Retroperitoneal venous malformation mimics paraganglioma on triple imaging modalities.

Here, we report a case of a woman in her 50s who was referred for an incidentally discovered lesion anterior to the origin of inferior vena cava suspicious for a paraganglioma following a CT scan for vaginal bleeding. A follow-up 68Ga-DOTATATE PET-CT and MRI of the abdomen reinforced the radiological impression of an extra-adrenal paraganglioma (EAP). The EAP was deemed non-functional given normal urine biogenic amine, supine plasma fractionated metanephrines and chromogranin A levels. The mass was resected laparoscopically without perioperative blockade. Histological examination revealed a venous malformation. Extrahepatic abdominal venous malformations are rare and can be indistinguishable from an EAP on imaging preoperatively. Although benign, the lesion nevertheless warrants excision as it is associated with a risk of haemorrhage.

A young patient with heart failure was diagnosed with extra-adrenal paraganglioma: a case report.

BACKGROUND: We present a case of pelvic paraganglioma that presented with heart failure as the primary symptom. CASE PRESENTATION: A 35-year-old man was admitted to hospital due to heart failure. Contrast-enhanced pelvic CT showed mass shadows in the posterior wall of the bladder and multiple enlarged lymph nodes in the retroperitoneal area. Ultrasound-guided puncture was performed, and the pathologic diagnosis was extra-adrenal paraganglioma. The patient refused any chemotherapy and died within six months of diagnosis. CONCLUSION: The possibility of neuroendocrine-related tumors, for example paragangliomas, should be considered in young patients with heart failure, especially those with concomitant hypertension and diabetes.

Case Report: An Unusual Case of Ectopic ACTH Syndrome Caused by Mediastinal Paraganglioma.

Ectopic adrenocorticotrophic hormone (ACTH) syndrome is not common, which is more unusual when caused by paraganglioma. We herein present a 40-year-old Chinese male who was diagnosed with ACTH-dependent Cushing's syndrome. However, the localization of the ACTH source was troublesome due to the inconsistent results of the high-dose dexamethasone suppression test and the desmopressin stimulation test. Bilateral inferior petrosal sinus sampling was performed, and ectopic ACTH syndrome was diagnosed. After 68Ga-DOTATATE-PET/CT and 18F-FDG-PET/CT were performed, it was localized in the anterior mediastinum. Post-operation histopathology demonstrated an ACTH-secreting mediastinal paraganglioma. The patient obtained complete clinical remission after a mediastinal tumorectomy.

Paraganglioma in the posterior mediastinum: a case report.

BACKGROUND: Paragangliomas are tumors that arise from extra-adrenal chromaffin cells. Herein, we present a rare case of a functional paraganglioma in the posterior mediastinum. CASE PRESENTATION: A 36-year-old man presented with paroxysms of chest pain and headache. At presentation, the patient's blood pressure was 190/120 mmHg. Chest computed tomography and magnetic resonance imaging revealed a left paravertebral mass in the posterior mediastinum. 123I-metaiodobenzylguanidine scanning revealed focally high tracer uptake in the left paravertebral area. The metanephrine level in the urine was elevated, confirming a rare, catecholamine-producing, functional paraganglioma in the posterior mediastinum. Before surgery, the patient was prepared by orally administering α- and ß-adrenergic blockers. The mass was then resected via a lateral thoracotomy. The metanephrine level in urine was normal 24 h after surgery. CONCLUSIONS: Paragangliomas in the posterior mediastinum are very rare, but more than half of all cases are functional. The associated symptoms are curable with complete resection, and long-term follow-up for recurrence is important.

Curious case of reversible left ventricular dysfunction in a young woman.

A 27-year-old woman presented with breathlessness on exertion of 3 months duration and was treated as dilated cardiomyopathy elsewhere. On evaluation, she was found to have left ventricular hypertrophy with ventricular dysfunction. CT angiogram revealed a peripheral-enhancing mass with central necrosis in the aortocaval region. Elevated serum metanephrine and uptake in 131iodine metaiodobenzylguanidine scan lead to the diagnosis of paraganglioma. Patient underwent open surgical excision of the tumour. Left ventricular function became normal and left ventricular hypertrophy resolved after surgery.

