• [Problems associated with general anesthesia in patients with thyroid cancer]

• Malignant pheochromocytoma with cutaneous metastases presenting with hemolytic anemia and pyrexia of unknown origin.

• Changing pattern of adrenalectomy at a tertiary referral centre 1970-2000.

• Laparoscopic adrenalectomy: a 6-year experience of 59 cases.

• Asymptomatic giant pheochromocytoma.

• Reversibility of catecholamine-induced cardiomyopathy in a woman with pheochromocytoma.

• Myocardial lesions in pheochromocytoma.

• [Malign pheochromocytoma behaving as a benign tumor]

• Intraoperative ultrasound aids in dissection during laparoscopic partial adrenalectomy.

• Congenital hemihypertrophy and pheochromocytoma, not a coincidental combination?

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

• Expression of PC2 and PC1/PC3 in human pheochromocytomas.

• Co-expression of PC2 and proenkephalin in human tumoral adrenal medullary tissues.

• Images in cardiovascular medicine. Pheochromocytoma of the urinary bladder.

• Cortical-sparing laparoscopic adrenalectomy in a patient with multiple endocrine neoplasia type IIA.

• Prognostic value of immunohistochemical expression of topoisomerase alpha II, MIB-1, p53, E-cadherin, retinoblastoma gene protein product,

• Calretinin and inhibin are useful in separating adrenocortical neoplasms from pheochromocytomas.

• Functional consequences of a SDHB gene mutation in an apparently sporadic pheochromocytoma.

1
UI - 12182031
AU - Wozniakowska W; Maka B; Laskowska M; Basek E
TI - [Problems associated with general anesthesia in patients with thyroid cancer]
SO - Wiad Lek 2001;54 Suppl 1():234-40
AD - Kliniki Chirurgii Onkologicznej, Centrum Onkologii-Instytut im. M. Sklodowskiej-Curie, Oddzial w Gliwicach.
The group of 100 patients with thyroid cancer operated in Institute of Oncology in Gliwice was described. The risk of anaesthesia, intraoperative complications and the results obtained depend on the preoperative preparation, especially on adequate treatment of endocrine disorders of thyroid, parathyroid and adrenal glands. It is necessary to estimate the presence of multiendocrine disorders in medullary thyroid carcinoma, especially of those with pheochromocytoma, because of its high mortality in the non alfa-blockers prepared patients.

2
UI - 12186139
AU - Srinivasan R; Kini U; Babu MK; Jayaseelan E; Pradeep R
TI - Malignant pheochromocytoma with cutaneous metastases presenting with hemolytic anemia and pyrexia of unknown origin.
SO - J Assoc Physicians India 2002 May;50(5):731-3
AD - Department of Pathology, Dermatology and General Medicine, St John's Medical College and Hospital, Bangalore, India.
We describe a 43 year old female who presented with pyrexia of unknown origin associated with Coomb's negative hemolytic anemia and impaired liver function tests of six months duration. A routine abdominal computerised tomographic scan showed a mass in the left adrenal which was excised at laparotomy and histologically diagnosed as pheochromocytoma. The hemolysis continued to worsen with development of resistance to steroid therapy. Subsequently she developed multiple firm to hard painless cutaneous nodules which were aspirated for cytologic examination. The cytology picture was that of a neuroendocrine tumour with cell morphology similar to that of the adrenal pheochromocytoma. Such an association of hemolytic anemia, pyrexia and cutaneous metastases in a case of malignant pheochromocytoma has not been described earlier in the medical literature.

