Rapid Extension of Left Adrenocortical Carcinoma into the Right Atrium
John J. Lee, MD, Joel Kupfer, MD, Sharo Raissi, MD, Stephen A. Geller, MD, and Robert J. Siegel, MD, Los Angeles, California
Adrenocortical carcinoma is a rare tumor which can extend into the right heart via the inferior vena cava. We describe a case of a 26-year-old woman who had progressive shortness of breath, intermittent chest pain, and peripheral edema. A two-dimensional echo- cardiogram on hospital admission showed a large multinodular mass in the right atrium which extended
into the inferior vena cava. Further studies showed that this mass was adrenocortical carcinoma. An echocar- diogram performed 3 weeks before admission was com- pletely normal. It appears that an adrenocortical carci- noma is capable of rapid growth up the inferior vena cava and into the right atrium. (J Am Soc Echocardiogr 1998;11:86-8.)
Adrenocortical carcinoma is a rare tumor with an estimated incidence of about 1 case per 1,700,000 people.1 This tumor is similar to hepatocellular carci- noma and renal cell carcinoma by virtue of its ability to extend into the inferior vena cava and right atrium via venous tributaries. A limited number of such cases have been published.2-4 We report on a 26-year-old patient with a left adrenocortical carcinoma with rapid extension into the right atrium.
CASE REPORT
A 26-year-old white woman was admitted with a 2-month history of progressive shortness of breath, intermittent chest pain, and bilateral lower extremity edema. An outpa- tient two-dimensional echocardiogram obtained 3 weeks before admission was completely normal. Other significant symptoms included a 3-week history of abdominal bloating and discomfort, hirsutism, and irregular menses. The phys- ical exam was remarkable for facial and chest hair, and 2+ pitting edema extending up to the thighs. A repeat two- dimensional echocardiogram on hospital admission showed a large multinodular mass in the right atrium which extended into the inferior vena cava. A computed tomog- raphy scan of the abdomen showed a 9 x 9 x 10 cm mass inseparable from the upper pole of the left kidney with extension into the inferior vena cava. A magnetic resonance
imaging scan showed that the mass originated from the adrenal gland. A ventilation/perfusion lung scan showed major mismatched defects in the left lung consistent with multiple pulmonary emboli. The patient subsequently un- derwent surgical resection of the adrenal mass as well as the left kidney and spleen. The tumor in the inferior vena cava and right atrium was extracted with the patient on cardio- pulmonary bypass. Images from the intraoperative trans- esophageal echocardiogram are shown in Figure 1. The patient tolerated the surgery well and was discharged 3 weeks after the procedure. She is scheduled to receive adjuvant chemotherapy with ortho para DDD. The histo- pathology showed that the tumor invading the inferior vena cava was adrenocortical carcinoma. The gross pathol- ogy specimen is shown in Figure 2. The serum cortisol and DHEA-SO4 levels were normal, but the serum testosterone level was markedly elevated (615 ng/dl; normal 15 to 70 ng/dl) showing that the carcinoma was an androgen- secreting tumor.
DISCUSSION
The unusual features of this case are the extension of a left adrenocortical carcinoma into the inferior vena cava and right atrium and the rate at which the tumor grew into the right atrium. Nine of the ten previous reports of an adrenocortical carcinoma extending into the inferior vena cava involved the right adrenal gland.5 This finding is explained by the direct course of the short right adrenal vein into the inferior vena cava compared with the longer left adrenal vein that drains into the renal vein. Thus, a left adrenocortical carcinoma which ex- tends beyond the inferior vena cava and into the right atrium is distinctly rare. More striking is the rate at which the tumor grew into the right atrium from the inferior vena cava. Indeed, even on review, the two-
Division of Cardiology, Department of Cardiothoracic Surgery, and the Department of Pathology and Laboratory Medicine, Ce- dars-Sinai Medical Center and University of California, Los Ange- les, School of Medicine.
Reprint requests: Robert J. Siegel, MD, Cardiac Noninvasive Laboratory Cedars-Sinai Medical Center, 8700 Beverly Blvd., Rm. 5336, Los Angeles, CA 90048.
Copyright @ 1998 by the American Society of Echocardiography. 0894-7317/98 $5.00+0 27/4/82577
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dimensional echocardiogram obtained 3 weeks before admission failed to identify tumor in the right atrium or proximal inferior vena cava. It appears that an adreno- cortical carcinoma is capable of rapid growth up the inferior vena cava and into the right atrium. Therefore the possibility of a mass in the right atrium being an
adrenocortical carcinoma should not be discounted on the basis of its sudden appearance.
Clinical features of an adrenocortical carcinoma include those caused by the local mass effect, such as flank pain and abdominal fullness, or those caused by unregulated hormone production by the tumor.6
When cortisol is overproduced, signs and symptoms of Cushing’s syndrome is evident. Androgen secre- tion, which is less common, results in virilization, hirsutism, and amenorrhea. Estrogen- and aldoste- rone-secreting tumors have been reported, but are rare. The clinical features of right atrial involvement are due to central or pulmonary embolization and cavity and/or valve obstruction, often with associ- ated congestive heart failure. Complete surgical re- section is the only treatment that offers potentially long-term disease-free survival.6
Chemotherapy with ortho para DDD, which blocks the 11 beta-hydroxylase, is recommended for the treatment of residual or recurrent tumor after surgery or for patients with metastatic disease. Tem- porary regression of the tumor with prolonged sur- vival can be expected. However, the prognosis of patients with adrenocortical carcinoma is generally poor. The mean survival without treatment is approx- imately 3 months. With treatment, the 5-year survival rate is about 30%. Because of the paucity of reported cases, the effect on prognosis of right atrial involve-
ment or pulmonary metastases in unknown. How- ever, the limited information available suggests that this is a poor prognostic sign.
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