Recurrent Pheochromocytomas
in Children
By Sigmund H. Ein, Barry Shandling, David Wesson, and Robert M. Filler Toron to, Ontario 8 Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 8 years (1980 to 1988). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 8 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 8 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences. there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension. e 1990 by W.B. Saunders Company.
RESULTS
Between 1980 and 1986, five recurrences (Table 2) were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years of age. The boy presented again with headaches whereas the three girls were again hypertensive29; all symptoms and signs were similarly within 3 months. All radiological investigations were positive; three of four had CT scans. 3sS8,9 The only biochemical examination that was positive in all four children was the vanillylmandelic acid (VMA) test. Norepinephrines were positive in the three patients examined. Preoperative blockade was again used.” The three girls had tumors less than 3 cm found in the opposite adrenal; only one girl was left without any adrenal tissue. The boy was again found to have two new pheos (left adrenal and right thoracic paravertebral). There were no postoperative problems and they also went home normotensive. Follow-up of 2 to 8 years has been normal. DISCUSSION
INDEX WORD: Pheochromocytoma.
P
(pheo) is an uncomHEOCHROMOCYTOMA mon but exciting pediatric tumor.1’2 Twenty percent of all pheos are found in the pediatric population3 and more than one third of these children have multiple tumors4 most of which are recurrent. We reviewed our 29-year experience with this tumor and concentrated on the recurrent pheos. MATERIALS AND METHODS
From 1958 to 1987 inclusive, 13 children with 20 pheos were diagnosed and treated at the Hospital for Sick Children, Toronto. There were nine boys and four girls aged 8 to 17 years. Five of these 13 children had multiple (12) tumors, and four patients had recurrences. It is on these four patients that we are focusing our review; they had five recurrent pheos over a 6-year period (1980 to 1986). There were three girls and one boy, between the ages of 8 and 15 years. Their original pheos (Table 1) all presented between 1976 and 1984 with hypertension (episodic or sustained) and one girl had congestive heart failure.’ Symptoms and signs were present less than 3 months. Another girl had a past history of a cerebellar hemangioblastoma (von Hippel-Lindau disease).‘.5-7 The computed tomography (CT) scan was the only positive radiographic test in all four patients, although it missed a second tumor in the boy (found at the Journal of Pediatric Surgery, Vol 25, No 10 Kktober),
first laparotomy). At least one biochemical examination was diagnostic of a pheo in each patient, but there was still one normal test result per patient. All children received preoperative blockade; three of four pheos were adrenal. All were less than 6 cm. There were no postoperative problems and the children went home normotensive. Follow-up was maintained.
1990: pp 1063-1065
Many (30%) children have multiple pheos, either synchronous or metachronous. In our 29-year experience, we treated 13 children with pheos and four (30%) of them had recurrent pheos. In reviewing the presentation, investigation, and treatment of the original tumors, one cannot find any specific pattern that would have led to the suspicion that the children would return with a recurrent pheo. ii In spite of close follow-up after their first pheo was successfully treated and they returned home normotensive, none of the recurrences were picked up on routine checkups with the child completely asymptomatic. The four children had recur-
From the Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada. Presented at the 38th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Chicago. Illinois, October 21-23.1989. Address reprint requests to Sigmund H. Ein. MD, Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario h45G 1X8, Canada. 6 1990 by W.B. Saunders Company. 0022-3468/90/25-l O-001 1$03.00/O
1063
1064
EIN
ET AL
Table 1. Original Pheochromocytomas Patient No.
1
SeXlAW
M/l 1 yr
Year
Past Histciv
1976
-
Signs and Symptoms
Radiological
Biochemical
(Duration)
Studies
Studies
HT
(epis). vomiting,
IVP +, angio +.