3
UI - 12123500
AU - Sidhu S; Bambach C; Pillinger S; Reeve T; Stokes G; Robinson B;
TI - Delbridge L Changing pattern of adrenalectomy at a tertiary referral centre 1970-2000.
SO - ANZ J Surg 2002 Jul;72(7):463-6
AD - Endocrine Surgical Unit, Royal North Shore Hospital, St Leonards, New South Wales, Australia. sidhu@med.usyd.edu.au
BACKGROUND: In 1987, a report from this unit described the changing indications for open adrenalectomy over a 15-year period. The indications for adrenalectomy had switched from it being the principal therapeutic procedure used in advanced breast cancer in the early 1970s, to being predominately performed for Cushing's disease or incidental, asymptomatic, adrenal masses by the early 1980s. The aim of the present study was to evaluate the changes in the presentation and management of adrenal disease in the last 15 years and to compare these findings with our previously published results. METHODS: Information was gathered from a prospective database of all patients undergoing adrenalectomy in the University of Sydney Endocrine Surgical Unit at Royal North Shore obtained on patient presentation, diagnostic investigations, indications for surgery, procedure performed and surgical outcomes. Prior to 1987, information was gathered by retrospective review of case notes of patients who had undergone adrenalectomy at Royal North Shore Hospital. underwent adrenalectomy. Excluding the 68 adrenalectomies performed for breast cancer, left 168 patients who underwent adrenalectomy for functional or non--functional masses. There were 97 (58%) women and 71 (42%) men, with a mean age of 48 years. RESULTS: Of the 168 patients, the principal indications for surgery were hyperaldosteronism (32%), phaeochromocytoma (20%), hypercortisolism (20%), incidentaloma (16%), carcinoma (6%) and other reasons (6%). Examination of the number of cases in each pathological group for the periods 1970-1986 and 1987-2000, revealed an 8-fold increase in the number of operations for hyper-aldosteronism, and a 3-fold increase in cases of phaeochromocytoma. The number of operations for the other pathological groups remained steady. The annual incidence of adrenalectomy in the hospital has steadily risen since 1990, with a linear increase in the adrenalectomy rate since the introduction of laparoscopic adrenalectomy in 1995. There were fewer complications in either the open or laparoscopic group since 1987 compared with the pre-1987 cohort. CONCLUSIONS: In the past 5 years, there has been a linear increase in the number of adrenalectomies performed in this unit for hyperaldosteronism and to a lesser extent phaeochromocytoma. This is a reflection of increased clinical awareness, improved diagnostic modalities and the advent of laparoscopic adrenalectomy.

4
UI - 12123501
AU - Pillinger SH; Bambach CP; Sidhu S
TI - Laparoscopic adrenalectomy: a 6-year experience of 59 cases.
SO - ANZ J Surg 2002 Jul;72(7):467-70
AD - Endocrine Surgical Unit, Royal North Shore Hospital, St Leonards, New South Wales, Australia.
BACKGROUND: The aims of this paper were to review our experience with laparoscopic transperitoneal adrenalectomy, report on outcomes in comparison with the published literature, and demonstrate any learning curve with the technique. METHODS: A review of our database and patient Patient demographics, tumour characteristics, operating times, outcomes and lengths of stay were studied. Diagnostic tools, including a comparison between tumour size on computed tomography scanning and on pathological section were reviewed. A comprehensive literature review was conducted using MEDLINE. RESULTS: Indications for surgery included 33 patients with primary hyperaldosteronism (29 adenomas, 4 hyperplasias), 12 phaeochromo-cytomas, 7 cortisol-secreting adenomas, 4 non-secreting adenomas, 1 medullary cyst, 1 metastasis and 1 ganglioneuroma. The tumours ranged in size from 7 to 110 mm. All tumours were localized and lateralized preoperatively using standard techniques. Throughout the review period, six open procedures were undertaken electively, for various reasons. Three cases were converted to open procedures (5.1%). Thirty-eight left and 21 right procedures were undertaken. For all laparoscopic procedures, the average time in the operating theatre was 175.1 min. Men took 188.3 min compared with 165.7 min for women. Left-sided lesions took 178.72 min compared with 167.63 min for right-sided lesions. The average length of stay was 3.8 days. There was one wound infection, one blood transfusion and two readmissions: one for pain control in a patient with difficult home circumstances, and one patient suffered transient hypoadrenalism. CONCLUSION: The results achieved in our initial experience with this technique are comparable with the published literature. The results confirm that laparoscopic adrenalectomy is the method of choice for resection of benign adrenal pathology. The procedure has a learning curve and should be performed by a surgeon experienced in both open and laparoscopic adrenal surgery.

