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Association of hydrops fetalis with congenital neuroblastoma
Volume Number
132 8
Communications
that was followed by intrauterine fetal death. In the second case, at 43 weeks of gestation, the sinusoidal FHR was observed during early labor that followed an uncomplicated pregnancy. Despite the return to a normal heart rate a severely depressed infant was delivered. These case reports should strengthen the sense of ominous import of the sinusoidal pattern and should convince obstetricians to act decisively when the pattern is seen. We, along with Rochard and associates,z believe that the sinusoidal pattern represents absence of central nervous system control over the heart. This would represent the end stage of severe fetal distress and would explain the large percentage of fetal deaths* seen in association with the sinusoidal pattern. It would seem that, in conjunction with an appropriately high risk situation, this pattern does appear to be ominous; it warrants intensive treatment and probably delivery.
in brief
905
Coombs tests, negative; urinalysis, negative; total serum protein, 2.3 Gm. per 100 ml. (albumin, 1.4 Gm.); blood urea nitrogen; 21 mg. Per 100 ml.; and serum creatinine, 1.1 mg. per 100 ml. Blood glucose and serum electrolytes were normal. With an F~,,of 100 per cent, the PoZ was 100 mm. Hg; Pco,, 49 mm. Hg; and pH, 7.32. Chest roentgenogram showed diffuse opacification of both lung fields with indistinct
borders of the cardiac silhouette. Despite supportive therapy, the course
was progressively
downhill
and the baby died eight
days after birth. At autopsy, all organs were congested. The lungs strated
diffuse
chronic
manifest by fibrin-filled
obliterative
hyaline
macrophages
membrane
demondisease
and proliferation
of
fibroblasts. The heart and kidneys were normal grossly and microscopically. The thin strip of yellow cortex of the right adrenal gland was stretched over a round medullary nodule of brown tissue,
measuring
0.7 cm. in diameter.
Microscopically
form, hyperchromatic cells, some forming were associated with focal areas of hemorrhage This pattern was diagnostic of neuroblastoma
small, uni-
pseudorosettes, and necrosis. (Fig. 1).
REFERENCES
1. Modanlou, H. D., Freeman, R. K., Ortiz, O., et al.: Sinusoidal fetal heart rate pattern and severe anemia, Obstet. Gynecol. 49: 537, 1977. 2. Rochard, F., Schifrin, B. S., Goupil, F., Legrand, H., Blottiere, J., and Sureau, C.: Nonstressed fetal heart rate monitoring in the antepartum period, AM. J. OBSTET. GYNECOL. 126: 699, 1976.
Association of hydrops fetalis with congenital neuroblastoma THOMAS LEO
J. KAPLAN,
MOSS,
B.A.
M.D.
Departments of Pathology, Cedars-Sinai Medical Center and Universily of Calfoforniu, Los Angeles, School of Medicine, Los Angelrs, Colijornia HYDROPS FETALIS, usually associated with erythroblastosis fetalis, is also seen with a number of other conditions.’ One of these,. not widely recognized, is congenital neuroblastoma. There have been three such cases reported in the literature.*’ 3 We recently encountered a fourth case, which forms the basis of this report. The infant was the product of a 32 week gestational period and an uncomplicated delivery. Birth Weight was 3,200 grams. Severe hydrops fetahs was noted at birth. There was no evidence of Rh or ABO incompatibility. No congenital abnormalities were noted. Laboratory tests shortly after birth were as follows: hematocrit, 42 per cent; platelet count, 92,000 per cubic millimeter; Reprint requests: Thomas rics, University of California, cine, Los Angeles, California
