Wilms' Tumor With Acute Abdominal Pain Mark

Rosenfeld, MD; Bradley M. Rodgers, MD; James L. Talbert, MD

\s=b\ Acute abdominal pain is the presenting manifestation in approximately 30% of all patients with Wilms' tumor. In a small proportion of these patients, this pain is significant enough to engender a diagnosis of an acute surgical abdomen. Six of 38 patients with Wilms' tumors treated between the years 1965 and 1975 at the Shands Teaching Hospital of the University of Florida Medical Center have had significant pain. Our experience with these patients emphasizes the importance of thoroughly palpating the abdomen of any child with a suspected acute surgical condition, following induction of anesthesia and prior to initiating the operation. In the absence of any evidence of an acute surgical problem at the time of the exploratory laparotomy, it is also imperative that a careful intra-abdominal examination be performed to exclude the presence of conditions, such as Wilms' tumor of the kidney, that may occasionally present in this

manner.

(Arch Surg 112:1080-1082, 1977)

Although abdominal pain is sometimes associated with il Wilms' tumor of the kidney, in the vast majority of patients, the correct diagnosis can be determined without

difficulty. In the occasional case, however, the true cause of the patient's pain may be overlooked, and an emergency

for publication April 21, 1977. From the Department of Surgery, Division of Pediatric Surgery, University of Florida College of Medicine, Gainesville, Fla. Reprint requests to Box J286, JHM Health Center, Gainesville, FL 32610

Accepted

(Dr Rodgers).

exploratory laparotomy performed on the presumption of an acute surgical abdomen. In the ten-year period from 1965 through 1975, thirty-eight patients with Wilms' tumor have been treated at the University of Florida Medical Center. Six of these children came to their physician with acute abdominal pain and underwent exploratory laparot¬ omy prior to establishment of the proper diagnosis of Wilms' turmor. A clinical evaluation of these six patients forms the basis of the present report. REPORT OF CASES Case 1.—A 5-year-old boy came to his local physician with a history of right lower abdominal pain, nausea, and vomiting of 24 hours' duration. He underwent abdominal exploration with a preoperative diagnosis of acute appendicitis. At the time of operation, a normal-appearing appendix was encountered, but during the procedure, the surgeon palpated a mass in the right retroperitoneal area. No further exploration was performed. Two days later, an intravenous pyelogram was obtained that revealed a

Wilms' tumor. At the time of his admission to this medical center, he had a large mass palpable in the right flank. Initial laboratory studies were normal, including a normal bone

right-sided

marrow aspirate. A stage I right-sided Wilms' tumor was excised, weighing 375 gm. The patient received daily dactinomycin therapy for four days and subsequent courses of dactinomycin during the next two years. When last evaluated, four years following resec¬ tion, he was entirely free of disease. Case 2.—This 4-year-old girl was admitted to the medical center with diffuse abdominal pain, malaise, and anorexia. Past history revealed that she had been struck in the left upper quadrant of the abdomen with a board three days earlier. The physical examina-

