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Renal leiomyoma in a newborn infant
Renal
Leiomyoma
in a Newborn
FRANCES REEDER, B.S. AND THOMAS S. MORSE, From tbe Department of Surgery, Obio State Unicersity College of Medicine, and tbe Columbus Children’s Hospital, Columbus, Obio.
BDOMINAL masses in the newborn infant are relatively rare. Most of them are of renaI hydronephrotic or polycystic origin, usually kidneys [I]. SoIid renaI tumors are much Iess common in infants [2], and when they do occur they are nearIy aIways embryomas of the kidney. According to Deming [?I, onIy 6 per cent of soIid renaI tumors in infants are benign. One of these benign forms is the Ieiomyoma. Leiomyomas usuaIIy are subcapsuIar tumors measuring Iess than 3 mm. in diameter which escape detection during Iife. The foIlowing case report is of interest because of the rare finding of a Ieiomyoma of the kidney Iarge enough to be readiIy paIpabIe on the day of birth. REPORT
This 435 pound male premature baby was admitted to the CoIumbus ChiIdren’s HospitaI at fifteen hours of age because of a firm mass in the left upper quadrant of the abdomen. The prenatal history was noncontributory and delivery was uncompIicated. The baby had miId respiratory distress with an eIevated respiratory rate and suprasternal retractions. The chest roentgenogram was normaI, and the distress gradually subsided. Physical examination revealed an active premature baby in no apparent distress. Pulse was IZO; respirations, 52; and temperature 98°F. A moderate Ieft cephalhematoma was present. The chest was clear but with poor ventilation. Retractions were present. The heart was not enIarged, and no murmurs were present. The hands and feet were miIdIy cyanotic. In the Ieft upper quadrant of the abdomen was a firm movable mass which did not fee1 cystic and which couId not be transiIIuminated. HemogIobin, white blood ceII count and urinarysis were within norma Iimits. The indirect bilirubin was 4.6 mg. per cent. Plain roentgenograms of the
COMMENTS
In 1938 Be11 [4] reviewed 30,000 autopsies and found cIinicaIIy insignificant renaI Ieiomyomas to be reIativeIy common. These Ieiomyomas are usuaIIy subcapsuIar in Iocation and less than 6 mm. in diameter. Thus, they
Accepted for publication February Americm
Journal of Surgery, Volume 104. November rg6n
Columbus, Ohio
abdomen were noncontributory. Intravenous pyeIogram showed a retroperitonea1 mass on the Ieft side, causing distortion of the Iower caIycea1 system, presumably an embryoma of the kidney. Removal of the tumor was delayed because of a rising biIirubin which necessitated an exchange transfusion. FolIowing this procedure the baby’s abdomen was expIored through a Ieft transverse abdomina1 incision. The right kidney was norma on visualization. On the Ieft side a Iarge retroperitonea1 mass was found which extended into the Ieft Bank and included the kidney. The renaI pedicIe was identified and the renal veins and artery ligated. The adrena gIand was easily separated from the mass and was not removed. The kidney containing the tumor was well encapsuIated and was compIeteIy removed. There was no obvious Iymph node invoIvement. The patient tolerated the procedure we11 and made an uneventful recovery. The resected specimen measured 6 by 4.5 by 4 cm. and weighed 45 gm. One portion of the specimen resembIed norma renaI tissue. The major portion of the specimen was made up of a yelIow, firm, we11 encapsurated mass. On cut surface this mass had the appearance of fibrous or muscular tissue. (Fig. I .) From the kidney peIvis was a patent ureter. Microscopic examination showed interlacing bundles of what appeared to be smooth muscle. (Fig. 2.) Throughout the smooth muscIe were dispersed normal appearing glomeruli and renal tubules. AIthough severa mitotic figures were present, the smooth muscle fibers were normaIIy oriented. In some areas the tumor blended with the normal kidney parenchyma. The final pathoIogic diagnosis was Ieiomyoma of the kidney.
A
CASE
M.D.,
Infant
788
15, xg62.
Renal
Leiomyoma
FIG. I. Cut section showing normal kidney structure on left with attached smooth muscle tumor.
FIG. 2. Photomicrograph showing norma kidney parenchyma above and interlacing bundIes of smooth muscle forming the tumor below.
are asymptomatic and defy detection during life. It has been shown that 60 to 70 per cent of patients with tuberous sclerosis harbor smaII renaI Ieiomyomas. The cause of Ieiomyomas is obscure. Busse [_q] reported in I 899 that there is abundant smooth muscIe in the medulla of the embryonic kidney. A Ieiomyoma might resuIt from an overgrowth of this muscuIature, but the usua1 Iocation in 789
the subcapsular
region is not consistent with et aI. [6] suggested an origin from the smooth muscle of the renal blood vesseIs. Our patient had no unusual vasculature. Other suggestions have included this origin.
Zuckerman
an extreme
variation
of the differentiation
of
metanephric mesenchyme or a chronic inflammatory process. In our case the origin of the smooth muscIe remains in doubt.
Reeder
and Morse renaI tumors infants.
In 1947 Zuckerman et a1. [6] reported a renaI Ieiomyoma in a full term maIe newborn infant. This is the onIy previousIy reported case in a newborn infant of which we are aware. In 1954 Cottrell and HeckeI [7] reported a renaI hamartoma in a three month oId girl. They suggested that malignant degeneration of this type of tumor is rare. However, Golden and Stout [8] described eight large retroperitoneal smooth muscle tumors, of which five were malignant.
is the treatment
of choice
in
REFERENCES
L. A. and MARTIN, L. R. Abdominal masses in the newborn infant. Pediatrics, ZI : 596,
I. L.ONGINO,
1958. 2. MELICOW, M. M. and USON, A. C. PaIpabIe abdominal masses in infants and children: a report based on review of 653 cases. J. Ural., 81: 705, ‘959. 3. DEMING, C. L. The prognosis and probIems in renal tumors. J. urol., 55: 571, 1946. A. BELL, E. T. Classification of renaI tumors with observations on frequency of various types. J. Ural., 39: 238, 1938. _ 5. BUSSE. Cited by Fuchsman, J. J. and Angrist, A. Benign renaI tumors. J. Ural., 57: 167, 1948. 6. ZUCKERMAN, I. C., KERSHNER, D., LAYTNER, B. D. and HIRSCHL, D. Leiomyoma of the kidney. Ann. Surg., 126: 220, 1947. 7. COTTRELL, T. L. and HECKEL, N. J. Renal hamartoma. J. Pediat., 45: 206, 1954. 8. GOLDEN, T. and STOUT, A. P. Smooth muscIe tumors of G.I. tract and retroperitonea1 tissues. Surg. Gynec. &+Obst., 73: 784, 1941.
SUMMARY
The case of an infant with a renaI Ieiomyoma palpable on the day of birth is presented. These lesions are clinicalIy indistinguishable from the more common embryomas of the kidneys. While smalI Ieiomyomas tend to be benign, nearly half the reported tumors which were large enough to be palpable have been malignant. Prompt and complete excision of al1 soIid
790
Leiomyoma
in a Newborn
FRANCES REEDER, B.S. AND THOMAS S. MORSE, From tbe Department of Surgery, Obio State Unicersity College of Medicine, and tbe Columbus Children’s Hospital, Columbus, Obio.
BDOMINAL masses in the newborn infant are relatively rare. Most of them are of renaI hydronephrotic or polycystic origin, usually kidneys [I]. SoIid renaI tumors are much Iess common in infants [2], and when they do occur they are nearIy aIways embryomas of the kidney. According to Deming [?I, onIy 6 per cent of soIid renaI tumors in infants are benign. One of these benign forms is the Ieiomyoma. Leiomyomas usuaIIy are subcapsuIar tumors measuring Iess than 3 mm. in diameter which escape detection during Iife. The foIlowing case report is of interest because of the rare finding of a Ieiomyoma of the kidney Iarge enough to be readiIy paIpabIe on the day of birth. REPORT
This 435 pound male premature baby was admitted to the CoIumbus ChiIdren’s HospitaI at fifteen hours of age because of a firm mass in the left upper quadrant of the abdomen. The prenatal history was noncontributory and delivery was uncompIicated. The baby had miId respiratory distress with an eIevated respiratory rate and suprasternal retractions. The chest roentgenogram was normaI, and the distress gradually subsided. Physical examination revealed an active premature baby in no apparent distress. Pulse was IZO; respirations, 52; and temperature 98°F. A moderate Ieft cephalhematoma was present. The chest was clear but with poor ventilation. Retractions were present. The heart was not enIarged, and no murmurs were present. The hands and feet were miIdIy cyanotic. In the Ieft upper quadrant of the abdomen was a firm movable mass which did not fee1 cystic and which couId not be transiIIuminated. HemogIobin, white blood ceII count and urinarysis were within norma Iimits. The indirect bilirubin was 4.6 mg. per cent. Plain roentgenograms of the
COMMENTS
In 1938 Be11 [4] reviewed 30,000 autopsies and found cIinicaIIy insignificant renaI Ieiomyomas to be reIativeIy common. These Ieiomyomas are usuaIIy subcapsuIar in Iocation and less than 6 mm. in diameter. Thus, they
Accepted for publication February Americm
Journal of Surgery, Volume 104. November rg6n
Columbus, Ohio
