical practice throughout the EU countries such patients are more likely to be seen by an oncological surgeon in the Scandinavian countries hence the invitation to contribute to this study would have not reached them.
Data were collected for patients treated between 2000 and 2010. One centre reported data for 1992-1998 (AF). One contributor from Turkey (OM) reported data from four other universities in the same country.
Demographics, biochemical assessment, intraoperative details, intra and postoperative morbidity, size and stage of the tumor, pathology, pre- and postoperative adjuvant thera- pies and follow-up data were recorded. The number of adrenalectomies performed in each centre was also recorded.
Data are presented as range and median. Survival curves were analysed using XLStat software (Addinsoft, 2011).
Results
Replies were received from 18 centres in nine countries (Table 1) including two centers in which there were no eligible cases. Data regarding the volume of adrenal surgery were provided by surgeons from 11 centres who performed 2,160 adrenalectomies (range 70-350 cases per centre, median 167 cases over a decade).
ACC with IVC invasion was diagnosed in 38 patients (18F:20M, age 15-84 years, median 54 years). There were 16 non-functioning tumours and 22 functioning tumours secreting cortisol (n=14), cortisol and androgens (n=4), androgens (n=1) or oestrogens (n=1) or aldosterone (n=2). On cross-sectional imaging, tumours measured 18-255 mm (median 115 mm) and were predominantly on the right side (26R:12L). The smallest tumour in this series was an 18- mm-right aldosteronoma. Fourteen patients were found to have metastatic disease at presentation.
The IVC thrombus created partial obstruction of the IVC (n=36) with only two patients reported to have had complete obstruction. Tumour thrombus extended in the prehepatic IVC (n=21), subdiaphragmatic IVC (n=6) or into the superior vena cava/right atrium (n=3) (no details provided for eight patients).
An open approach was used for all cases. Fourteen patients underwent adrenalectomy only and 24 patients had en-bloc resection of adjacent viscera (nephrectomy n=16, liver resection n=14, splenectomy n=3, Whipple procedure n=2). The IVC was controlled locally (n=27), at supra- hepatic levels (n=6) or on cardiac bypass (n=5). Severe intraoperative blood loss was reported in five cases without
further details on estimated volume of blood loss. Postop- erative complications recorded were gas embolism (n=2), chyle fistula (n=2), IVC thrombosis and renal failure (n=1) and cardiorespiratory events (n=3).
Histology demonstrated T3 (n=14) or T4 (n=24) tumours according to ENSAT classification. Histology reports included a Weiss score in 22/38 cases and this score ranged from 3 to 9 (median 6). Complete resection (R0) was achieved in the majority of patients (n=20), there was microscopic persistent disease (R1) in seven patients and macroscopic residual disease (R2) in four patients (no details available for seven patients).
Perioperative 30-day mortality was 13% due to five patients who had severe intraoperative bleeding and postoperative coagulopathy. Postoperatively, Mitotane was used in 23 patients and chemotherapy in eight patients.
Twenty-five patients died 2-61 months after their operation (median 5 months). At the time of data collection, 13 patients were alive at 2-58 months (median 16 months) with known metastatic disease (n=7) or with no signs of distant disease (n=6). The overall survival in this cohort of patients was 24±4 months (Fig. 1). There was a trend towards better survival in female patients (29±6 vs. 15± 3 months, p=0.08 Wilcoxon test) but no difference in survival based on secretory activity (24±5 vs. 17± 4 months) or size above or below the median value of 11 cm (24±6 vs. 23±5 months). The longer survival of patients who received Mitotane treatment (36±5 vs. 4± 1 months, p<0.0001 Wilcoxon test) was due to the fact that Mitotane chemotherapy would not have been initiated in those who died within few weeks/months after their operation either because of perioperative complications or rapidly advancing disease.
Discussion
ACC is a very aggressive malignancy with poor prognosis. Because of the rarity of the disease, it was only in 2004 that the International Union Against Cancer defined the TNM criteria and published the first staging classification for ACC. This was found to have significant limitations and a revised classification with superior prognostic accuracy was proposed by the European Network for the Study of Adrenal Tumors (ENSAT) [9]. In this classification, stage III ACC is defined as the presence of positive lymph nodes, infiltration of surrounding tissue, or vascular tumour thrombus whereas stage IV is restricted to patients with distant metastasis.
