Paraganglioma



Paraganglioma











Mediastinal paraganglioma shows the typical nesting pattern of growth. Note the presence of a dilated vascular structure image. These findings are typical for paraganglioma.






Mediastinal paraganglioma shows the presence of marked nuclear atypia and macronuclei and a lack of mitotic activity.


TERMINOLOGY


Synonyms



  • Extraadrenal paraganglioma, carotid body tumor


Definitions



  • Neuroendocrine neoplasm


ETIOLOGY/PATHOGENESIS


Etiology



  • These tumors may originate from normal paraganglia


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Unusual tumors in mediastinal compartment


  • Age



    • May occur at any age


    • More common in 3rd decade of life


  • Gender



    • No gender predilection


    • Some studies have reported slight predominance in male patients


Site



  • Paragangliomas may occur in anterior and posterior mediastinum


Presentation



  • Chest pain


  • Cough


  • Difficulty in swallowing


  • Dyspnea


  • Headache


  • Hypertension


  • Diaphoresis


  • Part of the multiple endocrine neoplasia (MEN) syndrome


  • Some patients may be asymptomatic


Treatment



  • Complete surgical resection


  • Adjuvant therapy in selected cases


Prognosis



  • In majority of cases, complete surgical resection is curative


  • In some cases, paragangliomas behave aggressively with metastatic disease leading to death


MACROSCOPIC FEATURES


General Features



  • Soft, irregular


  • Tan to light brown


Size



  • Varies from 2 cm to > 10 cm in diameter


MICROSCOPIC PATHOLOGY


Histologic Features



  • So-called zellballen growth pattern


  • Nested pattern


  • Spindle cell pattern


  • Presence of large cells with macronuclei and bizarre shapes


  • Granular-like cells may be prominent


  • Melanin pigment may also be seen


  • Mitotic activity is rare


  • Extensive areas of hyalinization may be present


  • Ectatic vessels



DIFFERENTIAL DIAGNOSIS

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Paraganglioma

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