Urologia Internationalis
Urol Int 2011;87:182-185 DOI: 10.1159/000326942
Adrenal Tumors with Venous Thrombosis: A Single-Institution Experience
Yasser Osman Ahmed Haraz Mohsen El-Mekresh Abdul-Monem Gomha Mohamed Abou El-Ghar Ibrahim Eraky
Urology Department, Urology and Nephrology Center, Mansoura University, Mansoura, Egypt
Key Words
Adrenal tumors . Venous thrombosis . Management
Abstract
Objective: To assess the incidence, imaging, surgical ap- proach and prognosis of adrenal tumors associated with ve- nous thrombosis. Material and Methods: Charts of 206 pa- tients who underwent adrenal surgery were reviewed. Data of patients with pathologically confirmed venous thrombo- sis, utilized diagnostic modalities, operative treatment and prognosis were reviewed and analyzed. Results: Venous thrombosis was confirmed pathologically in 6 patients (2.9%). All were of male gender with age ranging between 2 and 54 years. The mean size of the masses was 11.5 ± 5.2 cm. Venous thrombosis was diagnosed preoperatively in 2 pa- tients, adrenal vein thrombosis in 1 patient, and renal vein thrombosis in the others. Masses were successfully excised via an open approach in association with nephrectomy in 3 cases. There was no operative mortality or gross morbidity. Pathologically, thrombosis was limited to the adrenal vein in 4 patients and extended to the renal vein in 2. Pathology of the masses revealed neuroblastoma in 2, pheochromocyto- ma in 2, adrenocortical carcinoma in 1, and pleomorphic sar- coma in 1 case. Metastasis developed within 6 months in 3 of these patients. Conclusion: Venous thrombosis with ad- renal tumors is a rare pathological condition in which open
surgery is the standard of care. Primary malignant adrenal masses with venous thrombosis have a poor prognostic out- come.
Copyright @ 2011 S. Karger AG, Basel
Introduction
Adrenal neoplasia associated with venous thrombosis is a rare clinical situation. Accurate diagnostic imaging should be performed preoperatively to help dictate the ideal surgical approach and to optimize successful treat- ment of the disease [1]. Although the risk of metastasis is high, long-term survival can be achieved after radical macroscopic resection [2]. In general, long-term progno- sis is poor owing to delayed diagnosis, frequent associ- ated metastatic disease and lack of effective adjuvant treatment [3]. Based on a single-institute experience, we aimed at assessing the incidence, imaging techniques, therapeutic options and prognosis of adrenal tumors as- sociated with venous thrombosis.
Patients and Methods
Between 1976 and 2005, 238 patients with 246 adrenal masses were diagnosed in a single institute. We retrospectively reviewed the histopathological term of adrenal masses with venous throm-
KARGER
Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com
Accessible online at: www.karger.com/uin
EN 12/1.11 0
F 455 566
Ir: 6+C
DFOV 2250G
12 Aug 200
51:
SIND
3
<V 120
A 270
arge
bosis in the clinical charts of 206 patients who underwent adrenal surgery. Six cases (2.9%) were diagnosed to harbor this pathology based on the final histopathology following radical adrenalecto- my. These patients were in the center of our study. Demographic data, mode of presentation, clinical and radiological findings, preoperative diagnosis, operative findings and pathological diag- nosis of the removed specimens were recorded.
Results
Venous thrombosis was confirmed pathologically in association with 6 masses of the excised 206 masses (2.9%). All patients were of male gender with a mean age of 25.7 ± 23.9 years (range 2-54). Masses were right-sided in 4 patients and left-sided in 2 patients. The presentation was loin pain in 3, abdominal mass in 2, while it was in- cidentally discovered in the remaining patient. General examination showed evidence of cachexia in 1 patient and hypertension in another. Serum catecholamine was re- quested for 2 patients and was proven normal in both. Al- though abdominal ultrasound could diagnose the adrenal masses in all, renal venous thrombosis was suspected in only 1 case. The size of the masses was 11.5 + 5.2 cm (range 4-20). All tumors showed extensive areas of hem- orrhage and necrosis. CT was performed in 4 patients and it could detect left renal vein thrombus reaching the infe- rior vena cava (IVC) (fig. 1) in only 1 patient. On the oth- er hand, MRI was requested for 2 patients and it con- firmed the diagnosis of a previously described thrombus in 1 patient in addition to an adrenal vein thrombus in the other. Venous involvement of the right renal vein was di- agnosed intraoperatively in an additional patient.