Tumor
VMA - , catechol +,
seizure, wt loss,
(CT + missed one
headache, blurred
tumor)
(1) R parirenal artery
metaneph -
1.5 cm: (2) L paraaorta 3 cm
vision (3 mo) 2
F/8 Yr
-
1979
HT
(epis), CHF (2 mo)
US +, IVP +. CT +
VMA + , catechol +,
L adrenal 5 cm
metaneph + , HVA 3 4
F/9 yr F/15 yr
1979
-
1984
8 yr cerebral
7 (elsewhere)
7 (elsewhere)
HT kust)
US -,
IVP -,
CT +
? (elsewhere)
R adrenal
VMA +, dopamine -,
L
hemangioblastoma
norapineph +,
Won Hipplelindau)
epineph +
adrenal 6 cm
Abbreviations: HT. hypertension; epis. episodic: wt. weight: IVP, intravenous pyelography; angio, sngiogrsphy; CT, computed tomography; VMA, vsnillYlmsn&lic acid: catechol. catecholamina; metanaph,
metanephrine; R, right: L, left; CHF, congestive heart failure; us,
ultrasound; HVA,
homovanillic acid: sust, sustained; norepineph, norepinephrine; epineph, epinephrina.
rence of their hypertension along with other signs and symptoms (not necessarily the same) of this endocrine tumor. Moreover, the time of the recurrence (1 to 6 years) did not fit a pattern. The original tumors were all diagnosed in the 1970s (except one in 1984) and each patient averaged three radiological studies. When a recurrence was suspected in the 198Os, three of these four children again had a CT scan to make the diagnosis, and in two of these three this was the only x-ray test performed. On the other hand, the average number of biochemical tests in the 1980s increased from four to five, but the percentage of positive results continued to average between 55% and 57%; the VMA was again the only positive test in all four recurrent pheos with norepinephrine next. The recurrent tumors were all adrena11S4~6*‘2*‘3 in the four patients (opposite adrenal in three) with the boy also having an intrathoracic paravertebral tumor.‘4-‘6 The recurrent tumor size was also smaller (2.4 cm) than the original (3.5 cm). In spite of the fact that 75% of the recurrences Table 2. Recurrent Patient NO.
1
SW/A00
M/17
yr
Year.
1982 (6 yr)
were in the opposite adrenal,16 only one of the three patients ended up without any remaining adrenal tissue. When there is a recurrence in the opposite adrenal gland, (or, indeed, in any adrenal) should the entire adrenal gland be removed, or should an attempt be made to leave some adrenal tissue behind?16 The answer must remain in the hands of the operating surgeon; if the tumor (primary or recurrent) can be excised completely with some functioning adrenal tissue left behind, so much the better for the patient. In the situation of a primary unilateral adrenal pheo, a total adrenalectomy is probably the procedure of choice. However, if a pheo exists as part of a multiple endocrine neoplasia type 2 syndrome, it has been recommended that these patients have bilateral adrenalectomies, because they have medullary hyperplasia or multicentric tumor involvement of both adrenal glands.2~3Y” Why do recurrences occur? The answer may never be known. Either there is a small nidus of tumor that Pheochromocytoma
Signs and Symptoms
Radiological
Biochemical
(duration)
Studies
Studies
Headache 1% mo)
US +, angio +, CT +
Tumw
(1)
VMA +, dopamina +, norepineph + , HVA -,
F/9 yr
1980 (1 yrl
HT k.ustf. sweating
US + , angio +
VMA +, matanaph + , dopamine -,
(1% mo)
HVA -, 3
F/14 yr
1984 (5 yr)
HT lapis). flushing,
paravert
6 yr
3 cm; (2) L adrenal
apineph +, plasma ranin + 2
Rchest
Follow-UP
2.7 cm R
adrenal 3 cm
8 yr
norepineph +,
epineph -
CT +
VMA +, catechol + , mataneph -
L adrenal 1.7 cm
4 yr (no adrenals)
CT +
VMA +, meteneph -,
R adrenal 1.3 cm
2 yr
sweating, blurred vision (3 mo) 4
F/ 17 yr
1986 (2 yr)
HT kust)
dopamina -,
norepineph +, HVA -, Abbreviations: US, ultrasound; angio, angiography; CT, computed tomography; VMA,
epineph -
vanillylmandalic acid; norepineph, norapinephrina; HVA,
homovanillic acid; epineph, epinephrine; R. right; paravert, paravertebral; L, left; HT. hypertension; sust, sustained; metaneph. metanephrine; epis, episodic; catechol, catecholamine. *Numbers in parentheses denote number of years after first pheochromocytoma was removed.
