Concomitant Intrarenal and Pararenal Angiomyolipomas*

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JOURNAL OF UROLOGY

\'ol. 74, No, 6, December !955 Printed in U.S.A.

COXCOlVIITA='iT INTRAREXAL AXD PAIL-\RE2'\AL A~GIOMYOLIPOMAS* HENRY BRODY

AND

HAROLD Lll'SHUTZ

Frnm lhc Departments of Laboratories and Umlogy, the .1/bert Binslc'in 11Iedic11l Center,

Northern Division, Philadelphia 41, Pa,

Lipomatous tumors of the retroperitoneal tissues are not lmcommon 1 · 2 The majority, according to J'l'Iayo and Dixon,3 occur between the lower pole of the kidney and the pelvic brim, Ackerman 4 states: "They have a predilection for the pararenal area, Their intimate relation to the kidney may necessitate its remoyal," Intrare11al lipomas, of sufficient size to be of diniral signific:anc-e, are rnre, ,\pproximately forty cases, not including the so-called ''capsulomaH," 5 have been reported, 6 Some have been pure lipomas; about half have had intermixed other components, aud have been called fibrolipoma, angiomyolipoma, hamart.oma, etc, \Ve have been unable to find a report of concomitant occurrence of renal and pararenal liporna, The fol]O\\'ing case is of interest in that it demonc;trates not only the occurrence of lipomatous tumors, both \Vithin and in the vicinity of the kidney, but also the recurrence of a pararrnal tumor more thall (en years after the original resection, .\cccpted for publication l\fay 31, 1955, * This case was demorrntrnted at the Slide Seminar during the ninth Annual Tumor Da,·, November l 954, at the Albert Einstein Medical Center, Dr, N, C, Foot (Come]! Fniverni1 v :Vfoemberton, J, de J, and J\IcCaughan, J, M,: Intrnrenal am! perirenal lipomata, St1t'g,, Gynec, & Obst., 56: 110-1L5, 1933, uJ Melen, D, R, and Gaspar, L: Intrnrenal cystic myxoliponrn, J, UroL, 31: 69-77, 1934, v) Potier, A, H,: ,\fossive renal lipoma, Urol, & Cutan Hev,, 40: 717-724, l!J:l6, \Y) Hobertson, T, D, and Hand, J, R,: Primar~- intrnrenal liponrn of 74]

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742

HENRY BRODY AND HAROLD LIPSHUTZ

FIG. 1. On left is bean-shaped pararenal lipomatous mass. On right is kidney showing fatty masses protruding both medially and laterally from lower half of kidney. CASE REPORT

In October 1941, a 29-year-old woman, because of abdominal symptoms, was surgically explored at another hospital. A large fatty tumor was removed from the vicinity of the lower pole of the left kidney. The pathological diagnosis was retroperitoneal leiomyolipoma. The patient was in good health and asymptomatic, for the following 10 years. In this period, she had two uneventful pregnancies. In February 1952, during a routine gynecologic checkup, a large abdominal mass was palpated. Gastrointestinal studies were negative. Excretory surgical significance. J. Urol., 46: 458-474, 1941. x) Latienda, R. I.: Lipoma del rinon. Arch. Soc. de Anat. Norm. y Path., 4: 31-37, 1942. y) Tahara, C. and Hess, E. :Massive renal fibrolipoma; report of two cases. J. Urol., 54: 107-115, 1945. z) Fuchsman, J. J. and Angrist, A.: Benign renal tumors. J. Urol., 59: 167-173, 1948. aa) O'Heeron, M. K.: Intrarenal lipoma. Urol. & Cut. Rev., 52: 73-76, 1948. bb) Herman, L.: Massive spontaneous hemorrhage into and around parenchymal lesions of kidney. J. Urol., 59: 544-552, 1948. cc) Heckel, N. J. and Penick, G. 0.: A mixed tumor of the kidney, lipomyohemangioma. J. Urol., 59: 572576, 1948. dd) Jentzer, A.: Grave hemorrhages provoked by solitary lipoma having perforated capsule of superior pole of kidney. Urol. & Cutan. Rev., 52: 612-613, 1948. ee), Morgan, G. S., Straumfjord, J. V. and Hall, E. J.: Angiomyolipoma of the kidney. J. Urol., 65: 525---527, 1951. ff) Rouvillois, C., Bordier, H. Poggioli, V., Djian, A. and Delarue, J.: Sur un cas de lipome du rein. J. d'urol., 57: 826-830, 1951. gg) Beadles, R. 0. Jr. and Urich, R. W.: Intrarenal lipoma: report of a case. J. Urol., 67: 460-463, 1952. hh) Rusche, C.: Renal hamartoma (angiomyolipoma): report of three cases. J. Urol., 67: 823-831, 1952. ii) Spillane, R. J., Singiser, J. E. and Prather, G. C.: Fibromyxolipoma of the kidney. J. Urol., 68: 811-814, 1952. jj) Thalheimer, M., Brux, J. M. and Daftari, B.: Rupture sousperitoneal d'un lipome intrarenal. Mem. Acad. Chir. Paris., 79: 114-116, 1953.

