CASE l%EiFORTS

SOLITARY LATE RECURRENCE OF RENAL CELL CARCINOMA PRESENTING AS DUODENAL ULCER

ALAN I. FREEDMAN, M.D. JOHN E. TOMASZEWSKI, M.D. KEITH N. VAN ARSDALEN, M.D.

From the Departments of Surgery, Division of Urology, and Pathology, University of Pennsylvania School of Medicine, Philadelphia Pennsylvania

ABSTRACT-The natural history of renal cell carcinoma is often unpredict- able and even bizarre. We report a case of solitary late recurrence of renal cell curcinoma presenting as a duodenal ulcer and review the relevant literature.

The natural history of renal cell carcinoma has been the subject of intensive study, yet its be- havior remains unpredictable and poorly un- derstood. Renal cell carcinoma may remain sta- ble for long periods of time without growing or metastasizing, has the second highest sponta- neous r’egression rate of all solid tumors, and metastases may develop many years after re- moval of the primary lesion. l We present a case of a solitary late recurrence presenting as an up- per gastrointestinal (UGI) bleed and review the role of ,aggressive surgical management.

Case Report A sixty-five-year-old man underwent a right

radical nephrectomy for Stage I renal cell car- cinoma in 1976. Since that time he had been followed with periodic computerized tomog- raphy I(CT) scans and intravenous urography with no evidence of recurrent disease. Twelve years 1a:ter he presented to his general physician complaining of dark tarry stools and fatigue. He was found to be markedly anemic with a hemoglobin of 8.5 g/dL. Stool was maroon and positive for blood. He underwent esophagogas- troduodenoscopy and was found to have a bloody duodenal mass which had the appear- ance of a duodenal ulcer. He was treated medi- cally with. H, antagonists and antacids. How- ever, he continued to bleed with increasing transfusion requirements and subsequently un-

derwent repeat endoscopy four days later. On this occasion, active bleeding was observed in the area of the lesion, and the patient was emergently taken to surgery.

At exploration a suspicious friable mass was noted in the proximal duodenum. A biopsy of this was done, and vagotomy and pyloroplasty were performed. The patient recovered un- eventfully. However, the pathologic diagnosis from the biopsy specimen was adenocarcinoma consistent with renal primary carcinoma. The patient was then transferred to olur :institution for further treatment.

Initially an UGI x-ray film dernonstrated an ulcerated lesion in the proximal duodenum with associated deformity and rnass effect as well as irregular narrowing of the proximal duodenum with thickened folds. An arterio- gram revealed a hypervascular lesion in the re- gion of the duodenum receiving its blood supply from the superior mesenteric artery via the pan- creaticoduodenal artery (Fig. 1). A complete metastatic survey including CT scan, bone scan, and lung tomogram failed to -reveal any other metastatic lesions. At the tirne of surgical exploration, no definite mass was seen in the re- gion of the duodenum; there was no llymphade- nopathy. A pancreaticoduodenectomy (Whip- ple procedure) was performed. Gross examination of the specimen identified a 2-cm orange-yellow submucosal nodule located 2 cm

1fil

FIGURE 1. Arteriogram showing hypervascular mass (arrows) in region of duodenum, receiving its blood supply from inferior pancreaticoduodenal ar- tery.

distal to the ampulla that was surrounded by area of thick sclerosis. Microscopically, the duodenal mucosa was focally ulcerated. Imme- diately below the ulcer there was an accumula- tion of tumor cells in the submucosa that ex- tended into the muscularis and fat. Neoplastic cells were arranged as groups of ill-formed tu- bules and contained abundant clear cytoplasm. Nuclear pleomorphism was mild. Focal hemor- rhage was seen. The histopathology was con- sistent with metastatic clear cell carcinoma of renal origin (Fig. 2).

Comment The natural history of renal cell carcinoma is

unpredictable and even bizarre at times. One aspect of its unusual behavior is its tendency to remain dormant for long periods before demon- strating evidence of renewed growth or metas- tases. Although Hansen and Thybo2 reported that patients surviving disease-free for eight years following nephrectomy can be considered

FKURE 2. (A) Portion of duodenum with ulcer (ar- rows) and subjacent nodule of metastatic renal cell carcinoma. (B) Higher magnification of metastatic renal cell carcinoma showing clear cytoplasm and mild-moderate nuclear pleomorphism. (Hematoxy- lin and eosin, original magnification x 12 and x 150, respectively.)

cured, there are several reports in the literature of late recurrences developing ten to thirty years after nephrectomy.3 lo McNichols, Segura, and DeWeerd” in a large review reported that 11 percent of patients surviving ten years after nephrectomy experienced a late recurrence. However, the 18 patients with a late recurrence account for only 3.5 percent of the total 506 pa- tients in this series. Nakano et aZ.‘j reported 2 cases of recurrence more than ten years after curative nephrectomy out of 43 patients for an incidence of 4.7 percent.

The gastrointestinal (GI) tract is an unusual location for solitary late recurrence of renal cell carcinoma. The most common sites are lung, renal fossa, wound, and tracheal-bronchial tree.4,7 Willis’2 in a review reported 135 cases of metastatic disease involving the gastrointestinal tract. Of these, 10 originated in the kidney. GrahamI in a review of 195 cases of renal cell carcinoma, reported 3 cases of gastrointestinal metastases. None of these were located in the

462 UROLOGY I MAY 1992 I VOLUME XxX1X. NUMRER 5

small bowel. Willis’” reported that metastases to the gastrointestinal tract initially appear as submucosal nodules which then can spread to involve the mucosa and/or serosa. This descrip- tion is consistent with the pathologic findings in the case presented herein.

Of the reported cases of a solitary recurrence occurring more than ten years after nephrec- tomy, 5 have involved the GI tract. Starr and Miller” reported a case of renal cell carcinoma with metastasis to the jejunum twenty years af- ter nephrectomy. Their patient presented with melena and anemia. Khilnani and Wolf3 re- ported 6 cases of renal cell carcinoma involving the GI tract. Of these, 2 were cases of late re- currence ten or more years after nephrectomy. One appeared to be a right hilar mass extending into and through the wall of the esophagus. In the second case, at exploration a large mass was found which surrounded the duodenum and common bile duct. Liver metastases were also encountered. McNichols et al. ” reported that 2 of the 18 patients in their series with late recur- rences had metastases develop in the duodenum, but did not elaborate on their clini- cal course or treatment.

Although the number of cases is small, it would appear that survival is improved when an agglressive surgical approach is employed in patients with solitary metastatic lesions, partic- ularly when the lesion appears several years af- ter nephrectomy. In the McNichols et al.” se- ries, 13 patients underwent resection of a metastatic lesion one to fourteen years after nephrectomy with survival of 1.5 to nineteen years (mean 10 yrs). Five-year survival in this group was 69 percent. O’Dea et ~2.‘~ reported on 44 patients with solitary metastases from re- nal cell carcinoma. In 26 of the patients the metast,atic lesion appeared from one month to seven years after nephrectomy; 20 of these pa- tients underwent surgery for the metastasis and 18 lived more than two years. There were 6 pa- tients who survived more than five years. Tolia and Whitmore,15 in their report on patients with solitary metastases, found a five-year sur- vival of 29 percent in those treated aggressively. In this series both patients in whom the solitary metastasis appeared after nephrectomy sur- vived five years. Maldazys and DeKernion,16 in a review, concluded that the interval between

nephrectomy and the development of metas- tases was correlated with length of survival.

Late recurrence represents one aspect of the unpredictable behavior of renal cell carcinoma. It is clear that life-long follow-up is indicated for all patients with renal cell carcinoma. It also appears that an aggressive approach is war- ranted in cases of late recurrence.

Addendum This patient was alive and well with no evi-

dence of disease when recently seen in follow- up three and one-half years after the pancreati- coduodenectomy.

11i\isiori of Urology 3 Silverstein Pavillion

Hospital of the University of Pennsyl~~ania 3400 Spruce Street

Philadelphia, Pennsylvania 19104 (DR. V4n ARSDAI,EN)

Keferenct5

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2. Hansen JB, and Thybo E: I.ong!-term sIIrviva1 after nephrectomy for adenocarcinorna rrnis. Scantl J 1 Jr01 Nepluol 6: 47 (1972).

3. Khilnani MT. and Wolf BS: Late involvcmrnt ot the alimen- tary tract hy carcinoma of the kidney. Am J Dig Dis 5: 529 i 1960).

4. Bloom DA. Kaufman JJ, and Smith RB: I.ate rwxrcnw of renal tubular carcinoma, J Ural 126: ,546 (19X1)

5. Rowf BM, and Ruhin R: Metastasis from hy~wrncphroma twenty years aftc,r nephrectomy, JAMA 173: 116 (11170).

6. Nakano E. czt ~1: Late recurrrncc of wnal ccl1 carc~inoma after nephrectom): Eur Urol 10: 347 (1984).

7. Takatera H. rot al: Solitary late rcctlrwnc tl of rtnal cf,ll car- cinoma, J Ural 136: 799 (1986).

8. Craves KC. and Mahrey RE: Adenocarcinoma of kidney rc- current after tuvnty years, N Engl J Mcd 212: 416 (1935).

9). Starr A. and Miller GM: Solitary jejunal nlctastasis twent!. years after runoval of a renal cell carcinoma. V I~n~I J McLd 246: 250 (1952).

10. Kradjian RM. and Bennington Jr.: Renal carcinoma recur- rrnt 31 years after nephrectomy. Arch Surg 90: lCl2 (19f.31.

11. McNichol\ DW_ Scgura JW. and DrWtwtl JFI: Renal cdl carcinoma: long-term survi\.al and late rvctur~*nc~‘. J Ilrol 126: 17 (1981).

12. Willis HA: Secondary tumor\ of the mtv~tirrea, irk: ‘I’hc> Spread of Tumors in the Human Body cd 3. I,~lnd~n~. Brltterworth & Co., Ltd., chap 21, 1973, pp 20%21X

13. Graham AP: Malignancy of the kidney. sllrv,c\.of 19.5 cases. J Ural 58: 10 (1!947).

14. O’Dca MJ, Zincke II, Utz IX;. and Bernatz f’E: ‘I‘h<, trcat- ment of renal cell carcinonla with solitar! rwtastasis. J Urol 120: ,540 (1978).

15. T&a RM. and Whitmore WIT Jr: Solitar! nl(btahtasis from renal carcinoma. J Ural 114: 836 (19751.

16. Maldazys JD. and DeKernicm JB: E’rrqyostic fat tars in Inctastatic renal wll carcinoma, J I.rol 106: 376 i IOXR).

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