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Multimodality imaging of aortopulmonary paraganglioma

      Highlights

      • Secondary forms should always be kept in mind in the diagnostic work-up of hypertension
      • Hypertension associated to pain and palpitation should raise suspicion of underlying catecholamine secerning tumor
      • Multimodality imaging is extremely useful for characterization of unknown masses

      Keywords

      A 72-years old woman was admitted to our department for a long lasting (3-hours) thoracic pain episode and increasing palpitations during the last month. At admission clinical examination was unremarkable and laboratory test showed slightly increased urinary as well as plasma levels of catecholamines. Transthoracic echo (Fig. 1, panel A-B) showed a vascularized, hypo-echoic mass with a central hyperechoic region that did not appear to interfere with flow (Online Video 1) in the great vessels. Subsequent CT scan confirmed the presence of a 6 × 7 cm mass with a central necrotic core, located under the aortic arch, over the pulmonary trunk (Fig. 1, panel C-D) and vascularized by thyroid arteries. Total body iodine-131 meta-iodobenzylguanidine scintillation scan showed tracer accumulation in the anterior mediastinum (Fig. 1, panel E), which suggested the presence of chromaffin hyperfunctionating cells, thus confirming the diagnosis of aortopulmonary paraganglioma.
      Fig. 1
      Fig. 1Transthoracic echo (suprasternal window) showing a hypoechoic mass (yellow arrow) with a central hyperehoic region (yellow asterisk) (A), with abundant vascularization evident with color-doppler (yellow triangle) (B). CT scan showing a contrast-enhanced mass (red arrow) with a central necrotic core (red asterisk), sagittal plane (C), frantal plane (D). Total body iodine-131 meta-iodobenzylguanidine scintillation scan with tracer accumulation in the anterior mediastinum (yellow arrow) (E). Ao = aorta; PA = pulmonary artery; LA = left atrium; LV = left ventricle; RPA = right pulmonary artery; LPA = left pulmonary artery. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
      Paraganglioma are extremely rare neoplasm of the neural crest, originating from either parasympathetic or sympathetic paraganglia. Only 10% of sympathetic paragangliomas are located in the thorax and are usually associated with catecholamine excess, presenting with hypertensive crisis, headache, sweating and palpitations [
      • Ramlawi B.
      • David E.A.
      • Kim M.P.
      • Garcia-Morales L.J.
      • Blackmon S.H.
      • Rice D.
      • et al.
      Contemporary surgical management of cardiac paragangliomas.
      ]. However, some may be pauci-symptomatic and the diagnosis may be delayed until compressive symptoms occur [
      • Erickson D.
      • Kudva Y.C.
      • Ebersold M.J.
      • Thompson G.B.
      • Grant C.S.
      • van Heerden J.A.
      • et al.
      Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients.
      ]. High index of suspicion for malign forms should be maintained in patient with RET, VHL, SDHD and SDHB mutations [
      • Boedeker C.C.
      • Neumann H.P.
      • Maier W.
      • Bausch B.
      • Schipper J.
      • Ridder G.J.
      • et al.
      Malignant head and neck paragangliomas in SDHB mutation carriers.
      ]. Complete surgical excision with adequate pre-operative alfa and beta-blockade is the main stone of the therapy [
      • Ramlawi B.
      • David E.A.
      • Kim M.P.
      • Garcia-Morales L.J.
      • Blackmon S.H.
      • Rice D.
      • et al.
      Contemporary surgical management of cardiac paragangliomas.
      ].
      Although rare, catecholamine secreting tumours should always be researched in the diagnostic work-up of patients with hypertension, palpitation or chest pain, as in this case, remembering that some may be located in the mediastinum which can be initially explored by echocardiography from suprasternal view.
      The following are the supplementary data related to this article.
      (mp4, (0.53 MB)

      References

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        • David E.A.
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        • Blackmon S.H.
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        Contemporary surgical management of cardiac paragangliomas.
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        • Ebersold M.J.
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        Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients.
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        • et al.
        Malignant head and neck paragangliomas in SDHB mutation carriers.
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