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I:\"TR:\REKAL A_ND l'.-'._RARl,NAL ANGIO:\1YOLIPOJ\'IAS

743

urography demonstrated a "space-taking lesion," with distortion of the infnio1 aud middle calyces of the left kidney. Below the left kidne~- ,rn:;: a second soft tissue mass, its translucency suggesting lipid. A gynecologic consultant found, in addition, an enlarged, myomatous uterus. A_ left retroperitoneal exploration, through a left lmnbar incision, ,n1s carriPd out on :February 1952_ When the left kidney was explored, a large, orange, mass 1Yas found involving the lmrnr pole, extending into the hilus. -were several smaller, similar nodules. When the kidney ,vas a targe retroperitoneal tumor of 8imilar cmrnistency i\-as found_ TllP postoperative course was uneventful. In the three years since operation, there has been no evidence of recurrence. The patient has no demonstrable hpomas. She shows 1.10ne of the external components of the tuberous sclerosis complex. The surgical specimen (figs. 1 and 2) consisted of two portions. Ono was the left kidney, measuring 12 5 by 4 cm. The lower pole was enlarged; a 11urnber of lipomatous protrusions increased its dimensions to 8 cm. transversely and -Ci cm. anteroposteriorly. A segment. of attached ureter ,ms displaced posteriorly. Th(-, renal pelvis was extra.renal, and subdivided into three trunks, connected ,Yith three separate which were not distended. 011 longitudinal section, about one-third of the lower half of the kidney was replaced by fatty masses. The of these, continuous with the largest protruding mass, measured approximately 7 by .J crn. on its eut surface. Numbers of smaller fatty nod apparently discrete, were found scattered through the lower half of the kidney. The masses were without distinct lobulation, and had an orauge-yellcnY color. The large, separately removed tumor measurer! 17 by 7 by 5 cm,, was bea11·· shaped, with most of i_ts surface smooth and encapsulated. The fat had a son1e-· what orange cast, its cut surface was indistinctly lobulated, varying from ish to orange-yellow. On microscopic study (fig. 3, A, C) the pararenal mid

Fm. 2. Bisected kid1Jey, shfming !'atty t.nmor masses in Jo~·er hnlf ol' kidne)'.

744

HENRY BRODY AND HAROLD LIPSHUTZ

~FIG. 3. A, section from kidney showing adult adipose tissue separating renal parenchyma. B, showing smooth muscle related to a thick-walled blood vessel. C, small focus showing some degree of anaplasia in otherwise mature lipoma. D, focus of frank sarcoma in a retroperitoneal lipoma.

intrarenal tumors showed the same histological pictures. Mature adipose tissue formed the greatest part of the tumors. In the kidney, the tumor extended between and separated the normal renal elements. Scattered through the tumor were small foci of spindle-shaped cells, having the appearance and staining