RECURRENT PHEOCHROMOCYTOMAS
1065
IN CHILDREN
lays dormant and suppressed by the functioning pheo until the original suppressing tumor is excised”; or a new pheo starts de novo, uninfluenced by any other previous functioning tissue. None of the five recurrent pheos in this series was malignant, and the four
children remain well with a follow-up of 2 to 8 years. A close continuing annual follow-up is important, considering that if and when a recurrent pheo does return, it will probably be heralded by a return of the hypertension and other symptoms and signs.
REFERENCES 1. Fonkalsrud EW: The adrenal glands, in Welch KJ, Randolph JG, Ravitch MM, et al (eds): Pediatric Surgery. Chicago, IL, Year Book, 1986,~~ 1113-1124 2. Kaufman BH, Telander RL, Van Heerden JA, et al: Pheochromocytoma in the pediatric age group: Current status. J Pediatr Surg 18:879-884, 1983 3. Scott HW, Oates JA, Nies AS et al: Pheochromocytoma: Present diagnosis and management. Ann Surg 183587-593, 1976 4. Stackpole RH, Melicow MM, Uson AC: Pheochromocytoma in children. J Pediatr 63:315-330, 1963 5. Gilsanz FJ, Luengo C, Conerjo P, et al: Cardiomyopathy and pheochromocytoma. Anesthesia 38:888-891, 1983 6. Remine WH, Chong CC, Van Heerden JS, et al: Current management of pheochromocytoma. Ann Surg 179:740-748,1974 7. Bloom DA, Fonkalsrud EW: Surgical management of pheochromocytoma in children. J Pediatr Surg 12:157-163, 1977 8. Heikkinen ES, Akerblom HK: Diagnostic and operative problems in multiple pheochromocytomas. J Pediatr Surg 12:157-163, 1977
9. Van Heerden JA, Sheps SG, Hamberger B, et al: Pheochromocytoma: Current status and changing trends. Surgery 91:367-373, 1982 10. Pullerits J, Reynolds C: Pheochromocytoma: A clinical review with emphasis on pharmacologic aspects. J Clin Invest Med 5~259-265, 1982 11. Stringel G, Ein SH, Creighton R, et al: Pheochromocytoma in children-An update. J Pediatr Surg 15:496-500, 1980 12. Van Way CS III, Scott HW Jr, Page DL, et al: Pheochromocytoma, in Ravitch MM (ed): Current Problems in Surgery. Chicago, IL, Year Book, 1974, pp 35-36 13. Hume DM: Pheochromocytoma in the adult and in the child. Am J Surg 99:458-496, 1960 14. King RM, Telander RL, Smithson WA, et al: Primary mediastinal tumors in children. J Pediatr Surg 17:512-520, 1982 15. Prevot J, Schmitt M, Vidailhet M: Rare forms of pheochromocytoma in children. Prog Pediatr Surg 16:97-106, 1983 16. Marshall DG, Ein SH: Two boys with four pheochromocytomas each. J Pediatr Surg 21:815-817, 1986
Discussion J. Grosfeld (Indianapolis, IN): Do you think in the current year you would recommend just simply taking the blood pressure and watching the symptoms or would you use the MIBG scan as a method of following your patients? S. Ein (response): So far we haven’t been following them with the MIBG. A. Coran (Ann Arbor, MI): We’re going to report on 21 pheos over 15 years this coming November at the Western Surgical meeting, and we have found in that series that all of the cases that were evaluated during the MIBG era were positive. That is a scan with 100% positive pick-up rate of primary and recurrent tumors. I have one question for you. What are the details of your preoperative blockade? What drugs do you use and how long do you give them to the patient? S. Ein (response): Anesthesiologists examine these children and block them from 2 to 4 days. The
blockage is never 100% in spite of the fact that we originally thought so. M. Laquaglia (New York, NY): These are all recurrences in the contralateral adrenal and the question would be: why are they not just metachronous pheochromocytomas? Were calcintonins measured in these patients? Also, as a correlate to the discussion of Drs Grosfeld and Coran, have you had experience with treatment of unresectable disease? S. Ein (response): Calcintonins were not done. The CAT scans were all reviewed in retrospect and there was nothing there that we could see. Whether there was a small nidus, I can’t tell you. It certainly was not apparent either by CAT scan or by examination during the original operation. We have in our series three malignancies: one was resected a long time ago, another one presented with cord signs (he’s alive and well), and a third one was only partially resected. He’s alive, but the follow-up is only a couple of years.
in Children
By Sigmund H. Ein, Barry Shandling, David Wesson, and Robert M. Filler Toron to, Ontario 8 Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 8 years (1980 to 1988). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 8 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 8 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences. there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension. e 1990 by W.B. Saunders Company.