OOOZ-9378/78/240905+02$00.20/0
J. Moss, Department of PediatLos Angeles, School of Medi90024.
0 1978 The C. V. Mosby Co
Hydrops fetalis is usually, but not always, associated with erythroblastosis. A review of autopsy material at the University of California, Los Angeles Medical Center and the Cedars-Sinai Medical Center over the past 20 years disclosed 32 cases of hydrops fetalis and erythroblastosis fetalis was present in 28 of these. Two infants with hydrops had pulmonary hypoplasia without erythroblastosis and no significant associated findings were observed in the other two. Neuroblastoma, in association with hydrops fetalis, was first reported by Strauss and Driscoll,* in 1964. They observed two patients with bilateral adrenal congenital neuroblastoma. In both of these cases, there were extensive metastases to the liver, bone marrow, and placenta. A third case was reported in 1974 by Johnson and,Halbert.3 The tumor in this latter patient involved both adrenal glands with metastases to the liver, lungs, brain, and placenta. Previous hypotheses regarding the pathogenesis of hydrops fetalis with neuroblastoma include: (1) invasion of erythropoietic tissue by tumor cells, resulting in severe intrauterine anemia and congestive heart failure; (2) mechanical compression of the vena cava by the primary tumor or enlarged liver: and (3) impedance of blood flow by tumor cells in the hepatic and placental vasculature.** 3 A fourth possibility is intrauterine arrhythmia and heart failure due to excessive secretion of catecholamines. Instances of hydrops fetalis resulting from idiopathic paroxysmal tachycardia have been recorded in the literature.4-7 A fifth possibility is hypersecretion of aldosterone induced by pressure encroachment, irritation, etc., of the tumor on the adrenal cortex. Hypersecretion of aldosterone is a well-established, albeit rare, cause of edema, and a significant number of patients (less than 10 per cent) with primary hyperaldosteronism have
906
Communications
in brief
Fig. 1. Photomicrograph showing chromatic cells with pseudorosettes
section of neuroblastoma. Note the characteristic and focal areas of hemorrhage and necrosis.
edema.* Tumors of the adrenal medulla are occasion111~ associated with Cushing’s syndrome,g-” thereb) suggesting that the cortex can be triggered into a lypersecretory state. In the case presented here, al.hough the tumor was small, measuring 0.7 cm. in -diameter, it did demonstrate compression of’the over-
ying adrenal
cortex.
small
hyl>el.-
1 1. Wilson. R. J., et al.: Metabolic studies in a patient phaeochromocytoma associated with hypokalaemia hyperaldosteronism. J. Endocrinol. 56: 69. 1973.
with a and
Transitory fetal asci tes: An ultrasound diagnosis
REFERENCES
1. Driscoll, S. G.: Hydrops fetalis, N. Engl. J. Med. 275: 1432, 1966. 2. Strauss, L., and Driscoll, S. G.: Congenital neuroblastoma involving the placenta, Pediatrics 34: 23, 1964. 3. Johnson, A. T., Jr., and Halbert, D.: Congenital neuroblastoma presenting as hydrops fetalis, N. C. Med. J. 35:289, 1974. 4. Cowan, R. H., Waldo, A. L., Harris, H. B., et al.: Neonatal paroxysmal supraventricular tachycardia with hydrops, Pediatrics 55: 428, 1975. 5. Kesson, C. W.: Foetal paroxysmal auricular tachycardia. Br. Heart J. 20:552, 1658. ’ 6. Silber. D. L., and Dusin. R. E.: Intrauterine atrial tachvcardia associated with massive edema in the newborn, Am. J. Dis. Child. 117: 722, 1969. 7. Hedvall, G.: Congenital paroxysmal tachycardia-A re-
port of three cases, Acta Paediatr. Stand. 62: 550, 1973. 8. Williams, R. H.: Textbook of Endocrinology, Philadelphia, 1968, W. B. Saunders Company. 9. i(aplan, L. I.: Sympathicoblasto&a w&h metastases associated with the clinical picture of Cushing’a syndrome: Report of a case, Arch. Neurol. Psychiatry 62: 696, 1949.
0. Kitaiim, W., Saruta, T., Kondo, K.. et al.: A case of serondiry Ural.
aldosteronism induced 141, 1975.
114:
by pheochromocytoma,
J.
LAWRENCE D. PLAT-I, M.D JOSEPH V. COLLEA, M.D. DANIEL M. JOSEPH. M.D.
~I‘HE USE OF diagnostic ultrasound in the practice of obstetrics has become an invaluable tool for the clinician. This technique has proved useful in the antenatal diagnosis of many congenital abnormalities, including fetal ascites.’ A case is presented in which the diagnosis of fetal ascites was made on multiple ultrasonic examinations, but ascites was not present at birth.
Supported in part by National Institutes of HeaItB Grant 070X6. Reprint requests: Dr. Lawrence D. Platt, !Vomen‘s Hospital. jK-22. 1240 N. Mission Road, In-1s Angeles. California 90033.
HD
000”-1~978/7X/240906+0$$00.:40/0
0
I%8
I~t>e C:. \‘. Mvstw
Co.