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tion revealed a spleen palpable 4 cm below the left costal margin, and laboratory indices showed an hematocrit level of 33%. Twelve hours later, the left upper quadrant mass had enlarged to 13 cm below the left costal margin and the hematocrit level had fallen to 27%. A subcapsular hematoma of the spleen was suspected and laparotomy was performed. At the time of exploration, free intraperitoneal blood was noted and there was a large, ruptured, left-sided Wilms' tumor. The spleen was invaded by the tumor and there was a metastatic nodule in the right lobe of the liver, making this a stage IV neoplasm. A radical left-sided nephrectomy and splenectomy were performed, and the patient treated immediately postoperatively with dactinomycin and radiation therapy to the left flank. The postoperative course was complicated by a leftsided subphrenic abscess that required operative drainage. Dacti¬ nomycin was administered for two years postoperatively. Fifteen months following the laparotomy, a liver scan and celiac arteriogram revealed no residual tumor. Seven years after resec¬ tion, this patient is asymptomatic, without evidence of recurrent disease. Case 3.—This 5-year-old girl was admitted to a local emergency room with a history of persistent postprandial vomiting. A mass was noted in the left flank and a diagnosis of a ruptured spleen was made. At the time of exploratory laparotomy, a large leftsided Wilms' tumor was encountered and a radical nephrectomy was performed. During the operation, a "cyst" was felt on the inferior pole of the right kidney, but was not biopsied. Postopera¬ tively, the patient received dactinomycin and radiation therapy to the left flank. Eight days prior to her admission to this medical center (ten months following the left-sided nephrectomy), a large right flank mass was noted. On physical examination, this mass was approximately 10 cm in diameter. Arteriography revealed a large tumor of the right kidney. She underwent exploratory laparotomy and a right lower pole heminephrectomy, with removal of a 175-gm Wilms' tumor. The patient received postoperative vincristine sulfate and dactinomycin. Chemotherapy was contin¬ ued for two years following her operation and, when last seen four years following resection, she was free of all evidence of disease. Case 4.—This 4-year-old girl came to her local physician complaining of right lower quadrant abdominal pain. She under¬ went an exploratory laparotomy with a preoperative diagnosis of acute appendicitis. A completely normal appendix was encoun¬ tered, without other pathology being evident at the time of the laparotomy. During convalescence, a right flank mass was noted and the patient was returned to the operating room for a radical right-sided nephrectomy for a stage I Wilms' tumor. Postopera¬ tive dactinomycin and vincristine chemotherapy was employed, in addition to radiation to the right flank. Six months later, she was noted to have a right pulmonary lesion. The dactinomycin chemo¬ therapy was intensified, and she received bilateral whole lung radiation. The patient was referred to the M. D. Anderson Hospital in Houston, where doxorubicin hydrochloride chemo¬ therapy was initiated. At the time of admission to this medical center two years following her nephrectomy, the patient complained of increasing malaise. A metastatic evaluation consisted of a normal brain and liver scan and a pulmonary angiogram demonstrating displace¬ ment of the right pulmonary vein with a large right hilar mass. Because she had received the maximum doses of radiation ther¬ apy, a right-sided pneumonectomy and mediastinal dissection was performed. Postoperatively, she was placed on dactinomycin and vincristine chemotherapy. She remained asymptomatic and free of detectable disease for 13 months, when she was seen with complaints of pain in the right shoulder; a roentgenogram

revealed a left-sided posterior mediastinal mass. Metastatic eval¬ uation revealed no evidence of disease elsewhere, and she was begun on an experimental chemotherapy protocol with razoxane. Her subsequent clinical course was progressively downhill, and she died three months later with diffuse mediastinal involvement. Case 5.—This 3'/2-year-old boy came to his local physician with left upper quadrant abdominal pain three hours following abdom¬ inal trauma sustained in a wrestling match. He underwent exploratory laparotomy, with a preoperative diagnosis of ruptured spleen. At the time of operation, a large left-sided retroperitoneal mass was encountered and biopsied. Examination of the specimen revealed a Wilms' tumor and he was referred to this medical center for definitive therapy. On physical examination, there was an ill-defined mass in the left upper quadrant that was tender to palpation. The intravenous pyelogram showed inferior displacement and distortion of the left kidney by an upper pole mass. A radical left-sided nephrectomy was performed, with the finding of a stage II Wilms' tumor with invasion through the renal capsule and into the renal vein. Regional lymph nodes were free of tumor. Dactinomycin chemo¬ therapy was instituted postoperatively, in addition to left flank radiation. Four months postoperatively, the liver was noted to be enlarged and nodular and a liver scan was suggestive of metas¬ tatic disease. A chest roentgenogram showed bilateral pulmonary métastases and the patient was started on vincristine therapy in addition to dactinomycin. Whole lung radiation was begun, but this treatment was discontinued because of weakness in both lower extremities. A myelogram revealed block at the T-6 level, and this area was selectively radiated. The patient experienced very little clinical improvement and suffered a progressive decline. He died eight months after resection with extensive

pulmonary tumor.

Case 6.-This 20-month-old girl came to her local physician six weeks prior to her admission to this medical center with right lower quadrant pain. She underwent exploratory laparotomy with a preoperative diagnosis of acute appendicitis. A normal appendix was encountered, and no other pathology noted. She continued to suffer abdominal pain and increasing abdominal girth. Four weeks later, she began having spiking fevers and a left flank mass was noted. An intravenous pyelogram revealed a large left-sided Wilms' tumor. On physical examination at the time of admission to this hospital, there was a large left-sided mass that crossed the midline. Evaluation for metastatic disease was negative. At the time of laparotomy, a large stage II Wilms' tumor was encoun¬ tered, with extension into the left hemidiaphragm. The periaortic lymph nodes were free of tumor. A radical left-sided nephrectomy was performed and the patient was started immediately on dactinomycin and vincristine therapy, in addition to radiation therapy to the left side of the abdomen and diaphragm. Chemo¬ therapy was maintained for 18 months and the patient remains asymptomatic and free of disease now, 26 months postoperative-

ly.