All of the masses were approached via an open ap- proach (thoracoabdominal in 3, abdominal in 2, dorso- lumbar in 1). Adrenalectomy was successfully accom- plished in all cases and it was associated with nephrec- tomy in 3 because of direct infiltration in 1 and associated renal vein thrombosis in 2. There was no op- erative mortality or gross morbidity with postoperative hospital stay of 5.3 ± 1.3 days (range 4-7). Pathologi- cally, venous thrombosis was limited to the adrenal vein in 4 patients and extended to the renal vein in 2. There was no evidence of wall invasion in any patient. Pathol- ogy of the masses was: neuroblastoma in 2, pheochromo- cytoma in 2, adrenocortical carcinoma in 1, and pleo- morphic sarcoma in the remaining patient. Metastasis developed within 6 months in 3 of these patients, who died within the first year postoperatively.
Discussion
Adrenal tumors with venous thrombosis are rare. It is difficult to determine an accurate incidence as most of the published cases are case reports or small series. We believe that the possible advantage of this contribution is that it could provide an accurate incidence as it depends upon pathological confirmation rather than radiological or clinical detection of the thrombi. Moreover, we includ- ed thrombi limited to the adrenal vein. We reported 6 cases of the excised 206 masses representing an incidence of 2.9%. All of our patients were of male gender. Con- versely, female predominance was observed in two inde- pendent series [1, 3] while an equal sex prevalence was reported in another report [4]. There are no age limits for venous involvement of adrenal tumors as has been re- ported for extremes of age - it may occur as young as 2 years [5] and as old as 80 [3]. The youngest age in our se- ries was 2 years, while the oldest was 56 years.
There is no side predominance for venous involve- ment in case of adrenal tumors. Nevertheless, right-sided masses are commonly associated with IVC thrombus ex- tension. This was explained by the longer length of the left adrenal vein that drains into the left renal vein in comparison with the short right vein draining directly to the IVC, which makes the thrombus way to the IVC easier and shorter on the right side than on the left side [1]. However, even right atrial involvement was reported secondary to left adrenal tumors [6]. The venous wall could be affected by either compression or direct inva- sion or by intraluminal extension in the form of throm- bus [3]. In this series, 4 of the masses were right-sided
while 2 were left-sided and none extended beyond the renal vein.
Patients with venous involvement may present with manifestations other than venous obstruction. It may be discovered incidentally [4], presented with non-specific manifestations such as abdominal pain, flank mass, fever, weight loss and wasting syndrome [7]. It could also be presented with manifestations of excess steroid secretion such as Cushing syndrome or with metastatic manifesta- tions [3, 4]. Our patients’ presentation ranged from inci- dentally discovered up to metastatic cachexia. In this se- ries, among 87 incidentaloma cases, only 1 patient (1.1%) had a pathologically proven venous thrombosis. Preop- erative diagnosis of venous involvement in case of adrenal tumors is troublesome, particularly if major veins are not involved. In this investigation, among 4 cases with ve- nous thrombosis limited to the adrenal vein, only 1 case could be diagnosed preoperatively with the aid of MRI. This could be of major clinical relevance because missing adrenal venous thrombosis could carry the risk of in- traopertive thrombus detachment with initial rough ma- nipulation of the gland. Thus we advise MRI to be our primary diagnostic modality and ask our radiologists to reconsider adrenal venous involvement in cases with ex- tensive hemorrhage and necrosis. Ryan et al. [8] reported a case of bilateral adrenal vein thrombosis that was diag- nosed by MRI while it could not be identified using US or CT.
