			Home About us Contact

Malignant Pheochromocytomas (malignant + pheochromocytoma)

Distribution by Scientific Domains

Medical Sciences	100%

Selected Abstracts

Malignant pheochromocytoma with progressive paraparesis in von Hippel,Lindau disease

EUROPEAN JOURNAL OF NEUROLOGY, Issue 4 2000
R. Mössner
Pheochromocytomas are a feature of the von Hippel,Lindau disease spectrum, a multisystem disorder of autosomal dominant inheritance. Pheochromocytomas are, however, observed during life with a lower frequency than other features of this disease, such as retinal angiomas, haemangioblastomas of the CNS, and renal carcinomas. We present the highly unusual case of a patient who required an emergency operation for an intradural extramedullary thoracic tumour which was clinically suggestive initially of neurinoma. We present evidence from NMR, histological and isotope scan investigations of this being a pheochromocytoma metastasis and of an additional right-sided paraganglioma at the same height. A detailed history revealed that this patient had suffered from four other pheochromocytomas and two other paragangliomas, in addition to retinal angiomatosis of von Hippel,Lindau disease. This case is extraordinary due to (i) the unusual site of the metastasis, (ii) the neurological requirement for an emergency operation of pheochromocytoma, (iii) metastasis of pheochromocytoma in von Hippel,Lindau disease (only eight previous cases), and (iv) the number of recurrent pheochromocytomas. It clearly demonstrates the necessity for frequent and life-long follow-up in von Hippel,Lindau disease. [source]

Clinical and pathological data of 10 malignant pheochromocytomas: Long-term follow up in a single institute

INTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2007
Kuo-How Huang
Background: Malignant pheochromocytomas are rare. Many controversies still exists in clinical practice. We report the clinical and histological data of long-term follow up in 10 patients with malignant pheochromocytoma. Methods: The clinical charts of 10 patients with malignant pheochromocytoma from a single institute were reviewed. The diagnosis of pheochromocytoma was confirmed at surgery. All patients had metastases in sites where chromaffin tissue was normally absent. Results: The median follow-up period was 5.5 years (range, 2,20 years). Extra-adrenal tumors occurred in four patients including paraganglioma tumors in three cases and bladder tumor in one case. Regional lymph node metastases were noted in six patients. Distant metastases were located in the lungs of two patients, in the bones of two patients and in the liver of one patient. Histological characteristics were not helpful for diagnosis of malignancy and for prediction of prognosis. Metastases were present in postoperative pathology in seven patients. In another three patients, metastases were discovered 6 months to 10 years after surgery. Three patients received chemotherapy and one patient received combination therapy of high-dose 131I-meta-iodobenzylguanidine (131I-MIBG) therapy and chemotherapy. All patients achieved long-term survival except for two who died of metastasis 1.5 years and 2 years after diagnosis. Conclusions: Early complete resection and adjunctive lymphadenectomy can cure malignancy. Close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma. The possibility of malignancy should be kept in mind even though the initial pathology is benign. [source]

High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma

CANCER, Issue 2 2003
Brian Rose M.D.
Abstract BACKGROUND 131I-Metaiodobenzylguanidine (131I-MIBG) can be used systemically to treat malignant pheochromocytoma. To improve outcome, the authors used higher levels of activity of 131I-MIBG than previously reported. The authors reported the response rates and toxicity levels in patients with malignant pheochromocytoma or paraganglioma who were treated with high-dose 131I-MIBG. METHODS Following debulking surgery and stem cell harvest, 12 patients with malignant pheochromocytoma or paraganglioma were treated with 131I-MIBG. Five had received previous external beam radiation and/or chemotherapy. The median single treatment dose was 800 mCi (37 gigabecquerels; range, 386,866 mCi) or 11.5 mCi/kg (range, 5.6,18.3 mCi/kg). The median cumulative dose was 1015 mCi (range, 386,1690 mCi). RESULTS Three patients had a complete response, two of whom had soft tissue and skeletal metastases. Their median follow-up was 45 months (range, 23,101 months). Seven patients had a partial response (PR), with a median follow-up 43 months (range, 6,47 months). Two patients without a response died with progressive disease (PD) and 2 patients with an initial PR died of PD at 13 and 11 months, respectively. Grade 3 thrombocytopenia occurred after 79% (15 of 19) of treatments had been administered. Grade 3 and 4 neutropenia followed 53% (10 of 19) and 19% (4 of 19) of treatments, respectively. One patient required stem cell infusion, and one developed primary ovarian failure. CONCLUSIONS The single and cumulative doses of 131I-MIBG were approximately 2,3.5 times higher than those used at other centers. Unlike previous reports, two patients with both skeletal and soft tissue metastases had a complete response. Hematologic toxicity was significant but tolerable. High-dose 131I-MIBG may lead to long-term survival in patients with malignant pheochromocytoma. Cancer 2003;98:239,48. © 2003 American Cancer Society. DOI 10.1002/cncr.11518 [source]

Circulating EM66 is a highly sensitive marker for the diagnosis and follow-up of pheochromocytoma

