On extrarenal retroperitoneal tumors

ON EXTRARENAL

RETROPERITONEAL

TUMORS*

HEINRICH L. WEHRBEIN BROOKLYN, NEW YORK

S

INCE

reporting a case of gangIioneuroma in 1929, which was retroperitonea1 and extrarena1, we have encountered five other tumors so characterized by their Iocation. There were three neuroblastomata in chiIdren, one Ieiomyosarcoma in an eIderIy woman, and one metastatic tumor in a middIe aged maIe which arose from a tiny embryoma of the testis. In a11 cases uroIogic structures were invoIved and a11 cases except the second had compIete preoperative uroIogic examinations. The diagnostic diffIcuIties in the fourth and the fifth case deserve particuIar attention. The surgica1 diff<ies were a11 due to bIood suppIy contro1. The aorta was intimateIy invoIved in the third case, so much so that remova was impossibIe. It was aIso invoIved in the fifth case, but not so intimateIy. The renaI vesseIs were the main source of blood suppIy in the first and the fifth case making the operations fairIy simpIe. In the second case branches from the interna iliac artery suppIied the tumor. The vena cava was invaded in the fourth case, thereby causing inoperabiIity. The surgica1 approach was transperitonea in the first three cases and retroperitonea1 in the Iast two. One of the patients died shortIy after the operation and one died two months Iater. The other three are aIive and apparentIy we11 today. CASE REPORTS CASE I. The patient was a femaIe child, aged 4 months. She was brought to the hospita1 because of vomiting, loss of weight, and mass in the abdomen noticed for the preceding three weeks. A Iarge, round hard mass was found in the Ieft side of the abdomen, extending * From the urologica

services, Brooklyn

from the Costa1 margin to the iliac crest, and from the midline to the Ioin. The mass was painIess and movabIe. Cystoscopy showed P.S.P. function from both sides in eight minutes. A Ieft pyeIogram showed dispIacement of the Ieft ureter mediaIIy and the Ieft kidney high up in the abdomen. The Ieft kidney peIvis and the caIices showed crowding. (Fig. I.) The chest and the Iong bones were roentgenoIogicaIIy negative. An operation for remova of the mass was undertaken. Through a T-shaped abdomina1 incision and a transperitonea1 approach it was possibIe to free the tumor compIeteIy and to determine its attachment to the Iower part of the Ieft kidney capsuIe. Due to the bIood suppIy it was necessary to remove the kidney aIong with the tumor. On pathoIogic examination the tumor proved to be g cm. in each of its three diameters and to be attached to the Iower and mesia1 surface of the kidney capsuIe. The kidney itseIf was not invoIved. The histoIogic report was as follows: “The tumor is divided into lobules of varying size and shape, separated by connective tissue bands. The ceIIs resembIe meduIIary neural epitheIium; they are for the most part smaI1 and of varying s’hape. Many of them produce gIia1 fibers and in some foci we11formed rosettes are seen. Aside from the rosettes the genera1 architecture is that of strands of primitive neura1 epithelium found in some teratomata.” (Fig. 2.) A diagnosis of neurobIastoma was made. The postoperative recovery of the patient was uneventfu1 and the chiId is reported to be aIive and we11 four years after operation. CASE II. A female child, aged 18 months, was brought to the hospita1 with the chief compIaint of a mass in the Iower abdomen which had been noticed for eight months and which had been sIowIy increasing in size. Examination reveaIed a large, hard, nodular tumor in the right hypogastrium. It was very sIightIy movabIe and not painfu1; on recta1 examination it appeared fixed to the right

and Lutheran

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BrookIyn,

New York.

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sacroihac synchondrosis. Cystoscopy was not undertaken, because the tumor couId be outIined so detinitely by palpation. The preoperative diagnosis was retroperitonea1 neurocytoma.

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Iumbar and the first sacra1 nerves. The chiId is we11 today over four years after the operation. ApparentIy there is no recurrence and the foot drop has disappeared.

FIG. I. DispIacement of Ieft ureter and left kidney. The operative approach was through an incision sIightIy to the right of the midIine. The posterior peritoneum was incised mediaIIy to the right ureter which was anterior to the tumor. Dissection was fairIy easy at first; the externa1 iIiac vesseIs and the ureter were easiIy separated and the tumor was then found to emerge from the first sacra1 foramen and to derive its bIood suppIy from the interna iIiac or hypogastric artery. It was necessary to cut numerous vessels arising from this artery and aIso severa nerve bundIes which were thought to come from S I and 2. The operation was very bIoody and removal of the tumor was not thought to be compIete, smaI1 portions of it apparentIy remaining in the first sacra1 foramen. The tumor measured 7 by 8 by 4 cm. and had a thin capsule. On cross section it showed grayish-white noduIes in a dark red stroma. Sections showed a tumor made up of smaI1, deep staining ceIIs, arranged in nests or IobuIes which were separated by a fibrous stroma. The ceI1 bodies were rather widely separated by masses of gIia1 fibers, which were very deIicate and cobweb-like in texture. In some areas the tumor closely resembIed cerebra1 tissue with occasional gangIionic ceIIs. The growth did not appear to be very maIignant, but was definiteIy of neurocytic origin, a neurobIastoma. (Fig. 3.) The only postoperative compIication was a foot drop on the right; the neuroIogic consuItant thought this due to injuries to the fifth

