National Cancer Institute®
Last Modified: November 21, 2001
UI - 21281567
AU - Jacobs JM; Hawes MJ
TI - From eyelid bumps to thyroid lumps: report of a MEN type IIb family and review of the literature.
SO - Ophthal Plast Reconstr Surg 2001 May;17(3):195-201
AD - Department of Ophthalmology, University of Colorado Health Sciences Center, Denver, USA.
PURPOSE: We present a two-generation family with multiple endocrine neoplasia (MEN) type IIb diagnosed by their ophthalmologists based on characteristic ophthalmic findings. METHODS: A family consisting of a 33-year-old female proband and her 8- and 7-year-old children had prominent corneal nerves; eyelid, lip, and tongue nodules; and a characteristic facies. A polymerase chain reaction-based genetic assay was obtained to detect the genetic mutation most commonly associated with MEN type IIb. Serum calcitonin and urine catecholamine studies were obtained. RESULTS: Molecular genetic studies detected in all 3 patients a mutation at codon 918 of the RET proto-oncogene known to be present in 95% of the cases of MEN type IIb. Serum calcitonin was elevated in the proband and her son. Urine catecholamine levels were elevated in the proband. Surgical treatment and histologic analysis confirmed pheochromocytoma and medullary thyroid carcinoma (MTC) in the proband. Surgical exploration revealed the MTC to be metastatic to the liver. CONCLUSIONS: This family demonstrates the characteristic findings of MEN type IIb: prominent corneal nerves in a clear stroma and multiple submucosal neuromas of the conjunctiva, eyelids, lips, and tongue. Ophthalmologists have a critical role to play in recognizing these signs, because the early diagnosis of medullary thyroid carcinoma and pheochromocytoma may be life saving.
UI - 21367648
AU - Winter C; Schmidt-Mutter C; Cuny R; Soulas S; Dupeyron JP; Steib A
TI - Fatal form of phaeochromocytoma presenting as acute pyelonephritis.
SO - Eur J Anaesthesiol 2001 Aug;18(8):548-53
AD - Department of Anaesthesiology, Hopital Civil, Hopitaux Universitaires, 67091 Strasbourg Cedex, France.
We report the case of a young man who presented with a clinical picture of acute pyelonephritis. Within 3 h of admission, the patient developed acute respiratory distress associated with tachycardia and shock, and he was transferred to the intensive care unit. Mechanical ventilation of the lungs and symptomatic treatment were started immediately. Abdominal ultrasound revealed the presence of an adrenal tumour with central necrosis indicating a probable phaeochromocytoma. There was no sign of pyelonephritis. Ventricular fibrillation followed by asystole occurred soon after admission. The suddenness of the patient's death did not allow time for further investigation and therapy. The severity of the clinical signs was probably related to a massive release of catecholamines because of necrosis of the tumour, which may have been worsened by the diagnostic procedures performed to investigate the clinical symptoms and signs of acute pyelonephritis.
UI - 21457581
AU - Bullough AS; Watters M
TI - Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
UI - 21457582
AU - Fawcett WJ; Edkins CL
TI - Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
UI - 20383129
AU - Prys-Roberts C
TI - Phaeochromocytoma--recent progress in its management.
SO - Br J Anaesth 2000 Jul;85(1):44-57
AD - Sir Humphry Davy Department of Anaesthesia, Bristol Royal Infirmary, UK.
UI - 21268711
AU - Yeh CN; Jeng LB; Chen MF; Hung CF
TI - Nonfunctioning malignant pheochromocytoma associated with dermatomyositis: case report and literature review.
SO - World J Urol 2001 Apr;19(2):148-50
AD - Department of Surgery, Chang Gung Memorial Hospital, 5, Fu-Hsing Street, Kwei-Shan, Taoyuan, Taiwan.
A 42-year-old man with dermatomyositis presented with right-upper-quadrant dull pain and normal blood pressure for 10 days. Abdominal ultrasonography, abdominal computed tomography, and angiography revealed a retroperitoneal tumor with direct invasion to the liver and the diaphragm. The diagnosis of nonfunctioning malignant pheochromocytoma was made on the basis of clinical evidence. The tumor was removed en bloc with part of the diaphragm, the right lobe of the liver, the right adrenal gland, and the right kidney. Pathologic examination with immunohistochemical staining revealed a malignant pheochromocytoma growing exophytically from the right adrenal gland and invading the right lobe of the liver and the diaphragm. The postoperative course has been uneventful and no recurrence has been noted over a 6-month follow-up period.
UI - 21434076
AU - Wu KD; Chen YM; Chu TS; Chueh SC; Wu MH; Bor-Shen H
TI - Expression and localization of human dopamine D2 and D4 receptor mRNA in the adrenal gland, aldosterone-producing adenoma, and pheochromocytoma.