Complete primary resection is the only treatment to improve survival in ACC and incomplete resection is associated with a 5-year survival of less than 10% [3]. This paper reports the outcome of operations on a selected
| Centre | Gender | Age | Secreting | Biochem | CT size | Side | Thrombus size | TNM | En-block resection | IVC control | IVC repair | Resection | Histo weight | Recurrence site | Comments | Survival/ FU |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| AUSTRIA, | F | 39 | Yes | Cortisol | 120 | L | Prehepatic | 3 0 0 | Adrenal | Local | Venotomy | R0 | 354 | Lung | Deceased | 57 |
| Vienna | IVC | only | ||||||||||||||
| ENGLAND, | F | 28 | Yes | Cortisol | 110 | R | Prehepatic | 3 x 1 | Kidney | Local | Primary | R0 | 450 | Lung, | Alive with | 20 |
| Oxford | IVC | repair | liver | disease | ||||||||||||
| ENGLAND, | F | 31 | Yes | Cortisol | 120 | L | Prehepatic | 3 X X | Kidney | Bypass, | Venotomy | R0 | 410 | None | Alive without | 44 |
| Oxford | IVC | extracorporeal | disease | |||||||||||||
| circulation | ||||||||||||||||
| ENGLAND, | F | 34 | Non | 140 | R | Prehepatic IVC | 3 X X | Kidney | Local | Venotomy | R0 | 1,020 | Lung | Alive without | 36 | |
| Oxford | disease | |||||||||||||||
| ENGLAND, | F | 51 | Non | 70 | R | Prehepatic | 3 x 1, lung | Kidney | Local | Primary | R0 | 237 | Lung, | Deceased | 32 | |
| Oxford | IVC | repair | liver | |||||||||||||
| ENGLAND, | M | 25 | Yes | Cortisol | 200 | L | Between | 3 X X | Kidney, | Bypass, | Primary | R1 | 3,240 | Deceased | 2 | |
| Oxford | hepatic | spleen | circulation | repair | (perioperative) | |||||||||||
| veins and atrium | maintained | |||||||||||||||
| ENGLAND, | F | 57 | Yes | Cortisol and | 80 | R | Between | 4 0 0 | Adrenal | Suprahepatic | Primary | R0 | 195 | Lung, | Deceased | 18 |
| Sheffield | testosterone | hepatic | only | repair | liver | |||||||||||
| veins and atrium | ||||||||||||||||
| FRANCE, | M | 68 | Non | 78 | R | Prehepatic | 4 X X | Adrenal | Local | Primary | R0 | 380 | None | Alive without | 7 | |
| Lille | IVC | only | repair | disease | ||||||||||||
| FRANCE, | M | 37 | Non | Cortisol+ | 150 | R | 30 MM | 4 X X | Kidney, | Bypass, | Venotomy | R0 | 1,816 | Deceased | 1 | |
| Lille | androgen | liver, | all methods | (perioperative) | ||||||||||||
| Whipple | ||||||||||||||||
| FRANCE, | M | 65 | Non | 150 | R | 30 MM | 4 0 0 | Adrenal | Suprahepatic | Venotomy | R2 | Deceased | 1 | |||
| Lille | only | (perioperative) | ||||||||||||||
| FRANCE, | F | 15 | Yes | Androgen | 160 | R | Prehepatic | 3 0 0 | Adrenal | Local | Primary | R0 | 1,065 | Liver | Deceased | 5 |
| Poitiers | IVC | only | repair | |||||||||||||
| FRANCE, | M | 53 | Yes | Cortisol+ | 60 | R | Prehepatic | 3 0 0 | Adrenal | Local | Venotomy | R0 | Liver | Deceased | 24 | |
| Poitiers | androgen | IVC | only | |||||||||||||
| GERMANY, | M | 77 | Non | 60 | R | Prehepatic | 4 X 1 liver | Liver | Local | Venotomy | R2 | 1,150 | Alive with | 5 | ||
| Halle | IVC | disease | ||||||||||||||
| GERMANY, | M | 55 | Non | 180 | R | Prehepatic | 3 X 0 | Liver | Local | Venotomy, | R1 | 1,800 | None/cardiac | Deceased | 4 | |
| Halle | IVC | repair | death | |||||||||||||
| GERMANY, | F | 58 | Yes | Cortisol+ | 80 | R | 3 0 X | Liver | Suprahepatic | Primary | R0 | Lung | Deceased | 16 | ||
| Hamburg | androgen | repair | ||||||||||||||
| GERMANY, | M | 45 | Yes | Oestrogen | 120 | R | 3 0 X | Kidney, | Local | Vein graft, | R0 | Lung | Deceased | 3 | ||
| Hamburg | liver | autologus | ||||||||||||||
| patch | ||||||||||||||||
| GERMANY, | F | 59 | Yes | Androgen | 130 | R | Prehepatic | 4 0 0 | Kidney, | Local | Venotomy, | R0 | Lung | Deceased | 3 | |
| Hamburg | IVC | liver | repair | |||||||||||||
| ITALY, | M | 67 | Yes | Cortisol | 95 | R | Under R | 4 X 1 | Liver | Local | Primary | R1 | 309 | Bone | Alive with | 19 |
| Padua | hepatic | repair | disease | |||||||||||||
| vein | ||||||||||||||||
