Primary Leiomyosarcoma of the Kidney: Report of a Case and Review of the Literature

Primary Leiomyosarcoma of the Kidney: Report of a Case and Review of the Literature

THE JoURNAL UROLOGY Copyright© by The Williams & Wilkins Co. PRIMARY LEIOMYOSARCOMA OF THE KIDNEY: REPORT OF A CASE AND REVIEW O.F THE LITERATURE...

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THE JoURNAL

UROLOGY

Copyright©

by The Williams & Wilkins Co.

PRIMARY LEIOMYOSARCOMA OF THE KIDNEY: REPORT OF A CASE

AND REVIEW O.F THE LITERATURE ROBERT C. LOOMIS From the Eugene Hospital and Clinic, Eugene, Oregon

leiomyosarcoma of the kidney is rare but has been recognized since 1919 to the best of my knowledge, 40 cases have been reported. Herein I shall report on another case and briefly review the literature. CASE REPORT

practical nurse was admitted to on March 23, 1970 with left lower pain, nausea and vomiting 6 in duration. She had been seen on JVIarch 21 an internist of left flank pain, pyuria and fever A diagnosis of left pyelonephritis was made and treatment with ampicillin was Urologic revealed multiple difficulties with stress and a JVIarshall-I\!Iarchetti urethral had been <>rtnrrn,c,n in March 1969 at another hospital. No excretory urograms (IVPs) were taken at that time. retrograde pyelograms had been obtained 5 years previously during a diagnostic for recurrent cystitis (fig. 1). One month prior to the present hospitalization the had undergone cystoscopy, cystography cystometrography at another hospital in followup of the J\farshall-1\farchetti procedure because of persistent difficulties with stress inconwas essentially tinence. The remaining except for a chronic state and a of peptic ulcer. examination revealed an acutely ill, pale young woman with a fever of 105F and marked left flank tenderness. A chest film was negative. White blood count was 16,600 with a definite shift to the left. Urinalysis revealed 100 to 200 white blood cells, lO to 20 red blood cells and many bacteria. A urine culture yielded more than colonies per ml. Proteus mirabilis, sensitive to chloromycetin, kanmnycin, neomycin, and rnandelamine Hemoglobin was 12.8 gm. per cent and hematocrit was 40. Blood cultures were negative. IVP revealed a non-functioning large left kidney, thought to be hydronephrotic from a non-opaque stone (fig. 2, A). Retrograde showed a large mass in the upper of the left kidney, the pelvis and obstruction (fig. 2, . Transfemoral aortogram disclosed a probable malignancy of the (fig. 3). Treatment was changed to kanamycin injections and operation was scheduled. At operation, a firm, obviously malignant kidney was removed en bloc with approximately 6 cm. of ureter and all perirenal and left retroperitoneal fat. On frozen Accepted for publication

19, 1971.

Fm. 1. Retrograde preoperatively. Note of left kidney. II section the diagnosis of was made. The tumor seemed to arise from the renal pelvis, almost replacing it (fig. 4). There was no tumor outside the specimen or in received lymph node:i. Further extraperitoneal exploration revealed no distant metastatic tumor. The kidney measured by 7 by 6 cm. and 220 gm. There wa:-o no involvement of the renal vessels. _;vricroscopic examination revealed a well differentiated spindle cell tumor with and pleomorphism with mitotic figures. There was considerable inHammation (fig. 5). Convalescence was uneventful and the patient was discharged from the hospital 10 postoperatively. It is now 8 months since aml. she is free of signs of recurrence. Chest film remains negative. The stress incontinence has

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LOO:.VIIS

Fm. 2. A, enlargement of left kidney (60-minute IVP). B, left retrograde pyelogram. Note upper pole mass.

Fm. 4. Leiomyosarcoma replaces renal pelvis

Fm. 3. Transfemoral left renal arteriogram. Note tumor stain (ureteral catheter still in place). DISCUSSION

Most investigators believe the 72-year-old man reported by Berry to be the first reported case that

was documented by pathologic slides. 1 He reported this case in 1919 and mentioned 2 cases of benign renal le:omyoma reported by Larkin and Larkin in 1901. The first tumor thought to be malignant was reported by Pick in a dissertation quoting Renke. That tumor was characterized as a soft mass about the size of a child's head, arising from the left kidney of a 3-year-old boy who presented with signs and symptoms of hydronephrosis 9 months in duration. Nevinny quoted Brandt2 as presenting 2 1 Berry, F. B.: Report of three cases of combined tumors of the kidney in adults. J. Med. Research, 40: 4.59, 1919. 2 Brandt, F.: Uber 2 Falle von Myosarkon in der

PRHIARY LEIOJVIYOSARCOMA OF KIDNEY

_Fie.