FIG. 2. Case I. Neuroblastoma. CASE III. This was a female child, aged 26 months. The chief compIaint was an abdomina1 sweIIing which had been noticed for five weeks and which was growing rapidIy. Examination showed a huge, noduIar, firm, and not tender mass which aImost flied the abdomen and which was not movabIe. It was more prominent in the midIine, but extended we11 into both flanks. Roentgen pIates showed the boweIs dispIaced by the mass and an enIarged Iiver. Chest pIates showed a marked mediastinal shadow protruding into the Ieft Iung. The long bones were negative. On cystoscopy the P.S.P. function on the right was eight minutes and on the Ieft fourteen. PyeIogram demonstrated marked displacement of both ureters IateraIIy and compression of both ureters. (Fig. 4.) A transperitonea1 expIoratory operation was undertaken in spite of the apparent hopeIessness of the case. The mass was found to be retroperitonea1 and extended from the IeveI of the gastrohepatic omentum down to the bifurcation of the common iIiac arteries. The ureters as we11 as the kidneys were pressed IateraIIy by the mass. There were many enIarged Iymph glands in front of the tumor. Since remova was impossibIe, two of the en-

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larged lymph gIands were removed for histologic examination and the abdomen was cIosed. The histologic examination of the lymph

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of the abdomen which had been present for eIeven months and which caused a great dea1 of pain. She stated that the mass has been

FIG. 3. Case II. Neuroblastoma.

glands showed the nodes replaced by tumor tissue, composed of smaIl ceIIs with deepIy stained round vesicuIar nucIei. The ceI1 bodies were ill defined and consisted of narrow rims of cytopIasm which streamed into fine processes, forming an interceIIuIar gIia1 network. OccasionaIIy the ceils were arranged in smaI1 rosettes. Numerous mitotic figures were present. The

FIG.

4.

Case

III. Displacement ureters.

of both

tumor, a neuroblastoma, was highly vascular and appeared to be very malignant. (Fig. 3.) The chiId had an uneventfu1 postoperative course, but the tumor continued to grow and the patient died two months after the expIoratory operation. CASE IV. This woman, 70 years of age, had as chief complaint a swelling in the right side

FIG. 3. Case III. NeurobIastoma.

growing sIowIy, but that lately the pain had been getting very much worse. Her genera1 condition was poor and she had suffered for many years from hypothyroidism and adiposity. Examination showed a huge mass in the right side of the abdomen. It was irreguIar in outIine, not movabIe and quite tender. Distinct fIuctuation couId be feIt. As far as we couId tell it extended from the Costa1 margin to somewhere below the iliac crest and seemed to be retroperitonea1; intestines couId be palpated in front of the mass. A barium mea1 mereIy showed displacement of the bowel by the mass and a vagina1 examination seemed to rule out tumor arising from the pelvic organs. On cystoscopy a steady drip of hazy urine was observed to come from the right kidney, which continued during observation, namely for 30 minutes. P.S.P. appeared from the Ieft in four minutes, but none appeared from the right during the period of observation. A right pyelogram with 20 C.C. of contrast medium showed a huge misshapen peIvis, but no caIices. The outline of the kidney couId not be discerned. Because of the pain and in spite of the patient’s poor genera1 condition, operation was undertaken for drainage of the hydronephrotic sac. Under IocaI anesthesia the lumbar ab-

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dominal wail was dissected down to the mass and 1,300 C.C. of a Iight brown watery fluid drained off. At the end of the drainage blood

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American

appeared through the trocar. A gauze pack and compression seemed to control the bIeeding, but the patient died a few hours later. Th e post-mortem examination revealed a very compIex state of affairs. The nucleus of the pathoIogic changes found was a large hard noduIar tumor in the right lumbar gutter. It extended from the IeveI of the second lumbar vertebra to the upper margin of the sacrum. Its upper pole was attached to the right kidney and had invaded and compressed the vena cava. The compression of the vena cava had produced numerous huge varices of the veins of the abdominal waI1. The fluid drained off during the operation had been in a retroperitonea1, but extrarenal cavity. It is probable that it formed on the basis of an oId hematoma. The operative hemorrhage was due to a ruptured varix. The right kidney was totahy hydronephrotic, and the right ureter was compressed along its upper abdominal portion by the tumor. Histologic examination showed Iarge spindleshaped celIs arranged in broad interIacing bundles. The nuclei were generaIly ova1 and vesicular, but many of them were very Iarge, hyperchromatic or showing mitotic figures. OccasionaI muItinucIear forms were seen. The cel1 bodies were Iarge and stained yelIow by the

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Van Gieson method. A scanty connective tissue stroma distributed throughout the growth carried a fairIy abundant vascuIar suppIy. The

FIG. 7. Case v. Note compression

FIG. 6. Case IV. Leiomyosarcoma.

