International Journal of Surgery Case Reports 3 (2012) 302-304
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International Journal of Surgery Case Reports
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INTERNATIONAL JOURNAL OF SURGERY CASE REPORTS Advancing surgery through shared experience
Adrenocortical carcinoma extending into the inferior vena cava in a patient with right kidney agenesis: Surgical approach and review of literature
Roberto Luca Meniconi*,1, Roberto Caronna, Monica Schiratti, Giuseppe Dinatale 1, Gabriele Cosimo Russillo, Alessia Liguori, Piero Chirletti
Department of Surgical Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy
ARTICLE INFO
Article history: Received 8 February 2012 Received in revised form 13 March 2012 Accepted 22 March 2012 Available online 3 April 2012
Keywords: Adrenocortical carcinoma Inferior vena cava Thrombectomy
ABSTRACT
INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis and the association with tumor thrombus into the inferior vena cava (IVC) is not common. The best treatment is represented by radical surgery.
PRESENTATION OF CASE: We describe a case of a large ACC of the left adrenal gland extending into the IVC through the left renal vein in a young patient with agenesis of the right kidney and signs of acute renal failure. A midline laparotomy was performed, subsequently extended by a left thoracophrenotomy through the 7th intercostal space in order to control the proximal surface of the mass and the thoracic aorta. The tumor was completely excised preserving the kidney, and thrombectomy was performed by a cavotomy with a temporary caval clamping, without cardiopulmonary by-pass (CPB).
DISCUSSION: We discuss surgical approaches reported in literature in case of ACC with intracaval exten- sion. The tumor must be completely resected and the thrombectomy can be performed by different approaches: cavotomy with direct suture, partial resection of caval wall without reconstruction, resec- tion of vena cava with graft reconstruction. These procedures could require a CPB, with an increased mortality. In our case we preserved the kidney and a thrombectomy without CPB was performed. CONCLUSION: Intracaval extension of ACC does not represent a contraindication to surgery. The best treatment of intracaval thrombus should be the cavotomy with direct suture. The CPB is not always required. In presence of renal agenesis, the preservation of the kidney is mandatory.
@ 2012 Surgical Associates Ltd. Published by Elsevier Ltd. Open access under CC BY-NC-ND license.
1. Introduction
Adrenocortical carcinoma (ACC) is a rare malignancy (0.5-2 cases per million/year) with a poor prognosis (5-year overall sur- vival: 32-45%) related to delay in diagnosis and to lack of effective adjuvant treatment.1 Although prognostic factors are unclear, sev- eral studies indicate that radical surgical resection can improve survival rate.2 The prognostic value of intravenous extension is still controversial and debated but does not represent a contraindica- tion to surgery.3-5 Involvement of the inferior vena cava (IVC) was reported in only single case reports and small series with the excep-
tion of Chiche’s paper,6 but no work about ACC with caval extension and contralateral renal agenesis has been described.
We present the case of a large ACC of the left adrenal gland extending into the IVC through the left renal vein in a young patient with agenesis of the right kidney and signs of renal injury. The aim of this report is to consider the best surgical approach and the oper- ative strategy to perform a complete surgical resection of the tumor preserving the left kidney without the need of a cardiopulmonary by-pass (CPB).
2. Case presentation
2.1. Medical history
A 43-year-old man, without any other pathologies, noticed the presence of a left varicocele, confirmed at Doppler ultrasound. An abdominal ultrasonography showed the presence of a bulky retroperitoneal mass on the left side and a right renal agene- sis. A full-body CT scan confirmed that the mass (15 cm x 10 cm) originated from the left adrenal gland, displacing the left kid- ney medially and the spleen laterally, adhering to the stomach, the left hemidiaphragm and the abdominal aorta. The presence of a neoplastic thrombus in the left renal vein extending into
* Corresponding author at: Department of Surgical Sciences, University of Rome, “La Sapienza”, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy. Tel .: +39 6 49970385.
