Adrenocortical carcinoma with extension into inferior vena cava and right atrium: report of 3 cases in children
L. B. Godine1, W. E. Berdon2, R. C. Brasch3 and J. C. Leonidas4
1 Department of Radiology, Westchester County Medical Center,
2 Department of Radiology, Babies Hospital, Columbia Presbyterian Medical Center, New York
3 Department of Radiology, University of California, San Francisco, Calif., and
4 Department of Radiology, Schneider Children’s Hospital, New Hyde Park, New York, USA
Abstract. We present three cases of adrenocortical carci- noma, a rare tumor in children, with extension into the inferior vena cava and the right atrium. The diagnosis is facilitated by use of ultrasonography, computed tomog- raphy and magnetic resonance imaging.
Adrenocortical carcinoma in children is a highly malig- nant tumor. It tends to rapidly extend locally by vascular and visceral invasion, with distant metastases to lung, bone and brain. There have been a few isolated cases of extension into the right atrium in children and adults [1- 3]. We report three new cases of adrenocortical carcinoma extending into the right atrium, two of whom had mag- netic resonance imaging (MRI).
Case 1: virilization of one years duration in a 13-year-old girl
The patient presented to an ENT specialist because of hoarseness and deepening voice. Speech therapy was recommended. Over the next year she developed increasing hirsutism, acne and clitoro- megaly. Androgen levels revealed marked elevations of testosterone and dihydroepiandrosterone stearate. Further laboratory tests showed abnormal levels of adrenal steroids, including testosterone, androstenedione, 17-hydroxy-progesterone, and dihydroepiandro- strone. Cortisol and urinary catecholamines were normal. Physical examination revealed a thin, hirsute girl with a deep voice, pre- auricular and submental facial hair, bitemporal balding, moderate facial, chest and back acne, abundant pubic hair in a male distribu- tion and an enlarged clitoris.
Ultrasound examination showed an 8×7×6 centimeter com- plex right abdominal mass containing punctate calcifications and echogenic linear septa, separate from and depressing the right kid- ney, with extension into the inferior vena cava and right atrium (Fig. 1 a).
Examination was performed on a 1.5 T magnet, using T1 weighted images in coronal plan and T2 in sagittal and axial planes
(Fig. 1 b, c). The T1 weighted images showed a large, inhomogene- ous supraranal mass with an overall signal similar to that of liver but with a higher intensity than liver on T2 weighted images. The mass displaced the right kidney inferiorly but did not invade it (Fig. 1 b). Occupying most but not all of the inferior vena cava was a large thrombus that extended into the right atrium (Fig. 1c). Distinction between bland and tumor thrombus could not be made.
Surgery was performed by a combined thoracoabdominal ap- proach with the patient on cardiopulmonary bypass. A large right adrenal tumor was removed as well as the tumor thrombus within the inferior vena cava and right atrium. The atrial part of the tumor thrombus, largely acellular, was particularly friable and necrotic. The patient has subsequently developed areas of pulmonary and mediastinal recurrent tumor.
Case 2: functioning adrenocortical carcinoma with unsuspected RA extension
A 2-year-old white female was seen because of dehydration and seizures. The patient had a recent history of abdominal enlargement. Physical examination revealed a lethargic febrile patient with a BP of 130/88. A 10×6 cm abdominal mass was felt in the left upper quadrant. The patient had genital enlargement with pubic hair. The breasts showed mild enlargement with buds. The patient had acne and hirsutism.
Abdominal CT showed a left suprarenal partly calcified mass with caval extension. Laboratory data showed hypoglycemia and an hematocrit of 48. At surgery a left subcostal incision was made and the tumor was mobilized. It was felt that the left kidney had to be removed to adequately excise the adrenal mass. The in- ferior vena cava was examined and the tumor mass was found to extend above the hepatic vena cava into the right atrium. This had not been recognized in the preopoerative evaluations. A Fogarty catheter was passed in an attempt to remove the caval-atrial exten- sion.