SO - J Clin Endocrinol Metab 2001 Sep;86(9):4460-7
AD - Department of Internal Medicine, National Taiwan University Hospital, Taipei 100, Taiwan. firstname.lastname@example.org
Aldosterone secretion is evidently regulated by a dopaminergic inhibitory mechanism. Pharmacological characterization and autoradiographic studies revealed D2-like receptors in the adrenal cortex, especially in the zona glomerulosa. However, the subtype of the dopamine receptors involving this regulation has not been elucidated. To investigate which subtype of receptors expresses in the adrenal cortex, we examined the messages of D2-like receptors, D2, D3, and D4, by RT-PCR and in situ hybridization of adrenal glands and adrenal neoplasm. Both D2 and D4 receptors were expressed in normal adrenal glands, pheochromocytoma, and aldosterone-producing adenoma. However, the D2 receptors were not universally expressed, in contrast with the D4 receptors that were detected in all cases of aldosterone-producing adenoma and adrenal remnant. No D3 receptor message was detected by RT-PCR in any adrenal sample. Both D2 and D4 receptors were expressed in significant amounts in the adrenal medulla and pheochromocytoma. In the adrenal cortex, the expression of the D2 receptors was in the zona glomerulosa and zona reticularis, with no different signal intensities between the two zones. D4 receptors were mainly localized in the zona glomerulosa and, to a lesser extent, in the zona reticularis. Both receptors were expressed at low levels in the zona fasciculata. In aldosterone-producing adenoma, the expression of D2 and D4 was especially found in nonzona fasciculata-like cells. To elucidate which dopamine receptor regulates aldosterone secretion, the effects of specific D2 and D4 antagonists, raclopride and clozapine, respectively, were examined in cultured NCI-H295 cells. Dopamine further increased angiotensin II-induced aldosterone secretion by 20%. In the presence of 1 microM dopamine and angiotensin II, 10(-5)-10(-7) M clozapine decreased aldosterone levels by 40-55%. The decrease in aldosterone secretion by clozapine was completely reversed when raclopride was added simultaneously. These data suggest that dopamine exerts dual effects on aldosterone secretion in NCI-H295 cells. Activation of D4 receptors can increase aldosterone secretion, whereas an inhibitory effect is mediated via D2 receptors. In summary, we demonstrated the existence of both D2 and D4 receptors in the human adrenal gland and adrenal neoplasm. Both receptors play significant roles in the modulation of aldosterone secretion, but in opposite directions.
UI - 21459805
AU - Giarelli E
TI - Ethical issues in genetic testing. The experiences of one family diagnosed with an inherited cancer syndrome.
SO - J Infus Nurs 2001 Sep-Oct;24(5):301-10
AD - University of Pennsylvania School of Nursing, 420 Guardian Drive, Philadelphia, PA 19104, USA. IMLNG@aol.com
This article describes selected ethical issues faced by members of a family with genetic predisposition to the cancer syndrome multiple endocrine neoplasia type 2a. The bioethical principles of autonomy, nonmaleficence, beneficence, and justice are the moral guides for nurses and other health professionals, who may apply them to help patients, family members and peers resolve ethical issues and moral dilemmas. Sometimes, however, issues that are morally problematic cannot be settled by simply referring to standards of practice and bioethical norms. In these cases all stakeholders must struggle together to resolve the conflict.
UI - 21290106
AU - Rizzo S; Bonomo S; Moser A; Bottura D; Castellini C; Mazzola F; Lauro E;
TI - Vicenzi L; Betresini B; Angeli G; Brazzarola P; D'Azzo G; Rosa G [Bilateral pheochromocytoma associated with duodeno-jejunal GIST in patient with von Recklinghausen disease: report of a clinical case]
SO - Chir Ital 2001 Jan-Feb;53(2):243-6
AD - Dipartimento di Scienze Chirurgiche e Gastroenterologiche Cattedra di Semeiotica e Metodologia Chirurgica, Universita degli Studi di Verona.
The authors present the case of a 60-year-old male patient suffering from von Recklinghausen's disease (neurofibromatosis type I, NF1) with bilateral pheochromocytoma and occasional intraoperative reports of duodenojejunal GIST (GastroIntestinal Stromal Tumour). Through a review of the literature the authors analyze the frequency and the features of bilateral pheochromocytoma and its rare histological variant, the so-called composite pheochromocytoma, characterized by the combination of pheochromocytoma and ganglioneuroma or ganglioneuro-blastoma. Bilaterality of pheochromocytoma is more frequent in patients with familiarity for pheochromocytoma without NF1. Composite pheochromocytoma accounts for about 3% of total pheochromocytomas. In addition, the authors summarize the present knowledge about gastrointestinal stromal tumours and investigate the possible association between them and NF1 or pheochromocytoma, concluding that any such association is purely casual, while confirming the well known, genetically determined association between NF1 and pheochromocytoma.
