Myelolipoma of the Adrenal Gland and Kidney Adenocarcinoma: Clinical Case

Vol. 98, Oct.

T'HE JouRN_tL oF UROLOGY

Copyright © Hl67 by The Williams & VVilkins Co.

erinted in fJ.S.A...

MYELOLIPOl\IA OF THE ADRENAL GLAI\D AND KIDKEY ADEI\OCARCINOMA: CLINICAL CASE RAUL LOPEZ ENGELKING, XAVIER IBARRA ESPARZA, DAVID JIMENEZ VELASCO AND EUGENIA MALDONADO

From the Seclion of Urology, Hospital General, Mexico D. F., Mexico

Myelolipoma is a lesion of variable size located in the cortex of the adrenal glands. The tu1nor contains hematopoietic and adipose tisSlW. It is an extremely rare lesion and is generally found on autopsy, since it develops without any characteristic clinical picture. Some authors associate the lesion with cases of severe anemia. :Vlyelolipomas are not considered true neoplastic lesions. De N avasquez stated that the hematopoietic tissue in the adrenal cortex is only a manifestation of the potential hematopoietic action. According to Plaut, if the lesions do not constitute nodules partially or totally encapsulated and circumscribed, they should be considered heterntopic marrow. Since it is not clear whether myelolipomas are foci of hematopoiesis with adipose tissue cells or alterations similar to tumorn, Natera proposed that they should be classified as choristomas. ::VIyelolipomas occur more frequently in male patients and often develop in association with adenocarcinoma of the prostate, carcinomatosis, renal disease or arteriosderosis and hypertension. Patients range in age from 17 to 93 years, the average age being 60. In some cases endocrine changes have been found (i.e., hyper-aldosteronism, symptoms of Addison's disease, hermaphroditism); however, it is not believed that an endocrine correlation exists but that the adrenal cortex, in the presence of severe endocrine disease, is subject to myelolipomas. Of the 50 cases studied, 28 patients were obese, suggesting an alteration of the lipid metabolism. Theories regarding, the origin of myelolipornas include: 1) foci of extramedullary hematopoiesis, 2) blood embolism of the bone marrow, 3) metaplasia of the reticulo-endothelial cells or of the adrenocortical cells and 4) inclusions of embryonic remains in the cortex. Selye and Stone noted lymphocytes and polyrnorphonuclear cells in adrenal glands adAccepted for publication September 27, 1966. Head at annual meeting of South Central Section, American Urological Association, Inc., Omaha, Nebraska, September 26-29, 1965.

jacent to experimentally produced tumors. Lewi;, stated that the corticotrophic action of the anterior hypophysis, provoked by the action of toxic products, stimulates hematopoiesis in the adrenal cortex. He noted that several cases of myelolipoma have beeE observed in people who have been badly burned. McDonnell found 4 cases in 2000 autopsies (0.2 per cent) and stated that only 27 cases had been reportecl prior tu 1956. Plaut studied 50 cases from the Arnwd Forces Institute and 50 cases from the literature. In 1958 Natera published the only case 1u the Mexican literature. The case herein reported corresponds to the case described by ),mold in 1866. Arnold believed the lesion was of the bone marrow. Ir\ 1905 Gierke reported case in which the lesion was not considered a tumor until 1929. The term myelolipoma was used for the first time by Oberling. CASE REPORT

A.A.G., a 63-year-old white, obese man, had a past history of malaria, acute appendicitis, calculous cholecystitis (cholecystectomy 7 years before his present illness) and right retropcritoneal exploration because of total hematuria with no other manifestations. The patient stated that he had a tumor which had not been biopsied. From 1962 to 1964 he had experienced total hematuria at irregular periods of 4 to 6 days on 5 occasions. These episodes were accompanied burning on urination, mild pain at the hypochondrium and right flank, and fever. In 1964 a prostatectomy had beell done because of an adenorna. No renal tumor was noted during the operation and the patient was discharged from the hospital. Total hematuria recurred in August. 'J'lw patient was ambulant, apparently well built and obese when he was admitted to our in February 1965. On examination it was noted that the carotid was hardened and that the abdornen had very thick adipose panniculus. A.n infra-umbilical scar and a right lumbar scar, 419

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LOPEZ ENGELKING AND ASSOCIATES

extending anteriorly to 1 cm. from the external border of the anterior rectus were seen. A hard, painless and movable mass extended downward for about 8 cm. below the hypochondrium on the right, upward to the anterior axillary line and to about 2 cm. from the medial abdominal line. A plain film of the kidneys, ureters and bladder revealed a huge, irregular right kidney shadow below the iliac crest; the upper pole had indefinite limits. The left kidney shadow was normal. On excretory urography the right kidney was better visualized and upward deviation of the calyces was seen (fig. 1). Good left kidney function and anatomy were noted. On cystoscopy the bladder appeared to have a normal capacity; trabeculation and normal ureteral orifices were seen but no bleeding was observed. Routine urinalysis was negative. A urine culture revealed Klebsiella pneumoniae. The blood count included erythrocytes, 4,800,000; hemoglobin, 11.8; hematocrit, 40; bands, 2; monocytes, 3; segmented, 61; neutrophiles, 63;

Fm. 1. Excretory urogram of right kidney shows calyces deviated upward; good left kidney function.

