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NCI CANCERLIT^® Search: Pheochromocytoma - September 2001

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Last Modified: November 1, 2001

• [Hemodynamic instability and paraplegia after thoracotomy for excision of a cardiac pheochromocytoma]

• [Pigmented pheochromocytoma. Case report with immunohistochemical and electron microscopic characterization]

• Factitious pheochromocytoma-like hypertensive bouts caused by the valsalva maneuver.

• A "pheo" lurks: novel approaches for locating occult pheochromocytoma.

• A new variant of Carney's triad: phaeochromocytoma and chondrosarcoma.

• Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA. A case report.

• [Low dose CVD chemotherapy as a tumor dormancy therapy for extra-adrenal malignant pheochromocytoma: a case report]

• Pheochromocytoma coexisting with renal artery lesions.

• Re: Pheochromocytoma coexisting with renal artery lesions.

• [Unusual form of adrenal pheochromocytoma: asymptomatic cystic mass]

• Pheochromocytoma presenting after cardiac transplantation for dilated cardiomyopathy.

• Intestinal pseudoobstruction as a presenting feature of a pheochromocytoma.

• Pheochromocytoma during pregnancy: a case report.

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1
UI - 21364959
AU - Decoene C; Tavernier B; Jegou B; Pol A; Proye C
TI - [Hemodynamic instability and paraplegia after thoracotomy for excision of a cardiac pheochromocytoma]
SO - Ann Fr Anesth Reanim 2001 Jun;20(6):567-9
AD - Service d'anesthesie-reanimation en cardiologie, CHRU, 59037 Lille, France. c-decoene@chru-lille.fr
We report the occurrence of a sustained hypotension (vasoplegia) following thoracic phaeochromocytoma surgery. Diagnosis of spinal cord injury was done by magnetic nuclear resonance (MNR) showing surgical "Horsley wax" inside the vertebral canal and ischaemic signal inside the anterior part of the spine cord. Removal of "Horsley wax" dramatically improved arterial blood pressure but did not correct all neurologic disorders. Haemodynamic disorders related to spinal cord injury are rare after thoracotomy, and may have been enhanced by the haemodynamic instability typically associated with phaeochromocytoma surgery. Early diagnosis has to be done by MNR.

2
UI - 21384301
AU - Langner C; Hoffmann JG; de Geeter P; Rompel R; Ruschoff J
TI - [Pigmented pheochromocytoma. Case report with immunohistochemical and electron microscopic characterization]
SO - Pathologe 2001 Jul;22(4):276-80
AD - Institut fur Pathologie, Karl-Franzenz-Universitat Graz. Auenbruggerplatz 25, 8036 Graz, Osterreich. cord.langner@kfunigraz.ac.at
We present a case of pigmented adrenal paraganglioma in a 39-year-old female patient with associated neurofibromatosis type 1 (NF1). Histology showed features typical for phaeochromocytomas except for varying amounts of brown pigment within the cytoplasm of tumour cells, which proved to be melanin by histochemical and ultrastructural analysis. The occurrence of melanin is believed to reflect the origin of this neoplasm from multipotent cells of the neural crest. Pigmented phaeochromocytoma has to be taken in consideration in the differential diagnosis of pigmented neoplasms, especially in the adrenal gland, where it has to be discriminated from pigmented cortical adenoma (so-called black adenoma) and primary malignant melanoma.

3
UI - 21402305
AU - Boon D; Kraaijenhagen RA; van Montfrans GA
TI - Factitious pheochromocytoma-like hypertensive bouts caused by the valsalva maneuver.
SO - Ann Intern Med 2001 Aug 21;135(4):304-5

4
UI - 21394080
AU - Pacak K; Goldstein DS; Doppman JL; Shulkin BL; Udelsman R; Eisenhofer G
TI - A "pheo" lurks: novel approaches for locating occult pheochromocytoma.
SO - J Clin Endocrinol Metab 2001 Aug;86(8):3641-6
AD - Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1583, USA. karel@mail.nih.gov
Most, but not all, pheochromocytomas can be localized by computed tomography or magnetic resonance imaging. Here we introduce two novel approaches for localization of pheochromocytoma in a patient in whom conventional imaging modalities failed to show the tumor. First, we establish that measurements of plasma free metanephrines coupled with vena caval sampling are useful for localizing occult pheochromocytoma, particularly when elevations in plasma catecholamines are slight or intermittent. Second, we show that positron emission tomographic scanning using the imaging agent 6-[18F]fluorodopamine as a substrate for the norepinephrine transporter offers a highly effective method for tumor localization. These novel approaches may be of value in difficult cases, where biochemical and clinical evidence of pheochromocytoma is compelling, yet conventional imaging modalities fail to locate the tumor.