Imaging of venous involvement of the renal vein with or without IVC is much easier. CT diagnosis of venous thrombosis is made using the indirect finding of massive enlargement of venous diameter with adjacent neoplasm or the direct finding of an intraluminal filling defect, while a definitive diagnosis is made using injection of in- travenous contrast material [9]. The role of US versus CT in the diagnosis of caval thrombosis has previously been studied. Didier et al. [10] found that the cranial extent of the tumor thrombus was better demonstrated by US studies whilst tumor thrombus was better outlined by CT, particularly when it extended beyond the limits of the IVC wall. On the other hand, neither method could ac- curately be used to predict IVC wall infiltration when the tumor thrombus remained within the confines of the IVC, nor could either method differentiate tumor from non-tumor thrombi. Recently, the combination of fluo- rine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) and CT (18F-FDG-PET/CT) technol- ogy for diagnosis of tumor thrombus extension was rec- ommended as it revolutionizes imaging by fusing func- tional and anatomical criteria for tumor staging [11].
The size of masses ranged from 4 to 20 cm and deter- mined our surgical approach. We prefer to use the thora- coabdominal approach above the 10th rib, when the mass is on the right side, and of huge volume. In such a situa- tion, the transpleural, transperitoneal approach provides adequate field exposure, rapid pedicle control and mini- mizes procedural complications and bleeding. In addi- tion, it facilitates IVC manipulation. In left-sided tumors we preferred the ventral approach, as it allows for rapid vascular control before dissection of the mass itself. Three of our patients underwent nephrectomy with adrenalec- tomy. In 1 case, the tumor was aggressive and infiltrated the kidney; there was no obvious plain of separation and definitive pathology confirmed renal involvement. In the remaining 2 cases, nephrectomy was indicated in view of renal vein thrombosis. Adrenal tumors associated with venous thrombosis are aggressive; most reported cases were associated with nephrecotmy [1, 3, 6]. In addition, partial hepatectomy, splenectomy and even distal pancre- atectomy might also be needed if these organs are infil- trated [1, 12, 13].
Involvement of the right atrium is extremely rare. Only a few reports have demonstrated involvement of the right atrium by tumor thrombus extension from adrenal tumors. In this critical situation, the thrombus is excised with the help of cardiopulmonary bypass (CPB) [14]. However, total vascular exclusion of the liver may replace CPB with minimal morbidity, which can be done by ap- plying the upper caval clamp across the right atrium [15]. If the tumor thrombus does not extend to the right atri- um, thrombectomy can be done without CPB [16].
Pathologically, we report only 1 case of adrenocortical carcinoma (ACC). ACC accounts for most published se- ries of adrenal tumors with venous involvement [1, 3, 7, 14, 16]. It is usually associated with metastatic disease [4]. Neuroblastoma was found in the final pathology of 2 cas- es. Neuroblastoma is the most poorly differentiated type of neuroblastic tumor and usually occurs in children. However, if it occurs in adults it has similar imaging fea- tures as ACC. Custodio et al. [12] reported a case of neu- roblastoma with tumor thrombus extension to the right atrium in a 32-year-old female patient. We report a case of pleomorphic sarcoma of the adrenal gland. Pleomor- phic sarcoma is an extremely rare mesenchymal tumor of the adrenal gland. To the best of our knowledge, this is the fourth case that is reported in the literature as only 3 case reports had been published before [17].
As a result of delayed diagnosis, frequent associated metastatic disease and lack of effective adjuvant treat- ment, the overall prognosis is poor [3]. Lucon et al. [16]
reported 7 cases with adrenal tumors and venous throm- bosis. In this report, prognosis was closely related to his- topathology. Among 5 patients with ACC, 4 developed metastatic disease within a few months of radical resec- tion. On the other hand, 2 out of 3 patients with pheo- chromocytoma enjoyed long-term survival. Half of our patients developed metastatic disease within 6 months of surgery; those with pheochromocytoma and 1 case with neuroblastoma were cured. Generally, Peix et al. [2] re- ported long-term survival after radical macroscopic re- section in half of their patient population.