| ITALY, | F | 60 | Yes | Cortisol | 70 | L | Prehepatic | 4 X 0 | Kidney | Local | Venotomy | R0 | 370 | None | Alive without | 2 |
| Padua | IVC | disease | ||||||||||||||
| ITALY, | M | 27 | Yes | Cortisol | 80 | L | Prehepatic | 3 X 1 liver | Adrenal | Local | Venotomy | R2 | Persistent | Deceased | 3 | |
| Padua | IVC | lung | only | disease |
| Centre | Gender | Age | Secreting | Biochem | CT size | Side | Thrombus size | TNM | En-block resection | IVC control | IVC repair | Resection | Histo weight | Recurrence site | Comments | Survival/ FU | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ITALY, | F | 33 | Yes | Cortisol | 90 | L | Prehepatic | 3 0 | 1 liver | Liver | Local | Venotomy | R0 | 450 | Bone, liver, | Deceased | 61 |
| Padua | IVC | lung | |||||||||||||||
| ITALY, | M | 48 | Non | 120 | R | Between r | 4 1 | 1 liver | Liver | Suprahepatic, | Resection, | R2 | 390 | Persistent | Deceased | 3 | |
| Padua | hep vein and | lung | intrapericardial, with | closure | disease | ||||||||||||
| atriun | circulation | ||||||||||||||||
| maintained | |||||||||||||||||
| ITALY, | F | 55 | Non | 65 | L | Prehepatic | 4 X | 1 | Kidney, | Local | Venotomy | R1 | 340 | Local, lung, | Deceased | 32 | |
| Padua | IVC | liver, | liver | ||||||||||||||
| spleen | |||||||||||||||||
| POLAND, | M | 75 | Non | 200 | R | Under R | 4 X | 0 | Adrenal | Local | Venotomy | R1 | 2,087 | Deceased | 1 | ||
| Krakow | hepatic | only | (perioperative) | ||||||||||||||
| vein | |||||||||||||||||
| POLAND, | M | 71 | Yes | Cortisol | 254 | R | under R | 4 X | 0 | Kidney, | Local | Venotomy | R1 | 2,370 | Deceased | 2 | |
| KRAKOW | hepatic | liver, | (perioperative) | ||||||||||||||
| vein | Whipple | ||||||||||||||||
| SPAIN, | M | 22 | Yes | Cortisol | 122 | R | 4 0 | 0 | Liver | Suprahepatic | Vein graft | 1,700 | Alive with | 10 | |||
| Barcelona | disease | ||||||||||||||||
| SPAIN, | M | 42 | Non | 150 | R | 4 1 | 1 liver | Kidney, | Local | Alive with | 13 | ||||||
| Barcelona | lung | liver | disease | ||||||||||||||
| SPAIN, | F | 59 | Non | 88 | R | 4 1 | 1 liver | Kidney, | Suprahepatic | Primary | Alive with | 3 | |||||
| Barcelona | liver | repair | disease | ||||||||||||||
| SPAIN, | M | 45 | Non | 240 | L | Prehepatic | 4 0 | 1 lung | Kidney | Local | Primary | R0 | Alive with | 58 | |||
| Granada | IVC | repair | disease | ||||||||||||||
| SPAIN, | F | 74 | Yes | Cortisol | 100 | R | Prehepatic | 4 1 | X | Adrenal | Local | Primary | R1 | 87 | Liver, right | Deceased | 32 |
| Granada | IVC | only | repair | renal hilum | |||||||||||||
| SPAIN, | F | 19 | Yes | Cortisol | 90 | R | 3 0 | 0 | Adrenal | Local | Venotomy | 275 | Retroperitoneal | Deceased | 12 | ||
| Madrid | only | LN, lung, | |||||||||||||||
| bone | |||||||||||||||||
| SPAIN, | M | 60 | Non | 150 | L | Prehepatic | 4 0 | 0 | Kidney, | Extracorporeal | Primary | R0 | Spine | Deceased | 24 | ||
| Tenerife | IVC | spleen, | circulation | repair | |||||||||||||
| pancreas | |||||||||||||||||
| TURKEY, | F | 58 | Yes | Aldosterone | 18 | R | Under R | 4 X | lung | Adrenal | Local | Deceased | 7 | ||||
| Amir | hepatic | only | |||||||||||||||
| vein | |||||||||||||||||
| TURKEY, | M | 62 | Yes | Aldosterone | 110 | L | Under R | 4 X | 1 lung | Adrenal | Local | Deceased | 4 | ||||
| Amir | hepatic vein | only | |||||||||||||||
| TURKEY, | M | 53 | Non | L | Prehepatic | 4 X | 0 | Kidney | Local | Venotomy, | R0 | None | Alive without | 24 | |||
| Bursa | IVC | repair | disease | ||||||||||||||
| TURKEY, | F | 42 | Yes | Cortisol | 200 | L | Prehepatic | 4 0 | 0 | Adrenal | Suprahepatic | Vein graft | R0 | 168 | Alive without | 12 | |
| Ege | IVC | only | disease | ||||||||||||||
| TURKEY, | M | 68 | Non | 40 | R | Prehepatic | 4 1 | 0 | Adrenal | Local | Venotomy | R0 | Retroperitoneal | Deceased | 12 | ||
| Ege | IVC | only | LN | ||||||||||||||
| TURKEY, | F | 84 | Yes | Cortisol | R | Under R | 4 X | 1 lung, | Adrenal | Local | Deceased | 6 | |||||
| Istambul | hepatic | bone | only | ||||||||||||||
| vein |
A.
