Humans ; Paraganglioma/complications* ; Retroperitoneal Neoplasms/diagnostic imaging* ; Retroperitoneal Space ; Tachycardia

Carotid Body Tumor ; complications ; Female ; Glomus Jugulare Tumor ; complications ; Humans ; Middle Aged ; Paraganglioma ; complications

Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
Case Report ; Corticotropin/secretion+ACo- ; Cushing Syndrome/etiology+ACo- ; Female ; Human ; Mediastinal Neoplasms/therapy ; Mediastinal Neoplasms/secretion ; Mediastinal Neoplasms/complications+ACo- ; Middle Age ; Paraganglioma, Extra-Adrenal/therapy ; Paraganglioma, Extra-Adrenal/secretion ; Paraganglioma, Extra-Adrenal/complications+ACo-

Gallbladder paraganglioma is a very rare tumor and so far only a few cases have been reported. Most of these were asymptomatic and were found incidentally during operation. Recently, we experienced a gallbladder paraganglioma that gave rise to hemorrhage, which in turn caused acute cholecystitis. Our case involved a 45 year-old female patient complaining of an intermittent right upper abdominal pain. After a preoperative evaluation, cholecystectomy and lymphadenectomy were performed under the impression of gallbladder cancer with acute cholecystitis. Postoperative pathologic examination revealed a hemorrhagic gallbladder paraganglioma accompanied by acute cholecystitis. Immunohistochemical staining of the chief cells for neuron specific enolase, chromogranin and synaptophysin were positive. Sustentacular cells also stained positively for S100 protein.
Acute Disease ; Case Report ; Cholecystitis/*etiology ; Female ; Gallbladder Neoplasms/*complications/pathology ; Hemorrhage/*complications ; Human ; Immunohistochemistry ; Middle Age ; Paraganglioma/*complications/pathology

OBJECTIVETo explore the clinical implications of left ventricular hypertrophy (LVH) in patients with pheochromocytoma and paraganglioma (PH/PGL).

METHODSSeventy-eight PH/PGL patients receiving medical attention at Peking Union Medical College Hospital from October 2010 to April 2013 were included in the study.Forty-six healthy people who had no hypertension, pathoglycemia, dyslipidemia history, and with normal chest X-ray, electrocardiogram and echocardiography results served as control group. Clinical symptoms and signs, levels of 24-hour urinary catecholamine, electrocardiogram and echocardiography of participants were recorded and analyzed.

RESULTS(1)Left ventricular ejection fraction, cardiac output index, left ventricular mass index (LVMI), left atrial volume index (LAVI) and mitral E/E' were all significantly higher while mitral annulus lateral E' velocity was significantly lower in PH/PGL group than in control group (all P < 0.05). (2) LVH was associated with more prevalent acute left-sided heart failure episodes (12% (3/25) vs. 0, P = 0.030), sustained LVEF depression (12% (3/25) vs. 0, P = 0.030) and ECG ST-T segment alterations (60% (15/25) vs. 21% (11/53) , P = 0.001) in PH/PGL patients. LAVI ((30.2 ± 8.8) ml/m(2) vs. (23.8 ± 4.7) ml/m(2), P = 0.007) and mitral E/E' (11.2 ± 6.0 vs. 7.2 ± 1.4, P = 0.003) were significantly higher in patients with LVH than in patients without LVH. Mitral annulus septal ((7.8 ± 2.6) cm/s vs. (10.4 ± 3.2) cm/s, P = 0.001) and lateral ((9.3 ± 3.3) cm/s vs. (12.9 ± 2.9) cm/s, P < 0.001) E' velocity, averaged S' velocity((7.9 ± 1.6) cm/s vs. (8.8 ± 1.7) cm/s, P = 0.036) were significantly lower in LVH patients comparing to patients without LVH. (3) According to multiple linear regression analysis, age (t = 3.491, P = 0.001), gender (t = 2.899, P = 0.005), heart rate (t = 2.255, P = 0.027), and 24-hour urinary norepinephrine level (t = 3.369, P = 0.001) were independent factors affecting LVMI of PH/PGL patients.

CONCLUSIONLeft ventricular hypertrophy is associated with acute left-sided heart failure, left ventricular diastolic dysfunction and elevated left ventricular filling pressure in PH/PGL patients.

Echocardiography ; Electrocardiography ; Heart Failure ; Heart Rate ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; complications ; pathology ; Mitral Valve ; Paraganglioma ; complications ; Pheochromocytoma ; complications ; Ventricular Function, Left

Acute Kidney Injury ; diagnostic imaging ; etiology ; Aged ; Humans ; Male ; Necrosis ; diagnostic imaging ; etiology ; Paraganglioma ; complications ; Postoperative Complications ; Retroperitoneal Neoplasms ; complications ; Skin ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.
Adult ; Carney Complex/*chemically induced/complications ; Female ; Gastrointestinal Stromal Tumors/*diagnosis/pathology ; Humans ; Korea ; Paraganglioma/*diagnosis/pathology ; Retroperitoneal Neoplasms/*diagnosis/pathology ; Stomach Neoplasms/*diagnosis/pathology

OBJECTIVES: Jugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma. METHODS: Retrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications. RESULTS: Most common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications. CONCLUSION: Neurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients.
Angiography ; Compliance ; Cranial Nerve Diseases ; Hemorrhage ; Hoarseness ; Humans ; Hypogonadism ; Mitochondrial Diseases ; Neurologic Examination ; Ophthalmoplegia ; Paraganglioma ; Postoperative Complications ; Radiosurgery ; Retrospective Studies ; Tinnitus

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Appendectomy ; Appendiceal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Appendicitis ; etiology ; surgery ; Diagnosis, Differential ; Female ; Granular Cell Tumor ; complications ; metabolism ; pathology ; surgery ; Humans ; Paraganglioma ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; complications ; genetics ; metabolism ; pathology ; surgery ; Endolymphatic Sac ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; Middle Aged ; Paraganglioma ; metabolism ; pathology ; Point Mutation ; Von Hippel-Lindau Tumor Suppressor Protein ; genetics ; von Hippel-Lindau Disease ; complications ; genetics ; metabolism