Fm. 2. Postoperative view of abdomen shows scar.

eosinophiles, 1; lymphocytes, 33. The blood chemistry values were urea, 27 mg.; glucose, 115 mg. and creatinine, 1.29 mg. Diagnosis was right renal tumor. Because of the previous lumbotomy, a thoraco-abdominal, transpleural, transdiaphragmatic, transperitoneal incision was made. This approach allowed removal of the entire tumor occupying the lumbar fossa and reaching the iliac crest, adherent to the diaphragmatic pleura, the psoas muscles and peritoneum. Early li~ation of the pedicle was done and 3 renal veins were found. The incision, which was started at the 11th rib, was moved in a forward direction over the rib up to the external border of the anterior rectus on the right side of the abdomen. Incision of skin, cellular tissue, wide dorsal, upper serratus, major, minor and transverse obliques was made. Subcostal resection of the 11th rib, opening of pleura to anterior reflexion, opening of diaphragm and peritoneum and application of a Finochetti separator were accomplished. Dissection and separation were made towards the line of the second and third portions of the duodenum and liver angle of the colon. The parietal peritoneum (posterior) was opened perpendicular to the vena cava at the level of the renal vessels. Dissection of the renal pedicle revealed an artery and 3 renal veins, the latter about 1 cm. in diameter. Dissection of the tumor was started, cutting it with scissors. The upper pole was adherent to the diaphragmatic dome. The surgeon noticed the flow of a yellow and orange-colored substance of soft consistency. The posterior surface was intimately adherent to the psoas muscle. The ureter was dissected down to the bladder, cutting it at that level. The tumor was removed together

MYELOLIPO:\IA OF ADRENAL GLAND AND KIDNEY ADENOCARCI2'0MA

with the 1mrietal 11eritoneun1 that covered it. The hepati<:'. angle of the colon was Sl1tured to the muscles. Suture of diaphragm, parietal pleura and muscles of chest wall was made, leaving a water seal with an outlet of a catheter iu the 10th intercostal space at the level of the axillary line of the abdomen. A drain wa~ inserted in the cavity in front of the pleural reflexion on the abdominal portion of the incision. The water seal ,vas removed after 48 hours

4:21

inasm.uch as a ehest x-ray verified that the pulmonary field was nmmal. In 5 days the drain was remm,ed and the wound appeared to be healed 9 tively (lig, 2). Eight clays patient suffered bronchopncumonia with bilateral pulmonary lesiom which was treated with antibiotics; ox)'gen was required. The patient was referred to the x-ray rn.ent arn l was \reated as an outpatient. One year

Fro, 3. A, specimen of kidney and adrenal. B, kidney adenocarcinoma

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LOPEZ EI'sGELKING AND AciSOCIA TES

Fm. 4. A, adrenal rnyeloliporna. B, myelolipoma tumor sectioned.

postoperatively the ratient was in good health and had had no recurrence. Macroscopicallv the specimen weighed 1,250 gm. and measured 27 by 15 by 8.5 cm. Upon removing the perirenal fatty tissue, a tumor of the upper pole of the kidney was seen which was removed without cutting the parenchyma (fig. S, A). The kidney measured 16.5 by 11 by 8 cm. A tumor in the inferior pole measured 12 by 10 by 7 cm., was yellow with white and dark reel areas (fig. 3, B). The mass was causing deformity of the cnlyces. The varenchyma of the UJJJJer

pole showed a loss of the cortico-meclulla relation; a tumor al thrombus was inside the renal vein. The mass, located above the upper renal pole, was oval and measured 10 by 8 by 6 cm. (niyelolipoma). It opened in a zone where yellow and orange material was seen which was friable (fig. 4, A). The outer surface of the tumor was smooth in some areas and irregular in others. It had adhesions and orange-colored tissue which resembled the adrenal cortex. A discontinuous fibrous capsule and reddish-gray material were inside (fig. 4, B).

:\1YELOLIP0l\1A OF ADRE'\/AL GLAND A '.\D KID'.\EY ADENOCA RCI:'\Oi\IA

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lVIicroscopically the renal tumor was composed

iuter,titial ti,~ue. There were trnnor cells incide

of cells of a clear cytoplasm and eccentric nuclei which were arranged in an alveolar form or in cord formation (fig . .5). There were necrotic and hemorrhagic zones. Tlw remaindet· of the renal

the renal Ycin. On sectioning of the upper pole of the kidney, a mixture of adult

parenchyma had hyalinizrcl glomeruli and abunchronic inflammatory infiltrate in the

danl,

hematopoietic tissue in intimate with the adrenal cmtcx cells was uot.ed (fi.p;. f\ A). Sometimes a band of tissue separated the myclo-adipose G,

·B\G. 5. A, microscopic findings of renal tumor cells of clear cytoplasm and eccentric m1clei arra.ng,·d in alveolar or cord formation. B, high powered field of part. il.