abdomen were noncontributory. Intravenous pyeIogram showed a retroperitonea1 mass on the Ieft side, causing distortion of the Iower caIycea1 system, presumably an embryoma of the kidney. Removal of the tumor was delayed because of a rising biIirubin which necessitated an exchange transfusion. FolIowing this procedure the baby’s abdomen was expIored through a Ieft transverse abdomina1 incision. The right kidney was norma on visualization. On the Ieft side a Iarge retroperitonea1 mass was found which extended into the Ieft Bank and included the kidney. The renaI pedicIe was identified and the renal veins and artery ligated. The adrena gIand was easily separated from the mass and was not removed. The kidney containing the tumor was well encapsuIated and was compIeteIy removed. There was no obvious Iymph node invoIvement. The patient tolerated the procedure we11 and made an uneventful recovery. The resected specimen measured 6 by 4.5 by 4 cm. and weighed 45 gm. One portion of the specimen resembIed norma renaI tissue. The major portion of the specimen was made up of a yelIow, firm, we11 encapsurated mass. On cut surface this mass had the appearance of fibrous or muscular tissue. (Fig. I .) From the kidney peIvis was a patent ureter. Microscopic examination showed interlacing bundles of what appeared to be smooth muscle. (Fig. 2.) Throughout the smooth muscIe were dispersed normal appearing glomeruli and renal tubules. AIthough severa mitotic figures were present, the smooth muscle fibers were normaIIy oriented. In some areas the tumor blended with the normal kidney parenchyma. The final pathoIogic diagnosis was Ieiomyoma of the kidney.
A
CASE
M.D.,
Infant
788
15, xg62.
Renal
Leiomyoma
FIG. I. Cut section showing normal kidney structure on left with attached smooth muscle tumor.
FIG. 2. Photomicrograph showing norma kidney parenchyma above and interlacing bundIes of smooth muscle forming the tumor below.
are asymptomatic and defy detection during life. It has been shown that 60 to 70 per cent of patients with tuberous sclerosis harbor smaII renaI Ieiomyomas. The cause of Ieiomyomas is obscure. Busse [_q] reported in I 899 that there is abundant smooth muscIe in the medulla of the embryonic kidney. A Ieiomyoma might resuIt from an overgrowth of this muscuIature, but the usua1 Iocation in 789
the subcapsular
region is not consistent with et aI. [6] suggested an origin from the smooth muscle of the renal blood vesseIs. Our patient had no unusual vasculature. Other suggestions have included this origin.
Zuckerman
an extreme
variation
of the differentiation
of
metanephric mesenchyme or a chronic inflammatory process. In our case the origin of the smooth muscIe remains in doubt.
Reeder
and Morse renaI tumors infants.
In 1947 Zuckerman et a1. [6] reported a renaI Ieiomyoma in a full term maIe newborn infant. This is the onIy previousIy reported case in a newborn infant of which we are aware. In 1954 Cottrell and HeckeI [7] reported a renaI hamartoma in a three month oId girl. They suggested that malignant degeneration of this type of tumor is rare. However, Golden and Stout [8] described eight large retroperitoneal smooth muscle tumors, of which five were malignant.
is the treatment
of choice
in
REFERENCES
L. A. and MARTIN, L. R. Abdominal masses in the newborn infant. Pediatrics, ZI : 596,
I. L.ONGINO,
1958. 2. MELICOW, M. M. and USON, A. C. PaIpabIe abdominal masses in infants and children: a report based on review of 653 cases. J. Ural., 81: 705, ‘959. 3. DEMING, C. L. The prognosis and probIems in renal tumors. J. urol., 55: 571, 1946. A. BELL, E. T. Classification of renaI tumors with observations on frequency of various types. J. Ural., 39: 238, 1938. _ 5. BUSSE. Cited by Fuchsman, J. J. and Angrist, A. Benign renaI tumors. J. Ural., 57: 167, 1948. 6. ZUCKERMAN, I. C., KERSHNER, D., LAYTNER, B. D. and HIRSCHL, D. Leiomyoma of the kidney. Ann. Surg., 126: 220, 1947. 7. COTTRELL, T. L. and HECKEL, N. J. Renal hamartoma. J. Pediat., 45: 206, 1954. 8. GOLDEN, T. and STOUT, A. P. Smooth muscIe tumors of G.I. tract and retroperitonea1 tissues. Surg. Gynec. &+Obst., 73: 784, 1941.
SUMMARY
The case of an infant with a renaI Ieiomyoma palpable on the day of birth is presented. These lesions are clinicalIy indistinguishable from the more common embryomas of the kidneys. While smalI Ieiomyomas tend to be benign, nearly half the reported tumors which were large enough to be palpable have been malignant. Prompt and complete excision of al1 soIid
790