AMERICAN JOURNAL OF PERINATOLOGY/VOLUME 8, NUMBER 3

May 1991

CONGENITAL MESOBLASTIC NEPHROMA, HEMORRHAGIC SHOCK, AND DISSEMINATED INTRAVASCULAR COAGULATION IN A NEWBORN INFANT Terence L. Zach, M.D., Raul F. Cifuentes, M.D., and Robert L. Strom, M.D.

ABSTRACT

Congenital mesoblastic nephroma is an uncommon tumor of infancy that is generally characterized as a benign tumor. It has no sex predilection and affects more often the right than the left kidney. However, atypical forms, some of which are aggressive, have been described.1 Although maternal polyhydramnios and neonatal hypertension have been reported in association with congenital mesoblastic nephroma, it usually presents as an asymptomatic abdominal mass. Hemorrhage and necrosis of the tumor may occur, especially in the atypical form, but these are often clinically silent. We report a case of a newborn infant with a congenital mesoblastic nephroma who presented with an abdominal mass in association with hypotension, shock, and disseminated intravascular coagulation. CASE REPORT

Patient is a 38 weeks' gestation, 3720 g black male born to a 24-year-old gravida 3 para 1101 mother. Pregnancy was uncomplicated; however, polyhydramnios was noted at the time of the rupture of membranes. First stage of labor lasted 8 hours 3 minutes and the second stage lasted 2 minutes. The infant was delivered precipitously, vaginally, from

Downloaded by: National University of Singapore. Copyrighted material.

Congenital mesoblastic nephroma is a rare tumor of infancy that usually presents as an asymptomatic abdominal mass. A full-term newborn infant with an atypical variant of this neoplasm developed hemorrhagic shock and disseminated intravascular coagulation. The stormy course was complicated by persistent fetal circulation and then the inability to withdraw ventilatory support due to the mass effect of the tumor. After the removal of the tumor at 10 days of age, transient conjugated hyperbilirubinemia developed. At 15 months of age, the infant was thriving without evidence of recurrence of the tumor.

vertex presentation. Apgar scores were 2 at 1 minute and 2 at 5 minutes of life. The patient was immediately intubated and a nasogastric tube was inserted. Abdominal distension was noted at the time of birth and increased over the first several hours of life. Other physical findings were paleness in a poorly perfused infant with a heart rate of 200 beats per minute and a blood pressure of 36/20 mmHg. The abdomen was massively distended, taut with no definite palpable mass and without tympanism. A chest and abdominal radiograph revealed clear lung fields, elevated diaphragms and a large right abdominal mass displacing the bowel to the left. Laboratory investigation on admission included normal plasma electrolytes, glucose and calcium levels. The total carbon dioxide was 16 mmol/liter. Plasma lactate was 4.7 mmol/liter; ALT, 16 IU/liter; AST, 164 IU/liter, creatine phosphokinase 465 IU/liter. A complete blood count disclosed 12,000 white blood cells, 40% polymorphonuclear, 2% bands, 49% lymphocytes, 6% monocytes, 2% eosinophils, and 1% myelocytes. The hemoglobin was 11.9 gm/dl and the hematocrit was 33.6%. Platelet count was 135,000 per mm3. The initial venous blood gases revealed pH 7.27; partial carbon dioxide pressure (PCO2), 47; partial oxygen pressure (PO2) 24; bicarbonate, 22. Urinalysis pH was 7.0, specific gravity 1.015; pro-

Division of Neonatology, Department of Pediatrics, Hennepin County Medical Center and University of Minnesota, Minneapolis, Minnesota Reprint requests: Dr. Cifuentes, Dept. Pediatrics, Division of Neonatology, Hennepin County Medical Center, 701 Park Ave. South, Minneapolis, MN 55415 Copyright © 1991 by Thieme Medical Publishers, Inc., 381 Park Avenue South, New York, NY 10016. All rights reserved.