INTERNATIONAL JOURNAL OF CANCER, Issue 8 2006
Johann Guillemot
Abstract We have previously demonstrated that measurement of tissue concentration of the novel secretogranin II-derived peptide EM66 may help to discriminate between benign and malignant pheochromocytomas. The aim of the present study was to characterize EM66 in plasma and urine of healthy volunteers and pheochromocytoma patients, in order to further evaluate the usefulness of this peptide as a circulating marker for the management of the tumors. HPLC analysis of plasma and urine samples demonstrated that the EM66-immunoreactive material coeluted with the recombinant peptide. In healthy volunteers, plasma and urinary EM66 levels were, respectively, 2.6 (1.9,3.7) ng/ml and 2.9 (1.9,4.6) ng/ml. In patients with pheochromocytoma, plasma EM66 levels were 10-fold higher than those of healthy volunteers (26.9 (7.3,44) ng/ml), and returned to normal values after removal of the tumor. In contrast, urinary EM66 levels were not significantly different from those of healthy volunteers (3.2 (2.2,3.9) ng/ml). Measurement of total or free plasma metanephrines and 24 hr urinary metanephrines in our series of patients revealed that these tests, taken separately, are less sensitive than the EM66 determination. Pheochromocytes in primary culture secreted high levels of EM66, suggesting that the chromaffin tumor was actually responsible for the increased plasma peptide concentrations in the patients. These data indicate that EM66 is secreted in the general circulation and that elevated plasma EM66 levels are correlated with the occurrence of pheochromocytoma. Thus, EM66 is a sensitive plasma marker that should be considered as a complementary tool in the management of pheochromocytoma. © 2005 Wiley-Liss, Inc. [source]

Clinical and pathological data of 10 malignant pheochromocytomas: Long-term follow up in a single institute

VEGF in 105 pheochromocytomas: enhanced expression correlates with malignant outcome

APMIS, Issue 4 2003
KAISA SALMENKIVI
Pheochromocytomas are rare sympathoadrenal tumors that are highly vascular. Their malignancy is extremely difficult to estimate on the basis of histopathological features. Vascular endothelial growth factor (VEGF) is one of the most important angiogenic factors involved in both tumor growth and metastasis. In our search for new prognostic markers, we investigated the expression of VEGF in normal adrenal gland, in 105 primary pheochromocytomas, and in 6 metastases by using immunohistochemistry and Northern blot analysis. We also calculated the microvessel density of these tumors by staining the endothelial cells with monoclonal CD34 antibody. VEGF messenger ribonucleic acid was found in all pheochromocytomas studied. Immunohistochemically, VEGF was not found in normal adrenal medullary cells. Interestingly, all malignant pheochromocytomas (n=8), regardless of their primary location, had strong or moderate VEGF immunoreactivity, while most benign adrenal pheochromocytomas (26 of 37, 70.3%) were either negative or only weakly positive. The staining was heterogenous in extraadrenal pheochromocytomas as well as in a group of tumors that had histologically suspicious features but had not metastasized, here called borderline tumors (n=29). The microvessel density varied greatly in all of the tumor groups, and no statistical difference was found between these groups. Here we report moderate to strong VEGF expression in malignant pheochromocytomas, and negative or weak expression in benign adrenal pheochromocytomas. Normal medullary cells are immunohistochemically negative. Thus, low VEGF expression in pheochromocytomas favors a benign diagnosis. [source]

Immunohistochemical localization of somatostatin receptor subtypes in benign and malignant adrenal tumours

CLINICAL ENDOCRINOLOGY, Issue 6 2008
Nicole Unger
Summary Background, Somatostatin mediates its action through five receptor subtypes (sst1,5) that are widely distributed in various endocrine tissues and tumours. Because of the inhibitory effects of somatostatin, long-acting analogues have been synthesized. In contrast to their well-established use in neuroendocrine and pituitary tumours, little is known about their potential use in adrenal tumours. Objective, We examined somatostatin receptor protein expression in adrenal tumours of various aetiologies. Immunostaining was performed with specific polyclonal antibodies for sst1,5. Design, Seven benign and eight malignant pheochromocytomas (PHEOs), eight aldosterone-secreting adenomas (APAs), nine cortisol-secreting adenomas (CPAs), seven nonfunctioning adrenal tumours (NFAs) and 25 adrenal carcinomas (CAs) as well as eight normal adrenal glands were investigated. Measurements, Staining pattern, distribution and subcellular localization of the somatostatin receptor subtypes were evaluated. Results, In the majority of normal cortices the expression of sst1,5 was limited to the reticular zone. The medulla was predominantly positive for sst3. Most cortical adenomas were positive for all five subtypes. However, in the majority of these tumours, less than 30% of cells were positively stained. A high expression of sst4 was found in CPAs but only very few cortical carcinomas exhibited sst immunostaining. All benign PHEOs were positive for sst3. The majority presented with more than 60% of tumour cells stained. By contrast, only six out of eight malignant PHEOs were positive for sst3. Conclusions, Somatostatin receptor subtypes are expressed in PHEOs as well as in tumours of the adrenal cortex with tumour-specific distribution patterns. This may offer new diagnostic and therapeutic possibilities. [source]