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Thread: Granular Cell Tumor Pictures - Atlas of Colon and Ileum

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    Default Granular Cell Tumor Pictures - Atlas of Colon and Ileum

    Pathophysiology
    Granular cell tumors are typically solitary, smaller than 3 cm, and located in the dermis or subcutis and less frequently in the submucosa, smooth muscle, or striated muscle. Granular cell tumors are also found in the internal organs, particularly in the upper aerodigestive tract. Benign and malignant counterparts are known; the latter are rare, comprising fewer than 2% of all granular cell tumors

    International
    The lesion is uncommon. Exact figures are unavailable. Much of the literature on granular cell tumors consists of reports of single cases.

    Race-, Sex-, and Age-related Demographics
    Granular cell tumors appear to be more common in black persons. Multiplicity of lesions is definitely more common in black persons. Up to 10% of granular cell tumors are multiple (ie, two to four lesions).
    A slight female predominance exists. The female-to-male ratio is estimated at approximately
    Granular cell tumors affect persons of varying ages, and the range is wide. Most patients are middle-aged, with a peak incidence in the fourth through sixth decades of life.

    History
    Benign lesions manifest as nonulcerated and usually painless nodules with an insidious onset and slow growth rate. They are rarely larger than 3 cm and usually have been noted for less than 6 months.

    Malignant lesions
    Malignant granular cell tumors are rare. By convention, granular cell tumors are classified as malignant when their constituent cells show cytologic features of malignancy or when a morphologically benign granular cell tumor metastasizes to regional lymph nodes or distant sites or otherwise causes death.

    Malignancy is encountered more often in deep-seated lesions in adults, with a mean patient age of 50 years. Sex and race distribution of malignant tumors mirrors that of benign lesions. A history of long clinical duration and rapid recent growth has been observed in some cases, suggesting a possibility of malignant transformation from a preexisting benign granular cell tumor.

    The lesions are usually larger (ie, 4-15 cm) and may be locally destructive, thus causing symptoms (eg, pressure, obstruction, hemorrhage, ulceration, secondary infection) depending on the site. Metastases to regional lymph nodes and/or distant organs (most common site is the lungs) and concomitant symptoms may be present.

    Approach Considerations
    Laboratory tests may be necessary to assess the functional effects of visceral lesions (eg, liver function tests in lesions along the biliary tree). However, the diagnosis of granular cell tumors centers on analysis of biopsy specimens.

    Anatomic pathology
    Gross features include pale white/yellow, nonencapsulated, and variably (well to poorly) circumscribed nodules with solid, fleshy cut surfaces that are devoid of liquefaction, necrosis, or bleeding. The overlying skin or mucosa is thickened and may have a cobblestone appearance.
    Granular Cell Tumor Pictures Atlas attachment.php?s=b377384d0a5b28c1941462e824c65f03&attachmentid=2083&d=1442249398

    Microscopic features of benign granular cell tumors are remarkably uniform, regardless of the site. Granular cell tumors are sometimes located near a nerve twig, usually within the perineurium, and are variably circumscribed at the periphery. Approximately half of all granular cell tumors have poorly defined or infiltrative margins. The nodules are composed of large polyhedral cells arranged in sheets, nests, lobules, or trabeculae and are surrounded by variable stroma. A reticulin framework may be around individual cells or small groups of cells. Occasionally, granular cell tumors are extensively collagenized.

    The tumor cells have abundant granular eosinophilic cytoplasm with centrally located vesicular or pyknotic nuclei. Markedly enlarged lysosomes in tumor cells may be observed as eosinophilic globules surrounded by a clear halo; some are extruded from cells and may be phagocytosed by histiocytes. In such cases, they are termed angulate bodies. Usually, the granules stain positive with periodic acid-Schiff (PAS) staining and are resistant to diastase. They also stain with Sudan black B and are magenta in trichrome preparations. Multinucleation, plentiful mitotic activity, nuclear pleomorphism, and prominent nucleoli are uncommon features. Squamous epithelium overlying the peripheral superficial lesions exhibits acanthosis and pseudoepitheliomatous hyperplasia.

    Immunohistochemical findings
    Granular cell tumors have an uncertain histogenesis. Many immunohistochemical and ultrastructural studies suggest a Schwann cell origin.

    The tumor cells stain positively for S-100 protein, neuron-specific enolase, and NK1-C3 in almost all cases. Positivity with stains for myelin-associated P0 and P2 proteins, myelin basic protein, and Leu-7 is less consistent.

    The tumor cells are nonimmunoreactive for epithelial, muscle, endothelial, and glial cell markers. This is useful for differentiating a granular cell tumor from other diagnostic possibilities.

    Ultrastructural findings
    Ultrastructural findings with granular cell tumors are highly characteristic. Pleomorphic secondary lysosomes are observed within the cytoplasm of tumor cells.

    Features indicating neural derivation of granular cell tumors (eg, myelin residues, long-spacing collagen, arrays of neuritic processes among tumor cells) may be observed

    Procedures
    Kobara et al reported that endoscopic imaging under direct view has potential diagnostic value for submucosal tumors in the gastrointestinal (GI) tract. The two granular cell tumors in this study were both white, cloudy, round, and elastic, with no visible tumor coating. Final pathological diagnosis was obtained by core biopsy using the submucosal endoscopy with mucosal flap method.

    Staging
    Universally recommended and accepted staging schemes specific for granular cell tumors do not exist. A general staging scheme developed by the American Joint Committee on Cancer for use with other soft tissue tumors may be followed.
    Granular Cell Tumor Pictures Atlas attachment.php?s=b377384d0a5b28c1941462e824c65f03&attachmentid=2084&d=1442249424

    Pathologic differential diagnoses
    Some schwannomas and neurofibromas may show granular changes in parts, but the changes are never extensive enough to create a major diagnostic challenge. Moreover, schwannomas are encapsulated, and other stigmata of von Recklinghausen disease associated with neurofibromas are absent in patients with granular cell tumors.

    Benign granular cell tumors may exhibit some superficial resemblance to rhabdomyomas and hibernomas. However, upon critical analysis, they do not show cytoplasmic striations or vacuoles and are negative for skeletal muscle markers and fat stains.

    Intracranial granular cell tumors (the posterior pituitary is a noteworthy site for granular cell tumors) may be mistaken for granular variants of glial tumors but can be differentiated based on their negativity for glial fibrillary acid protein.

    Granular cell variants of basal cell carcinoma, melanoma, leiomyoma, leiomyosarcoma, dermatofibrosarcoma, angiosarcoma, fibrous histiocytoma, and ameloblastoma can sometimes be indistinguishable from granular cell tumors if examined with routine light microscopy. A battery of immunohistochemical stains is needed to make a specific diagnosis. Granular cell tumors are positive for S-100 protein and negative for epithelial, melanocytic, smooth muscle, dendritic cell, and endothelial markers.

    Malignant granular cell tumors can sometimes mimic alveolar soft part sarcoma because of their organoid growth pattern and periodic acid-Schiff (PAS)–positive intracellular crystalloids. The rhomboid crystalloids with their characteristic lattice pattern, observed ultrastructurally in alveolar soft part sarcoma, are absent in granular cell tumors.

    References:
    http://emedicine.medscape.com/article/282430-workup#c7











    Last edited by Medical Photos; 09-14-2015 at 04:51 PM.

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