Jour~~d

Ieft hydronephrosis of left ureter.

and

tissue was rather edematous. Diagnosis was leiom yosarcoma. (Fig. 6.) CASE v. The patient was a maIe 39 years of age whose chief compIaint was pain in the region of the left kidney of three months’ duration. His abdomen was too fat to ahow accurate palpation. A pyeIogram showed marked

FIG. 8. Case

v. Metastatic embryona1 testis.

carcinoma

of

hydronephrosis of the Ieft kidney with decreased function and marked and persistent compression of the Ieft ureter below the kidney pelvis. (Fig. 7.) On operation a hydronephrotic kidney was found. The compression of the ureter was due to an extrarena tumor which extended from the pedicle of the kidney down to the third lumbar vertebra. The tumor was smooth, white and firm. It was denseIy attached to the aorta. RemovaI of the kidney and the tumor was undertaken, but it could not be

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separated from the aorta waII. A smaII strip of tumor tissue had to be left behind, measuring about 7 cm. in length. The tumor was composed of broad sheets and nests of rounded or polyhedra1 ceIIs, separated by a deIicate stroma network and occasional broader trabecuIar bands. The tumor ceIIs were of fairIy uniform size and shape. Their nucIei were rounded and vesicular; the ceI1 bodies were pale and vacuoIated. In the stroma were abundant lymphocytes. (Fig. 8.) The tumor probabIy diagnosis was metastatic arising from an embryona1 carcinoma of the testicle. Examination of the testis showed no increase in size and no noduIes, but the left epididymis was distinctly thickened and indurated. Nevertheless, it was decided to remove the testicIe. Even after the testis was taken out of the scrotum i’t was impossibIe to discern any tumor, and onIy after remova and cross-section, was a pea sized, yeIIow tumor found in the center of the testicle. HistoIogic sections of this tumor showed marked centraI necrosis, but the margina portions showed the same histoIogic picture as the metastatic tumor. The patient made an uneventful recovery, and was then treated with deep Roentgen rays. He is alive and apparentIy we11two years after the operation. SUMMARY

Three cases of retroperitonea1 neurobIastomata in chiIdren are reported. ApparentIy they differ wideIy in their degree of malignancy. RemovaI of such tumors should be attempted since cure has been effected in two og the three cases reported here. The second case is particuIarIy rebecause remova of the tumor markable,

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seemed to be incompIete and yet the chiId made a compIete recovery. One case of retroperitonea1 Ieimyosarcoma is reported which is remarkabIe on account of the compIexity of the pathoIogic findings. One case of metastatic testicuIar tumor in the retroperitonea1 space is reported. It tends to corroborate the fact that such tumors can be cured by deep roentgen therapy. GratefuI acknowledgment is due to Drs. D. D. Davis and W. H. Field for some of the clinica materia1 reported here and to Dr. J. A. deVeer for the carefu1 anaIysis of the pathologic findings. REFERENCES I. ALYEA, E. P. Medulloblastoma invoIving kidney. Soutb. M. J., 26: 753, 1933. 2. BARRINGER, B. S. Radiosensitive kidney tumors. J. Ural., 38: I, 1937. 3, BIGLER, J. A., and HOYNE, A. Ganglioneuroma. Am. J. Dis. Cbild., 43: 1552, 1932. 4. BOTHE, A. E. Neurocytoma of the kidney. Ann. Surg., 101: 418, 1935. 5. CULVER, H., and DAVISON, M. Tumors of the adrenal gland. J. urol., 32: 428, 1934. 6. HOFFMAN, S. J. Neurocytoma. Am. J. Dis. Child., 49: 135. 1935. 7. JACKSON,C., and BABCOCK,W. W. RetroperitoneaI ganghoneuroma. S. Clin. Nortb America, I I: 1233, 1931. 8. JERGENSEN, F. H. Hypertension and retroperitonea ganglioneuroma. Arch. Path., 16: 340, ‘933. 9. LEWIS, D., and GESCHICKTER,C. F. Tumors of the sympathetic nervous system. Arch. Surg., 28: 16, 1934. IO. MCFARLAND, J. Ganglioneuroma. Arch. Path., I I: 118, 1931. 1I. MORGAN, E. K., and STONE, C. M. Primary retroperitoneal Ieiomyosarcoma. J. Ural., 39: 63, 1938. 12. WEHRBEIN, H. L. GangIioneuroma of the retroperitoneal space. Long Island M. J., vol. 23, 1929.