F1G,, 6. A., adrenal myelolipoma, mixture of adult adipose and hematopoietic tissue in intimate reJa. tionship with adrenal cortex cells. B, adrenal myelolipoma, band of fibrous connective t.issue limiting myelo-adipose cells.

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LOPEZ ENGELKING AND ASSOCIATES

Fm. 7. A, plasmocytes, Russell's bodies in myelolipoma tissue. B, megakaryocytes and lymphocytic cells in myelolipoma tissue.

Fm. 8. Inside tumor there are vessels with thickened walls There were abundant cells of the granulocytic series, numernus plasmocytes, Russell's bodies (fig. 7, A), megakaryocytes (fig. 7, B), lymphocytes and erythrocytic foci. No adrenal marrow was observed. Inside the tumor were vessels with thickened walls (fig. 8). There were no calcifications nor osseous trabeculae. DISCUSSION

The characteristics of the tumor herein described are similar to those described as peculiar to Soo's type 2. That is, macroscopically it appears reddish-gray or reddish-brown and microscopically it shows a predominance of bonemarrow cell elements, within which the myeloid

element is more apparent than the erythroblastic element. There are adult adipose cells in lesser proportion than in Soo's type 1, in which the adipose tissues predominate with a few myeloid elements and abundant erythroblasts. Some myelolipomas may be similar to lipomas when the myeloid tissue is abundant, as in our case. The cut surface is reddish-gray and the appearance is greatly altered by hyperemia and hemorrhage. Generally, there is a single nodule in a suprarenal but sometimes it can occur bilaterally. The mass can be spherical or oval and mea,ures up to 8 cm. The lesion is circumscribed and, generally speaking, it is surrounded by a capsule. Calcification or ossification foci may be found.

MYELOLIPOi\IA OF ADRENAL GLAND AND KIDNEY ADE?-.;OCAHCIXOJ\IA

Vascularization of myelolipoma~ is clifficult to evaluate ~ince the collapsed capillaries may not, be reeognized. j\Iarkecl thickening of the walls of the vessels that irrigate this mass has been oh· served. The ll1)'elolipoma in the cortical tissue, although one eannot state precisely in whid1 of its 3 layers, since the small and circumscribed lesions are rare. However, the inner poetion of the fascicular zone seems to he the main sik for the myelo-adipose c'.hanges. ~\Iicro:scopically, these are comprised of 2 t.,·1ws of elements. Adiposcc and hcmatopoietic cells arc mixed in varying; proportions. The: cell arrangement is characteristic of bone marrow with er)·thrnblasts that are relatively scan·c in rdatirm to the lymphocyteo which are more abundant than is u-mal in hone marrow. There arc numerous pre-mydocytes and polymorphonuclcar eells as well as a few SUMMARY

A caKc of myeloli]Joma is herein presented. It 1s the first one reported with a combination of an

425

adrenal myeloli]Joma and a renal adenocarcinorna. The advantages of our surgical route an: rli.,cu~scrl. ),clrenal myclolipomas are rare. This the seconrl case reported; the le.-:ion is the turn.or seen to elate. ILEFEHENCE8 CoLLil\S, JJ. C. · Formation of hone nrnrrow in snprnrcnal gland. Amer. J. Path., 8: 97-100, rn:32. DE NAYAS(lUEZ,

S.: A case of myclo-lipoma (bone-

marrow heteropia) of the snprarenal gland. Guy's Hosp. Hep., 85: 237-240, 19:fa G1FFEC'i, I!. K.: Myelolipoma of the adrenals; Report of seven m,ses. Amer. J. Path., 23: (il3-625, 1\)47.

HoLLID,IY, T. D.S.: :Massive bone-marrow heterotopia in both adrenal glands. J. Path. & Bact. 70: 2:30, 1955.

McDONNELL, W. V.: Myeloliponrn of adreirnL Arch. Path., 61: 416-41\l, 1D56. N,,TEIU, E. R.: Mielolipoma de la glandula suprarrenal. Rcvista Invest. clin. 10: :-l41-3'17, Hl58.

U1cn,1.nn;;oN, J. C.: A tumour of the adrenal gland composed of (he elements of bone marrow tissue. Amer. J. Cancer, 25: 74G-752, 1D35. SELYE, H. AND STONE, H.: Hormonally induced transformation of adrenal into myeloid Li~· sne. Arner. J. Path., 26: 211-234, l\l50.