203

AMERICAN JOURNAL OF PERINATOLOGY/VOLUME 8, NUMBER 3 May 1991

tein, 1 +; blood, 3 +; leukocyte esterase, positive; and HENNEPIN COUNTY MED CENT SOMATOM DR the remainder normal. The microscopic analysis reBOY NB ©773149 VI HC1 BABY vealed greater than 100 red blood cells, 0 to 2 hyaline ^•^•^^B 1 22 08-MAR-88 FRONT 11=17=04 H/SP casts, and 0 to 2 granular casts per high power field. : DU2 024 • Hypotension developed during this period and the L SCAN 24 E patient required dobutamine, dopamine, packed F red blood cells, and fresh frozen plasma for cardioT vascular support. Disseminated intravascular coagulation developed and the hemoglobin decreased to 5 CM ! 8.9 gm/dl at 6 hours of age. The prothrombin time was 20.6 seconds, partial thromboplastin time was greater than 150 seconds, fibrinogen was 54 mg/dl, fibrin degradation products were greater than 500 (xg/liter, and platelet count was 76,000 per mm3. The 224 KV 125 ^ coagulation abnormalities and the anemia were cor119 AS .41 ^ F c SL 8 rected after a double volume exchange transfusion GT 0 was performed. Persistent pulmonary hypertension TP 109 IV CONTRAST developed and was managed with hyperventilation Figure 2. Computed tomography scan of the abdomen and 100% oxygen. He required peak inspiratory confirmed the findings of the ultrasound scan in Figure 1. pressure of 32 cm H 2 O and a rate of 70 bpm. This abated by day 4. Abdominal ultrasound revealed a large 8 by 8 cm mass in right upper quadrant in the region of the renal fossa. The mass was well encapsulated with measured 9 x 6.5 X 6 cm and weighed 211 g. The cut mixed attenuation (Fig. 1). Computed tomography surface showed soft nodular tan tissue with multiple (CT) scan of the abdomen confirmed these findings, cystic areas. Some of the cysts contained blood clots suggesting a renal or adrenal tumor with extensive or loose myxomatous mucoid tissue. The neoplasm hemorrhage or necrosis (Fig. 2). Urinary vanillylreplaced the central portion of the kidney and commandelic and homovanillic acids levels were 5.5 and pressed the cortex. Thrombosis of intralobular arte4.5 mg/gr creatinine, respectively, both within norries and hemorrhagic necrosis of the renal cortex mal limits. Plasma creatinine at 24 hours of age was were also seen. Histopathologically, the tumor was 1.7 and peaked at 2.1 mg/dl on day 4 of life. By day highly cellular and made up of sheets and vaguely 16, it had become completely normal at 0.4 mg/dl. defined bundles of monomorphic spindle cells with The initial gross hematuria cleared by day 2. The oval clear nuclei. In somefieldsa high mitotic activity patient's condition stabilized by day 4; however, bewas observed (Fig. 3). These findings are characteriscause the mass compromised diaphragmatic movetic of an atypical mesoblastic nephroma.2 Postoperament, the patient remained ventilator dependent. tively, the patient developed transient direct hyperOn day 10, an exploratory laparotomy revealed an bilirubinemia, which peaked to 11.0 mg/dl for total encapsulated tumor involving the entire right kidand 7.0 mg/dl for the direct fraction at 21 days, ney. A right nephrectomy was performed. The mass presumably secondary to edema of the common bile

Figure 1. Ultrasound scan of the abdomen revealing a large 8 by 8 mass in the upper right quadrant in the region of the renal fossa. The mass is well-encapsulated with mixed 204 attenuation.

Figure 3. Histologic examination revealed a highly cellular tumor with sheets of highly defined bundles of monomorphic spindle cells with oval clear nuclei. Note the high mitotic activity.

Downloaded by: National University of Singapore. Copyrighted material.

it

CONGENITAL MESOBLASTIC NEPHROMA/Zach, Cifuentes, Strom

DISCUSSION

Abdominal masses in the newborn occur in 0.2 to 0.6% of live births and the majority of these are related to the urogenital tract.3-5 Renal tumors account for only 5% of the renal masses in this period of life. One of these is congenital mesoblastic nephroma, from which a subset, based on histopathologic findings, has been labeled as atypical.l Hemorrhage, necrosis, or local invasion of adjacent structures and microscopicfindingsof increased cellularity with high mitotic index characterize this atypical form. Moreover, these atypical forms may have different clinical and radiologic manifestations and, more importantly, different prognosis than the typical congenital mesoblastic nephroma.6 However, some reservations have been raised regarding this classification.2 Our patient had both microscopic and gross features of an atypical congenital mesoblastic nephroma. These tumors can present without symptoms and may be detected by physical examination.7 Sometimes the antecedent of polyhydramnios may suggest their presence.8 In addition to the palpable mass, arterial hypertension, hematuria, vomiting, jaundice,7 and hypercalcemia9 have been described. In the past intravenous pyelography was the preferred radiologic examination. This has been replaced by renal ultrasonography and CT. Chan et al6 reported a distinctive echogenic "ring sing" on renal ultrasound with typical congenital mesoblastic nephroma, whereas in atypical congenital mesoblastic nephroma the tumor was more likely to have an inhomogeneous sonographic appearance. Intratumor hemorrhage, necrosis, and cyst formation account for this mixed attentuation pattern. The renal ultrasound in our patient was consistent with this atypical pattern. The abdominal CT scan may complement the sonographic findings. This case represents an unusually fulminant presentation of an atypical form of congenital mesoblastic nephroma. We hypothesize that the process of labor and precipitous vaginal delivery resulted in hemorrhage of the tumor. This is based on a hemoglobin of 11.9 gm/dl at 2 hours of age, which decreased even further despite packed red blood cell transfusion. The hemorrhage resulted in hypotension and early cardiovascular shock with the ultimate development of disseminated intravascular coagulation. However, the role of tumor necrosis or tumor tissue injury with release of factors precipitating or