RESULTS
Between 1980 and 1986, five recurrences (Table 2) were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years of age. The boy presented again with headaches whereas the three girls were again hypertensive29; all symptoms and signs were similarly within 3 months. All radiological investigations were positive; three of four had CT scans. 3sS8,9 The only biochemical examination that was positive in all four children was the vanillylmandelic acid (VMA) test. Norepinephrines were positive in the three patients examined. Preoperative blockade was again used.” The three girls had tumors less than 3 cm found in the opposite adrenal; only one girl was left without any adrenal tissue. The boy was again found to have two new pheos (left adrenal and right thoracic paravertebral). There were no postoperative problems and they also went home normotensive. Follow-up of 2 to 8 years has been normal. DISCUSSION
INDEX WORD: Pheochromocytoma.
P
(pheo) is an uncomHEOCHROMOCYTOMA mon but exciting pediatric tumor.1’2 Twenty percent of all pheos are found in the pediatric population3 and more than one third of these children have multiple tumors4 most of which are recurrent. We reviewed our 29-year experience with this tumor and concentrated on the recurrent pheos. MATERIALS AND METHODS
From 1958 to 1987 inclusive, 13 children with 20 pheos were diagnosed and treated at the Hospital for Sick Children, Toronto. There were nine boys and four girls aged 8 to 17 years. Five of these 13 children had multiple (12) tumors, and four patients had recurrences. It is on these four patients that we are focusing our review; they had five recurrent pheos over a 6-year period (1980 to 1986). There were three girls and one boy, between the ages of 8 and 15 years. Their original pheos (Table 1) all presented between 1976 and 1984 with hypertension (episodic or sustained) and one girl had congestive heart failure.’ Symptoms and signs were present less than 3 months. Another girl had a past history of a cerebellar hemangioblastoma (von Hippel-Lindau disease).‘.5-7 The computed tomography (CT) scan was the only positive radiographic test in all four patients, although it missed a second tumor in the boy (found at the Journal of Pediatric Surgery, Vol 25, No 10 Kktober),
first laparotomy). At least one biochemical examination was diagnostic of a pheo in each patient, but there was still one normal test result per patient. All children received preoperative blockade; three of four pheos were adrenal. All were less than 6 cm. There were no postoperative problems and the children went home normotensive. Follow-up was maintained.
1990: pp 1063-1065
Many (30%) children have multiple pheos, either synchronous or metachronous. In our 29-year experience, we treated 13 children with pheos and four (30%) of them had recurrent pheos. In reviewing the presentation, investigation, and treatment of the original tumors, one cannot find any specific pattern that would have led to the suspicion that the children would return with a recurrent pheo. ii In spite of close follow-up after their first pheo was successfully treated and they returned home normotensive, none of the recurrences were picked up on routine checkups with the child completely asymptomatic. The four children had recur-
From the Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada. Presented at the 38th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Chicago. Illinois, October 21-23.1989. Address reprint requests to Sigmund H. Ein. MD, Division of General Surgery, The Hospital for Sick Children, Toronto, Ontario h45G 1X8, Canada. 6 1990 by W.B. Saunders Company. 0022-3468/90/25-l O-001 1$03.00/O
1063
1064
EIN
ET AL
Table 1. Original Pheochromocytomas Patient No.
1
SeXlAW
M/l 1 yr
Year
Past Histciv
1976
-
Signs and Symptoms
Radiological
Biochemical
(Duration)
Studies
Studies
HT
(epis). vomiting,
IVP +, angio +.
