Adrenal Cysts: Report of 3 Cases and Review of Literature

Vr)L lJS. ,\pr. Prhded in U.{·{ A.

Tnr~ .JOURNAL OF UROLOGY

Copy-right (Q) 1968 by The ·w]l.liams & \Vi.lkins Cn

ADRENAL CYSTS: REPORT OF 3 CASES AND REVIEW OI<' LITEH.ATUR !VI. JVIIKAIL

AND

A. KIRSHMAN

Frmn the Department of Pathology, Albert Einstein ll!feclical Center,

of the adrenal glands are rare; to date, 171 cases have been reported. The first case was described Greiselius in 1670 and cited by O'Connell. 1 In 1959 .Abeshouse am! associates reviewed 152 cases and a.dded 3 of their own. 2 Since that time 15 cases have been reported various authors. 3 • 4 Three new cases are herein presented. CASE RJ,;PURTS

Case .1. A hyperfo@ive (1.5-year-old white woman, known to have Parkiuson's disease, was admitted because of fever of unknown origin, :I m.outh in duration. Clinical and radiological examination revealed a non-functioning left kidney with .;;econdary nephrosis due to staghorn calculus. The carbon dioxide concentration was 26 nwq. per liter; chloride, 100 meq. per L; sodium, 141 meq. per L; potasRrnm, ;c; meq. pet· Land the blood urea nitrogen (13Ul\) was 14 mg. per cent. A left nephrectomy and left adrenalectomy were performed. Postoperatively, a respiratory infection developed and the Bl:N rose to 71 mg. per cent. The patient died lO clays postoperat1vel.v. Permission for an autopsy was not granted. The specimen received in the laboratory consisted of a left kidney and left adrenal gland. The adrenal gland measured 6 1.8 1.5 cm. On cut surface a small was noted within the cortex (fig. l, A). The measured 0.7 by 0.6 cm. in greatest dimensions and contained a yellowish gelatinous material. The wall was very thiu Elsewhere, the adrenal cortex was of uniform thickness and golden yellow in appearance. The rnedulla was unremarkable. JV1icroscopic examination revealed a thin and delicate fibrous wall 1967. Accepted for publication 1\.pril Brit. J. O'Connell, N. D.: Adrenal Radio!., 32: 490-492, 19-59. 2 Abeshouse, G. A., Goldstein, R. B. and Abes.house, B. S.: Adrenal cysts: Review of the literature and report of three cases. J. Urol., 81: 7Ili19, 1959. 3 Tehrani, l'IL, Wolgin, vV. and Niedwich, A.: .Adrenal cyst: Report of two cases. J. Urol., 93: ,127-429, 1965 ., Plaut, A.: Locally invasive lyrnphangioma of adrenal gland, Cancer, 15: 1165-1169 1962. 1

Philadelphia, I'ennsyfoan/a

lined by a siugle la.yer of uni[orm cuboidal cPlls having the appearance of adrenal cortical cel.b (fig. 1, B). In certain areas the lining assumed stratified appearance. Fat stain revealecl minu I e granules indicative of fat within the cytoplasm of theoe cells. The lumen was filled with an amor phous eosinophilic substance. Case 2. A 70-year-olcl white womau snffrred sudden, sharp pain in the dorsal spine rndiat.i1t12: Io the neck, starting a few hours prior to admis.oiun. She had moderate cardiac hypertrophy and extensive dissecting aortic aneurysm. An OJJen1. tion was performed for the latter. Three of cardiac arrest occurred before and during 1he, operation necessitating resuscitation. tively, pulmonary atelccta3is and pa.raly~is the right side developed; the patient brccarne comatose and died 5 clays after aclmissiou .Autopsy showed the cau~e of death to be due Ln extensive cerebral infarct,. A left. adn-nal gland which weighed 36 gm. was an inciden1al 6nding. On cut surface the gland revmlecl Yarious sized cysts ranging in diameter from 0.2 to 2 cm. (fig. 2). The contents of the varied from clear yellowish fluid to pale gelatinous makria.L On the wall of one of i.he cysts a cakific plaque wa,, noted. N orrnal adrenal tissue was afrio ~cen. Histologic examination revealed the spaces lined by a single layer of uniform enclo thelial cells (fig. 3). The spac,'.S wne divided or· separated from one another delicate fibrnu, septa in whiGh, at times, remnants of u11altered or compressed adrenal cortical tissue were noted. Small foci of calcification could be seen iu certain areas of the walls. The spaces were filled with homogeneous eosinophilic rn.ateriaL Case 3. A 61-year-old hypertensive white man complained of left flank pain. An urogram showed a large calculus at the uretero vesical junction and a large left suprarenal mass with a calcified superior border sugge~tivc of adrenal tumor. A left renal angiograrn. demon· strated a left supra.renal mass measuring 8 t" cm. (fig. 4, A). Selecti\'e left venogrnphy indicated that the ma,s was or

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FIG. 1. Case 1. A, adrenal cortical cyst. Cyst is surrounded by adrenal cortex and medulla. It is filled with homogeneous eosinophilic material. 8X. B, cyst is lined by cuboidal cells with large nuclei. Lining is separated by thin hand of connective tissue from adrenal cortex. 450X.