5
UI - 12240860
AU - Jain SK; Agarwal N
TI - Asymptomatic giant pheochromocytoma.
SO - J Assoc Physicians India 2002 Jun;50():842-4
AD - Department of Medicine, Dr. Ram Manohar Lohia Hospital, New Delhi.
An interesting case of pheochromocytoma is being presented which remained asymptomatic despite attaining a size of 16 x 11 cm. On intensive investigations, it was found to be a dopamine secreting tumour with certain features in favour of malignancy. The case was successfully operated and advised regular follow up.

6
UI - 2634991
AU - Sadowski D; Cujec B; McMeekin JD; Wilson TW
TI - Reversibility of catecholamine-induced cardiomyopathy in a woman with pheochromocytoma.
SO - CMAJ 1989 Nov 1;141(9):823-4
AD - Department of Medicine, University Hospital, Saskatoon, Sask.

7
UI - 2295041
AU - Silver MD
TI - Myocardial lesions in pheochromocytoma.
SO - CMAJ 1990 Jan 15;142(2):99-100

8
UI - 12183918
AU - Jarvelainen H; Viikari J
TI - [Malign pheochromocytoma behaving as a benign tumor]
SO - Duodecim 2001;117(20):2045-50
AD - Hope Heart Institute, 1124 Columbia Street, Floor 7, Seattle, WA 98104, USA. hannu.jarvelainen@utu.fi

9
UI - 12352391
AU - Pautler SE; Choyke PL; Pavlovich CP; Daryanani K; Walther MM
TI - Intraoperative ultrasound aids in dissection during laparoscopic partial adrenalectomy.
SO - J Urol 2002 Oct;168(4 Pt 1):1352-5
AD - Urological Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
PURPOSE: Adrenal cortical sparing surgery is a relatively new approach to adrenal tumors. Laparoscopic partial nephrectomy is a technically feasible but challenging operation. We describe the use of intraoperative ultrasound to facilitate laparoscopic partial nephrectomy in a population with a hereditary predisposition to multifocal pheochromocytoma. MATERIALS AND METHODS: All patients underwent a history, physical examination, serum and urine catecholamine determinations, abdominal computerized tomography-magnetic resonance imaging and metaiodobenzylguanidine scan. The adrenal gland was exposed using a standard 3 or 4 port approach. Intraoperative ultrasound was performed using a 7.5 MHz. 10 mm. transducer placed through a 12 mm. port. After imaging the whole gland and adjacent structures partial adrenalectomy was performed based on intraoperative ultrasound images using a harmonic scalpel or alternatively using a cut and sew technique that provided a 5 mm. margin. Tumors were removed intact and sent for pathological examination. RESULTS: Since 1998, 11 patients have undergone laparoscopic partial adrenalectomy. Intraoperative ultrasound was performed in 7 patients with suspected multiple adrenal masses, including 4 and 3 who underwent unilateral and bilateral laparoscopic partial nephrectomy, respectively. Six of the 7 patients had germline defects in the von Hippel-Lindau disease gene and 1 had no identifiable alteration in the von Hippel-Lindau disease or RET gene. A solitary tumor was identified in 3 cases and multiple lesions were noted in 4. In 1 case intraoperative ultrasound localized a tumor not identified on preoperative imaging that was not readily visible via laparoscopy. Intraoperative ultrasound guided surgical dissection in all cases and identified an extra-adrenal mass in 1 patient with bilateral adrenal masses. There were no complications due to intraoperative ultrasound and no conversions to open surgery. Mean operative time was 335 minutes and mean estimated blood loss was 179 ml. Histological study demonstrated pheochromocytoma in 14 lesions and the extra-adrenal mass proved to be a splenic rest. At short-term followup no recurrences have been noted. All patients retained sufficient adrenal cortical function to avoid steroid replacement therapy. CONCLUSIONS: Laparoscopic ultrasound differentiates normal adrenal parenchyma from adrenal tumors and facilitates laparoscopic partial adrenalectomy.