Tumor
VMA - , catechol +,
seizure, wt loss,
(CT + missed one
headache, blurred
tumor)
(1) R parirenal artery
metaneph -
1.5 cm: (2) L paraaorta 3 cm
vision (3 mo) 2
F/8 Yr
-
1979
HT
(epis), CHF (2 mo)
US +, IVP +. CT +
VMA + , catechol +,
L adrenal 5 cm
metaneph + , HVA 3 4
F/9 yr F/15 yr
1979
-
1984
8 yr cerebral
7 (elsewhere)
7 (elsewhere)
HT kust)
US -,
IVP -,
CT +
? (elsewhere)
R adrenal
VMA +, dopamine -,
L
hemangioblastoma
norapineph +,
Won Hipplelindau)
epineph +
adrenal 6 cm
Abbreviations: HT. hypertension; epis. episodic: wt. weight: IVP, intravenous pyelography; angio, sngiogrsphy; CT, computed tomography; VMA, vsnillYlmsn&lic acid: catechol. catecholamina; metanaph,
metanephrine; R, right: L, left; CHF, congestive heart failure; us,
ultrasound; HVA,
homovanillic acid: sust, sustained; norepineph, norepinephrine; epineph, epinephrina.
rence of their hypertension along with other signs and symptoms (not necessarily the same) of this endocrine tumor. Moreover, the time of the recurrence (1 to 6 years) did not fit a pattern. The original tumors were all diagnosed in the 1970s (except one in 1984) and each patient averaged three radiological studies. When a recurrence was suspected in the 198Os, three of these four children again had a CT scan to make the diagnosis, and in two of these three this was the only x-ray test performed. On the other hand, the average number of biochemical tests in the 1980s increased from four to five, but the percentage of positive results continued to average between 55% and 57%; the VMA was again the only positive test in all four recurrent pheos with norepinephrine next. The recurrent tumors were all adrena11S4~6*‘2*‘3 in the four patients (opposite adrenal in three) with the boy also having an intrathoracic paravertebral tumor.‘4-‘6 The recurrent tumor size was also smaller (2.4 cm) than the original (3.5 cm). In spite of the fact that 75% of the recurrences Table 2. Recurrent Patient NO.
1
SW/A00
M/17
yr
Year.
1982 (6 yr)
were in the opposite adrenal,16 only one of the three patients ended up without any remaining adrenal tissue. When there is a recurrence in the opposite adrenal gland, (or, indeed, in any adrenal) should the entire adrenal gland be removed, or should an attempt be made to leave some adrenal tissue behind?16 The answer must remain in the hands of the operating surgeon; if the tumor (primary or recurrent) can be excised completely with some functioning adrenal tissue left behind, so much the better for the patient. In the situation of a primary unilateral adrenal pheo, a total adrenalectomy is probably the procedure of choice. However, if a pheo exists as part of a multiple endocrine neoplasia type 2 syndrome, it has been recommended that these patients have bilateral adrenalectomies, because they have medullary hyperplasia or multicentric tumor involvement of both adrenal glands.2~3Y” Why do recurrences occur? The answer may never be known. Either there is a small nidus of tumor that Pheochromocytoma
Signs and Symptoms
Radiological
Biochemical
(duration)
Studies
Studies
Headache 1% mo)
US +, angio +, CT +
Tumw
(1)
VMA +, dopamina +, norepineph + , HVA -,
F/9 yr
1980 (1 yrl
HT k.ustf. sweating
US + , angio +
VMA +, matanaph + , dopamine -,
(1% mo)
HVA -, 3
F/14 yr
1984 (5 yr)
HT lapis). flushing,
paravert
6 yr
3 cm; (2) L adrenal
apineph +, plasma ranin + 2
Rchest
Follow-UP
2.7 cm R
adrenal 3 cm
8 yr
norepineph +,
epineph -
CT +
VMA +, catechol + , mataneph -
L adrenal 1.7 cm
4 yr (no adrenals)
CT +
VMA +, meteneph -,
R adrenal 1.3 cm
2 yr
sweating, blurred vision (3 mo) 4
F/ 17 yr
1986 (2 yr)
HT kust)
dopamina -,
norepineph +, HVA -, Abbreviations: US, ultrasound; angio, angiography; CT, computed tomography; VMA,
epineph -
vanillylmandalic acid; norepineph, norapinephrina; HVA,
homovanillic acid; epineph, epinephrine; R. right; paravert, paravertebral; L, left; HT. hypertension; sust, sustained; metaneph. metanephrine; epis, episodic; catechol, catecholamine. *Numbers in parentheses denote number of years after first pheochromocytoma was removed.
