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Manuscript Accepted Early View Article
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Early View Article: Online published version of an accepted article before publication in the
final form.
Journal Name: International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD)
Type of Article: Case Report
Title: Adrenal Cortical Carcinoma with Pulmonary Emboli: A Unique Presentation of a
Rare Tumor with Extensive Tumor Thrombus and Inferior Vena Cava Extension
Authors: Hoylan T. Fernandez, Peter T. W. Kim, Giuliano Testa
doi: To be assigned
Early view version published: March 18, 2016
How to cite the article: Fernandez H T, Kim P T W, Testa G. Adrenal Cortical Carcinoma
with Pulmonary Emboli: A Unique Presentation of a Rare Tumor with Extensive Tumor
Thrombus and Inferior Vena Cava Extension. International Journal of Hepatobiliary and
Pancreatic Diseases (IJHPD). Forthcoming 2016.
Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the
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Manuscript Accepted Early View Article
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TYPE OF ARTICLE: Case Report 1
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TITLE: Adrenal Cortical Carcinoma with Pulmonary Emboli: A Unique Presentation 3
of a Rare Tumor with Extensive Tumor Thrombus and Inferior Vena Cava Extension 4
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AUTHORS: 6
Hoylan T. Fernandez1, MPH, Peter T. W. Kim1, Giuliano Testa1, MBA 7
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AFFILIATIONS: 9
1MD, From the Annette C. and Harold C. Simmons Transplant Institute, Baylor 10
University Medical Center, Dallas, Texas. 11
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CORRESPONDING AUTHOR DETAILS 13
Hoylan T. Fernandez, MD 14
Annette C. and Harold C. Simmons Transplant Institute, Baylor University Medical 15
Center, 3500 Gaston Avenue, Dallas, TX 75246 United States 16
Phone number: 214-820-2050; 17
Email: hoylan.fernandez@baylorhealth.edu. 18
Fax: 214-818-6491 19
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Short Running Title: IVC Reconstruction for Adrenal Cortical Carcinoma 21
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Guarantor of Submission : The corresponding author is the guarantor of 23
submission. 24
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Manuscript Accepted Early View Article
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TITLE: Adrenal Cortical Carcinoma with Pulmonary Emboli: A Unique Presentation 33
of a Rare Tumor with Extensive Tumor Thrombus and Inferior Vena Cava Extension 34
35
ABSTRACT 36
37
Introduction 38
Adrenal cortical carcinoma (ACC) is rare, and presenting symptoms vary widely 39
depending on functional or non- functional endocrine status. These tumors are most 40
commonly treated with surgical resection and post-operative mitotane administration. 41
42
Case Report 43
This is an unusual presentation of a 23 year old female with no significant past 44
medical history, admitted to the hospital with syncope and dyspnea. CTA 45
demonstrated extensive bilateral pulmonary embolisms, with an associated 16cm 46
assumed right lobe hepatic mass with suprahepatic vena cava tumor thrombus 47
extension beyond the level of the hepatic veins. The patient underwent a complete 48
resection of the right adrenal mass, with inferior vena cava resection, thrombectomy, 49
and placement of caval interposition graft without the use of bypass. Pathology was 50
consistent with adrenal cortical carcinoma. 51
52
Conclusion 53
This case of an adrenal cortical carcinoma, with a rare presentation of bilateral 54
pulmonary embolisms, was treated with a surgical R0 resection. This included a 55
right adrenalectomy with IVC resection and interposition graft. Tumors with IVC 56
involvement and tumor thrombus can be treated with surgical resection and IVC 57
grafting, without the use of bypass. 58
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Keywords: inferior vena cava, adrenalectomy, adrenal cortical carcinoma 60
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Manuscript Accepted Early View Article
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TITLE: Adrenal Cortical Carcinoma with Pulmonary Emboli: A Unique Presentation 64
of a Rare Tumor with Extensive Tumor Thrombus and Inferior Vena Cava Extension 65
66
INTRODUCTION 67