10
UI - 11998914
AU - van den Akker EL; de Krijger RR; de Herder WW; Drop SL
TI - Congenital hemihypertrophy and pheochromocytoma, not a coincidental combination?
SO - Eur J Pediatr 2002 Mar;161(3):157-60
AD - Department of Paediatric Endocrinology, Sophia Childrens Hospital, Erasmus University Medical Centre, Rotterdam, The Netherlands. vandenakker@alkg-azr.nl
We describe a 19-year-old female, known to have congenital hemihypertrophy, who presented with bilateral benign pheochromocytoma. This is the second time that this combination has been reported in the literature. We speculate that the combination of congenital hemihypertrophy and pheochromocytoma is not coincidental and could be part of the clinical spectrum of the Beckwith-Wiedemann syndrome. CONCLUSION: in patients with congenital hemihypertrophy, the physician should be aware of the symptoms of pheochromocytoma. Besides screening for abdominal tumours, analysis of plasma and/or urinary catecholamines and/or their metabolites should be considered.

11
UI - 11354841
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.

12
UI - 8206338
AU - Konoshita T; Gasc JM; Villard E; Takeda R; Seidah NG; Corvol P; Pinet F
TI - Expression of PC2 and PC1/PC3 in human pheochromocytomas.
SO - Mol Cell Endocrinol 1994 Mar;99(2):307-14
AD - Inserm Unit 36, College de France, Paris.
Expressions of two Kex2-related proteases, Pc2 and PC1/PC3, and of one of their possible substrates, proenkephalin, were examined in normal (n = 7) and various pathological (n = 48) human adrenal tissues. Northern blot analysis detected the expression of these genes in pheochromocytomas only. In the 20 pheochromocytomas studied with this technique, PC2, PC1/PC3 and proenkephalin were expressed in 85%, 50% and 90%, respectively. The presence of PC2 and PC1/PC3 was further confirmed using the sensitive RT/PCR techniques. Other evidence of human tumoral adrenal medullary PC2 expression was provided by in situ hybridization and immunohistochemistry. In addition, proenkephalin was expressed only in the pheochromocytomas expressing PC2 and/or PC1/PC3. These results demonstrate that functional Kex2-related endoproteases are expressed in human pheochromocytomas and may be involved in the processing of proenkephalin.

13
UI - 7819329
AU - Konoshita T; Gasc JM; Villard E; Seidah NG; Corvol P; Pinet F
TI - Co-expression of PC2 and proenkephalin in human tumoral adrenal medullary tissues.
SO - Biochimie 1994;76(3-4):241-4
AD - INSERM Unit 36, College de France, Paris.
Expression of PC2, a Kex2-related protease, and of one of its possible substrates, proenkephalin, was examined in normal adrenal glands (n = 7) and pheochromocytomas (n = 20). PC2 could only be detected in normal adrenal glands using the sensitive RT/PCR technique. By Northern blot, PC2 and proenkephalin were expressed in 85% and 90% of the 20 pheochromocytomas studied, respectively. Moreover, in situ hybridization and immunohistochemistry confirmed expression of PC2 in human tumoral adrenal medullary tissue. These results show for the first time expression of PC2 in human pheochromocytomas which may be involved in the processing of proenkephalin.