132 8
Communications
that was followed by intrauterine fetal death. In the second case, at 43 weeks of gestation, the sinusoidal FHR was observed during early labor that followed an uncomplicated pregnancy. Despite the return to a normal heart rate a severely depressed infant was delivered. These case reports should strengthen the sense of ominous import of the sinusoidal pattern and should convince obstetricians to act decisively when the pattern is seen. We, along with Rochard and associates,z believe that the sinusoidal pattern represents absence of central nervous system control over the heart. This would represent the end stage of severe fetal distress and would explain the large percentage of fetal deaths* seen in association with the sinusoidal pattern. It would seem that, in conjunction with an appropriately high risk situation, this pattern does appear to be ominous; it warrants intensive treatment and probably delivery.
in brief
905
Coombs tests, negative; urinalysis, negative; total serum protein, 2.3 Gm. per 100 ml. (albumin, 1.4 Gm.); blood urea nitrogen; 21 mg. Per 100 ml.; and serum creatinine, 1.1 mg. per 100 ml. Blood glucose and serum electrolytes were normal. With an F~,,of 100 per cent, the PoZ was 100 mm. Hg; Pco,, 49 mm. Hg; and pH, 7.32. Chest roentgenogram showed diffuse opacification of both lung fields with indistinct
borders of the cardiac silhouette. Despite supportive therapy, the course
was progressively
downhill
and the baby died eight
days after birth. At autopsy, all organs were congested. The lungs strated
diffuse
chronic
manifest by fibrin-filled
obliterative
hyaline
macrophages
membrane
demondisease
and proliferation
of
fibroblasts. The heart and kidneys were normal grossly and microscopically. The thin strip of yellow cortex of the right adrenal gland was stretched over a round medullary nodule of brown tissue,
measuring
0.7 cm. in diameter.
Microscopically
form, hyperchromatic cells, some forming were associated with focal areas of hemorrhage This pattern was diagnostic of neuroblastoma
small, uni-
pseudorosettes, and necrosis. (Fig. 1).
REFERENCES
1. Modanlou, H. D., Freeman, R. K., Ortiz, O., et al.: Sinusoidal fetal heart rate pattern and severe anemia, Obstet. Gynecol. 49: 537, 1977. 2. Rochard, F., Schifrin, B. S., Goupil, F., Legrand, H., Blottiere, J., and Sureau, C.: Nonstressed fetal heart rate monitoring in the antepartum period, AM. J. OBSTET. GYNECOL. 126: 699, 1976.
Association of hydrops fetalis with congenital neuroblastoma THOMAS LEO
J. KAPLAN,
MOSS,
B.A.
M.D.
Departments of Pathology, Cedars-Sinai Medical Center and Universily of Calfoforniu, Los Angeles, School of Medicine, Los Angelrs, Colijornia HYDROPS FETALIS, usually associated with erythroblastosis fetalis, is also seen with a number of other conditions.’ One of these,. not widely recognized, is congenital neuroblastoma. There have been three such cases reported in the literature.*’ 3 We recently encountered a fourth case, which forms the basis of this report. The infant was the product of a 32 week gestational period and an uncomplicated delivery. Birth Weight was 3,200 grams. Severe hydrops fetahs was noted at birth. There was no evidence of Rh or ABO incompatibility. No congenital abnormalities were noted. Laboratory tests shortly after birth were as follows: hematocrit, 42 per cent; platelet count, 92,000 per cubic millimeter; Reprint requests: Thomas rics, University of California, cine, Los Angeles, California