5 . .iViicroscopic section of leiomyosarcoma (high power)

cases. However, one was probably benign and therefore is not included in this review. l\lacKeuzie and Parkins reported 011 one case. 3 described a 26-year-old woman who had had pain in the right loin for 14 months. 4 She was well years after nephrectomy. Crosbie and Pinkerton described a 35-year-old woman whose presenting complaints were urinary frequency and right lumbar pain 6 months in duration. 5 Cooke described a 60-year-old woman who had noticed a lump in the right loin 5 years in duration. 6 Hematuria was her presenting complaint, and at operation the entire renal substance was found to be replaced tumor. The patient died within 24 hours of anesthetic pneumonia and autopsy revealed metastases to the ornentum, peritoneum and liYer. Swan reported on one leiomyosarcoma among 65 ca.ses of renal neoplasm. 7 His main symptom was hematuria. Smith 40 of his own cases of renal malignancy of the upper ur;nary tract and found one example of leiomyosarcorna. 8 Patch described a 55- - - - - ~ ----~-

Genend d. Nierenbeckens. 1naugural Dissertation, Greifswald, 1919. Cited by Lazarus and Friedmann. 22 3 MacKenzie, D. W. and Parkins, G. A.: Renal tumonrs; brief review of series of cases from Department of Urology, Hoyal Victoria Hospital. Cmrnd. Med. Ass. 20: 616, 1929. 4 Berry, E.: of the kidney. Canad. Med. Ass. J., 20: 5 Crosbie, A. H. and H.: Malignant leiomyoma of the kidney. J. Ural., 27, 1932. 6 Cooke, W. E.: Malignant leiomyoma of kidney. J. Path. Bact., 37: 1933. 7 Swan, H. H. J.: of the kidney with analysis of 65 cases. Brit. J., 1: 606, 1933. 8 Smith, G. G.: Neopfasms of the and ureter· a report of 40 cases. Amer. J. Surg., 30: 1935. '

man with right renal colic and henrnturia 2 weeks in duration. 9 l\Iintz reported en 2 ca,0es of leiornyosarcoma 10 and later added 2 more. and associates found :-i ca,es of originating from the renal pelvis among 35 caceo of renal sarcoma. 11 Crabtree ,·m,nHc,rl on a with a kidney weighing 1,000 gm. at nephrectomy who had recurrence 3 years later.12 Tetelman and Lisa reported on 2 case,; found at autopsy; one of the died of a cardiovascular accident and the other of bronchopneumonia while studied for right abdominal maso. 13 I3ag;o1an also on a case of leiomyosarcoma diagnosed at autopsy. 14 In 1951 Blum and Fruhling found 2 cam,; of sarcoma, as did Petkovic. 15 • 16 Bhende 3 cases found at Kretschmer, Bruce and 9 Pat.ch, F. S.: Three unusual tumors; cancer in the crossed heterolateral kidney, without fusion malignant leiomyoma · in hydronephrotic sac. 19;37. 10 Mintz, E. H.: Sarcoma kidney in adult. Ann. Surg., 105: 1937. 11 Weisel, W., T. Sarcoma of the kidney. , 50: 12 Crabtree, F:. G.: Leiomyorna of sociated with hemorrhagic J. Urol., 52: 480, 13 Tetelman, M. M. and IL: coma of the kidney: report cases. J. 54: 224, 194.S. 14 Bagolan, P.: Su un caso raro de leiomyosarcoma bilaterale dci reni. Tumori, 24: 19.50. E. and Frnhling, L.: observatiom-; de sarcomes leiomyoblastiqnes du rein. J. urol., 57: 46, 1951. 16 Petkovic, S.: tumors of kidney. Urol. &Cntan. 55: 1951. 11 M.: muscle tumors of kidney Sci., 6: 747, 1952.