FIG. 3. Case 2. Cyst is lined by compressed connective tissue and flattened endothelium. lOOX.

FIG. 2. Case 2. A, mnltiloculated lymphangiectatic cyst of adrenal with numerous dilated lymphatic channels at center. Some cysts are intracortical, others are in periadrenal fat with outburst of adrenal cortex. B, cortex is noted in scattered dark areas along walls of cysts. C, cysts also show areas of calcification. 8X.

necrotic. Other tests including histamine tests and vanillyl mandelic acid titers were within normal limits. Ureterolithotomy and, later, left adrenalect01ny were performed. The specimen was an irregularly-shaped mass of fibro-fatty tissue measuring 8 by 5 by 3 cm. Within the mass a portion of an adrenal gland was identified which measured approximately 3 by 2.5 by 2 cm. Within the adrenal gland a large collapsed cystic mass with an estimated diameter of 3.5 cm. was noted. The adrenal gland formed part of the cyst wall and the opposite wall was composed of thin membranous tissue. Minimal calcification along one

ADRENAL CYSTS

FR1o 4. Case 3. A left renal arteriogram demonstrates vessels bordering adrenal cortical adjacent to and partly lined by cortex. It extends into adrenal remnants and calcification. 8X. ,odge of the wall wa~ 1101,cd. Tlw adrenal yellmYorang;e <'ortex n1ea~ured 0.1 cnL 1\Licro~copic examinat,ion showed a relatiYely large pseudocyst arising from the adrenal gland extending into the periadreual fat (fig. 4, B) in which some small vessels aud remnants of smooth 1.nusclc were seen. The pseudocyst revealed no clear lining and the wall "·as eomposed of collagenons fibrous tissue. At times attenuated cortical tissue and foci of caleification were noted in the cyst wall. The Jurnen 11·as monolocnlar and empty. The uninvolved part of the adrenal, concentrated at 011c edge of the lesion, consist,ed of cortex and medulla, ~orne,Yhat compressed by the cyst. DISCUSSfOl\

rn not clear. The nature of adrenal Levison' and Terrier and Lecene 6 lmYe classified adrenal cyst:-; into 5 groups aernrding to probable etiology: 1) Glandular or retention cysts are exceedingly rare. Their presence in man is in 6 Levison, P": A case of bilateral adrenal cyst. Endocrinology, 17: 372-87G, 1933. Terrier, F. and Lecene, P .. Les grands cysts de la capsule surrenale. Rev. de d1ir., Paris, 34: 321-327. 190fi.

doubt but they han'. been noter! in animals" Embryologic origim are postulated for thes;," t~·pes of cysts. 2) C:,Aic adeuomas a.n• al~o mm" 3) Pseudoeysts are due to hemorrhage into normal or diseased adreual glands, fo!JowPd by the formation of a hematoma aud subc:equent degeneration. This type of cyst may contain clra,r or bloody fluid. The etiologic factorn for hemorrhage include: crnsh injury, toxic manifo,,tatiou of infedion (Waterhmrne-Friderichoen), shock, benign or malignant tumors, etc ..\.ccording to some authors, benign tumor~ sucb a.s hamartomas are the most common and lymphangioma and hemangioma are rare i.!l Urn, group. Pseudocyst formation as a result, of degeneration of hamartomas has been reported. 4) Lymphangiectatic or hemangiomatous cysts haw'. no real distinction from pseudocysts, and (,he differential diagnosis is rather difficult. might be the result of ed.asia of vessels. "Whether their origin is in eorte:x or medulla is unsettled, although they are noted i1, both cortex and medulla.. An infiltrative type into the central vein and/or into the surrouncling adrenal fat has hee11 noted by Pla,ut. 4 Our thi.rd