5
UI - 21364021
AU - Wahid ST; Jones R; Chawla SL; Connolly VM; Kelly WF; Bilous RW
TI - A new variant of Carney's triad: phaeochromocytoma and chondrosarcoma.
SO - Postgrad Med J 2001 Aug;77(910):527-8
AD - South Tees Acute Hospitals NHS Trust, Middlesbrough, UK. s.wahid@virgin.net
A 61 year old hypertensive woman presented in 1986 with a right scapular chondrosarcoma. She developed type 1 diabetes mellitus in 1991 and suffered a stroke in 1991. Chest radiography showed pulmonary metastases in 1997. Further radiological staging detected a right sided phaeochromocytoma, which was subsequently removed in 1998. Before this, repeated urine estimations of vanillylmandelic acid had been normal. Her diabetes was cured by adrenalectomy. It is believed that the combination of phaeochromocytoma and extrapulmonary chondrosarcoma represents a new variant of Carney's triad.

6
UI - 21254130
AU - Tewari KS; Steiger RM; Lam ML; Rutgers JK; Berkson RA; DiSaia PJ
TI - Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA. A case report.
SO - J Reprod Med 2001 Apr;46(4):385-8
AD - Divisions of Gynecologic Oncology and Maternal-Fetal Medicine and Department of Obstetrics and Gynecology, University of California, Irvine, Medical Center, Orange, 101 The City Drive, Orange, CA 92868, USA.
BACKGROUND: Multiple endocrine neoplasia syndrome type IIA (MEN IIA) has rarely been encountered in pregnancy. CASE: A 22-year-old, nulliparous woman developed bilateral pheochromocytomas during pregnancy. This finding aroused suspicion for MEN IIA, and close endocrinologic follow-up was arranged. Four years later, hyperparathyroidism developed, and the diagnosis was established. The patient underwent prophylactic total thyroidectomy with parathyroid exploration. CONCLUSION: This was the first case of MEN IIA in pregnancy in which the diagnosis was established prior to the development of medullary thyroid cancer, thereby allowing prophylactic thyroidectomy. The presence of bilateral neoplastic disease in young patients may be indicative of a hereditary predisposition to malignancy.

7
UI - 21409381
AU - Maezawa T; Yonese J; Tsukamoto T; Isii N; Hasegawa Y; Ishikawa Y; Fukui I
TI - [Low dose CVD chemotherapy as a tumor dormancy therapy for extra-adrenal malignant pheochromocytoma: a case report]
SO - Nippon Hinyokika Gakkai Zasshi 2001 Jul;92(5):593-6
AD - Department of Urology, Cancer Institute Hospital, Tokyo, Japan.
A 38 year-old man presented with upper abdominal mass and hypertension pointed out at a medical examination. Blood pressure was 170/90 under medication of an alpha-blocker. Abdominal CT scan showed an 8 x 8 cm inter-aortocaval mass displacing pancreas head ventrally, and further a 4 x 4 cm mass at the aortic bifurcation, but there was no tumorous lesion in bilateral adrenal glands. Plasma nor-epinephrine level and urinary VMA excretion were excessive but plasma adrenaline level was within normal limits. MIBG scintigram showed hot spots in the 4th and 9th thoracic vertebrae. The destructive change of the 9th vertebra on magnetic resonance imaging strongly suggested metastasis of the tumor. Histologic and immunohistochemical findings of the biopsy specimen taken from the lower abdominal tumor in addition to the above clinical data led to the diagnosis of extra-adrenal malignant pheochromocytoma with spinal metastases. Since 2 cycles of full dose CYVADIC chemotherapy had no effects on lowering the high blood pressure and reducing the tumor size, low dose (60% of the full dose) CVD (cyclophosphamide, vincristine and dacarbazine) was given as a palliative chemotherapy on an out-patient clinic approximately every 4 weeks. After 4 cycles of the chemotherapy, his backache due to spinal metastasis markedly improved, hypertension as well as the plasma dopamine level was normalized and nor-epinephrine level was markedly decreased, though the tumor size was not reduced. Thereafter, no medication for hypertension was necessary. During 3 years and 6 months until now, 36 cycles of the chemotherapy has been repeated with no significant side effects. He has been at full-time work with quality of life being well preserved. Low dose CVD regimen appears to be an effective tumor dormancy therapy for advanced extra-adrenal pheochromocytoma.

