Granular Cell Tumor



Granular Cell Tumor


Cyril Fisher, MD, DSc, FRCPath










Prominent pseudoepitheliomatous hyperplasia image can be seen overlying granular cell tumors. The tumor is unencapsulated, creating a sheet-like distribution of neoplastic granular cells.






High magnification shows that the cells are polygonal, with a slightly spindled appearance. The cytoplasm contains numerous eosinophilic granules and the nuclei are small, round to oval, and hyperchromatic.


TERMINOLOGY


Abbreviations



  • Granular cell tumor (GCT)


Synonyms



  • Granular cell myoblastoma


  • Abrikossoff tumor


Definitions



  • Benign tumor composed of poorly demarcated proliferation of plump granular cells



    • Distinct from congenital epulis of newborn (gingival granular cell tumor of infancy)


ETIOLOGY/PATHOGENESIS


Schwannian Derivation



  • Thought to arise from Schwann cells



    • Positive with neural-associated antibodies


    • Granules represent senescent change with accumulation of autophagocytic lysosomes


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare


  • Age



    • All ages



      • Peak between 40-60 years


  • Gender



    • Female > male (2:1)


  • Ethnicity



    • Blacks affected more often than whites


Site



  • Over 50% occur in head and neck region



    • Up to 70% of these develop in oral cavity (mucosa, tongue, hard palate)


    • Tongue is most common site



      • Dorsum more often than lateral margin


  • Skin


  • Nerve


  • Esophagus


  • Biliary tract


  • Neurohypophysis


  • Up to 20% of patients have multiple lesions


Presentation



  • Most present as a single painless nodule



    • Usually have symptoms for < 12 months


  • Rarely, may present with Eagle syndrome



    • Elicitation of pain on swallowing, turning head, or extending tongue



      • Syndrome is thought to be caused by irritation of glossopharyngeal nerve


Treatment



  • Surgical approaches



    • Complete excision with narrow margins


    • Laser excision can be performed


Prognosis



  • Excellent long-term prognosis


  • Recurrence/relapse/persistence is uncommon (˜ 10%)


  • Malignant GCTs very rare



    • Local recurrence in more than 30%


    • 50% metastasize, mainly to lymph nodes, lung, bone


MACROSCOPIC FEATURES


General Features



  • Smooth-surfaced submucosal or subcutaneous nodule



    • Poorly demarcated


  • Cut surface firm, pale yellow or cream colored



  • In mouth, concurrent Candida infection can form white plaque


Size



  • Mean: 1-2 cm

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Granular Cell Tumor
Premium Wordpress Themes by UFO Themes
%d bloggers like this: