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Renal pelvic tumors in childhood
RENAL PELVIC TUMORS IN CHILDHOOD
CHARLES GARY
VINOCUR,
HITZIG,
CHARLES TERRY
M.D.
M.D.
MARBOE,
M.D.
W. HENSLE,
M.D.
From the Departments of Urology, Surgery, and Pathology, Babies Hospital, Columbia-Presbyterian Medical Center, New York. New York
ABSTRACT - A capillary hemangioma in the renal pelvis of a twelve-year-old child is presented. The diagnosis and management of renal pelvic tumors in childhood is reviewed. M7e believe this pathologic entity should be included in the differential diagnosis of hematuria and ureteropelvic junction obstruction in a child.
Capillary hemangioma of the renal pelvis is rare. Its presence in the pediatric age group is virtually unknown. We recently have encountered this unusual condition in a twelveyear-old child. Case Report A previously healthy twelve-year-old white girl had two episodes of gross, painless hematuria and intermittent pink urine. She was otherwise asymptomatic. Her past medical history and physical examination were unremarkable. She was seen at another hospital where an intravenous urogram demonstrated a filling defect in the right renal pelvis (Fig. 1); a retrograde pyelogram with ureteral brushings revealed that this was a fixed mass. Urine cytologic findings (~3) were negative for malignant cells. The patient was referred to Babies Hospital, Columbia-Presbyterian Medical Center, where repeat IVP and plain film tomography again suggested a large, noncalcified, broad-based sessile lesion on the medial wall of the right renal pelvis. It had a cauliflower appearance on the free margin, suggestive of tumor. At the time of exploration the right kidney was mobilized and the right renal pelvis identified. The intrapelvic mass could be both seen
UROLOGY
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W:TOBER
1980
/
VOLUXIE
XVI.
NUMBER
FIGURE 1. Preoperative lesion (arrow);
4
intravenous pyelogram of vessel. lower indentation is crossing
393
FIGURE 2. (A) Tumor (arrow) within unopened renal pelvis. (B) Lobular mass with stalk. (C) Polypoid mass shows superficial ulceration and contains medium-sized vascular channels surrounded by lobular aggregates of smaller vessels. (D) Highpower view shows branching capillary-sized channels.
and palpated with ease (Fig. 2A). The renal pelvis was opened sharply and the tumor mass excised in toto, including a portion of the medial wall of the pelvis (Fig. 2B). The renal pelvis was then reconstructed over a no. 8 infant feeding tube. The closure was done with interrupted 6-O Dexon sutures in a watertight manner. Postoperatively the patient did very well. An intravenous urogram done on postoperative day 7 revealed a small amount of extravasation from the lower end of the suture line. The patient had no clinical symptoms, and her periureteral drain site had remained dry. The drain was removed on postoperative day 8, and the paA follow-up intravenous tient was discharged. urogram done three months later revealed excellent function of the right kidney without obstructive changes and a smooth contour to the right renal pelvis (Fig. 3).
394
Pathologic findings Pathologic examination of the specimen revealed a 0.9 by 0.8 by 0.4-cm. lobular, polypoid mass with a red, granular surface (Fig. 2C). Microscopically it consisted of aggregates of small, capillary-sized vascular channels (Fig. 2D). Cleft-like branching channels were seen with some larger channels present centrally in the mass of capillaries. Near the surface, lymphocytes and polymorphonuclear leukocytes were seen in conjunction with an area of superficial erosion. It had the over-all appearance of a capillary hemangioma which had undergone superficial erosion. Comment Hemangiomas have been noted in all portions of the renal parenchyma and collecting system.’ They are found most commonly in
UROLOGY
/
OCTOBER
1980
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VOLUME XVI, NUMBER 4
These lesions can be approached more conservatively than tumors of the renal pelvis in adults.6,7 In children, these tumors have, to date, always been benign. It therefore appears justified to open the renal pelvis or ureter surgically in such cases and do an excisional biopsy of the lesion with the adjoining ureteral wall. If the lesion appears less well localized, more extensive excision may be necessary. If, however, a frozen section confirms the benign nature of the tumor, no more radical procedure need be performed, and the prognosis should be excellent. FIGURE
Intravenous
3.