A 46-Year-Old Woman With a Mediastinal Mass.

CASE PRESENTATION: A 46-year-old otherwise healthy woman visited the ED twice over a period of 4 days for chest discomfort, midback pain, and dyspnea. The pain was localized, constant, nonpleuritic in nature, moderate in severity, and was not relieved by over-the-counter medications.

Update on Pheochromocytoma and Paraganglioma from the SSO Endocrine and Head and Neck Disease Site Working Group, Part 2 of 2: Perioperative Management and Outcomes of Pheochromocytoma and Paraganglioma.

This is the second part of a two-part review on pheochromocytoma and paragangliomas (PPGLs). In this part, perioperative management, including preoperative preparation, intraoperative, and postoperative interventions are reviewed. Current data on outcomes following resection are presented, including outcomes after cortical-sparing adrenalectomy for bilateral adrenal disease. In addition, pathological features of malignancy, surveillance considerations, and the management of advanced disease are also discussed.

Caveat for Vascular Surgeons: Lesson Learned from Acute Onset of a Rare Aortic Paraganglioma in a Young Boy.

BACKGROUND: Paragangliomas (PGs) are rare neuroendocrine tumors arising from the extra-adrenal autonomic paraganglia that are tiny organs formed by bundles of neuroendocrine cells derived from the embryonic neural crest and capable of catecholamines secretion. Diagnosis and treatment of aortic PGs could be a challenging issue when they present as an emergency setup (sudden abdominal pain and radiological images resembling a vascular emergency). CASE REPORT: We present a rare case of a 16-year-old man with a symptomatic and bleeding left para-aortic mass, treated in emergency with embolization, before a staged videolaparoscopic resection. Histology of the mass showed the presence of a large aortic PG. CONCLUSIONS: In case of active bleeding, in emergency, vascular consultants are always involved. Sometimes, circumstances are very atypical; therefore, it is essential to keep in mind rare pathologies. In such settings, multidisciplinary approach is primary to obtain a prompt diagnosis and appropriate treatment.

[Glomuvenous malformations can be very painful and cause diagnostic challenges].

This case report describes three different cases of glomuvenous malformations, which is a rare, autosomal dominant inherited cutaneous venous disease. There is a broad variation in the clinical appearance of these lesions, from flat blue elements to raised purple element with cobblestone appearance. One of the patients in this report experienced intense pain in her lesions, but after surgical resection the pain resolved. All diagnoses were confirmed with biopsy. If in doubt of the diagnosis, biopsy of vascular lesions is important.

Polymyalgia rheumatica and vagal paraganglioma.

INTRODUCTION: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome. CASE REPORT: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica. CONCLUSION: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis.

Adalimumab therapy in a patient with Crohn's disease with a giant pelvic paraganglioma after chemotherapy.

A 23-year-old man was diagnosed with a giant pelvic paraganglioma in September 2013, and a 6-month chemotherapy course was performed. The chemotherapy resulted in stable disease of the tumor for about 1 year. However, in April 2015, the patient complained of fever and diarrhea of more than ten times a day. Endoscopy showed serpiginous (snake-like) ulcers in the cecum, ascending, descending, and sigmoid colons, with granulomas without caseation histologically. The patient was diagnosed with the active stage of Crohn's disease (CD) in June 2015. Oral mesalazine (3000 mg/day) and an elemental diet (900 kcal/day) led to temporary clinical remission. At the beginning of January in 2016, an abdominal abscess and fistula were detected by computed tomography, which needed surgical treatment. Adalimumab administration was started at the beginning of February, since active lesions were detected endoscopically. A second endoscopy showed improvement of the inflammatory lesions 3 months after induction therapy with adalimumab. Clinical remission has been maintained with adalimumab administration, with stable disease of the tumor and no adverse events. To the best of our knowledge, this is the first report of a patient with a paraganglioma who developed CD after chemotherapy. The patient was successfully treated with adalimumab after surgery for his CD.