The patient suddenly became cyanotic and a pulmonary embolus was diagnosed. Emergency thoracotomy was performed. The pa- tient became hypotensive and cardiac arrest occurred. Resuscitation was unsuccessful. The primary tumor was quite necrotic and, in the course of the abdominal exploration, evidently the atrial tumor broke up and fragments occluded the pulmonary outflow tract and pulmonary arteries.
a
85573
001/01
Mag Factor :
P
005/013
1.5
b
: 03970
H 10. 0 mm
: 001/01
: 009/002
Mag Factor : 1.5
C
a
b
a
b
Case 3: non-functioning adrenal cortical carcinoma
A 17-year-old girl was seen in February 1983 for abdominal pain, myalgias, and loose stools. Physical examination revealed a large right sided abdominal mass plus signs of ascites. The right sided ab- dominal mass was non-calcified. Venography showed inferior vena caval invasion; computerized tomography confirmed this. MRI in March 1983 showed the mass to extend into the right heart through the inferior vena cava (Fig.3 a). The liver was free of metastases.
Biopsy of the primary tumor showed an adrenocortical carcino- ma with extensive necrosis. Though there was some elevation of 17 hydroxycorticosteroids (not suppressed by dexcamethasone) the tumor was felt to be basically nonfunctioning. Chemotherapy (Mito- tane, Ortho para DDD) was utilized and 5000 rads of radiation was also given; there was no obvious tumor shrinkage and liver meta- stases developed (Fig.3b). The patient died nine months after diag- nosis with widespread metastases.
Discussion
Most pediatric patients with adrenocortical carcinoma have hormonally active tumors (unlike adults where the masses are usually “silent”). Girls present with viriliza- tion, while boys have precocious puberty. Less often, there is Cushing’s syndrome or hyperaldosteronism.
The diagnosis of the non-functioning tumors is often delayed until the mass becomes huge or systemic symp- toms arise; this is true in children as well as in adults. The tumor has been observed in association with hemihyper- trophy as well as the Beckwith-Wiedemann syndrome. It rapidly invades locally, spreading to regional lymph nodes, kidney, liver, diaphragm, and renal vein. Inferior vena caval and right atrial extension is uncommon [1-3]. Distant metastases to lungs, bone and brain occur in many cases. Prognosis is very poor in all age groups.
The ultrasound and CT features of the primary tumor in our three cases are similar to those described in the adult literature. The primary tumors are large, and the complex echo pattern on ultrasound correlates with the central necrosis, hemorrhage and calcification. Radiating septa or an echogenic, capsule-like rim can be seen.
The CT appearance of adrenocortical carcinoma is non-sepcific. The primary cancers, as in our cases, usually show an inhomogeneously enhancing mass with central areas of lower attenuation secondary to hemorrhage, ne- crosis or bright areas of calcification. However, none of
these parameters (size, inhomogeneous enhancement, central necrosis, calcification, enhancing capsule) is abso- lutely reliable in distinguishing a malignant from benign adrenal lesion. Only the presence of metastases can answer this.
Two of the three patients in this report had MRI exam- ination. The primary tumor was iso-intense to liver on T1 sequences but the caval thrombus was readily seen as it re- placed the normal “signal void” black inferior vena cava- gram image (Fig.1c, 3a). The atrial extent was easily identified (Fig. 1c, 3b) contrasted with the “black” right atrial flow void. The small foci of calcification in Case 1, seen on CT, were not evident on the MR. The ultrasound in Case 1 readily showed the IVC right atrial extension. Case 2 had angiography and CT, but MR was not available and the right atrial extension of the tumor was not realized prior to surgery.
The friable necrotic nature of these tumors, especially in the caval and atrial thrombus, is most important to em- phasize. Case 1 had cardiopulmonary bypass; the tumor in the atrium fragmented during surgical excision but this was controlled by the bypass procedure. Case 2 died in the operating room during mobilization of the tumor throm- bus. Case 3, because of malignant ascites, did not have sur- gical excision attempted.
References
1. Davies RP, Lam AH (1987) Adrenocortical neoplasm in children; Ultrasound appearance. J Ultrasound Med 6: 325
2. Falke THM, Peetoom JJ, de Roos A, van de Velde CJH, Mazer M (1988) Gadolinium-DTPA enhanced MR imaging of in- travenous extension of adrenocortical carcinoma. JCAT 12: 331
3. Schramek P, Dunser E, Bhargabha A, Hruby W, Umek H (1985) Adrenal Cortical carcinoma: preoperative demonstration of right atrial extension by sonography and computerized tomography. J Urol 133: 260
Accepted: 14 June 1989
Dr. W. E. Berdon Columbia Presbyterian Medical Center Babies Hospital 3959 Broadway New York, NY 10032 USA