NEUROBLASTOMA
PRESENTING
RENAL CELL CARCINOMA
GEORGE
C. BAUMGARTNER,
AS
IN AN ADULT
M.D.
JOHN GAETA, M.D. ZEW WAJSMAN, CLAUDE
M.D.
MERRIN,
M.D.
From the Departments of Urology and Pathology, Roswell Park Memorial Institute, New York State Department of Health, and the State University of New York at Buffalo, New York
ABSTRACT - A rare case of a fifty-six-year-old female with neuroblastoma is presented. Possible implications as to etiology are discussed, and the radiographic similarities between renal cell carcinoma and neuroblastoma are presented.
In 1972, Varkarakis, Bhanalaph, and Murphy’ reported their series of neuroblastoma which involved the kidney. Eleven of 62 patients had renal invasion on postmortem examination, most frequently from an abdominal or adrenal neuroblastoma. The oldest patient in their series was twenty years old. Deming and Howard2 stated that 80 per cent of neuroblastomas occur before the age of three years. Isolated instances of neuroblastoma have been reported, however, up to the eighth decade.2 An unusual case is reported herein of a neuroblastoma which occurred in a fifty-six-year-old woman who presented with findings of renal cell carcinoma. Case Report A fifty-six-year-old white female complained of back pain and weakness of both lower extremities for six months. Past medical history was negative for hypertension, sweating, or palpitations. She had had anorexia and mild weight loss. Physical examination revealed a blood pressure of 140/70 mm. Hg and pulse rate of 96. Findings on urinalysis and cytologic examination were negative. Admitting hemogram was 8.4 Gm. of hemoglo-
376
bin, 11,700 white blood cell count, and 110,000 platelets. Chest x-ray film showed only increased interstitial markings. Bone survey revealed small lucent areas in the calvaria. Intravenous pyelogram and renal arteriogram were almost identical to the usual radiographic appearance of cystic renal cell carcinoma (Figs. 1 and 2).3 Selective renal arteriogram was done which was compatible with cystic adenocarcinoma of the upper pole of the left kidney. A left nephrectomy was performed. Pathologic examination revealed neuroblastoma. On the fourth postoperative day, bleeding was noted from the drains, nose, and mouth. A coagulogram was reported as consistent with platelet deficiency. The patient died of generalized hemorrhage despite infusions of platelets and blood transfusions. Postmortem examination revealed evidence of hemorrhagic diathesis with neuroblastoma metastasis to the lungs, liver, and bones (Fig. 3). Comment Older age patients with neuroblastoma are of interest because they reflect exceptions to the
UROLOGY
/ SEFI-EMBER
1975 / VOLUME
VI, NUMBER
3
FIGURE 1. (A) lntravenous pyelogram showing duplicated upper collecting system on left with distortion of upper pole calyces by suprarenal mass. (B) Selective left renal at-teriogram showing cystic, relatively avascular mass, without calcification involving upper pole of kidney; this venous phase film shows irregular tumor vessels along medial and inferior border of cystic mass involving renal parenchyma with persistent tumor stain. (C) Selective left renal arteriogram with adrenalin; this film again demonstrates lesion with its abnormal vessels and lack of vasoconstriction.
FIGURE EC!iYO;Ll
2. 1Lateral x-rau skull; note mlvltipie cent areas over
calvaria.
usual age incidence of the disease and may be of practical importance in understanding factors which may inhibit or stimulate this tumor. The prenatal and genetic origins of this tumor have been proposed. 4,5 No definite viral material has been isolated from these tumors.6 A viral or carcinogen etiology would certainly be more probable in a patient of this age. In situ neuroblastomas have been discussed in children by Beckwith and Perrin.7 This malig-
UROLOGY
/ SEPTEMBER1975
/ VOLUMEVI,
NUMBER3
nant-appearing lesion in the adrenal disappears however before becoming clinically apparent. The possibility here is that an occasional in situ lesion becomes manifest after many years.7 The fact that this tumor, however, has the highest spontaneous remission rate of any tumor and cases of maturation of the tumor from neuroblastoma or sympatheticoblastoma to ganglioneuroma have been reported make it possible that cases such as this are the long-term results
377
FIGURE 3. Hematoxylin and eosin stain. (A) Low power, showing separation of kidney from tumor tissue by fibrous capsule of kidney; (B) high power and (C) medium power showing sympathicoblast cells with pseudorosette f&nation characteristic of neuroblastoma.
