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Excerpta Medica
The American Journal of Surgery 192 (2006) 209-210 Clinical image
Virilizing adrenocortical carcinoma with cavoatrial extension
Michael W. Yeh, M.D.ª,*, Dean Lisewski, M.B.B.S., F.R.A.C.S.b, Peter Campbell, M.B.B.S., F.R.C.S., F.R.A.C.S.b
a Endocrine Surgical Unit, Wallace Freeborn Level 2, Royal North Shore Hospital, Pacific Hwy., St. Leonards, New South Wales 2065, Australia
b Endocrine Surgical Unit, Liverpool Hospital, Liverpool, New South Wales, Australia
Manuscript received July 15, 2005; revised manuscript January 1, 2006
A 53-year-old postmenopausal woman presented with hir- sutism, temporal balding, weight gain, and acne. Serum laboratory values included the following: testosterone, 14.3 nmol/L (normal, 1.0-4.5 nmol/L); free androgen index, 57% (normal, 1-8%); dihydroxyepiandosterone sulfate, 25 umol/L (normal, <5.2 umol/L); androstenedione, 90 nmol/L (normal, <3.0 nmol/L); cortisol, 616 nmol/L (nor- mal, 138-650 nmol/L). Computed tomography (Fig. 1) and positron emission tomography (Fig. 2) imaging showed a 12-cm right adrenal tumor with cavoatrial extension. Trans- esophageal echocardiography showed intravascular tumor occupying the inferior vena cava (Fig. 3, white arrow) and right atrium (Fig. 3, white arrowhead) and prolapsing across the tricuspid valve (Fig. 3, black arrow) during systole.
A palliative operation was performed with the goal of preventing sudden death from tumor embolization and intractable heart failure. The tumor was resected via an anterior midline approach. After gentle mobilization of the abdominal portion of the tumor from the adjacent liver and infrahepatic vena cava, we encountered the segment of vena cava directly invaded by the tumor. The affected patch of vein was excised near the origin of the right renal vein and the defect was closed longitudinally. Next, a sternotomy was performed and the patient was placed on cardiopulmonary bypass. The intravascular tu- mor mass then was extracted transatrially during 2 brief periods of hypothermic circulatory arrest. Fig. 4 shows the ex vivo specimen in its original orientation, with a tongue of intravascular tissue extending superiorly from the main body of the tumor. The patient made an un- eventful recovery and was treated with conventional che- motherapy. More than a month postoperatively, asymp- tomatic thrombosis of the inferior vena cava was
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detected. This was treated with warfarin. At the 6-month follow-up evaluation, the patient’s androgen levels had diminished from the preoperative levels but remained higher than normal. She reported mild improvement in her virilization. No locally recurrent or metastatic disease has been detected on repeat imaging to date.
Comments
Adrenocortical carcinomas are rare tumors, with an annual incidence of 1 per million. Most are functional and present with hypercortisolism, sex steroid excess, or a combination thereof. These tumors generally present at a late stage and have a poor prognosis, with the 5-year survival rate estimated at 25% [1]. Given the extent of disease in this case, it is likely that subclinical pulmonary metastases were present at the time of presentation.
Surgery is the mainstay of treatment for adrenocortical
* Corresponding author. Tel .: +61-2-9926-7014; fax: +61-2-9906- 3385.
carcinoma. Involvement of the vena cava may complicate up to 30% of cases, and the detection of a large (>9 cm) right-sided lesion should prompt preoperative caval imag-
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ing. Resection of adrenocortical carcinomas with caval and/or atrial extension generally should be performed using cardiopulmonary bypass. Without this measure, sudden in- traoperative death from embolization of the tumor or asso- ciated thrombus may occur [2]. In the present case, the main tumor mass was mobilized (using a no-touch technique) before placing the patient on bypass so that the patient’s coagulation system would be intact for the early phase of the operation. Although an argument could be made for placing the patient on bypass at the start of the procedure to eliminate any chance of embolization, we believed that manipulation of the abdominal portion of the tumor would add little to the agitation already being caused by the free prolapse of intravascular tumor into the right ventricle.
Even in the absence of cure, aggressive surgery for adrenocortical carcinoma provides relief from symptoms of steroid excess in up to one third of patients and may prolong survival [3].
References
[1] Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169:5-11.
[2] Hedican SP, Marshall FF. Adrenocortical carcinoma with intracaval extension. J Urol 1997;158:2056-61.
[3] Kopf D, Goretzki PE, Lehnert H. Clinical management of malignant adrenal tumors. J Cancer Res Clin Oncol 2001;127:143-55.