RECURRENT PHEOCHROMOCYTOMAS
1065
IN CHILDREN
lays dormant and suppressed by the functioning pheo until the original suppressing tumor is excised”; or a new pheo starts de novo, uninfluenced by any other previous functioning tissue. None of the five recurrent pheos in this series was malignant, and the four
children remain well with a follow-up of 2 to 8 years. A close continuing annual follow-up is important, considering that if and when a recurrent pheo does return, it will probably be heralded by a return of the hypertension and other symptoms and signs.
REFERENCES 1. Fonkalsrud EW: The adrenal glands, in Welch KJ, Randolph JG, Ravitch MM, et al (eds): Pediatric Surgery. Chicago, IL, Year Book, 1986,~~ 1113-1124 2. Kaufman BH, Telander RL, Van Heerden JA, et al: Pheochromocytoma in the pediatric age group: Current status. J Pediatr Surg 18:879-884, 1983 3. Scott HW, Oates JA, Nies AS et al: Pheochromocytoma: Present diagnosis and management. Ann Surg 183587-593, 1976 4. Stackpole RH, Melicow MM, Uson AC: Pheochromocytoma in children. J Pediatr 63:315-330, 1963 5. Gilsanz FJ, Luengo C, Conerjo P, et al: Cardiomyopathy and pheochromocytoma. Anesthesia 38:888-891, 1983 6. Remine WH, Chong CC, Van Heerden JS, et al: Current management of pheochromocytoma. Ann Surg 179:740-748,1974 7. Bloom DA, Fonkalsrud EW: Surgical management of pheochromocytoma in children. J Pediatr Surg 12:157-163, 1977 8. Heikkinen ES, Akerblom HK: Diagnostic and operative problems in multiple pheochromocytomas. J Pediatr Surg 12:157-163, 1977
9. Van Heerden JA, Sheps SG, Hamberger B, et al: Pheochromocytoma: Current status and changing trends. Surgery 91:367-373, 1982 10. Pullerits J, Reynolds C: Pheochromocytoma: A clinical review with emphasis on pharmacologic aspects. J Clin Invest Med 5~259-265, 1982 11. Stringel G, Ein SH, Creighton R, et al: Pheochromocytoma in children-An update. J Pediatr Surg 15:496-500, 1980 12. Van Way CS III, Scott HW Jr, Page DL, et al: Pheochromocytoma, in Ravitch MM (ed): Current Problems in Surgery. Chicago, IL, Year Book, 1974, pp 35-36 13. Hume DM: Pheochromocytoma in the adult and in the child. Am J Surg 99:458-496, 1960 14. King RM, Telander RL, Smithson WA, et al: Primary mediastinal tumors in children. J Pediatr Surg 17:512-520, 1982 15. Prevot J, Schmitt M, Vidailhet M: Rare forms of pheochromocytoma in children. Prog Pediatr Surg 16:97-106, 1983 16. Marshall DG, Ein SH: Two boys with four pheochromocytomas each. J Pediatr Surg 21:815-817, 1986
Discussion J. Grosfeld (Indianapolis, IN): Do you think in the current year you would recommend just simply taking the blood pressure and watching the symptoms or would you use the MIBG scan as a method of following your patients? S. Ein (response): So far we haven’t been following them with the MIBG. A. Coran (Ann Arbor, MI): We’re going to report on 21 pheos over 15 years this coming November at the Western Surgical meeting, and we have found in that series that all of the cases that were evaluated during the MIBG era were positive. That is a scan with 100% positive pick-up rate of primary and recurrent tumors. I have one question for you. What are the details of your preoperative blockade? What drugs do you use and how long do you give them to the patient? S. Ein (response): Anesthesiologists examine these children and block them from 2 to 4 days. The
blockage is never 100% in spite of the fact that we originally thought so. M. Laquaglia (New York, NY): These are all recurrences in the contralateral adrenal and the question would be: why are they not just metachronous pheochromocytomas? Were calcintonins measured in these patients? Also, as a correlate to the discussion of Drs Grosfeld and Coran, have you had experience with treatment of unresectable disease? S. Ein (response): Calcintonins were not done. The CAT scans were all reviewed in retrospect and there was nothing there that we could see. Whether there was a small nidus, I can’t tell you. It certainly was not apparent either by CAT scan or by examination during the original operation. We have in our series three malignancies: one was resected a long time ago, another one presented with cord signs (he’s alive and well), and a third one was only partially resected. He’s alive, but the follow-up is only a couple of years.
