ADRENAL REST CARCINOMA IN HILUM OF KIDNEY
ELIAHU GOREN, M.D. ISAAC S. ENGELBERG, M.D. AVRAHAM EIDELMAN, M.D.
From the Department of Urology, Assaf Harofeh Medical Center, Zerifin, and the Department of Pathology, Chaim Sheba Medical Center, and the Sackler School of Medicine, Tel-Aviv University, Israel
ABSTRACT-Adrenocortical rests are uncommon in adults and infrequently undergo malignant changes. Most of the published cases are those of functional tumors allowing preoperative diagnosis on the basis of the resulting endocri- nopathy. Most nonfunctioning tumors were found accidentally at operation or autopsy. A case is presented of a parapelvic nonfunctioning adrenocortical rest carcinoma situated in the renal hilus in a fifty-year-old woman. No such tumor has been described previously.
Adrenal rest tumors are uncommon. Most of these are functional and therefore have been diagnosed preoperatively. Less frequently the tumors are nonfunctional and have been dis- covered accidentally or at autopsy. Rarely do these aberrant tumors undergo malignant changes.
We report a case of nonfunctional aberrant adrenocortical carcinoma in hilar region com- pressing the kidney from the outside in a woman aged fifty years whose preoperative ra- diologic picture seemed typical of renal tumor.
Case Report
A fifty-year-old woman was first hospitalized in November 1982 for elective surgical repair of left ureteropelvic obstruction as demonstrated on intravenous urography and diuretic reno- gram. At operation a left malrotated kidney with markedly dilated pelvis due to idiopathic pelviureteral stenosis was found, the remainder of the ureter being normal. Dismembered pyeloplasty was performed. The postoperative course was uneventful.
One year later the patient underwent elective cholecystectomy for cholelithiasis. Six months after the cholecystectomy she began to com-
plain of intermittent left lumbar pain and intermittent low-grade fever. Intravenous urog- raphy while demonstrating satisfactory postop- erative pelviureteral funnelling and emptying, revealed a space-occupying lesion in the left kidney. The patient was readmitted to the urol- ogy department for investigation. Findings on physical examination were unremarkable ex- cept for left loin tenderness. Laboratory find- ings were normal apart from urinalysis which showed 10-12 erythrocytes and 80-100 leuko- cytes/high-power field. Ultrasonography dem- onstrated an echogenic mass measuring 8 x 4 x 5 cm in the medial part of the lower half of the left kidney (Fig. 1). Computerized tomog- raphy (CT) similarly showed the mass bulging from the medial side of the left kidney displac- ing the pelvicalyceal system outward and con- taining a number of hypodense areas suspected of being necrotic foci. The addition of intrave- nous contrast material caused enhancement of the mass (Fig. 2). Left selective angiography showed a radial spoke-wheel arterial distribu- tion suggestive of renal oncocytoma although the otherwise diffuse vascular blush was con- sistent with the more common renal cell car- cinoma (Fig. 3). Venocavography, chest x-ray film, and bone and liver scans were normal.
L
At operation through a transverse laparot- omy left radical nephrectomy was performed. No enlarged retroperitoneal nodes or visceral metastases were found.
The operative specimen consisted of left kid- ney, measuring 8 × 8 x 15 cm, weighing 360 g.
It was covered with perirenal fat. Cut surface showed areas of hemorrhage and foci of necro- sis. The mass was well encapsulated and did not form part of the renal parenchyma nor did it arise from it. A normal adrenal gland was present at the upper pole (Fig. 4).
Microscopically (Fig. 5), the tumor consisted chiefly of adrenal cortical cells, chiefly corre- sponding to zona fasciculata, regularly ar- ranged and with negligible dysplasia and without mitoses. There was no extension into the pseudocapsule and no tumor emboli in blood vessels.
The postoperative course was uneventful. The arterial blood pressure remained normal; laboratory examination values were unchanged compared with preoperative values. After one year of follow-up the patient feels well and is clinically free of disease.
Comment
Culp’ was the first to differentiate between true adrenal heterotopia and aberrant adrenal
rests, the latter being characterized by (1) dis- tribution related to abdominal viscera and go- nads, (2) small size and lack of adrenal me- dulla, and (3) the added presence of a normal orthotopic adrenal gland on the same side.
Aberrant adrenal rests are thought to be formed by the separation of cortical fragments displaced by the migration of medullary ele- ments from the sympathochromaffin tissue (at the 11-mm embryo stage) into the preformed cortical primordium.2
The incidence of adrenal cortical rests may be as high as 50 percent in children, but these
generally undergo atrophy and disappear within a few years.3 Aberrant adrenal rests may be found anywhere along the path of embry- onic migration and include the kidney,3.4 peri- adrenal and periaortic fatty tissue, in proximity to pelvic organs such as ovary, uterus, broad lig- ament, spermatic cord and vessels,5-7 and tes- tis.8 Additional less common sites include pan- creas, liver, transverse colon, retroperitoneum, and hernial sacs. 7,9,10
Generally, the diagnosis of malignancy and hence prognosis is based on analyzing histologic features such as mitotic activity and venous in- vasion. However, in cases of adrenocortical tu- mors or aberrant adrenocortical tumors, other criteria must be evaluated. Tang and Gray !! found that aside from histologic criteria the fea- ture most consistently correlated with outcome is weight (over 95 g-malignant). Weiss12 sug- gested that a combination of nine histologic cri- teria was the most useful in distinguishing ma- lignant from benign tumor. Those patients with four or more of these criteria had either recur- rence or metastases. In our case although there is uniformly innocuous histologic appearance the weight of 150 g is more consistent with the diagnosis of carcinoma.
Functional adrenal rest tumors are usually diagnosed because of the resulting endocri- nopathy. In contrast little attention has been paid to incidentally discovered nonfunctional aberrant adrenal tumors. On review of the lit- erature we have not found any mention of cases
resembling ours; thus there is no previous expe- rience regarding optimal therapy. In a review of 8 cases of nonfunctioning orthotopic adreno- cortical carcinoma, 4 patients underwent adrenalectomy including excision of tumor mass, in 1 case biopsy with radiotherapy, and 1 each underwent biopsy and radiotherapy alone.13 Six patients died of tumor progression within nine months and 1 patient at three years.
Our patient with a nonfunctioning aberrant adrenocortical rest carcinoma compressing the left kidney is well and clinically tumor-free one year after radical excision. Judging by the weight of the tumor, prognosis must be guarded, reserving further treatment, includ- ing adjuvant therapy for possible future re- quirements.
Department of Urology Assaf Harofeh Medical Center Zerifin 70300, Israel (DR. GOREN)
References
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