Adrenal cortical carcinoma (ACC) is an extremely rare condition, with an incidence of 68
1 to 2 per million populations [1–5]. Patients may present with symptoms of steroid 69
surplus such as Cushing’s syndrome, virilization, hirsutism, or oligomenorrhea. In 70
atypical cases, presentations may include hypertension or electrolyte disturbances 71
associated with hyperaldosteronism. However, nonfunctioning tumors may present 72
with abdominal or back pain and vague gastrointestinal symptoms. These are 73
extremely aggressive tumors requiring a complete R0 resection and adjuvant 74
treatment with mitotane. Large-vessel invasion has been demonstrated as a poor 75
prognostic indicator, with a <30% 3-year survival rate.2,3 Resection of ACC with caval 76
extension and tumor thrombus has been described previously and has required a 77
cavotomy for thrombus removal or venous patch, conducted with the use of cardiac 78
bypass or veno-veno bypass [4,5]. This report describes a right ACC with rare 79
presentation of initial bilateral pulmonary embolisms and with suprahepatic vena 80
cava tumor thrombus extension above the level of the hepatic veins. The patient 81
required a right adrenalectomy, inferior vena cava (IVC) resection, thrombectomy, 82
and IVC replacement with composite graft, conducted without the use of bypass. 83
84
CASE REPORT 85
A 23-year-old woman with no significant past medical history was admitted to the 86
hospital with dyspnea and syncope. The admitting laboratory values included: Na 87
141meq/L, Cr 0.79mg/dL, glucose 129mg/dL, troponin 0.412ng/mL, D dimer 88
10.4ug/mL FEU, lactate 2mmol/L. A computed tomography scan of the head was 89
normal. However, a computed tomography angiogram of the chest demonstrated 90
extensive bilateral pulmonary emboli in the right and left main pulmonary arteries, 91
with extension into lobar, segmental, and subsegmental branches. Doppler 92
ultrasound of the lower extremities was negative for venous thrombosis. An 93
echocardiogram showed significant right atrial and ventricular dilation with markedly 94
reduced systolic function but preserved ejection fraction and a well-circumscribed 95
Manuscript Accepted Early View Article
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mass in the IVC consistent with thrombus. She was treated with tissue plasminogen 96
activator and continuous heparin infusion. 97
Ultrasound of the abdomen revealed a 16-cm mass thought to arise from the right 98
hepatic lobe with a 5-cm IVC thrombus. On subsequent magnetic resonance 99
imaging, a 16.6-cm mass was noted in the right upper quadrant adjacent to the 100
posterior right hepatic lobe with extension into the intrahepatic IVC and prominent 101
tumor thrombus extending just distal to the right atrium (Figure 1A–1C). Urine 102
metanephrine and normetanephrines were negative, but the free cortisol level was 103
elevated. Her dehydroepiandrosterone level was significantly elevated, while her 104
morning cortisol level was within normal limits. Tumor activity was negative for 105
adrenocorticotropic hormone, alpha-fetoprotein, and carbohydrate-associated 106
antigen 19-9. A transjugular biopsy of the tumor thrombus was attempted; however, 107
a large multilobular filling defect in the IVC was noted extending beyond the level of 108
the hepatic veins, resulting in an 80% stenosis (Figure 1D). Percutaneous biopsy 109
was consistent with ACC, with positive immunohistochemistry staining for inhibin and 110
MART-1 but negative staining for adrenocorticotropic hormone. 111
The tumor was arising from the right adrenal gland with multiple enlarged venous 112
collaterals enveloping the surface and adherent to the inferior aspect of the liver with 113
no apparent tumor infiltration of the liver or kidney. There was clear tumor infiltration 114
of the IVC and thrombus extending from the suprarenal cava to the level of the 115
hepatic veins (Figure 2A). 116
The liver was mobilized off of the right diaphragm and IVC. The right kidney was 117
spared by dissecting the tumor from the adrenal, with preservation of the right renal 118
vein. The adrenal mass was completely mobilized from the retroperitoneum. Total 119
vascular exclusion of the liver was performed by circumferential dissection of the 120
infrahepatic suprarenal cava, and the suprahepatic vena cava just below the hepatic 121
veins. Vascular clamps were positioned at the supra and infrahepatic caval positions, 122