Conclusions
Adrenal tumors in association with venous thrombo- sis are a rare pathological condition. As no effective adju- vant treatment is currently available, we recommend ag- gressive surgery. However, surgical outcome of such tu- mors remains poor.
References
1 Figueroa A, Stein J, Lieskovsky G, Skinner D: Adrenal cortical carcinoma associated with venous tumour thrombus extension. Br J Urol 1997;80:397-400.
2 Peix J, Mancini F, Villard J, Van Box Som P: Malignant cortico-adrenal tumors with vena cava extension. Is surgical resection justi- fied? Ann Chir 1998;52:357-363.
3 Chiche L, Dousset B, Kieffer E, Chapuis Y: Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. Surgery 2006;139:15-27.
4 Ohwada S, Izumi M, Tanahashi Y, et al: Combined liver and inferior vena cava resec- tion for adrenocortical carcinoma. Surg To- day 2007;37:291-297.
5 Godine L, Berdon W, Brasch R, Leonidas J: Adrenocortical carcinoma with extension into inferior vena cava and right atrium: re- port of 3 cases in children. Pediatr Radiol 1990;20:166-169.
6 Yavascaoglu I, Yilmaz M, Kordan Y: Cardiac and caval invasion of left adrenocortical car- cinoma. Urol Int 2008;81:244-246.
7 Ortiz Gorraiz M, Tallada Buñuel M, Vicente Prados FJ, et al: Left adrenal carcinoma with caval thrombosis. Arch Esp Urol 2003;56: 485-489.
8 Ryan M, Murphy J, Jay R, Callum J, MacDon- ald D: MRI diagnosis of bilateral adrenal vein thrombosis. Br J Radiol 2003;76:566- 569.
9 Marks W, Korobkin M, Callen P, Kaiser J: CT diagnosis of tumor thrombosis of the renal vein and inferior vena cava. AJR Am J Roent- genol 1978;131:843-846.
10 Didier D, Racle A, Etievent J, Weill F: Tumor thrombus of the inferior vena cava second- ary to malignant abdominal neoplasms: US and CT evaluation. Radiology 1987;162:83- 89.
11 Lai P, Bomanji J, Mahmood S, et al: Detec- tion of tumour thrombus by 18F-FDG-PET/ CT imaging. Eur J Cancer Prev 2007;16:90- 94.
12 Custodio C, Semelka R, Balci N, Mitchell K, Freeman J: Adrenal neuroblastoma in an adult with tumor thrombus in the inferior vena cava. J Magn Reson Imaging 1999;9: 621-623.
13 Wei C, Chen K, Chen M, Lai H, Chang L: Adrenal cortical carcinoma with tumor thrombus invasion of inferior vena cava. Urology 1995;45:1052-1054.
14 Baguet J, Cracowski J, Chavanon O: Right atrial thrombus from an adrenocortical car- cinoma. Heart 1999;82:522.
15 Huguet C, Caporossi M, Gavelli A, Harb J, McNamara M: Neoplastic thrombosis of the inferior vena cava involving the right atrium caused by adrenal cortical carcinoma. A new indication for vascular exclusion of the liver. Ann Chir 1994;48:364-369.
16 Lucon A, Machado M, Pereira M, Mendonça B, Praxedes J, Arap S: Surgery for adrenal tu- mours with thrombus in the supra-dia- phragmatic infra-atrial inferior vena cava, with no cardiopulmonary bypass. BJU Int 2004;94:70-73.
17 Mohanty S, Balani J, Parwani A: Pleomor- phic leiomyosarcoma of the adrenal gland: case report and review of the literature. Urol- ogy 2007;70:591-597.
Copyright: S. Karger AG, Basel 2011. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.