pyelogram
three months’
postoperative.
patients under forty, presenting with either gross or microscopic hematuria. There is no racial or sexual predominance. This is the first reported case of a hemangioma at the ureteropelvic junction in a child. Other benign stromal tumors, however, including fibromas and polyps, have been described in this location as well as more distal in the ureter.2-5 Benign renal pelvic tumors most often present with hematuria, flank mass, or pain. Workup should include intravenous urography and tomography as an initial diagnostic procedure, followed by selective urine cytology, retrograde pyelography, and ureteral brushing for cytology and cell block. Arteriography is not usually beneficial.
UROLOGY
/
OCTOBER
1980
/
VOLUME XVI, NUMBER 4
Babies Hospital 3959 Broadway New York, New York 10032 (DR. HENSLE) References 1. Rappoport A: Hematuria due to papillary hemangioma of the renal pelvis, Arch. Pathol. 40: 84 (1975). 2. Vest SA: Conservative surgery in certain benign stromal tumors of the ureter, J. Urol. 53: 97 (1945). 3. Bennington JL: Mescdermal tumors of the renal pelvis and ureter, in: Atlas of Tumor Pathol., Washington, D.C., Armed Forces Institute of Pathology, 1975, sect. 12, p. 31.2. 4. Evans AT, Stevens RK: Fibroepithelial polyps of the ureter and renal pelvis; a case report, J. Urol. 86: 313 (1961). 5. Riley A, and Swain WJ: Angioma of the kidney, Urol. Rev. 45: 377 (1941). 6. Colgan JE, Sharst L, and Morrow JW: Benign ureteral tumors in childhood; a case report and a plea for conservative management, J. Urol. 109: 308 (1973). 7. MacKenzie DW, and Ratner M: Tumors of the renal pelvis, a review of the literature and report of a case, ihid. 28: 495 (1932).
395
CHARLES GARY
VINOCUR,
HITZIG,
CHARLES TERRY
M.D.
M.D.
MARBOE,
M.D.
W. HENSLE,
M.D.
From the Departments of Urology, Surgery, and Pathology, Babies Hospital, Columbia-Presbyterian Medical Center, New York. New York
ABSTRACT - A capillary hemangioma in the renal pelvis of a twelve-year-old child is presented. The diagnosis and management of renal pelvic tumors in childhood is reviewed. M7e believe this pathologic entity should be included in the differential diagnosis of hematuria and ureteropelvic junction obstruction in a child.
Capillary hemangioma of the renal pelvis is rare. Its presence in the pediatric age group is virtually unknown. We recently have encountered this unusual condition in a twelveyear-old child. Case Report A previously healthy twelve-year-old white girl had two episodes of gross, painless hematuria and intermittent pink urine. She was otherwise asymptomatic. Her past medical history and physical examination were unremarkable. She was seen at another hospital where an intravenous urogram demonstrated a filling defect in the right renal pelvis (Fig. 1); a retrograde pyelogram with ureteral brushings revealed that this was a fixed mass. Urine cytologic findings (~3) were negative for malignant cells. The patient was referred to Babies Hospital, Columbia-Presbyterian Medical Center, where repeat IVP and plain film tomography again suggested a large, noncalcified, broad-based sessile lesion on the medial wall of the right renal pelvis. It had a cauliflower appearance on the free margin, suggestive of tumor. At the time of exploration the right kidney was mobilized and the right renal pelvis identified. The intrapelvic mass could be both seen
UROLOGY
/
W:TOBER
1980
/
VOLUXIE
XVI.