contributing to the development of disseminated intravascular coagulation remains undetermined. Vigorous supportive therapy with volume expanders and inotropic agents were required to maintain adequate perfusion. An exchange transfusion was needed to correct the coagulopathy. Although hemorrhage from the tumor and consequent hypotension have been reported as intraoperative complication,7'8 this is the first case, to our knowledge, describing a severe hemorrhage with resultant shock in the immediate neonatal period. Persistent pulmonary hypertension developed in our patient, most likely as a consequence of the profound systemic hypotension, acidosis, and hypoxemia. Intraabdominal masses during intrauterine life, experimentally, do not cause pulmonary hypoplasia.10 Surgical removal of typical congenital mesoblastic nephroma is curative. Local recurrences and fatalities have been associated with the atypical form.1-6 With the increased availability and use of prenatal ultrasonography, congenital mesoblastic nephroma may now be diagnosed prenatally.811 A cautious obstetric management of such patients during labor and delivery is suggested.

Downloaded by: National University of Singapore. Copyrighted material.

duct. A sweat chloride and alphaj-antitrypsin tests were normal. He was extubated on day 15 and discharged on day 31, his weight was 3720 gm. At 15 months follow-up, the patient was growing and developing normally with no evidence of tumor recurrence.

REFERENCES

1. Joshi VV, Kasznica J, Walters TR: Atypical mesoblastic nephroma: Pathologic characterization of a potentially aggressive variant of conventional congenital mesoblastic nephroma. Arch Pathol Lab Med 110:100-106, 1986 2. Beckwith JB, Weeks DA: Congenital mesoblastic nephroma. When should we sorry? Arch Pathol Lab Med 110:9899, 1986 3. Sherwood DW, Smith RC, Lemmon RH, Vrabel I: Abnormalities of the genitourinary tract discovered by palpation of the abdomen of the newborn. Pediatrics 18:782786, 1956 4. Museles M, Gaudry CL, Bason MW: Renal anomalies in the newborn found by deep palpation. Pediatrics 47:97101, 1971 5. Melicow MM, Uson AC: Palpable abdominal masses in infants and children: A report based in a review of 653 cases. J Urol 81:705-712, 1959 6. Chan HSL, Cheng M-Y, Mancer K, et al: Congenital mesoblastic nephroma: A clinicoradiological study of 17 cases representing the pathologic spectrum of the disease. J Pediatr 111:64-70, 1987 7. Howell CG, Othersen HB, Kiviat NE, et al: Therapy and outcome in 51 children with mesoblastic nephroma: A report of the national Wilm's Tumor Study. J Pediatr Surg 17:826-831, 1982 8. Blank E, Neerhout RC, Burry KA: Congenital mesoblastic nephroma and polyhydramnios. JAMA 240:1504—1505, 1978 9. Shanbhogue LKR, Gray E, Miller SS: Congenital mesoblastic nephroma of infancy associated with hypercalcemia. J Urol 135:771-772, 1986 10. Sauer L, Harrison MR, Flake AW, Krummel TR: Does an expanding abdominal mass produce pulmonary hypoplasia? J Pediatr Surg 22:508-512, 1987 11. Appuzio JJ, Unwin W, Adhate A, Nichols R: Prenatal diagnosis of fetal renal mesoblastic nephroma. Am J Obstet Gynecol 154:636-637, 1986

205

Congenital mesoblastic nephroma, hemorrhagic shock, and disseminated intravascular coagulation in a newborn infant.

Congenital mesoblastic nephroma is a rare tumor of infancy that usually presents as an asymptomatic abdominal mass. A full-term newborn infant with an...
866KB Sizes 0 Downloads 0 Views