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Malignant pheochromocytoma

Hae-Young Lee, Chan-Soon Park, Sang-Hoon Na, Kyung-Jin Kim, Chan Joo Lee, Sungha Park
A 47-year-old woman was admitted via emergency department due to dyspnea NYHA Fc II-III aggravated for 2 months after upper respiratory infection. Her height and body weight were 161 cm / 67 kg. Initial vital signs were 110/70 mmHg - 112 BPM - 24/min - 36.5°C. Chest PA showed cardiomegaly and pulmonary congestion (Figure 1). B-natriuretic peptide level was markedly increased (2002 pg/mL, normal range ≤ 100 pg/mL). The echocardiographic examination showed severely dilated LV cavity (61/72 mm) and severe LV systolic dysfunction (EF 28%) with normal left ventricular wall thickness (9/11 mm) (Figure 2)...
September 2016: Journal of Hypertension
Letizia Canu, Silvia Pradella, Elena Rapizzi, Rossella Fucci, Andrea Valeri, Vittorio Briganti, Valentino Giachè, Gabriele Parenti, Tonino Ercolino, Massimo Mannelli
Metastatic pheochromocytomas (PHEOs) and paragangliomas (sPGLs) are rare neural crest-derived tumors with a poor prognosis. About 50% of them are due to germ-line mutations of the SDHB gene. At present, there is no cure for these tumors. Their therapy is palliative and represented by different options among which antiangiogenic drugs, like sunitinib, have been hypothesized to be effective especially in malignant SDHB mutated tumors. We report the effects of sunitinib therapy in a SDHB mutation carrier affected by a malignant sPGL...
October 10, 2016: Archives of Endocrinology and Metabolism
Hannah L Bader, Tien Hsu
BACKGROUND: Mutations in the tumor suppressor gene von Hippel-Lindau (VHL) underlie a hereditary cancer syndrome-VHL disease-and are also frequently observed in sporadic renal cell carcinoma of the clear cell type (ccRCC). VHL disease is characterized by malignant and benign tumors in a few specific tissues, including ccRCC, hemangioblastoma and pheochromocytoma. The etiology of these tumors remains unresolved. METHODS: Conditional inactivation of the VHL gene in mouse (Vhlh) was generated to examine the pathophysiological role of the VHL gene function...
October 12, 2016: BMC Cancer
Nobumasa Ohara, Yasuyuki Uemura, Naomi Mezaki, Keita Kimura, Masanori Kaneko, Hirohiko Kuwano, Katsuya Ebe, Toshio Fujita, Takeshi Komeyama, Hiroyuki Usuda, Yuto Yamazaki, Takashi Maekawa, Hironobu Sasano, Kenzo Kaneko, Kyuzi Kamoi
BACKGROUND: Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors. Hypertension secondary to pheochromocytoma is often paroxysmal, and patients occasionally present with sudden attacks of alternating hypertension and hypotension. Spontaneous, extensive necrosis within the tumor that is associated with catecholamine crisis is an infrequent complication of adrenal pheochromocytoma, but its pathogenesis remains unclear. CASE PRESENTATION: A 69-year-old Japanese man developed acute-onset episodic headaches, palpitations, and chest pains...
October 12, 2016: Journal of Medical Case Reports
Sheikh Asad, Jens Peters-Willke, Louise Nott
Paragangliomas (or extra-adrenal paragangliomas) are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. This is in contrast to pheochromocytomas, where tumors occur in the adrenal gland. Malignant paraganglioma is a very rare presentation. We present a case report of a 56-year-old woman with acute foot drop with no previous significant background history...
December 2015: J Spine Surg
W Breen, I Bancos, K C Bible, W F Young, N N Laack, R L Foote, C L Hallemeier
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Shan She, Shengtai Bian, Ruichao Huo, Kun Chen, Zehuan Huang, Jiangwei Zhang, Jian Hao, Yongge Wei
High efficacy and low toxicity are critical for cancer treatment. Polyoxometalates (POMs) have been reported as potential candidates for cancer therapy. On accounts of the slow clearance of POMs, leading to long-term toxicity, the clinical application of POMs in cancer treatment is restricted. To address this problem, a degradable organoimido derivative of hexamolybdate is developed by modifying it with a cleavable organic group, leading to its degradation. Of note, this derivative exhibits favourable pharmacodynamics towards human malignant glioma cell (U251), the ability to penetrate across blood brain barrier and low toxicity towards rat pheochromocytoma cell (PC12)...