UI - 21437013
AU - Takami H; Ikeda Y; Takayama J; Sasaki Y; Kan S; Niimi M; Inada E;
TI - Kameyama K Adrenal-sparing adrenalectomy in hereditary bilateral phaeochromocytoma.
SO - ANZ J Surg 2001 Oct;71(10):623-4
AD - First Department of Surgery and Department of Anesthesiology, Teikyo University School of Medicine and Division of Diagnostic Pathology, Keio University Hospital, Tokyo, Japan. email@example.com
UI - 21448255
AU - Pautler SE; Pavlovich CP; Mikityansky I; Drachenberg DE; Choyke PL;
TI - Linehan WM; Wood BJ; Walther MM Retroperitoneoscopic-guided radiofrequency ablation of renal tumors.
SO - Can J Urol 2001 Aug;8(4):1330-3
AD - Urologic Oncology Branch, National Cancer Institute and the Diagnostic Radiology Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892, USA.
OBJECTIVE: Minimally invasive approaches to the management of renal tumors are being studied intensively in urology. Herein, we describe the use of multiple organ-sparing techniques for the management of tumors in a patient with von Hippel Lindau disease (VHL). MATERIALS AND METHODS: A 42 year-old woman with VHL underwent a right partial adrenalectomy and a left renal radiofrequency ablation (RFA) of two renal tumors. RESULTS: A 2.2 cm solitary right adrenal pheochromocytoma was resected using a transperitoneal approach. A retroperitoneal approach to the left kidney was performed and RFA of the two renal tumors completed using sonographic guidance. On the 5-month follow-up CT scan, there was no evidence of residual adrenal tumors and both renal lesions lacked contrast enhancement. No complications occurred during the post-operative recovery. CONCLUSIONS: Multiple organ-ablative laparoscopic procedures may be performed in a single sitting. Laparoscopic partial adrenalectomy is an effective technique in patients with bilateral tumors or a familial syndrome predisposing to multiple adrenal tumors. Further study of renal RFA is required to assess the long-term durability of the procedure.
UI - 21464051
AU - Minei S; Yamashita H; Koh H; Satoh T; Kobayashi S; Furuhata M; Uchida T;
TI - Baba S [Giant cystic pheochromocytoma: a case report]
SO - Hinyokika Kiyo 2001 Aug;47(8):561-3
AD - Department of Urology, Kitasato University School of Medicine.
The patient, a 59-year-old woman, was referred to our University Hospital for evaluation of a left mass. Ultrasonography revealed a left adrenal cystic mass. On excretory urograms, the left kidney was pressed downward by a suprarenal mass, and computerized tomography (CT) and magnetic resonance imaging (MRI) confirmed an adrenal cyst. 131I-meta-iodo-benzylguanidine (MIBG) scintigraphy showed prominent accumulation in the left adrenal mass and the capsule. Considering the elevation of catecholamines in both blood and urine samples, we performed a left adrenalectomy with a presumptive diagnosis of pheochromocytoma (tumor size: 11.6 x 7.5 x 6.5 cm, tumor weight 720 g). The subsequent pathological examination confirmed a left giant cystic pheochromocytoma. 131I-MIBG scintigraphy was the most useful tool in the diagnosis of the cystic pheochromocytoma.
UI - 21486587
AU - Mazzocchi G; Malendowicz LK; Aragona F; Rebuffat P; Gottardo L;
TI - Nussdorfer GG Human pheochromocytomas express orexin receptor type 2 gene and display an in vitro secretory response to orexins A and B.
SO - J Clin Endocrinol Metab 2001 Oct;86(10):4818-21
AD - Department of Human Anatomy and Physiology, Section of Anatomy, University of Padua, Via Gabelli 65, I-35121 Padua, Italy. firstname.lastname@example.org
Orexins A and B are hypothalamic peptides, that act through two receptor subtypes, called OX1-R and OX2-R. OX1-R selectively binds orexin A, whereas OX2-R is nonselective for both orexins. High levels of OX1-R mRNA and low levels of OX2-R mRNA have been previously detected in the human adrenal cortex and medulla. Here we demonstrated by RT-PCR the expression of the OX2-R, but not the OX1-R, gene in 10 benign secreting pheochromocytomas. Both orexins A and B stimulated catecholamine secretion from pheochromocytoma slices; the maximal effective concentration was 10(-8) mol/liter. Orexins A and B (10(-8) mol/liter) increased IP3, but not cAMP production, by tumor slices, and the effect was blocked by the PLC inhibitor U-73122. The catecholamine response to 10(-8) mol/liter orexins A and B was abolished by either U-73122 or the PKC antagonist calphostin C and was unaffected by the adenylate cyclase inhibitor SQ-22536 and the PKA inhibitor H-89. Collectively, these findings suggest that orexins stimulate catecholamine secretion from human pheochromocytomas, acting through OX2-R coupled to the PLC-PKC signaling pathway.
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