International Journal of Cardiology. 34 (1992) 346-348
346 Q 1992 Elsevier
CARD10
Science
Publishers
B.V. All rights reserved
0167-5273/92/$05,00
14103
Malignant pheochromocytoma Abhay K. Pande Abhay Heart Clinic. Thapar Nursing Home, Ramdaspeth Nagpur. India (Received
17 June 1991; revision
accepted
25 September
1991)
We present an interesting case of paroxysmal hypertension in a young male caused by malignant pheochromocytoma. This patient, who had history of paroxysms of abdominal pain with severe hypertension, developed osseous metastasis in the first lumbar vertebra resulting in collapse of the vertebra and it caused paraplegia. The diagnosis of pheochromocytoma was confirmed on histopathology. Key words:
Pheochromocytoma;
Malignant
change;
Osseous
Introduction Pheochromocytoma is an important cause of secondary hypertension which continues to evoke interest because it is capable of mimicking a variety of syndromes. We report an interesting case of malignant pheochromocytoma with unusual clinical presentation.
Case Report A 23-year-old male presented with rapidly progressive weakness in the lower limbs for 15 days and a history of severe abdominal pain associated with vomiting and hypertension, palpitations and flushing, and a palpable abdominal mass of 8 years’ duration. Initially, the frequency of the abdominal pain with hypertension was 1 to 2 times per year which increased to 6 to 8 times in the last 2 years with a rapid increase in the size of abdominal mass. There was no history of injury to the spine, of fever, or of tuberculosis. Examination revealed a thin nervous patient with a gibbus at the first lumbar vertebra and a large irregular mass, firm in consistency, palpable in the left hypochondrium and iliac regions. Cardiovascular exam-
Correspondence to: Dr. Abhay Pande, Cardiology University Hospital, 1211, Geneva, Switzerland.
Center,
metastasis:
Unusual
presentation
ination was normal. Central nervous system examination revealed paraplegia. There was no evidence of cafe-au-lait spots, neurofibromatosis, tuberous sclerosis, or Sturge-Weber disease. Abdominal palpation, performed as a provocative test, triggered a hypertensive crisis with a blood pressure of 270/160 mmHg which was controlled with sublingual nifedipine. A plain X-ray of the abdomen showed paravertebral calcification (Fig. 1A). The lumbar spine showed localized collapse of the first lumbar vertebra. Other disc spaces were normal (Fig. 1B). An intravenous pyelogram revealed an upwardly displaced left kidney with hydronephrotic changes. An abdominal ultrasound demonstrated a large infrarenal mass 20-25 cm in size in the left hypochondrium, lumbar and iliac regions. The patient was stabilized with oral phenoxybenzamine, labetolol, and liberal intake of salt and water. Exploratory laparotomy revealed a large highly vascular mass of irregular consistency adherent to the aorta and inferior caval vein. It was not possible to resect the mass. Surgery was well tolerated without any hypertensive or hypotensive crisis. The biopsy from the mass showed polyhydral polygonal cells with finely granular eosinophilic cytoplasm, vesicular nuclei, and surrounded by a rich network of capillaries (Fig. 2) and it confirmed diagnosis of malignant pheochromocytoma with metastasis to the first lumbar vertebra. He died 6 months later at his native place. Autopsy studies could not be performed.
Fig. 1. A. Plain X-ray of abdomen
showing calcification
in the tumor mass (arrow).
collapse of first lumbar vertebra
Fig. 2. Histopathology
of the tumor mass revealing
B. X-ray of dorsolumbar
spine showing localized
(arrow).