14
UI - 12379587
AU - Lamarre-Cliche M; Hernigou A; Boutouyrie P; Plouin PF; Azizi M
TI - Images in cardiovascular medicine. Pheochromocytoma of the urinary bladder.
SO - Circulation 2002 Oct 15;106(16):2143-4
AD - Clinical Investigation Center 9201, Assistance-Publique des Hopitaux de Paris/INSERM, and Department of Hypertension, Hopital Europeen Georges Pompidou, Paris, France. lamarrm@ircm.qc.ca

15
UI - 12053093
AU - Porpiglia F; Destefanis P; Bovio S; Allasino B; Orlandi F; Fontana D;
TI - Angeli A; Terzolo M Cortical-sparing laparoscopic adrenalectomy in a patient with multiple endocrine neoplasia type IIA.
SO - Horm Res 2002;57(5-6):197-9
AD - Divisione di Urologia, Dipartimento di Scienze Cliniche e Biologiche, Universita degli Studi di Torino, Italia.
We describe the case of a patient affected by multiple endocrine neoplasia type IIA with a new diagnosis of an asymptomatic right pheochromocytoma. The patient underwent laparoscopic adrenalectomy with adrenal sparing. The removal of the tumor was successful with preservation of about one third of the adrenal gland. At the time of the last follow-up, the patient is well with partial hypoadrenalism without replacement therapy. The limitations to cortical-sparing adrenalectomy imposed by traditional open surgery (small tumor with peripheral location) can be reconsidered using the laparoscopic approach. Laparoscopic cortical-sparing adrenalectomy should become the gold standard for treatment of bilateral pheochromocytoma. The advantages of this technique are its efficacy and its reduced invasiveness with a low rate of complications either during the operation or in the postoperative period. Moreover, the preservation of a portion of the adrenal cortex may prevent the need for a life-long steroid replacement therapy. Copyright 2002 S. Karger AG, Basel

16
UI - 11127918
AU - Gupta D; Shidham V; Holden J; Layfield L
TI - Prognostic value of immunohistochemical expression of topoisomerase alpha II, MIB-1, p53, E-cadherin, retinoblastoma gene protein product, and HER-2/neu in adrenal and extra-adrenal pheochromocytomas.
SO - Appl Immunohistochem Mol Morphol 2000 Dec;8(4):267-74
AD - Department of Pathology, University of Utah Health Sciences Center, Salt Lake City 84132, USA.
No reliable pathologic criteria have been identified that predict clinical behavior in adrenal and extra-adrenal pheochromocytomas (PHEOs). Reliable prognostic markers for the prediction of clinical outcome are needed to assign optimal treatment for potentially malignant tumors. In this report, we evaluated several molecular markers (topoisomerase II alpha, E-cadherin, HER-2/neu, and retinoblastoma (RB) gene protein) that have not been previously studied in PHEOs. Paraffin-embedded, formalin-fixed tissue blocks from 50 cases of PHEO (30 benign and 20 malignant, 31 adrenal and 19 extra-adrenal) were obtained from University of Utah Health Sciences Center, Salt Lake City, and the Medical College of Wisconsin, Milwaukee. Gross (tumor size, weight, local extension, cyst formation, hemorrhage, necrosis), microscopic (pleomorphism, hyaline globules, intranuclear inclusion, mitotic count, capsular and vascular invasion, ganglionic/neuronal differentiation), and immunohistochemical features (topoisomerase II alpha, p53, MIB-1, E-cadherin, RB, and HER-2/neu) were studied. With the exception of vascular invasion (P = 0.025), there were no unequivocal gross or microscopic characteristics that distinguished benign from malignant lesions (P approximately = 0.11-0.71). Topoisomerase III and MIB-1 indices in malignant lesions were significantly higher than those observed in benign lesions (P = 0.012 and 0.019). Differences in p53 expression were not statistically significant (P = 0.082). Loss in RB protein product expression was significantly more common in malignant lesions (P = 0.001), E-cadherin loss and HER-2/-neu overexpression were not observed in any of the benign or malignant lesions. We studied the immunohistochemical expression of topoisomerase II alpha, MIB-1, p53, RB gene protein product, E-cadherin, and HER-2/neu in a series of adrenal and extra-adrenal PHEOs. Overexpression of topoisomerase II alpha and MIB-1 and loss of RB protein product were more common in malignant lesions, whereas p53, E-cadherin, and HER-2/neu do not seem to have diagnostic utility in the prediction of biologic behavior in these neoplasms.