OOOZ-9378/78/240905+02$00.20/0
J. Moss, Department of PediatLos Angeles, School of Medi90024.
0 1978 The C. V. Mosby Co
Hydrops fetalis is usually, but not always, associated with erythroblastosis. A review of autopsy material at the University of California, Los Angeles Medical Center and the Cedars-Sinai Medical Center over the past 20 years disclosed 32 cases of hydrops fetalis and erythroblastosis fetalis was present in 28 of these. Two infants with hydrops had pulmonary hypoplasia without erythroblastosis and no significant associated findings were observed in the other two. Neuroblastoma, in association with hydrops fetalis, was first reported by Strauss and Driscoll,* in 1964. They observed two patients with bilateral adrenal congenital neuroblastoma. In both of these cases, there were extensive metastases to the liver, bone marrow, and placenta. A third case was reported in 1974 by Johnson and,Halbert.3 The tumor in this latter patient involved both adrenal glands with metastases to the liver, lungs, brain, and placenta. Previous hypotheses regarding the pathogenesis of hydrops fetalis with neuroblastoma include: (1) invasion of erythropoietic tissue by tumor cells, resulting in severe intrauterine anemia and congestive heart failure; (2) mechanical compression of the vena cava by the primary tumor or enlarged liver: and (3) impedance of blood flow by tumor cells in the hepatic and placental vasculature.** 3 A fourth possibility is intrauterine arrhythmia and heart failure due to excessive secretion of catecholamines. Instances of hydrops fetalis resulting from idiopathic paroxysmal tachycardia have been recorded in the literature.4-7 A fifth possibility is hypersecretion of aldosterone induced by pressure encroachment, irritation, etc., of the tumor on the adrenal cortex. Hypersecretion of aldosterone is a well-established, albeit rare, cause of edema, and a significant number of patients (less than 10 per cent) with primary hyperaldosteronism have
906
Communications
in brief
Fig. 1. Photomicrograph showing chromatic cells with pseudorosettes
section of neuroblastoma. Note the characteristic and focal areas of hemorrhage and necrosis.
edema.* Tumors of the adrenal medulla are occasion111~ associated with Cushing’s syndrome,g-” thereb) suggesting that the cortex can be triggered into a lypersecretory state. In the case presented here, al.hough the tumor was small, measuring 0.7 cm. in -diameter, it did demonstrate compression of’the over-
ying adrenal
cortex.
small
hyl>el.-
1 1. Wilson. R. J., et al.: Metabolic studies in a patient phaeochromocytoma associated with hypokalaemia hyperaldosteronism. J. Endocrinol. 56: 69. 1973.
with a and
Transitory fetal asci tes: An ultrasound diagnosis
REFERENCES
1. Driscoll, S. G.: Hydrops fetalis, N. Engl. J. Med. 275: 1432, 1966. 2. Strauss, L., and Driscoll, S. G.: Congenital neuroblastoma involving the placenta, Pediatrics 34: 23, 1964. 3. Johnson, A. T., Jr., and Halbert, D.: Congenital neuroblastoma presenting as hydrops fetalis, N. C. Med. J. 35:289, 1974. 4. Cowan, R. H., Waldo, A. L., Harris, H. B., et al.: Neonatal paroxysmal supraventricular tachycardia with hydrops, Pediatrics 55: 428, 1975. 5. Kesson, C. W.: Foetal paroxysmal auricular tachycardia. Br. Heart J. 20:552, 1658. ’ 6. Silber. D. L., and Dusin. R. E.: Intrauterine atrial tachvcardia associated with massive edema in the newborn, Am. J. Dis. Child. 117: 722, 1969. 7. Hedvall, G.: Congenital paroxysmal tachycardia-A re-
port of three cases, Acta Paediatr. Stand. 62: 550, 1973. 8. Williams, R. H.: Textbook of Endocrinology, Philadelphia, 1968, W. B. Saunders Company. 9. i(aplan, L. I.: Sympathicoblasto&a w&h metastases associated with the clinical picture of Cushing’a syndrome: Report of a case, Arch. Neurol. Psychiatry 62: 696, 1949.
0. Kitaiim, W., Saruta, T., Kondo, K.. et al.: A case of serondiry Ural.
aldosteronism induced 141, 1975.
114:
by pheochromocytoma,
J.
LAWRENCE D. PLAT-I, M.D JOSEPH V. COLLEA, M.D. DANIEL M. JOSEPH. M.D.
~I‘HE USE OF diagnostic ultrasound in the practice of obstetrics has become an invaluable tool for the clinician. This technique has proved useful in the antenatal diagnosis of many congenital abnormalities, including fetal ascites.’ A case is presented in which the diagnosis of fetal ascites was made on multiple ultrasonic examinations, but ascites was not present at birth.
Supported in part by National Institutes of HeaItB Grant 070X6. Reprint requests: Dr. Lawrence D. Platt, !Vomen‘s Hospital. jK-22. 1240 N. Mission Road, In-1s Angeles. California 90033.
HD
000”-1~978/7X/240906+0$$00.:40/0
0
I%8
I~t>e C:. \‘. Mvstw
Co.