of spontaneous remission or possibly reactivation of an immunologically repressed tumor.8’g All clinical signs were suggestive of renal cell carcinoma. Therefore, workup for renal cell carcinoma was done with no evaluation of the catecholamine levels or vanilmandelic acid. Conclusion Neuroblastoma should be considered in the differential diagnosis of renal cell neoplasms even in older patients, especially if the upper pole of the kidneys are involved. 666 Elm Street Buffalo, New York 14203 (DR. BAUMGARTNER) References 1. VARKARAKIS, M. J., BHANALAPH, T., and MURPHY, in neuroblastoma, N.Y. G. P.: Kidney involvement State J. Med. 72: 2753 (1972).
378
2. DEMING, C. L., and HARVARD, B. M.: Neurogenic tumors - neuroblastoma, in Campbell, M. F., and Harrison, J. H., Eds.: Urology, 1970, p. 957. 3. NEY, C., and FRIEDENBERG, R. M. : Radiographic Atlas of the Genitourinary System, Philadelphia, J. B. Lippincott Company, 1966, pp. 381, 702-707. 4. Cox, D., YUNCKEN, C., and SPRIGGS, A. I.: Minute chromatin bodies in malignant tumors of childhood, Lancet, 2: 55 (1965). 5. VOUTE, P. A., JR., WADMAN, S. K., and VANPUTTEN, W. J.: Congenital neuroblastoma, Clin. Pediatr. 9: 206 (1970). a viral etiology? 6. MCALLISTER, R. M.: Neuroblastoma: J. Pediatr. Surg. 3: 138 (1968). 7. BECKWITH, J. B., and PERRIN, E. V.: In situ neuroblastomas: a contribution to the natural history of neural crest tumors, Am. J. Pathol. 43: 1089 (1963). 8. Fox, F., DAVIDSON, J,, and THOMAS, L. B.: Maturation of sympathicoblastoma into ganglioneuroma: report of 2 patients with 20 and 46 year survivals respectively, Cancer 12: 108 (1959). 9. SMITHERS, D. W.: Maturation in human tumors, Lancet 2: 949 (1969).
UROLOGY
/
SEI?-EMBER
1975 /
VOLUME VI, NUMBER 3
PRESENTING
RENAL CELL CARCINOMA
GEORGE
C. BAUMGARTNER,
AS
IN AN ADULT
M.D.
JOHN GAETA, M.D. ZEW WAJSMAN, CLAUDE
M.D.
MERRIN,
M.D.
From the Departments of Urology and Pathology, Roswell Park Memorial Institute, New York State Department of Health, and the State University of New York at Buffalo, New York
ABSTRACT - A rare case of a fifty-six-year-old female with neuroblastoma is presented. Possible implications as to etiology are discussed, and the radiographic similarities between renal cell carcinoma and neuroblastoma are presented.
In 1972, Varkarakis, Bhanalaph, and Murphy’ reported their series of neuroblastoma which involved the kidney. Eleven of 62 patients had renal invasion on postmortem examination, most frequently from an abdominal or adrenal neuroblastoma. The oldest patient in their series was twenty years old. Deming and Howard2 stated that 80 per cent of neuroblastomas occur before the age of three years. Isolated instances of neuroblastoma have been reported, however, up to the eighth decade.2 An unusual case is reported herein of a neuroblastoma which occurred in a fifty-six-year-old woman who presented with findings of renal cell carcinoma. Case Report A fifty-six-year-old white female complained of back pain and weakness of both lower extremities for six months. Past medical history was negative for hypertension, sweating, or palpitations. She had had anorexia and mild weight loss. Physical examination revealed a blood pressure of 140/70 mm. Hg and pulse rate of 96. Findings on urinalysis and cytologic examination were negative. Admitting hemogram was 8.4 Gm. of hemoglo-
376
bin, 11,700 white blood cell count, and 110,000 platelets. Chest x-ray film showed only increased interstitial markings. Bone survey revealed small lucent areas in the calvaria. Intravenous pyelogram and renal arteriogram were almost identical to the usual radiographic appearance of cystic renal cell carcinoma (Figs. 1 and 2).3 Selective renal arteriogram was done which was compatible with cystic adenocarcinoma of the upper pole of the left kidney. A left nephrectomy was performed. Pathologic examination revealed neuroblastoma. On the fourth postoperative day, bleeding was noted from the drains, nose, and mouth. A coagulogram was reported as consistent with platelet deficiency. The patient died of generalized hemorrhage despite infusions of platelets and blood transfusions. Postmortem examination revealed evidence of hemorrhagic diathesis with neuroblastoma metastasis to the lungs, liver, and bones (Fig. 3). Comment Older age patients with neuroblastoma are of interest because they reflect exceptions to the
UROLOGY
/ SEFI-EMBER
1975 / VOLUME
VI, NUMBER
3
FIGURE 1. (A) lntravenous pyelogram showing duplicated upper collecting system on left with distortion of upper pole calyces by suprarenal mass. (B) Selective left renal at-teriogram showing cystic, relatively avascular mass, without calcification involving upper pole of kidney; this venous phase film shows irregular tumor vessels along medial and inferior border of cystic mass involving renal parenchyma with persistent tumor stain. (C) Selective left renal arteriogram with adrenalin; this film again demonstrates lesion with its abnormal vessels and lack of vasoconstriction.