L\TRIKENAL AND PARARENAL ANGIOJl;JYOLIPOMAS

74fi

qualities of smooth muscle, and associated with blood vessels. In the pararenal tumor, one small focus showed some cellular irregularity, with occasional nuclei. The diagnosis was intrarenal and pararenal angiomyolipomas. A number of questions are raised by this case. The first problem concerns the relation of the tumor removed in 1941 to those found in 1952. The histological appearance of the original tumor, and of the recurrence, was that of a benign tumor. Farbman 1 in his review of retroperitoneal fatty tumors, wrote "therP have been cases recorded in which recurrent tumors have been removed five or six times." Windholz (his case 1)7 reported the recurrence of multiple retroperitoneal lipomas l ~Z years after the original removal. There was no further followup. Ockuly and Douglas' refer, without specific citation, to cases of recurrence, in 1vhich two or three subsequent removals were necessary. Pertinent is the report De w·eerd and Dockerty 2 that two patients with benign retroperitoneal liporna ultimately succumbed to recurrent tumor more than 10 years after original complete removal. In our case, the more recent tumors were histologically similar to the original tmnor. It is unlikely that the tumors, in the form found at the second operation, were present at the initial exploration. It is also unlikely that the small anaplastic focus iu the recurrent tumor is necessary proof of malignancy. Liposarcoma usually arises as liposarcoma, ab initio. 9 There have been a few reports, however, of liposarcomatous change in benign lipoma. 10 , 11 One of us recently saw a small focus of liposarcoma in a histologically benign-appearing retroperitoneal Iipoma which accompanied a separate typical retroperitoneal liposarcorna. In that case, the histological evidence of malignancy in the small eentral focus was clear (fig. ::i, It ici unlikely that a large intrarenal liporna was present in 1941; there might have been, at that time, a very small tumor. The intrarenal tumor was completely separate from the pararenal tumor, though the two were practically identical histologically. Multiple lipomas are not infrequent; they may be synchronous or metachronous. Tedeschi12 has reported a case of apparently benign lipornas with multiple "metastases" and fatal outcome. Primary liposarcoma of the kidney has been even less frequently reported than intrarenal lipoma. Of considerable interest is the high degree of association of tuberous sclerosis with liposarcoma of the kidney .13 • 14 Our patient gives uo evidence of tuberous sclerosis. Windholz, F . : Roentgen diagnosis of retroperitoneaJ lipoma. Am. J. Roentgen., 56: 1946. E. A. a.nd Douglas, F. M.: Retroperitoneal pcrirerrnl lipomata. J. Urol., 37: 519-630 9 Sto'ut, A. l'.: Liposarcoma: Malignant tumor of lipoblasts. Ann. Surg., 119: 86-107, 1944. 10 Wright, C. J.E. Liposarcoma. a.rising in a simple lipoma. J. Path. & Bact., 60: 48::\· 7

487, 1948 .

Sternberg, 8. · Liposarcorna a.rising within a subcutaneous lipoma. Cancer, 5: 9751952. Tedeschi, C. G. · Systemic multicentric lipoblnstosis. Arch. Path., 42: 320-3:37, 1946. 13 Fish, G. W. and McLaughlin, W. L.: Liposarcoma, of kidney: report of a case present an 1mus1rnl syndrome . .J. Urol., 55: 28-·3.5, 1946. Newnrnn, B. and Reed, T.: Liposarcorna of the kidne)·. J. lJrol., 62: 292--294, 1949. 11

746

HENRY BRODY AND HAROLD LIPSHUTZ

Three years have passed since the nephrectomy. There 1s no evidence of recurrence. More than ten years separated the first and second operations. It is not possible to be certain that there will be no further recurrence. This would be so even if the small cellular focus had not been demonstrated. In the series of forty retroperitoneal and two intrarenal lipomatous tumors reported from the Mayo Clinic in 1933 by Pemberton and McCaughan 6-t there were 12 cases with recurrence; six of these were in cases which had originally been considered benign. It is of interest that 1 case was operated upon 4 times, and 1 case 3 times, for recurrence. In the more recent report from the Mayo Clinic by De W eerd and Dockerty 2 they mention 1 case of benign lipomatous retroperitoneal tumor in which recurrence occurred 5 years after operation. Another of their cases with a benign retroperitoneal tumor had two subsequent operations for recurrent tumor, and died four years after the initial operation. Another, alive after 10 years, had "extensive incapacitating recurrence." Still another died 18 years later with a retroperitoneal lipofibrosarcoma. In Farbman's review1 of retroperitoneal fatty tumors reported between 1937 and 1947, he states that six of the fourteen benign tumors in which there were followup records showed recurrence, and three of these were sarcomatous. Only time will tell if cure has been obtained. There is little indication that irradiation is of any value,1 · 2 although Stout9 has suggested that this mode of therapy should not be abandoned. SUMMARY

A case of intrarenal and pararenal lipomas is reported, in which the pararenal tumor represents a recurrence more than 10 years following primary resection. We wish to thank Dr. Charles Maser who performed the original surgery for the clinical records concerning the original operation and Dr. David JVIeranze for the opportunity of studying the slides of the original tumor, removed at the then Mount Sinai Hospital, Philadelphia. We also wish to thank Mr. Joseph Poppel, Jefferson Medical College, for the taking of the photomicrographs.