ensuring the suprahepatic clamp was placed superior to the thrombus. The right 123
adrenal gland and IVC was removed en bloc (Figure 2B). A polyester composite 124
graft (Hemashield Gold 22 mm- Maquet Getinge Group) was used to reconstruct the 125
IVC in an end-to-end fashion (Figure 2C, 2D). Once the suprahepatic caval 126
Manuscript Accepted Early View Article
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anastomosis was completed, the clamp was moved below the hepatic veins to allow 127
for early reperfusion of the liver and to aid with overall hemodynamics. 128
Pathology was consistent with an 18-cm, 928-g right ACC with IVC and liver capsule 129
invasion. The margins were negative, with no evidence of perineural invasion. The 130
patient did well postoperatively with no immediate complications and was discharged 131
on a steroid taper. She commenced treatment with mitotane as an adjuvant therapy 132
and therapeutic lovenox for treatment of her pulmonary emboli. 133
134
DISCUSSION 135
Adrenal cortical carcinoma is an extremely rare disease, whose incidence has been 136
reported at 0.78 per million, though other sources may report a higher incidence. It 137
has been noted to be more prevalent in women, occurring either in childhood or in 138
the fourth or fifth decade. They may be glucocorticoid, androgen, estrogen, or 139
aldosterone producing. However, in rare cases these masses may not produce 140
hormones, appear clinically inactive, and are found incidentally. Symptoms of 141
inactive tumors may include abdominal or back pain, fever, weight loss, or may have 142
no apparent clinical symptoms associated with their presence. 143
Diagnosis of ACCs involves both hormonal work up and imaging studies. If 144
glucocorticoid production is suspected, the following should be performed: 145
dexamethasone suppression test, 24 hr urinary cortisol, serum cortisol, and serum 146
ACTH. In the case of androgen or estrogen production a serum DHEA-S, 17-OH 147
progesterone, androstenedione, testosterone, and a 17β estradiol should be 148
obtained. In cases of aldosterone production serum potassium, and an 149
aldosterone/renin ratio are required. A pheochromocytoma should be excluded in 150
the work up. In regards to imagine, CT or MRI offer high specificity, and a PET scan 151
may be helpful in identification of metastatic disease. The presence of tumor 152
thrombus may require Doppler ultrasound, or echocardiography to define the 153
margins of extension. Pursuing a biopsy is discouraged as hormonal work up and 154
imaging are sufficient for diagnosis, and may create the risk of metastasis. However, 155
if the mass is not amenable to surgical intervention, a biopsy may be required for 156
diagnostic purposes to dictate appropriate medical therapy. 157
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By definition, an ACC with tumor thrombus is classified as a T4 tumor, and stage III 159
cancer. In most cases, these tumors are amenable to surgical resection and come 160
with a high (60-80%) risk of recurrence even with a complete resection. 161
Thrombectomy is performed with the aid of IVC cross clamping, hepatic vascular 162
exclusion, or the use of cardiopulmonary bypass, determined by the margins of 163
thrombus extension. In some cases, replacement of the IVC by use of a vascular 164
graft may be necessary if the thrombus has invaded the vascular wall, or cannot be 165
easily removed, and should be performed if an R0 resection is anticipated. Adjuvant 166
therapy with mitotane is recommended and radiotherapy may be considered. Large 167
vessel invasion independently represents a poor prognostic factor, and confers an 168
inferior disease free survival as well as overall survival. Imaging with CT, and in 169
some cases PET, is recommended every 3 months for at least 2 years. Overall 170
prognosis is poor with a 5 year survival rate of <35% in complete resections, and 171
<10% in incomplete resections. Median survival for stage III cases with large vessel 172
invasion are thought to be approximately 9 months, with a significantly decreased 173
disease specific survival with the presence of tumor thrombus. 174
Surgical resection is the current standard of care in the treatment of ACC, with 175
extent of resection dictated by local invasion of surrounding organs and large vessel 176
invasion. A small number of case reports, case series, and reviews have described 177
resection of ACCs with caval extension and caval tumor thrombus requiring 178