17
UI - 11893039
AU - Jorda M; De MB; Nadji M
TI - Calretinin and inhibin are useful in separating adrenocortical neoplasms from pheochromocytomas.
SO - Appl Immunohistochem Mol Morphol 2002 Mar;10(1):67-70
AD - Department of Pathology, University of Miami/Jackson Memorial Medical Center, Florida 33136, USA. mjorda@med.miami.edu
Most adrenocortical neoplasms and pheochromocytomas can be diagnosed by a combination of clinical findings and morphologic features. Occasionally, however, this histologic differential diagnosis requires ancillary tests, such as immunohistochemistry. Both tumors are generally negative for epithelial markers but express synaptophysin. Inhibin and chromogranin are used for the diagnosis of adrenocortical neoplasms and pheochromocytomas, respectively. Both antigens, however, are expressed focally and may be completely negative, particularly in small biopsies. The authors investigated the potential value of adding calretinin to inhibin in the differential diagnosis of these tumors. Fifty-five primary adrenal neoplasms including 33 adrenocortical tumors (21 adenomas and 12 carcinomas), 22 pheochromocytomas, and 7 healthy adrenal glands were examined immunohistochemically for the expression of calretinin and inhibin. Inhibin was demonstrated in 24 (73%) adrenocortical neoplasms. When calretinin was added, the number of tumors staining positively for the two markers alone or in combination increased to 31 (94%). Both antigens showed a focal pattern of distribution in many cases. None of the pheochromocytomas reacted for any of these two markers. Healthy adrenal gland showed a distinct positive and negative pattern of immunoreactivity for both antigens in cortex and medulla, respectively. There were no differences between staining patterns of calretinin and inhibin in healthy adrenal cortex, adrenocortical adenomas, and adrenocortical carcinomas. The authors conclude that the addition of calretinin to inhibin increases the sensitivity of the diagnosis of adrenocortical neoplasms. When used together, they are highly specific and sensitive for the differential diagnosis of these tumors from pheochromocytomas. These markers, however, do not distinguish between benign and malignant adrenocortical neoplasms.

18
UI - 12364472
AU - Gimenez-Roqueplo AP; Favier J; Rustin P; Rieubland C; Kerlan V; Plouin
TI - PF; Rotig A; Jeunemaitre X Functional consequences of a SDHB gene mutation in an apparently sporadic pheochromocytoma.
SO - J Clin Endocrinol Metab 2002 Oct;87(10):4771-4
AD - Departement de Genetique Moleculaire, Hopital Europeen Georges Pompidou, Assistance Publique/Hopitaux de Paris, Paris, France. Anne-Paule.Gimenez@hop.egp.ap-hop-paris.fr
Three genes encoding for mitochondrial complex II proteins are linked to hereditary paraganglioma. We have recently shown that an inactivation of the SDHD gene is associated with a complete loss of mitochondrial complex II activity and a stimulation of the angiogenic pathway (Gimenez-Roqueplo, A. P., J. Favier, P. Rustin, J. J. Mourad, P. F. Plouin, P. Corvol, A. Rotig, and X. Jeunemaitre, 2001, Am J Hum Genet 69:1186-1197). Here, we relate the case of a malignant sporadic pheochromocytoma induced by a germline missense mutation of the SDHB gene. Within the tumor, a loss of heterozygosity at chromosome 1pter led to a null SDHB allele and to a complete loss of complex II enzymatic activity. In situ hybridization and immunohistochemistry experiments showed a high expression of hypoxic-angiogenic responsive genes, similar to that previously observed in inherited-SDHD tumors. This observation highlights the role of the complex II mitochondrial genes in the oxygen-sensing pathway and in the regulation of angiogenesis of neural crest-derived tumors.

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