E-mail addresses: roberto.meniconi@gmail.com (R.L. Meniconi),
roberto.caronna@uniroma1.it (R. Caronna),
monica.schiratti@uniroma1.it (M. Schiratti),
giuseppedinatalef4@tiscali.it (G. Dinatale), gabriele.russillo@libero.it (G.C. Russillo), quasar177@yahoo.it (A. Liguori),
piero.chirletti@uniroma1.it (P. Chirletti).
1 General Surgery Residency.
2210-2612 @ 2012 Surgical Associates Ltd. Published by Elsevier Ltd. Open access under CC BY-NC-ND license. http://dx.doi.org/10.1016/j.ijscr.2012.03.026
the infrahepatic IVC was also shown (Fig. 1). A whole-body scintigraphy was negative for bone metastases. Laboratory tests showed creatinine levels of 2.1 mg/dl (normal range: 0.5-1.2 mg/dl) and glomerular filtration rate (GFR) of 54 ml/min (normal range: 100-130 ml/min) while plasmatic levels of cortisol, aldosterone, 17-OH-progesterone, DEAH-S and delta-4-androstenedione were normal. Considering the presence of renal failure signs in a young patient with congenital solitary kidney, we decided for a surgical approach.
2.2. Surgical intervention
A retrohepatic IVC filter was placed preoperatively by a right trans-jugular access. The operating room was equipped for intraop- erative blood salvage and for a CPB. The surgical procedure started by a midline laparotomy. Since it was confirmed that the exten- sion of the tumor thrombus involved only the infrahepatic IVC and the mass had close adherences with the abdominal aorta and the left hemidiaphragm, we decided to extend the incision by a left thoracophrenotomy through the 7th intercostal space in order to take control of the proximal extremity of the mass and the descending thoracic aorta for a possible emergency aortic clamp- ing. After mobilization of the spleen and the pancreas tail, we proceeded with an en bloc resection of the mass and the para- aortic lymph nodes, preserving the kidney. The infrahepatic and retrohepatic IVC was prepared and venous control was achieved by placing three tourniquet (two on the IVC under and above the renal vein and one around the left renal vein). After caval clamp- ing, the thrombectomy was performed by a longitudinal incision of the cava, without the need of a CPB. The IVC thrombus was dissected by forceps while the renal thrombus was removed insert- ing a Fogarty catheter into the left renal vein with immediate resumption of blood flow after the procedure. The caval inci- sion was closed by a direct suture. The post-operative course was uneventful, the renal function improved rapidly and the patient was
discharged in the 12th postoperative day with creatinine serum levels of 1.1 mg/dl and a GFR of 102 ml/min. Histological examina- tion revealed extensive necrosis and epithelioid cells with a clear and eosinophilic cytoplasm resembling adrenocortical cells with atypical nuclei and large nucleoli (Fuhrman’s grade III/IV) and high mitotic index. The mass resulted completely excised with nega- tive margins and associated to neoplastic thrombus of IVC and left renal vein with no involvement of para-aortic lymph nodes. Five months after surgery, the patient developed liver metastases and initiated adjuvant treatment with mitotane, an adrenocorticolytic drug.
3. Discussion
The approach to a patient with ACC extending into the IVC is certainly complex. Considering that prognosis depends strictly on a radical resection, the main aim is to obtain a complete exere- sis of the tumor.1,2 Therefore an aggressive approach is required, which should preserve the omolateral kidney whenever possible, performing a regional lymphadenectomy and thrombectomy.3,4 It is a surgery that requires some technical skills.
The first issue is to choose the best surgical incision: mid- line laparotomy with or without sternotomy for the CPB, sub-costal incision with midline extension, thoracophrenolaparo- tomy, laparotomy + thoracotomy (2 different incisions). In Chiche’s paper,6 of 15 patients with ACC extending into the IVC, they were performed 3 sterno-laparotomies, 2 thoracophrenolaparotomies, 4 sub-costal incisions + midline extension, 2 sub-costal incisions, 3 subcostal incisions + sternotomy, 1 thoracoabdominal incision. Most authors suggest that the subcostal incision guaranties a valid surgical approach in terms of radical exeresis, lymphadenectomy and eventual liver resections.1 The length of the incision may be adapted to each case and prolonged bilaterally. In our case we decided to start with a midline laparotomy to verify the possibility of opening the infrahepatic IVC without CPB. Then, we extended the incision to the 7th left intercostal space (thoracophrenola- parotomy) in order to control the descending thoracic aorta if an emergency aortic clamping could be needed during the dissection of the mass. Moreover, in our case, the mass size and its exten- sion to the diaphragmatic hiatus suggested an adequate access to the supradiaphragmatic aorta in order to control any bleed- ing.