Paraganglioma masquerading as acute myocardial infarction and cardiogenic shock.

Paragangliomas, extra-adrenal pheochromocytomas, are rare catecholamine-secreting tumor. A 34-year-old lady admitted with diagnosis of ST elevation acute myocardial infarction with cardiogenic shock. Left ventricular function, severely depressed, returned to normal after initial stabilization. Coronary angiogram was normal. A para-aortic paraganglioma was diagnosed during the patient's work-up with biochemical studies, computed tomography of abdomen and functional radioisotopes imaging and was eventually surgically resected. This case shows that acute myocardial infarction may be the initial manifestation of these neuroendocrine tumors. Hypertensive emergency, much less elevated blood pressure may not be present at time of presentation.

Retroperitoneal Functioning Paraganglioma--A Rare Case of Secondary Diabetes.

INTRODUCTION: Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, which can derive from either parasympathetic or sympathetic paraganglia and are closely related to pheochromocytomas. CASE REPORT: We present the case of a young male patient of 37 years old, who was admitted for hypertensive crisis and palpitations. His medical history included medically controlled type 2 diabetes mellitus, (diagnosed 10 months ago), Hepatitis A. Hormonal evaluation revealed elevated urinary metanephrines and normetanephrines, with mainly increased normetanephrines (2330 ug/24 h). Plasmatic metanephrins were in normal range, but levels of plasmatic normetanephrins were elevated (952 pg/ml). The assessment of pituitary and aldosterone-renin axis values were within normal limits. Abdominal computed tomography showed left adrenal nodular lesion on the external arm, bilobulated, size 32/33 mm with maximum axial and cranio-caudal diameter of approx. 45 cm, suggestive of a benign lesion, keeping the cleavage plane to vecinatate structures. Left adrenalectomy was performed by laparoscopic approach. We mention that immediately after induction of anesthesia were recorded blood pressures of 298/143 mmHg. Histopathologic and immunohistochemical examination diagnose paraganglioma, without invasion of adjacent tissues. The patient evolution was favorable, with the remission of the symptoms and normalization of hormonal markers. It is imperative to note the remission of diabetes in the postoperative period. DISCUSSION: This is the case of a young patient with functional retroperitoneal paraganglioma, who presented with symptoms of pheochromocytoma. Compared to pheochromocytomas, paragangliomas are rarely symptomatic and functional. Association with diabetes is even more rare. Specialized investigations allowed the proper diagnosis and the therapeutic approach above was the result of a multidisciplinary cooperation.

Pelvic paraganglioma: a rare and unusual clinical presentation of paraganglioma.

Paraganglioma of the urinary bladder is a rare tumour of the urinary bladder causing palpitation, headache, paroxysmal hypertension, tachycardia, blurring of vision and haematuria. Patients may present with these exaggerated symptoms during or just after micturition. We present a case of a 15-year-old girl who presented to us with accelerated hypertension, headache, palpitation and blurring of vision. On the basis of a positive family history, laboratory investigations and imaging studies, she was diagnosed to have an extra-adrenal paraganglioma. Complete enucleation of the tumour with preservation of the bladder was done. This case is reported because of the rarity of the disease in urology.

18F-FDG PET/CT in a Patient With Glomus Vagale Paraganglioma and Eisenmenger Syndrome: Searching for the Missing Link?

There is emerging evidence linking cyanotic heart disease and pheochromocytoma-paraganglioma through a proposed hypoxia pathway. We present a 59-year-old woman with known Eisenmenger syndrome secondary to delayed diagnosis of ventricular septal defect, in whom F-FDG PET/CT provided comprehensive evaluation of both an F-FDG-avid left glomus vagale paraganglioma and also increased right ventricular myocardial glucose metabolism in response to severe pulmonary hypertension in the chronic shunting state.