2016: Scientific Reports
Hae-Young Lee, Chan-Soon Park, Sang-Hoon Na, Kyung-Jin Kim, Chan Joo Lee, Sungha Park
A 47-year-old woman was admitted via emergency department due to dyspnea NYHA Fc II-III aggravated for 2 months after upper respiratory infection. Her height and body weight were 161 cm / 67 kg. Initial vital signs were 110/70 mmHg - 112 BPM - 24/min - 36.5°C. Chest PA showed cardiomegaly and pulmonary congestion (Figure 1). B-natriuretic peptide level was markedly increased (2002 pg/mL, normal range ≤ 100 pg/mL). The echocardiographic examination showed severely dilated LV cavity (61/72 mm) and severe LV systolic dysfunction (EF 28%) with normal left ventricular wall thickness (9/11 mm) (Figure 2)...
September 2016: Journal of Hypertension
Ann Kari Grindheim, Anni Vedeler
Annexin A2 (AnxA2) is present in multiple cellular compartments and interacts with numerous ligands including calcium, proteins, cholesterol, negatively charged phospholipids and RNA. These interactions are tightly regulated by its post-translational modifications. The levels of AnxA2 and its Tyr23 phosphorylated form (pTyr23AnxA2) are increased in many cancers and the protein is involved in malignant cell transformation, metastasis and angiogenesis. Our previous studies of rat pheochromocytoma (PC12) cells showed that reactive oxygen species (ROS) induce rapid, simultaneous and transient dephosphorylation of nuclear AnxA2, most likely associating with PML bodies, while AnxA2 associated with F-actin at the cell cortex undergoes Tyr23 phosphorylation...
July 2016: Communicative & Integrative Biology
Daniel Ching, Eleni Anastasiadis, Sarb Sandhu
INTRODUCTION: Non-urothelial tumours are rare and account for less than 5% of all bladder tumours. Bladder paragangliomas also known as extra-adrenal pheochromocytomas are of the non-urothelial subgroup. We present an unusual case of asymptomatic bladder paraganglioma. CASE REPORT: A 77year old lady presented with acute abdominal pain was found to have an incidental enhancing nodule in the bladder. During cystoscopy and transurethral resection the patient experienced significant fluctuations in blood pressure that required anaesthetic adjustments...
2016: International Journal of Surgery Case Reports
Kimitsugu Usui, Terukazu Hirasawa, Masataka Kobayashi, Kouichi Shioi, Kazuki Kobayashi, Naoki Sakai, Sumio Noguchi, Yukio Tsuura
We present a case of synchronous malignant pheochromocytoma in bilateral adrenal glands. A 73- year-old man presented to our hospital with bilateral adrenal masses incidentally found during abdominal ultrasonography examination for an unrelated issue. The patient had a 30-year history of hypertension and paroxysmal atrial fibrillation. Computed tomography and magnetic resonance imaging showed heterogeneous tumors in bilateral adrenal glands and an enlarged para-aortic lymph node. Hormonal examinations revealed a high value of urinary catecholamines...
June 2016: Hinyokika Kiyo. Acta Urologica Japonica
Shingo Moriyama, Hideki Takeshita, Saori Araki, Takuo Tokairin, Makoto Kagawa, Koji Chiba, Akiko Adachi, Akira Noro
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...
August 2016: Oncology Letters
Martin Fassnacht, Wiebke Arlt, Irina Bancos, Henning Dralle, John Newell-Price, Anju Sahdev, Antoine Tabarin, Massimo Terzolo, Stylianos Tsagarakis, Olaf M Dekkers
: By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system...
August 2016: European Journal of Endocrinology
Maria E Barmpari, Christos Savvidis, Anastasia D Dede, Haridimos Markogiannakis, Christina Dikoglou, Paraskevi Xekouki, Constantine A Stratakis, Andreas Manouras, Sofia Malaktari-Skarantavou
OBJECTIVE: Adrenal masses usually represent benign and nonfunctional adrenal adenomas; however, primary or metastatic malignancy should also be considered. Discovery of an adrenal mass needs further evaluation in order to exclude malignancy and hormonal secretion. We present a rare case of a possibly primary adrenal malignant melanoma with imaging and biochemical features of a pheochromocytoma. CASE REPORT: A 61-year-old male farmer was referred for evaluation of a mass in the right supraclavicular region and a left adrenal lesion...
