Expression Profile of the Telomeric Complex Discriminates between Benign and Malignant Pheochromocytoma

Boltze, Carsten; Mundschenk, J; Unger, Nicole; Schneider‐Stock, Regine; Peters, Brigitte; Mawrin, Christian; Hoang‐Vu, Cuong; Roessner, Albert; Lehnert, Hendrik

doi:10.1210/jc.2002-021299

Cited by 74 publications

(53 citation statements)

References 36 publications

(38 reference statements)

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“…Therefore, they have not yet had a significant impact on changes in pathological diagnosis and follow-up management. Previous studies have suggested that the Ki-67 index may be valuable in the diagnosis of malignant PCC (13)(14)(15). The classification using the Ki-67 index alone may be considered as simple and easy to apply in the clinical setting.…”

Section: Discussionmentioning

confidence: 99%

Carcinoma-like nonfunctional pheochromocytoma in the right adrenal gland: A case report

et al. 2016

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Abstract. Evaluation of the malignant potential of a pheochromocytoma (PCC) remains controversial. PCC is regarded as a neuroendocrine tumor (NET), and the classification of NETs has gradually been defined over the last decade, particularly for gastroenteropancreatic NET. The present study describes a case of locally advanced, carcinoma-like, nonfunctional PCC, which may be regarded as neuroendocrine carcinoma (NEC) rather than a malignant PCC. A 72-year-old man was referred to Saitama Red Cross Hospital (Saitama, Japan), presenting with a 2-month history of right flank pain. Computed tomography revealed a right adrenal gland tumor, which measured 6.0 cm in diameter, invading the hilum of the right kidney, liver and inferior vena cava (IVC). Radical surgery was performed with en bloc resection of the right kidney, and adjacent parts of the liver and IVC. Immunohistochemical examination demonstrated that all of the resected tissues were positive for cytokeratin AE1/AE3, chromogranin A, synaptophysin, cluster of differentiation 56 and Ki-67, and the specimen had a Ki-67 index of 80%. A diagnosis of carcinoma-like PCC or NEC of the adrenal gland was confirmed. Reports of NEC of the adrenal gland are extremely rare in the literature, and classification of PCC as a NET has not yet been fully discussed. The present case may therefore contribute to the classification of NETs in the adrenal gland.

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Section: Discussionmentioning

confidence: 99%

Carcinoma-like nonfunctional pheochromocytoma in the right adrenal gland: A case report

et al. 2016

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“…Underlying genetic mutations and molecular alterations associated with malignancy have led to the identiication of molecular therapeutic targets that include the mammalian rapamycin (mTOR) protein kinase, which is known to be upregulated in malignant pheochromocytomas [65], angiogenesis-mediated growth factors such as vascular endothelial growth factor (VEGF) and heat shock protein 90 (Hsp90), which are also overexpressed in malignant pheochromocytomas [66]. The mTOR inhibitor, Everolimus, has been used in a number of patients with malignant pheochromocytomas, but results have been disappointing [67].…”

Section: Targeted Therapymentioning

confidence: 99%

Pheochromocytomas

Heneghan¹,

Prichard²

2017

Clinical Management of Adrenal Tumors

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“…The sum of the scores groups adrenal pheochromocytomas into those with potential for biologically aggressive behavior (PASS) and those likely to behave in a benign fashion (PASS Ͻ4). [117], heat shock protein 90 [118], and telomerase complex proteins [119] have all been studied with varying degrees of success.…”

Section: Histological Scoring Systemsmentioning

confidence: 99%

Molecular and Therapeutic Advances in the Diagnosis and Management of Malignant Pheochromocytomas and Paragangliomas

et al. 2013

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Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies. TheOncologist2013; 18:391-407 Implications for Practice: Malignant pheochromocytoma and paraganglioma represent a significant management challenge.The diagnosis of malignancy is frequently made in retrospect and traditional treatment modalities have been limited. Recent exciting advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors. Increasing knowledge of genotype-phenotype interactions facilitates more accurate determination of malignant potential and has prompted investigation into targeted therapeutics with promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for pheochromocytoma. Due to the rarity of this tumor, large-scale clinical studies that would progress clinical practice are rare, and the requirement for international collaboration is crucial. The need for large-scale international multicenter studies to effectively exploit the molecular and genetic knowledge gained in this area is highlighted in this review.

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Expression Profile of the Telomeric Complex Discriminates between Benign and Malignant Pheochromocytoma

Cited by 74 publications

References 36 publications

Carcinoma-like nonfunctional pheochromocytoma in the right adrenal gland: A case report

Carcinoma-like nonfunctional pheochromocytoma in the right adrenal gland: A case report

Pheochromocytomas

Molecular and Therapeutic Advances in the Diagnosis and Management of Malignant Pheochromocytomas and Paragangliomas

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