A malignant pheochromocytoma with ileus, polyuria and hypercalcemia: A case of recurrence 17 years after the initial operation.

Baba, Suneharu; Machida, Koji; Ozaki, Isamu; Okushima, Toshimi; Murabayashi, S; Kamata, Yoshimasa; Imamura, K.; Takebe, Kazuo

doi:10.1507/endocrj1954.32.337

Cited by 13 publications

(8 citation statements)

References 22 publications

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“…The paucity of cohort studies with a long-term follow-up is probably due to the fact that SDH gene mutations have only been described quite recently. However, it is reported that metastatic disease can occur up to 20 years after initial diagnosis of PGL 53 54. The lack of long-term follow-up in the two included cohort studies may have resulted in an underestimation of the incidence of malignant PGL.…”

Section: Discussionmentioning

confidence: 96%

Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis

et al. 2012

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The main objective of this study was to perform a systematic review and meta-analysis on the risk of developing malignant paraganglioma (PGL) in SDHB-mutation and SDHD-mutation carriers. PubMed, EMBASE, Web of Science, COCHRANE and Academic Search Premier (2000-August 2011) and references of key articles were searched to identify potentially relevant studies. The main outcomes were the pooled incidence and prevalence of malignant PGL in SDHB-mutation and SDHD-mutation carriers. A meta-analysis was performed with an exact likelihood approach using a logistic regression with a random effect at the study level. Twelve studies were included. The pooled incidence of malignant PGL in populations comprising both asymptomatic mutation carriers and mutation carriers with manifest non-malignant PGL was 17% (95% CI 10 to 28) for SDHB-mutation carriers and 8% (95% CI 2 to 26) for SDHD-mutation carriers. The pooled risk in prevalence studies was 13% (95% CI 4 to 34) and 4% (95% CI 2 to 7), respectively. In studies comprising only mutation carriers with manifest disease, the pooled prevalence was 23% (95% CI 16 to 33) for SDHB-mutation and 3% (95% CI 1 to 10) for SDHD-mutation carriers. Incidence and prevalence of malignant PGL are higher in SDHB-mutation than in SDHD-mutation carriers, but lower in SDHB-mutation carriers than hitherto appreciated.

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Section: Discussionmentioning

confidence: 96%

Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis

et al. 2012

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“…Since malignant disease can occur 17 or more years after diagnosis of the initial tumor, with patients asymptomatic during this interval, 11,12 it is realistic to assume that some tumors classified as benign in this study may later turn out to metastasize. Such misclassification may lead to a diminished yield of differentially expressed genes, but would not be expected to result in any increase in falsepositive rates of gene detection.…”

Section: Discussionmentioning

confidence: 99%

Gene Expression Profiling of Benign and Malignant Pheochromocytoma

Brouwers

Elkahloun

Munson

et al. 2006

Annals of the New York Academy of Sciences

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There are currently no reliable diagnostic and prognostic markers or effective treatments for malignant pheochromocytoma. This study used oligonucleotide microarrays to examine gene expression profiles in pheochromocytomas from 90 patients, including 20 with malignant tumors, the latter including metastases and primary tumors from which metastases developed. Other subgroups of tumors included those defined by tissue norepinephrine compared to epinephrine contents (i.e., noradrenergic versus adrenergic phenotypes), adrenal versus extra-adrenal locations, and presence of germline mutations of genes pre-disposing to the tumor. Correcting for the confounding influence of nora-drenergic versus adrenergic catecholamine phenotype by the analysis of variance revealed a larger and more accurate number of genes that discriminated HHS Public Access Author Manuscript Author ManuscriptAuthor Manuscript Author Manuscript benign from malignant pheochromocytomas than when the confounding influence of catecholamine phenotype was not considered. Seventy percent of these genes were underexpressed in malignant compared to benign tumors. Similarly, 89% of genes were underexpressed in malignant primary tumors compared to benign tumors, suggesting that malignant potential is largely characterized by a less-differentiated pattern of gene expression. The present database of differentially expressed genes provides a unique resource for mapping the pathways leading to malignancy and for establishing new targets for treatment and diagnostic and prognostic markers of malignant disease. The database may also be useful for examining mechanisms of tumorigenesis and genotype-phenotype relationships. Further progress on the basis of this database can be made from follow-up confirmatory studies, application of bioinformatics approaches for data mining and pathway analyses, testing in pheochromocytoma cell culture and animal model systems, and retrospective and prospective studies of diagnostic markers.

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“…However, the development of acute abdominal emergencies such as paralytic ileus [7, 8, 9, 10, 11, 12], pseudo-obstruction [6, 13, 14], intestinal ischemia and necrosis [15, 16, 17, 18, 19], gastrointestinal bleeding [20], and perforation of the colon [13, 14]appears to be very rare. The prognosis of patients with these gastrointestinal manifestations is poor because of the increased risk from failing to establish the correct diagnosis or from proceeding with surgical exploration, while unaware of the presence of a pheochromocytoma.…”

Section: Discussionmentioning

confidence: 99%

“…As to the preoperative preparation, phentolamine among various alpha- or beta-adrenergic receptor blocking agents may be recommended. Of the 12 cases of pheochromocytoma associated with ileus treated with alpha- or beta-adrenergic receptor blocking agents for improving blood pressure and ileus [6, 7, 8, 9, 10, 11, 12, 13, 14, 17], 7 cases showed improvement in ileus. Especially, the use of phentolamine alone or in combination with other adrenergic receptor blocking agents appears to be effective [6, 7, 8, 10, 13].…”

Section: Discussionmentioning

confidence: 99%

Pheochromocytoma Presenting as an Abdominal Emergency: Association with Perforation of the Colon

Mazaki

Hara²,

Watanabe³

et al. 2002

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A pheochromocytoma is a rare tumor derived from the adrenal medulla (or from chromaffin cells of sympathetic ganglia). Its symptoms derive mostly from the excessive release of catecholamines (adrenaline and noradrenaline). Hypertension is the most recognized feature of this disease, but gastrointestinal manifestations can on rare occasions be just as serious and life threatening. This report describes a rare case of a 70-year-old woman with pheochromocytoma who developed an acute abdominal emergency with shock and panperitonitis as a result of perforation of the descending colon which was effectively treated by surgical removal of the tumor and the perforated colon. There have been 2 such cases in the English literature in whom a pheochromocytoma was associated with perforation of the colon. Successful surgical removal of such a pheochromocytoma has been not reported previously. Our case demonstrates the importance of recognizing that a pheochromocytoma presents with a wide spectrum of manifestations, and rapid treatment brings improvement to the patient.

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A malignant pheochromocytoma with ileus, polyuria and hypercalcemia: A case of recurrence 17 years after the initial operation.

Cited by 13 publications

References 22 publications

Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis

Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis

Gene Expression Profiling of Benign and Malignant Pheochromocytoma

Pheochromocytoma Presenting as an Abdominal Emergency: Association with Perforation of the Colon

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