April 2016: Hormones: International Journal of Endocrinology and Metabolism
Helena Leijon, Kaisa Salmenkivi, Ilkka Heiskanen, Jaana Hagström, Johanna Louhimo, Päivi Heikkilä, Ari Ristimäki, Timo Paavonen, Saara Metso, Hanna Mäenpää, Caj Haglund, Johanna Arola
Pheochromocytomas and paragangliomas are rare, neural crest-originating, neuroendocrine tumors. HuR is an mRNA-binding protein of the ELAV/Hu-protein family, which participates in posttranscriptional regulation of many cancer-associated genes. HuR expression has been connected with aggressive behavior of several malignancies. Cyclooxygenase-2 (COX-2) is also expressed in several malignant tumors, and its expression is regulated by HuR. Tissue microarray of 153 primary pheochromocytomas and paragangliomas was investigated for the expression of HuR and COX-2 proteins by immunohistochemistry using two different HuR antibodies (HuR19F12 and HuR3A)...
September 2016: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Takeshi Namekawa, Takanobu Utsumi, Takashi Imamoto, Koji Kawamura, Takashi Oide, Tomoaki Tanaka, Naoki Nihei, Hiroyoshi Suzuki, Yukio Nakatani, Tomohiko Ichikawa
Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor...
July 2016: Asian Journal of Surgery
Dong You, Ruizhen Ren, Ercheng Chen, Shulin Chen, Dawei Wang, Jianhui Liu
Malignant pheochromocytoma is a rare tumor, for which there is currently no effective therapy. Cytoreductive surgery is recommended to reduce tumor burden and relieve the symptoms of catecholamine excess, although complete eradication of the lesions is often not feasible. In patients with advanced disease, for whom surgical resection is not an option, systemic chemotherapy, radiotherapy and treatment with iodine-131-meta-iodobenzylguanidine may be used to achieve symptomatic relief. Although malignant pheochromocytoma is considered to be unresponsive to radiotherapy, a limited number of case reports, although not large patient samples, have been published on the effectiveness of radiotherapy for the treatment of this disease...
June 2016: Molecular and Clinical Oncology
Yun-Lin Ye, Xiao-Xu Yuan, Ming-Kun Chen, Yu-Ping Dai, Zi-Ke Qin, Fu-Fu Zheng
BACKGROUND: To demonstrate clinical characteristics of adrenal incidentaloma in South China and explore its comprehensive management. METHODS: The clinical data of patients with adrenal neoplasm from Jan 1998 to Dec 2012 were retrospectively analysed. Patients with suspicion of adrenal abnormalities or those in whom adrenal abnormalities were detected in the staging procedures of other cancers were excluded. Most patients with adrenal incidentaloma chose to have adrenalectomy, and some chose surveillance...
June 8, 2016: BMC Surgery
Irina Bancos, Shrikant Tamhane, Muhammad Shah, Danae A Delivanis, Fares Alahdab, Wiebke Arlt, Martin Fassnacht, M Hassan Murad
OBJECTIVE: To perform a systematic review of published literature on adrenal biopsy and to assess its performance in diagnosing adrenal malignancy. METHODS: Medline In-Process and Other Non-Indexed Citations, MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trial were searched from inception to February 2016. Reviewers extracted data and assessed methodological quality in duplicate. RESULTS: We included 32 observational studies reporting on 2174 patients (39...
August 2016: European Journal of Endocrinology
Danae A Delivanis, Dana Erickson, Thomas D Atwell, Neena Natt, Spyridoula Maraka, Schmit D Grant, Patrick W Eiken, Mark A Nathan, William F Young, Irina Bancos
OBJECTIVE: The role of percutaneous adrenal biopsy in a high risk population for adrenal malignancy has not been fully investigated. Our aim was to describe the clinical presentation leading to the adrenal biopsy and evaluate the diagnostic performance, complications and non-diagnostic rate of adrenal biopsy. DESIGN: Single center, retrospective cohort study. PATIENTS AND MEASUREMENTS: Medical records of patients who underwent adrenal biopsy between 1994 and 2014 were reviewed...
June 1, 2016: Clinical Endocrinology
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