About the approach to the IVC, there are some consideration to make. The first issue is how to achieve a complete venous control in order to perform safely the thrombectomy. Chiche et al.6 reported their experience of thrombectomy by caval clamping alone (26.7% of cases), hepatic vascular exclusion (33.3%) and CPB with or with- out hypothermic heart arrest (40%) depending on the upper limit of the thrombus (infrahepatic, retrohepatic or suprahepatic IVC, respectively) and the involvement of the right atrium. Postop- erative mortality was higher in CPB group as also reported by others.7-10 In our patient, the extension of tumor thrombus into the infrahepatic IVC allowed us to perform a direct caval approach without recurring neither to CPB nor to hepatic vascular exclusion, even if the operating room was equipped for that. It is relatively simple and advisable to clamp downstream of the thrombus if it does not extend beyond the infrahepatic IVC. On the contrary it is necessary to perform liver vascular exclusion and CPB, if a more extended thrombus is present.6,11,12 There is no agreement on the indication to place an IVC filter. It reduces the risk of pulmonary embolism due to thrombus fragmentation but we believe that it could be an obstacle to caval clamping and expose to the risk of filter dislocation in the right atrium.
Another issue is how to remove the caval thrombus: thrombec- tomy by cavotomy, partial resection of caval wall without
reconstruction, resection of vena cava with graft reconstruction. As reported,6 a thrombectomy was possible in 86.6% of cases, a resection of the IVC with direct suture in 6.7% and a resection of the IVC with graft reconstruction in 6.7%. On the other hand the massive involvement of the IVC is generally considered a contraindication for resection of retroperitoneal tumors.4,13 Caval clamping is indi- cated only if the thrombus does not exceed the suprahepatic veins but it is mandatory to provide a rapid volemic expansion just before and during clamping in order to avoid a cardiac arrest. Thrombus has a colloidal appearance and is very friable but it is generally easy to dissect from the caval wall. Direct suture of the vena cava is not complex, but it should avoid a narrowing more than 50%.1 In our patient a prolonged caval clamping and the positioning of IVC filter allowed us to perform also a thrombectomy of the left renal vein in order to avoid a left nephrectomy.
Some studies showed that survival and quality of life of these patients improve after radical surgery.1,6 Local stage, curative surgery, age less than 35 years and no other organ resections are significantly associated with a better survival.1 On the other hand, an aggressive approach is not recommended for patients with metastases. In fact, in these cases, even after radical surgery, 2-year-survival rate is less than 20%.1
About chemotherapy, mitotane seems to be moderately effec- tive and is used for metastatic disease as in our patient after developing liver metastases.
4. Conclusions
Surgical resection of ACC extending into the IVC is feasible and does not represent a contraindication to surgery. Surgical access, especially in case of large tumors extending up to the diaphragmatic hiatus, should provide a control of the descend- ing thoracic aorta (thoracophrenolaparotomy). Short or partial caval clamping is more advisable than CPB because of less post- operative complications and mortality. In case of unilateral renal agenesis, a kidney sparing surgery is mandatory. Unfortunately long-term prognosis of ACC extending into the IVC remains poor owing the delay in diagnosis and lack of effective adjuvant treat- ment.
Conflict of interest statement
The author(s) declare that they have no competing interests.
Funding
None.
Ethical approval
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in- Chief of this journal on request.
Author’s contributions
RLM and RC collected the data, reviewed the literature and drafted the manuscript; MS, GD, GCR and AL were involved in the patient’s care; PC, RC and RLM performed the operation; PC reviewed the manuscript for intellectual content. All authors read and approved the final manuscript.
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