cavotomy or limited IVC resection with emphasis on radical resection and requiring 179
cardiac or veno-veno bypass. While long term prognosis of these progressed ACCs 180
remains poor due to delayed diagnosis, frequent presence of advanced disease, and 181
lack of effective adjuvant treatment, the best management available is complete 182
surgical resection. 183
This case illustrates the use of radical resection with removal of suprahepatic vena 184
cava tumor thrombus above the level of the hepatic veins and composite graft 185
placement without the use of bypass. 186
187
CONCLUSION 188
An R0 resection and large-vessel venous reconstruction of these extensive 189
adrenalcortical carcinomas can be achieved without the aid of cardiac or veno-veno 190
Manuscript Accepted Early View Article
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bypass. Imaging is imperative in pre-operative planning, and a multi-disciplinary 191
team approach may be necessary. 192
193
ABBREVIATIONS 194
ACC, adrenal cortical carcinoma; IVC, inferior vena cava 195
196
CONFLICT OF INTEREST 197
There is no conflict of interest to report, including manuscript preparation or funding 198
by a commercial organization. 199
200
AUTHOR’S CONTRIBUTIONS 201
Hoylan T. Fernandez – Substantial contributions to conception and design, 202
acquisition of data, analysis and interpretation of data, drafting the article, revising 203
it critically for important intellectual content, final approval of the version to be 204
published 205
Peter T. W. Kim – Analysis and interpretation of data, revising it critically for 206
important intellectual content, final approval of the version to be published 207
Giuliano Testa- Analysis and interpretation of data, revising it critically for important 208
intellectual content, final approval of the version to be published 209
210
REFERENCES 211
1. Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis, and 212
treatment. Front Cell Dev Biol 2015; 3:45. 213
2. Gaujoux S, Brennan MF. Recommendation for standardized surgical 214
management of primary adrenocortical carcinoma. Surgery 2012; 152:123-215
32. 216
3. Turbendian HK, Strong VE, Hsu M, Ghossein RA, Fahey TJ 3rd. 217
Adrenocortical carcinoma: the influence of large vessel extension. Surgery 218
2010; 148:1057-64. 219
4. Chiche L, Dousset B, Kieffer E, Chapuis Y. Adrenocortical carcinoma 220
extending into the inferior vena cava: presentation of a 15-patient series and 221
review of the literature. Surgery 2006;139:15-27. 222
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5. Ochi T, Tanji N, Shimamoto K, Ikeda T, Toshino A, Yokoyama M. 223
Application of cardiopulmonary bypass for resection of renal cell carcinoma 224
and adrenocortical carcinoma extending into the right atrium. Int J Urol 225
2006; 13:202-5. 226
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FIGURE LEGENDS 228
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Figure 1: Magnetic resonance imaging demonstrating (A)- the right adrenal mass 230
involving the IVC with tumor thrombus; (B)- the origin arising from the right adrenal; 231
(C)- a coronal view showing IVC extension to the level of the hepatic veins; and (D)- 232
a venogram demonstrating IVC thrombus with resulting stenosis 233
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Figure 2: Surgical photos: (A) - right adrenal tumor in situ, with liver rotated medially 235
to expose the IVC; (B)- right adrenal tumor after removal; (C)- composite graft 236
reconstruction of the IVC due to tumor involvement; (D)- completion of IVC graft 237
reconstruction 238
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FIGURES 255
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Figure 1: Magnetic resonance imaging demonstrating (A)- the right adrenal mass 259
involving the IVC with tumor thrombus; (B)- the origin arising from the right adrenal; 260
(C)- a coronal view showing IVC extension to the level of the hepatic veins; and (D)- 261
a venogram demonstrating IVC thrombus with resulting stenosis 262
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Figure 2: Surgical photos: (A) - right adrenal tumor in situ, with liver rotated medially 278
to expose the IVC; (B)- right adrenal tumor after removal; (C)- composite graft 279
reconstruction of the IVC due to tumor involvement; (D)- completion of IVC graft 280
reconstruction 281
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