NUMBER
FIGURE 1. Preoperative lesion (arrow);
4
intravenous pyelogram of vessel. lower indentation is crossing
393
FIGURE 2. (A) Tumor (arrow) within unopened renal pelvis. (B) Lobular mass with stalk. (C) Polypoid mass shows superficial ulceration and contains medium-sized vascular channels surrounded by lobular aggregates of smaller vessels. (D) Highpower view shows branching capillary-sized channels.
and palpated with ease (Fig. 2A). The renal pelvis was opened sharply and the tumor mass excised in toto, including a portion of the medial wall of the pelvis (Fig. 2B). The renal pelvis was then reconstructed over a no. 8 infant feeding tube. The closure was done with interrupted 6-O Dexon sutures in a watertight manner. Postoperatively the patient did very well. An intravenous urogram done on postoperative day 7 revealed a small amount of extravasation from the lower end of the suture line. The patient had no clinical symptoms, and her periureteral drain site had remained dry. The drain was removed on postoperative day 8, and the paA follow-up intravenous tient was discharged. urogram done three months later revealed excellent function of the right kidney without obstructive changes and a smooth contour to the right renal pelvis (Fig. 3).
394
Pathologic findings Pathologic examination of the specimen revealed a 0.9 by 0.8 by 0.4-cm. lobular, polypoid mass with a red, granular surface (Fig. 2C). Microscopically it consisted of aggregates of small, capillary-sized vascular channels (Fig. 2D). Cleft-like branching channels were seen with some larger channels present centrally in the mass of capillaries. Near the surface, lymphocytes and polymorphonuclear leukocytes were seen in conjunction with an area of superficial erosion. It had the over-all appearance of a capillary hemangioma which had undergone superficial erosion. Comment Hemangiomas have been noted in all portions of the renal parenchyma and collecting system.’ They are found most commonly in
UROLOGY
/
OCTOBER
1980
/
VOLUME XVI, NUMBER 4
These lesions can be approached more conservatively than tumors of the renal pelvis in adults.6,7 In children, these tumors have, to date, always been benign. It therefore appears justified to open the renal pelvis or ureter surgically in such cases and do an excisional biopsy of the lesion with the adjoining ureteral wall. If the lesion appears less well localized, more extensive excision may be necessary. If, however, a frozen section confirms the benign nature of the tumor, no more radical procedure need be performed, and the prognosis should be excellent. FIGURE
Intravenous
3.
pyelogram
three months’
postoperative.
patients under forty, presenting with either gross or microscopic hematuria. There is no racial or sexual predominance. This is the first reported case of a hemangioma at the ureteropelvic junction in a child. Other benign stromal tumors, however, including fibromas and polyps, have been described in this location as well as more distal in the ureter.2-5 Benign renal pelvic tumors most often present with hematuria, flank mass, or pain. Workup should include intravenous urography and tomography as an initial diagnostic procedure, followed by selective urine cytology, retrograde pyelography, and ureteral brushing for cytology and cell block. Arteriography is not usually beneficial.
UROLOGY
/
OCTOBER
1980
/
VOLUME XVI, NUMBER 4
Babies Hospital 3959 Broadway New York, New York 10032 (DR. HENSLE) References 1. Rappoport A: Hematuria due to papillary hemangioma of the renal pelvis, Arch. Pathol. 40: 84 (1975). 2. Vest SA: Conservative surgery in certain benign stromal tumors of the ureter, J. Urol. 53: 97 (1945). 3. Bennington JL: Mescdermal tumors of the renal pelvis and ureter, in: Atlas of Tumor Pathol., Washington, D.C., Armed Forces Institute of Pathology, 1975, sect. 12, p. 31.2. 4. Evans AT, Stevens RK: Fibroepithelial polyps of the ureter and renal pelvis; a case report, J. Urol. 86: 313 (1961). 5. Riley A, and Swain WJ: Angioma of the kidney, Urol. Rev. 45: 377 (1941). 6. Colgan JE, Sharst L, and Morrow JW: Benign ureteral tumors in childhood; a case report and a plea for conservative management, J. Urol. 109: 308 (1973). 7. MacKenzie DW, and Ratner M: Tumors of the renal pelvis, a review of the literature and report of a case, ihid. 28: 495 (1932).
395