8
UI - 20353495
AU - Gill IS; Meraney AM; Bravo EL; Novick AC
TI - Pheochromocytoma coexisting with renal artery lesions.
SO - J Urol 2000 Aug;164(2):296-301
AD - Section of Laparoscopic and Minimally Invasive Surgery, and Departments of Urology and Nephrology/Hypertension, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
PURPOSE: Physiologically significant renal artery lesions in the presence of a pheochromocytoma comprise a confounding factor which may impact on the hypertension cure following excision of the pheochromocytoma. We present 10 cases of these dual lesions and review the literature on this entity. MATERIALS AND METHODS: From 1952 to 1999, 269 patients were diagnosed with pheochromocytoma at our institution. Hospital charts of these patients were reviewed retrospectively to identify those with coexisting renal artery stenosis. A Medline search was performed to review the available literature. RESULTS: Of the 269 patients with pheochromocytoma 10 (3. 7%) had coexisting renal artery lesions, including renal artery stenosis in 8, renal artery aneurysm in 1 and postangiographic dissection occlusion in 1. Pheochromocytoma was adrenal in 8 cases and ectopic in 2. Of the patients 9 have been treated to date by adrenalectomy in 4, nephroadrenalectomy in 3, adrenalectomy plus lysis of renal artery adhesions in 1 and adrenalectomy plus renal autotransplantation with bench repair in 1. Both lesions were diagnosed preoperatively in 9 cases and a hypovascular adrenal lesion was missed preoperatively in 1. A review of literature revealed a total of 87 cases of coexisting pheochromocytoma and renal artery lesions. CONCLUSIONS: There are multiple mechanisms that can lead to renal artery stenosis and pheochromocytoma. A high index of suspicion is necessary to enable both entities to be diagnosed preoperatively and allow proper planning of surgical therapy. Incomplete diagnosis may lead to persistent hypertension postoperatively.

9
UI - 21265326
AU - Walther MM; Choyke PL
TI - Re: Pheochromocytoma coexisting with renal artery lesions.
SO - J Urol 2001 Jun;165(6 Pt 1):2005-6

10
UI - 21294366
AU - Tazi K; Elmalki HO; Ei Fassi MJ; Koutani A; Hachimi M; Lakrissa A
TI - [Unusual form of adrenal pheochromocytoma: asymptomatic cystic mass]
SO - Prog Urol 2001 Apr;11(2):293-5
AD - Service d'Urologie B, CHU Ibn Sina, Rabat, Maroc. tazikarim@yahoo.com
We report an adrenal cyst discovered in a 26-year-old woman with abdominal pain. Imaging (ultrasonography, computed tomography) demonstrated a 12 cm adrenal cystic mass suggestive of hydatic cyst of adrenal. She undergoes surgical exploration, when blood pressure increases. Pheochromocytoma diagnosis is reconsidered. Cystic pheochromocytoma is a rare tumour of the adrenal gland that can pose a diagnostic challenge.

11
UI - 21342149
AU - Dalby MC; Burke M; Radley-Smith R; Banner NR
TI - Pheochromocytoma presenting after cardiac transplantation for dilated cardiomyopathy.
SO - J Heart Lung Transplant 2001 Jul;20(7):773-5
AD - Department of Transplant Medicine, Middlesex, United Kingdom.
Pheochromocytoma may present with a clinical picture indistinguishable from that of idiopathic dilated cardiomyopathy. We report 2 such patients who underwent cardiac transplantation following which we diagnosed and successfully treated pheochromocytoma.

12
UI - 21392119
AU - Salazar A; Naik A; Rolston DD
TI - Intestinal pseudoobstruction as a presenting feature of a pheochromocytoma.
SO - J Clin Gastroenterol 2001 Sep;33(3):253-4

13
UI - 21410967
AU - Norton KS; Girod W; Johnson LW
TI - Pheochromocytoma during pregnancy: a case report.
SO - J La State Med Soc 2001 Jul;153(7):354-7
AD - Louisiana Health Sciences Center, Shreveport, Louisiana, USA.