large polygonal polyhydral
cells characteristic
of pheochromocytoma
348 Discussion
The incidence of pheochromocytoma is 0.001% in the general population, and 0.5% in patients with hypertension [1,2]. Malignant change occurs in 3 to 14%, and it represents a particular diagnostic problem because of the potential for a multicentric origin and the absence of specific histologic criteria for malignant change [3]. The diagnosis is dependent on distant metastasis which occurs to lymph nodes, liver, bones, muscle, or lungs. The clinical presentation of pheochromocytoma is variable [2]. To the best of our knowledge a presentation with sudden onset paraplegia caused by collapse of the first lumbar vertebra due to osseous metastasis from the pheochromocytoma has not been reported previously. The treatment of the malignant form of the tumor comprises controlling excess circulating catecholamines with adrenergic blocking agents alone or in combination with labetolol and/or metyrosine. Therapeutic embolization to reduce tumor load and catecholamine
production can be attempted [2]. Chemotherapy with cyclophosphamide, vincristine and dacarbazine have not shown persistent benefits [4]. Malignant change in pheochromocytoma heralds poor prognosis with a 5 year survival of 44% as against 96% for benign tumors PI. References Beard CM, Sheps SG. Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc 1983;58:802-804. Sheps SG, Jiang NS. Klee GG, Van Heerdan JAV. Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 1990;65:88-95. Poutasse EF, Gifford GH. Pheochromocytoma: diagnosis and treatment. Prog Cardiovasc Dis 1965;8:235-252. Auerbuch SD, Steakly CS, Young RC, Gelman EP, Goldstein DS, Stull R, Keisser HR. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine and dacarbazine. Ann Intern Med 1980:109:267-273. Van Heerdan JAV, Sheps SG, Hamberger 8, Sheedy PF II. Poston JG, ReMine WH. Pheochromocytoma: current status and changing trends. Surgery 1982:91:367-373.
International Journal of Cardiology, 34 (1992) 348-350 0 1992 Elsevier Science Publishers B.V. All rights reserved 0167.5273/92/$05.00
CARD10 14104
Successful treatment of a nondeflatable septostomy catheter
balloon atria1
Siiheyla 6zkutlu and Nazan 6zbarlas Hacettepe UniLlersityChild Health Institute, Department of Pediatric Cardiology, Ankara, Turkey
(Received 29 July 1991; revision accepted 27 September 1991)
We present a rare complication of balloon atrial septostomy in arteries and review the management of this problem. The balloon the atria1 septum. We punctured the balloon with a stylet which double-lumen septostomy catheter. We suggest that this method employed.
Key words: Balloon atria1 septostomy; Transposition
Correspondence
to: Dr. Siiheyla Gzkutlu, Department
an infant with complete transposition of the great could not be deflated after the eighth pass across was passed through the circulation lumen of the should once be tried before other methods are
of the great arteries
of Pediatric Cardiology, Hacettepe University, Ankara, Turkey.
346 Q 1992 Elsevier
CARD10
Science
Publishers
B.V. All rights reserved
0167-5273/92/$05,00
14103
Malignant pheochromocytoma Abhay K. Pande Abhay Heart Clinic. Thapar Nursing Home, Ramdaspeth Nagpur. India (Received
17 June 1991; revision
accepted
25 September
1991)
We present an interesting case of paroxysmal hypertension in a young male caused by malignant pheochromocytoma. This patient, who had history of paroxysms of abdominal pain with severe hypertension, developed osseous metastasis in the first lumbar vertebra resulting in collapse of the vertebra and it caused paraplegia. The diagnosis of pheochromocytoma was confirmed on histopathology. Key words:
Pheochromocytoma;
Malignant
change;
Osseous
Introduction Pheochromocytoma is an important cause of secondary hypertension which continues to evoke interest because it is capable of mimicking a variety of syndromes. We report an interesting case of malignant pheochromocytoma with unusual clinical presentation.
Case Report A 23-year-old male presented with rapidly progressive weakness in the lower limbs for 15 days and a history of severe abdominal pain associated with vomiting and hypertension, palpitations and flushing, and a palpable abdominal mass of 8 years’ duration. Initially, the frequency of the abdominal pain with hypertension was 1 to 2 times per year which increased to 6 to 8 times in the last 2 years with a rapid increase in the size of abdominal mass. There was no history of injury to the spine, of fever, or of tuberculosis. Examination revealed a thin nervous patient with a gibbus at the first lumbar vertebra and a large irregular mass, firm in consistency, palpable in the left hypochondrium and iliac regions. Cardiovascular exam-
Correspondence to: Dr. Abhay Pande, Cardiology University Hospital, 1211, Geneva, Switzerland.