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case is probably similar to this one (fig. 4, B). 5) Parasitic cysts are exceedingly rare and almost exclusively due to echinococcal infestation. Barnett studied 1,617 cases of echinococcosis and showed that much less than 0.5 per cent of adrenal is involved. 7 The adrenal cysts can be seen in patients of any age with peak incidence at the third or fourth decade. Both sexes are equally involved. According to Wohl, as cited by O'Connell, the incidence of the cysts found at autopsy is 1: 1,155. 1 The cysts can occur on either side without predilection but the lesion is usually unilateral. Generally the cysts vary in size from microcysts to enormous ones containing several liters of fluid. A cyst with 5 liters of fluid has been reported. However, these cysts rarely exceed 10 cm. in diameter. They are mono- or multilocular. The latter type is noted mainly in cysts of lym.phatic origin. The wall is usually smooth and delicate, especially in the lymphangiectatic type, while in hematocysts the wall may be pigmented and have foci of calcification. The content of the cyst is usually clear but may be bloody or rusty in case of hemorrhage into the cyst, or milky in appearance in certain cases of lymphangiectatic cysts. In a reported case, the content of the cyst had a specific gravity of 1.020, cholesterol 33 mg. and albumin globulin ratio of 2.6 to 2.4. In our first case, the content of the cyst was analyzed by means of gas chromatography; it seemed to be a waxy, amorphous substance free of hormonal component. Histologically the cysts are n10st often lined by compressed cortical cells or endothelial cells depending on their origin. In others a true lining is absent. At times, foci of lymphocytes are seen in the fibrous septa or in adjacent adrenal tissue. In cases in which the cysts contain clear fluid, it appears as a homogeneous, light, eosinophilic substance. The clinical picture varies but most of the cases are asymptomatic and therefore remain undiagnosed clinically and are incidental findings at autopsy or during investigation for other diseases. However, certain cases are symptomatic. Large cysts may cause mechanical pressure resulting in back pain, sensation of pressure and indigestion. The character of these symptoms is often misleading. Pearse reported a case of abdominal 7 Barnett, L.: Hydatid cyst; their location in various organs and tissues of body. Australian & New Zealand J. Surg., 12: 240--248, 1943.

pain, shock and an externally palpable mass for several days which led to an erroneous diagnosis of cholelithiasis and pleurisy. 8 In Levison's case pleuri-glandular manifestations indicated probable parathyroid dysfunction. 5 In our third case flank pain was the chief complaint and on further study clinical diagnosis of adrenal cyst was made preoperatively. Diagnostic procedures which may be of some help include: tomography, retroperitoneal insufflation with carbon dioxide, arteriography, excretory urography and upper gastrointestinal x-ray. Excretory urography, in cases of large cysts, may show displacement of the kidney by adrenal or other retroperitoneal cysts. 9 Retroperitoneal carbon dioxide insuffiation and tomography may outline the mass. Arteriography differentiates between a solid tumor and cystic mass. An upper gastrointestinal series may show displacement of stomach and bowel by adrenal or retroperitoneal cysts. "\Vhenever adrenal cysts are suspected clinically it is advisable that honnonal studies (17ketosteroid, 17-hydroxycorticosteroid) and other laboratory procedures (electrolytes, eosinophil counts) be performed. In spite of these diagnostic aids diagnosis of adrenal cysts is rarely made with certainty. Of 171 cases found in the literature only five were diagnosed preoperatively. Our third case had been diagnosed preoperatively, the others two were incidental findings: one at necropsy and the other during the operation. In our cases as well as those reported in the literature clinical laboratory findings did not indicate adrenal insufficiency in cases of adrenal cysts in adult patients. However, a number of cases of temporary or pennanent adrenal insufficiency have been reported in infants and children by various authors. 10 , 11 Moore and Cermak described a child who had adrenal insufficiency with the corresponding clinical and laboratory changes.11 The child died at the age of 8 months. 8 Pearse, H. E.: Cyst of adrenal. Trans. West. Surg. Ass., 26: 329-336, 1916. 'Emmett, J_ L.: Clinical Urography. An Atlas and Textbook of Roentgenographic Diagnosis. Philadelphia: W. B. Saunders Co., 1964, 2nd edit., pp. 875, 884. 10 Jaudon, J. C.: Further observations concerning hypofunction of adrenals during early life; "salt and water" hormone deficiency. J. Pediat., 32: 641-669, 1948. 11 Moore, F. P., II and Cermak, E. G.: Adrenal cysts and adrenal insufficiency in infant with fatal termination. J. Pediat., 36: 91-95, 1950.

ADRENAL CYS'rs

At aniop~y, bilaleml adrenal cysts \Yen: found, surrounded by apparently sufficient and well prmc1Ted adrenal cortex. Other case.s of adrenal cyst~ without adrenal insuf±icicncy were reported in infants and children. ViThether the presence of adrenal cy~ts i~ related to enclor:rinopathy ha2 not been carefully studied. In the children, as previously mentioned, with bilateral adrenal cysts and adrenal i11~ufficiency, it ib difiicult to determine whether the cysts by mechanical pres~me or equinJcaJ activity of their content interfered with the biochemical function of the adrenal cortex. Biochemical study of the content of the cyst in our first case failed to rcYeal any hormone. but the pitt,ient 1Yas hypertensivt; and had hypokalernia. Our third. ca;;e was

also a hypertensive patient, who lmd histamine tests and vanillyl mandelic. acid deter· nriuations, In both cases, ihere may huvc bee11 certain relationships among the eyst,,, tbeir con te11t8 aud these clinical findings. HowAvrr, due Lo lack of enough materii,J ancl related to the conten1, of 1he nature and tbe effects of t,he sulJPlmice~ m UH,

SUMMARY

A brief review of the etiological, clinical 11,nd anatomical features of adrenal cysts is Three cases are added to 171 report;0d in i,Jie literature.