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LOOMIS

McNaught, Higbee and Atkins, and Kerr each reported one case. 18- 21 Lazarus and Friedmann reported on a case and reviewed the literature from 1893 to 1954.22 At that time they cited 16 other cases but 2 years later Frehling and Lev reported on a case and found 24 others.2 3 Gupta and associates reported on a single case. 24 Bazaz-Malik and Gupta reported on the only tumor which contained a stone and, in a review of the literature, found 31 cases preceding theirs. 25 Addison and Peach reported 2 cases of smooth muscle tumors in the kidney: one was benign and the other was a leiomyosarcoma in a 77-year-old woman. 26 To remove it they had to remove 10 cm. of the vena cava. The patient died of a myocardial infarction 6 months postoperatively. Becker and Gasteyer described a 56-year-old man with colic 10 months in duration.27 IVP, retrograde pyelography, radioisotope scan and renal arteriography were suggestive of a large tumor in the caudal pole of the left kidney. At nephrectomy peritoneal and mesenteric node metastases were found. Lloyd-Davies had a case remarkably similar to my case in that the patient was 23 years old and had a tumor on the left side. The pyelograms appeared similar to those in my case, and it was one of the few cases in which preoperative aortograms were obtained. 28 Through private correspondence I have learned that this patient was last seen on October 26, 1970 and was free of recurrence. In 1970 Islam and associates reported on a patient who was 38 months without recurrence. 29 The most recently reported case was that presented by Ziter and associates. 30 18 Kretschmer, H. L.: Leiomyosarcoma of the kidney. J. Urol., 68: 36, 1952. 19 Bruce, J. and McNaught, G. H. D.: Leiomyosarcoma of kidney. Brit. J. Urol., 25: 114, 1954. 20 Higbee, D.R. and Atkins, D. M.: Leiomyosarcoma in a double kidney. J. U rol., 71: 166, 1954. 21 Kerr, J. A.: Gastric and renal leiomyosarcoma. Brit. J. Surg., 41: 478, 1954. 22 Lazarus, J. A. and Friedmann, F.: Leiomyosarcoma of the kidney. Amer. J. Surg., 87: 251, 1954. 23 Frehling, S. and Lev, M.: Leiomyosarcoma of kidney. Arch. Surg., 73: 346, 1956. 24 Gupta, J.C., Nagrath, C. and Bhagwat, A.G.: A leiomyosarcoma of kidney. Indian J. Path. Bact., 6: 66, 1963. 25 Bazaz-Malik, G. and Gupta, D. N.: Leiomyosarcoma of kidney: report of a case and review of the literature. J. Urol., 95: 754, 1966. 26 Addison, N. V. and Peach, B.: Smooth muscle tumours of the kidney. (Report on two cases.) Brit. J. Urol., 38: 382, 1966. 27 Becker, H. and Gasteyer, K. H.: Leiomyosarkom der Niere. Med. Welt., 16: 1013, 1967. 28 Lloyd-Davies, R. W.: Leiomyosarcoma of kidney. Proc. Roy. Soc. Med., 62: 866, 1969. 29 Islam, M. U., Talibi, M. A., Boyd, P. F. and Laughlin, V. C.: Leiomyosarcoma of kidney. J.A.M.A., 212: 2266, 1970. 30 Ziter, F. M. H., Jr., Wieche, D.R. and McAn-

Some conclusions can be derived from a review of these 40 reported cases. Patient age range is 10 to 86 years, with an average of 50 years. The tumor occurs in women about twice as often as in men. The most common symptom is pain, sometimes colicky, in the flank, abdomen or back on the side of the lesion. Hematuria occurs but is a less common complaint. Occasionally pain and hematuria occur together. Frequency and weight loss are less common but do occur. Physical examination usually reveals a firm, round mass which may or may not be fixed and may or may not be tender. Abdominal x-ray occasionally shows calcification in the renal area, and IVP almost always reveals a filling defect or caliceal deformity but occasionally demonstrates hydronephrosis or non-function of the affected kidney. Nephrectomy was performed in most cases but the tumor was found at autopsy in at least 6 cases. In 1 case the tumor was inoperable and there is no information on one other case. The tumor is more often found on the right side (ratio 2: I) in either pole; one case of bilateral tumor has been reported.17 The tumor is usually large but can be small or nodular. It is encapsulated but occasionally grows out of its capsule, It spreads locally but can also spread to liver and lung. Histologically the tumor is similar to a leiomyosarcoma of any organ with elongated cells resembling muscle fibers, arranged in whorls and with cigar-shaped nuclei with considerable cytoplasm. Multinucleated giant cells are also noted. The amount of anaplasia varies. The pathologic grade of the tumor does not seem to alter the prognosis. If the tumor has invaded locally at the time of operation, there is -a greater chance of recurrence and mortality. It appears that the cases discovered early or incidentally have a better prognosis regardless of grade. SUMMARY

The forty-first case of leiomyosarcoma of the kidney is presented and the literature is reviewed. Similarities in incidence, symptoms, x-ray, surgical findings and followup are noted. The prognosis is poor when the lesion has spread either locally or distantly. Early diagnosis and complete removal of the tumor seem to yield the best prognosis. ADDENDUM

Patient was last seen on January 24, 1972 asymptomatic and without evidence of recurrent tumor. drews, J. F.: Renal leiomyosarcoma: a case report with angiographic findings. J. Urol., 105: 776, 1970.