1.00
0.75
0.50
0.25
0.00
0
12
24
36
48
60
Time (months postop)
B.
1.00
0.75
0.50
0.25
0
1
0.00
0
3
6
9
12
Time (months postop)
subgroup of patients presenting with radiological evidence of tumour invasion into the inferior vena cava (IVC). This dataset is limited by the lack of a denominator as it remains unknown how many other patients with ACC presenting with IVC invasion did not undergo surgery. Further bias might be created by the possibility that some surgeons might have felt uncomfortable to report poor outcomes hence the study group might be dominated by cases with a positive outcome.
The median age of 54 years in this cohort of patients corresponds to the age-dependent incidence of ACC world- wide. Median tumour diameter of 115 mm (range 18-255 mm) confirms previous reports of a correlation between increasing diameter and malignant behaviour, with a risk of malignancy of over 50% in tumours larger than 8 cm [10]. There was a balanced distribution of functioning and non-functioning tumours, confirming previous reports that hormonal status does not correlate with aggressive behaviour [11].
All patients in this series had open adrenalectomy as the complex venous control precludes the use of a laparoscopic
approach. Furthermore the operations involved resection of surrounding viscera in two thirds of patients. These findings confirm the general view that open adrenalectomy is the procedure of choice for patients with known or suspected malignant adrenal tumours and that good results obtained with laparoscopic procedures [12] are due to strict patient selection.
This study focused on patients with vascular extension into the IVC who needed caval clamping, hepatic vascular exclusion, or cardiopulmonary bypass. This selection criteria would explain why the majority of tumours were right sided as it would easier for a tumour thrombus to extend into the IVC through the short right adrenal vein compared with expansion through the longer left renal vein. In addition, ACC can affect the IVC by compression or direct invasion. The retrospective nature of this study precluded the collection of such precise intraoperative data and it remains unknown how common it is for large ACCs to create critical extrinsec compression the IVC in the absence of direct intravascular tumour extension.
The perioperative mortality observed in this study was related to massive intraoperative blood loss or coagulop- athy, known complications of bypass surgery. Otherwise the morbidity reported in this series is minimal. This could be related to the fact that responders to the questionnaire work in high-volume centres with facilities for support/ input from other surgical specialities, such as cardiac surgery or liver surgery. In view of the retrospective collection of data, it remains possible that the record of morbidity was not fully accurate.
Majority of patients achieved a complete R0 resection status, suggesting either a reporting bias (i.e. surgeons being more likely to report cases with favourable oncolog- ical outcome) or a yet-to be reported preponderance of some tumours to invade preferentially the large vessels in the absence of any aggressive infiltration of surrounding viscera. The small number of patients presenting with such tumours and the retrospective nature of the study make impossible to explore this hypothesis further.
To date, there have been limited data regarding the impact of large vessel extension on overall survival and recurrence-free survival in ACC. A previous literature review of 106 cases published by early 2000 reported an overall survival of 9 months [8]. In a retrospective review of 57 patients undergoing ACC resection with curative intent over a decade at the Memorial Sloan Kettering Cancer Center, the 3-year overall survival with and without large vessel extension was 29% vs. 93% and 3-year recurrence-free survival was 15% vs. 67% [13], demon- strating the negative impact on survival once the large vessels have been invaded by tumour. In contrast, the median survival of 16 months observed in this cohort of patients provides support for a proactive management of
patients with extensive vascular invasion. Even more encouraging is that some patients are alive without signs of metastatic disease over 1-year after their operation.
In summary, the relatively low perioperative mortality and the long disease-free survival achieved in some cases should encourage surgeons with adequate experience to offer surgical treatment to patients presenting with locally advanced adrenocortical cancers. Invasion in to IVC should not deter from referring such patients for surgical intervention in centres with appropriate facilities and experience.
Conflicts of interest None.
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