Center,
metastasis:
Unusual
presentation
ination was normal. Central nervous system examination revealed paraplegia. There was no evidence of cafe-au-lait spots, neurofibromatosis, tuberous sclerosis, or Sturge-Weber disease. Abdominal palpation, performed as a provocative test, triggered a hypertensive crisis with a blood pressure of 270/160 mmHg which was controlled with sublingual nifedipine. A plain X-ray of the abdomen showed paravertebral calcification (Fig. 1A). The lumbar spine showed localized collapse of the first lumbar vertebra. Other disc spaces were normal (Fig. 1B). An intravenous pyelogram revealed an upwardly displaced left kidney with hydronephrotic changes. An abdominal ultrasound demonstrated a large infrarenal mass 20-25 cm in size in the left hypochondrium, lumbar and iliac regions. The patient was stabilized with oral phenoxybenzamine, labetolol, and liberal intake of salt and water. Exploratory laparotomy revealed a large highly vascular mass of irregular consistency adherent to the aorta and inferior caval vein. It was not possible to resect the mass. Surgery was well tolerated without any hypertensive or hypotensive crisis. The biopsy from the mass showed polyhydral polygonal cells with finely granular eosinophilic cytoplasm, vesicular nuclei, and surrounded by a rich network of capillaries (Fig. 2) and it confirmed diagnosis of malignant pheochromocytoma with metastasis to the first lumbar vertebra. He died 6 months later at his native place. Autopsy studies could not be performed.
Fig. 1. A. Plain X-ray of abdomen
showing calcification
in the tumor mass (arrow).
collapse of first lumbar vertebra
Fig. 2. Histopathology
of the tumor mass revealing
B. X-ray of dorsolumbar
spine showing localized
(arrow).
large polygonal polyhydral
cells characteristic
of pheochromocytoma
348 Discussion
The incidence of pheochromocytoma is 0.001% in the general population, and 0.5% in patients with hypertension [1,2]. Malignant change occurs in 3 to 14%, and it represents a particular diagnostic problem because of the potential for a multicentric origin and the absence of specific histologic criteria for malignant change [3]. The diagnosis is dependent on distant metastasis which occurs to lymph nodes, liver, bones, muscle, or lungs. The clinical presentation of pheochromocytoma is variable [2]. To the best of our knowledge a presentation with sudden onset paraplegia caused by collapse of the first lumbar vertebra due to osseous metastasis from the pheochromocytoma has not been reported previously. The treatment of the malignant form of the tumor comprises controlling excess circulating catecholamines with adrenergic blocking agents alone or in combination with labetolol and/or metyrosine. Therapeutic embolization to reduce tumor load and catecholamine
production can be attempted [2]. Chemotherapy with cyclophosphamide, vincristine and dacarbazine have not shown persistent benefits [4]. Malignant change in pheochromocytoma heralds poor prognosis with a 5 year survival of 44% as against 96% for benign tumors PI. References Beard CM, Sheps SG. Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc 1983;58:802-804. Sheps SG, Jiang NS. Klee GG, Van Heerdan JAV. Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 1990;65:88-95. Poutasse EF, Gifford GH. Pheochromocytoma: diagnosis and treatment. Prog Cardiovasc Dis 1965;8:235-252. Auerbuch SD, Steakly CS, Young RC, Gelman EP, Goldstein DS, Stull R, Keisser HR. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine and dacarbazine. Ann Intern Med 1980:109:267-273. Van Heerdan JAV, Sheps SG, Hamberger 8, Sheedy PF II. Poston JG, ReMine WH. Pheochromocytoma: current status and changing trends. Surgery 1982:91:367-373.
International Journal of Cardiology, 34 (1992) 348-350 0 1992 Elsevier Science Publishers B.V. All rights reserved 0167.5273/92/$05.00
CARD10 14104
Successful treatment of a nondeflatable septostomy catheter
balloon atria1
Siiheyla 6zkutlu and Nazan 6zbarlas Hacettepe UniLlersityChild Health Institute, Department of Pediatric Cardiology, Ankara, Turkey
(Received 29 July 1991; revision accepted 27 September 1991)
We present a rare complication of balloon atrial septostomy in arteries and review the management of this problem. The balloon the atria1 septum. We punctured the balloon with a stylet which double-lumen septostomy catheter. We suggest that this method employed.
Key words: Balloon atria1 septostomy; Transposition
Correspondence
to: Dr. Siiheyla Gzkutlu, Department
an infant with complete transposition of the great could not be deflated after the eighth pass across was passed through the circulation lumen of the should once be tried before other methods are
of the great arteries
of Pediatric Cardiology, Hacettepe University, Ankara, Turkey.
