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Breast-nonmalignant

Benign tumors / changes

Clear cell “sugar” tumor of breast

 

Reviewer: Hind Nassar, M.D. in January 2009 (see Authors page)

Revised: 14 October 2012, last major update March 2010

Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.

 

Definition

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● Very rare tumor in breast of epithelioid to spindle cells with clear to eosinophilic granular cytoplasm and distinct cell borders, positive for melanocytic markers

 

Terminology

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● Perivascular Epithelioid Cell tumor, PEComa

 

Sites

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Etiology

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● Differentiates towards Perivascular Epithelioid Cell

● Other PEComas are angiomyolipoma, lymphangiomyoma, lymphangioleiomyomatosis, renal capsuloma and clear cell myomelanocytic tumor of the falciform ligament / ligamentum teres

 

Clinical features

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● Benign behavior

 

Case reports

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● 16 year old girl (Am J Surg Pathol 2002;26:670)

 

Treatment

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● Excision

 

Gross description (Macroscopy)

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Gross images

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Micro description (Histopathology)

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● Epithelioid to spindle cells with clear cytoplasm and distinct cell borders

 

Micro images

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Lung tumor is HMB45+

 

      

Rectal tumor is HMB45+

 

Virtual Slides

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Videos

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Cytology description

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Cytology images

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Positive stains

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● Melanocytic markers (HMB45, MelanA)

● PAS diastase sensitive (glycogen), PR

● Vimentin (focal)

 

Negative stains

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● S100, keratin (AE1-AE3, CAM5.2)

● Desmin, ER

 

Electron microscopy descriptions

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Electron microscopy images

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Rectal tumor shows premelanosomes

 

Molecular / cytogenetics description

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Molecular / cytogenetics images

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Differential Diagnosis

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Glycogen rich (clear cell) carcinoma: large nests of clear cells with hyperchromatic nuclei, mild/moderate atypia and pleomorphism; invasive; keratin+, PAS+ diastase sensitive; negative for HMB45 and MelanA

 

Additional references

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Virchows Arch 2008;452:119

 

End of Breast-nonmalignant > Benign tumors / changes > Clear cell “sugar” tumor of breast

 

 

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