A case of granular cell tumor
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  • Gross Cystic Disease Fluid Protein 15

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  • 1. CASE PRESENTATION BY Salma Tarek Mahmoud Assistant Fellow at Ahmed Maher Teaching Hospital
  • 2. A 22 ys old female patient complaining of left breast mass.  Excision was done 
  • 3. Gross examination •A fairly defined rubbery mass measured 10x7x7 mm. •Cut section was homogenous grayish white
  • 4. Microscopic examination
  • 5. PAS staining was done
  • 6. Cytokeratin
  • 7. c
  • 8. Diagnosis Granular cell tumor
  • 9. Granular cell tumor( GCTs) Uncommon tumor thought to be derived from schwann cells.  Abrikossoff first described this tumour in 1926 as ‘‘granular cell myoblastoma’’, assuming it was of myogenic origin; some even refer to the lesion as ‘‘Abrikossoffoma’’.  Considered benign, only rare reports of malignant variants Incidence  Occur in any age but are most common in fourth, fifth and sixth decades of life, rare in children.  It is about twice as common in women as in men. 
  • 10. Site  Arise in any organ as solitary painless nodule  Most frequently in oral cavity typically the tongue  Internal organs, particularly larynx, bronchus, stomach and bile duct. Clinically  Approximately 10–15% of patients have lesions at multiple sites.  Multiple lesions may appear synchronously or over a period of many years. Gross picture   Poorly circumscribed nodule less than 3 cm Cut section pale yellow-tan or gray.
  • 11. Microscopically      Infiltrating compact nests or sheets Bland looking large polygonal to spindle cells Eosinophilic cytoplasmic granules Nuclei are small dark monotypic +/- vacoulization and clearing
  • 12.  Pseudoepitheliomatous hyperplasia of the overlying squamous epithelium, should not be mistaken for squamous cell carcinoma.  In close proximity to peripheral nerve bundles
  • 13. Special Stains and Immunohistochemistry       S-100 protein: highlights cytoplasmic granularity with strong cytoplasmic and nuclear staining Carcinoembryonic antigen (CEA): diffuse immunoreactivity Cytokeratin and (EMA) are negative Myoglobin negative (ER) and (PR) are negative PAS positive diastease resistant cytoplasmic granules
  • 14. GCTs in breast Incidence  5%- 6% in breast.  Premenopausal women in 40’s, reported in adolescents, elderly women and men  May be more common in African-American women Site  Superior medial quadrant (course of supraclavicular nerve) Clinical features  Usually solitary unilateral, rarely multiple  Painless firm mass, may be associated with skin retraction and nipple inversion
  • 15. Gross  Cut surface is fairly defined white - gray to yellow  Less than 3 cm but reported up to 9 cm.
  • 16. Radiologically X-ray  Mammography Suggestive of malignancy due to apparent infiltration, stellate mass without calcification 
  • 17. Differential Diagnosis
  • 18. D.D Reactive histocytic lesion Invasive breast carcinoma  Apocrine carcinoma  Myoblastomatoid, Lobular carcinoma histocytic variant Alveolar soft part sarcoma Metastatic malignant melanoma Metastatic renal cell carcinoma
  • 19. Reactive Histocytic lesions  Dispersed histocytes with mixed population of inflammatory cells
  • 20. Apocrine carcinoma      Older age Usually in outer quadrant large cells with pleomorphic nuclei, prominent nucleoli, mitosis Typically associated with intra-ductal component IHC: Positive for cytokeratin, GCDFP-15
  • 21. Myoblastomatoid invasive lobular carcinoma, histocytic variant Older age  Associated with infiltrative component  Loosely cohesive tumor cells  IHC: Positive for cytokeratin 
  • 22. Alveolar soft part sarcoma • • • • • • Deep soft tissue Pleomorphic cells Cells are divided into packets by thin walled vessels Alveolar pattern if cells discohesive Vascular invasion common IHC: Positive for Myoglobin
  • 23. Metastatic malignant melanoma Old age  History of primary elsewhere  Nuclei usually show   nucleoli  Cytoplasmic pseudoinclusions  Melanin pigments IHC: Positive HMB- 45
  • 24. Metastatic renal cell tumor        History of primary elsewhere Nests, separeted by sinasoids Well defined cell membrane Nuclear pleomorphism Prominent nucleoli in high grades Clearing of cytoplasm IHC: positive for EMA
  • 25. Take home message         GCTs is an uncommon tumor occur in any age. 5%-6% incidence in breast, inner upper quadrant. Pose a real diagnostic challenge for physicians. Clinically and radiologically misdiagnosed as carcinoma Preoperative diagnosis with core needle biopsy is important because treatment is with wide excision , rather than mastectomy. Less than 1% of all GCTs, including mammary lesions, are malignant. The prognosis for benign GCT of the breast is excellent. Recurrence occurs in 2-8% of individuals after excision with wide margins