COMMENT Abdominal pain is the second most frequent symptom encountered in patients with Wilms' tumor, occurring in 26% to 33% of all reported cases.1"1 In our own series of patients with Wilms' tumor, pain was noted in 26% at their initial presentation (M. Rosenfeld, MD, B. M. Rodgers, MD, J. L. Talbert, MD, unpublished data, 1976). Snyder et a.V reported a similar incidence of pain in their patients with Wilms' tumor and, in 3% of these patients, an initial diagnosis of acute appendicitis was made. The abdominal

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pain

encountered in Wilms' tumor is thought to be due to invasion of the tumor into the renal capsule and the surrounding tissues, or to distention of the capsule by hemorrhage within the tumor itself.' Occasionally, ureteral obstruction by tumor may be the cause for persistent abdominal pain. In the series of 38 patients treated for Wilms' tumor at the Shands Teaching Hospital, six (16%) of these had signs and symptoms of an acute abdominal crisis and underwent exploratory laparotomy prior to a proper diagnosis of Wilms' tumor being made. Five of these laparotomies were performed at an outside hospital prior to referral to our medical center. In this retrospective review, there did not appear to be any characteristic of this pain that would raise the suspicion of a renal neoplasm. Three patients had relatively localized right lower abdominal pain and under¬ went exploration with the presumptive diagnosis of acute appendicitis. All three were noted to have normal appen¬ dices, but an abdominal mass was noted in only one patient at the time of surgery. In two of these patients, the pain was sufficiently diffuse to have possibly been secondary to capsular distention by the tumor. In the third patient, the localized right lower quadrant pain was most likely entirely unrelated to the presence of a left-sided Wilms' tumor. Nonetheless, the presence of this tumor might have been detected with a more thorough abdominal examination. These patients were referred to the University of Florida Medical Center from two days to ten months following their primary surgical procedure, and all had abdominal masses readily palpable on admission to our hospital. Three of the six patients underwent exploration for a presumptive diagnosis of splenic rupture, one being seen initially at the University of Florida Medical Center. All of these patients had a Wilms' tumor noted at the time of exploratory laparotomy, and two were treated immedi¬ ately with definitive resection, while one underwent initial biopsy of the tumor with delayed resection. The mortality in this entire group of six patients was 33%, not strikingly different from the expected mortality of patients with

similar tumor stages undergoing primary nephrectomy at our institution.1 Evaluation of acute abdominal conditions in infants and children can be quite difficult. In order to minimize the risk of overlooking other intra-abdominal pathology at the time of exploratory laparotomy for acute abdominal symptoms, two simple maneuvers should always be performed: 1. Careful abdominal and flank examinations should be performed in all patients after the induction of general anesthesia, in order to determine the presence of abdom¬ inal masses or organ enlargement. Under general anesthe¬ sia, both kidneys should be easily felt in children by using bimanual palpation of the flank. At least three of our patients would certainly have had the proper diagnosis suspected and the operative approach altered had such a maneuver been performed. 2. A thorough palpation of the retroperitoneum and remaining abdominal organs should be performed if no pathological diagnosis is made during the initial laparot¬ omy for significant abdominal symptoms. Such an exami¬ nation would most certainly have detected the Wilms' tumor in the two patients in our series with completely normal initial laparotomy.

Nonproprietary Names and Trademarks of Drugs Dactinomycin—Cosmegen. Doxorubicin hydrochloride-yldriarnî/cin. Vincristine sulfate-Omcowii.

References 1. Klapproth HJ: Wilms' tumor: A report of 45 cases and an analysis of 1,351 cases reported in the world literature from 1940 to 1958. J Urol 81:633\x=req-\ 648, 1969. 2. Ledlie EM, Mynors LS, Draper GJ, et al: Natural history and treatment

of Wilms' tumor: An analysis of 335 cases occurring in England and Wales 1962-1966. Br Med J 4:195-200, 1970. 3. Mohr RR, Murphy GP: Wilms' tumor: Thirty-year review of cases in Buffalo. NY State J Med. April 1974, p 660-665. 4. Snyder WH Jr, Hastings TN, Pollock WF: Retroperitoneal tumors, in Mustard WT, Ravitch MM, Snyder WH, et al (eds): Pediatric Surgery, ed 2. Chicago, Year Book Medical Publishers Inc, 1969, p 1026.

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Wilms' tumor with acute abdominal pain.

Wilms' Tumor With Acute Abdominal Pain Mark Rosenfeld, MD; Bradley M. Rodgers, MD; James L. Talbert, MD \s=b\ Acute abdominal pain is the presenting...
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