FIGURE EC!iYO;Ll
2. 1Lateral x-rau skull; note mlvltipie cent areas over
calvaria.
usual age incidence of the disease and may be of practical importance in understanding factors which may inhibit or stimulate this tumor. The prenatal and genetic origins of this tumor have been proposed. 4,5 No definite viral material has been isolated from these tumors.6 A viral or carcinogen etiology would certainly be more probable in a patient of this age. In situ neuroblastomas have been discussed in children by Beckwith and Perrin.7 This malig-
UROLOGY
/ SEPTEMBER1975
/ VOLUMEVI,
NUMBER3
nant-appearing lesion in the adrenal disappears however before becoming clinically apparent. The possibility here is that an occasional in situ lesion becomes manifest after many years.7 The fact that this tumor, however, has the highest spontaneous remission rate of any tumor and cases of maturation of the tumor from neuroblastoma or sympatheticoblastoma to ganglioneuroma have been reported make it possible that cases such as this are the long-term results
377
FIGURE 3. Hematoxylin and eosin stain. (A) Low power, showing separation of kidney from tumor tissue by fibrous capsule of kidney; (B) high power and (C) medium power showing sympathicoblast cells with pseudorosette f&nation characteristic of neuroblastoma.
of spontaneous remission or possibly reactivation of an immunologically repressed tumor.8’g All clinical signs were suggestive of renal cell carcinoma. Therefore, workup for renal cell carcinoma was done with no evaluation of the catecholamine levels or vanilmandelic acid. Conclusion Neuroblastoma should be considered in the differential diagnosis of renal cell neoplasms even in older patients, especially if the upper pole of the kidneys are involved. 666 Elm Street Buffalo, New York 14203 (DR. BAUMGARTNER) References 1. VARKARAKIS, M. J., BHANALAPH, T., and MURPHY, in neuroblastoma, N.Y. G. P.: Kidney involvement State J. Med. 72: 2753 (1972).
378
2. DEMING, C. L., and HARVARD, B. M.: Neurogenic tumors - neuroblastoma, in Campbell, M. F., and Harrison, J. H., Eds.: Urology, 1970, p. 957. 3. NEY, C., and FRIEDENBERG, R. M. : Radiographic Atlas of the Genitourinary System, Philadelphia, J. B. Lippincott Company, 1966, pp. 381, 702-707. 4. Cox, D., YUNCKEN, C., and SPRIGGS, A. I.: Minute chromatin bodies in malignant tumors of childhood, Lancet, 2: 55 (1965). 5. VOUTE, P. A., JR., WADMAN, S. K., and VANPUTTEN, W. J.: Congenital neuroblastoma, Clin. Pediatr. 9: 206 (1970). a viral etiology? 6. MCALLISTER, R. M.: Neuroblastoma: J. Pediatr. Surg. 3: 138 (1968). 7. BECKWITH, J. B., and PERRIN, E. V.: In situ neuroblastomas: a contribution to the natural history of neural crest tumors, Am. J. Pathol. 43: 1089 (1963). 8. Fox, F., DAVIDSON, J,, and THOMAS, L. B.: Maturation of sympathicoblastoma into ganglioneuroma: report of 2 patients with 20 and 46 year survivals respectively, Cancer 12: 108 (1959). 9. SMITHERS, D. W.: Maturation in human tumors, Lancet 2: 949 (1969).
UROLOGY
/
SEI?-EMBER
1975 /
VOLUME VI, NUMBER 3
