Lung tumor - Clear cell (sugar) tumor

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Lung tumor

Benign tumors

Clear cell (sugar) tumor

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 6 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Benign tumor derived from perivascular epithelioid cells, sheets of large cells with clear to eosinophilic granular cytoplasm and numerous PAS+ glycogen granules (similar tumors: angiomyolipoma, lymphangioleiomyoma, PEComa)
● Usually adults, may occur in children

Treatment
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● Simple excision

Gross description
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● Small, sharply outlined, red-tan mass, usually in peripheral lung

Gross images
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4 cm, gray-white, circumscribed tumor confined to the lower lobe under the pleura

Well circumscribed tumors

Micro description
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● Sheets of large cells with clear to eosinophilic granular cytoplasm and numerous PAS+ glycogen granules
● Small uniform nuclei, prominent vasculature, may have extracellular amorphous eosinophilic material with variable calcification, minimal stroma
● No fat, no mitotic figures
● Rarely reported with malignant histologic features (J Clin Oncol 2010;28:e626, Histopathology 2011;58:498)

Micro images
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Various images

(A) Core-needle biopsy shows neoplastic cells in nested and insular pattern surrounding numerous thinned wall sinusoid-like vessels; (B) intranuclear cytoplasmic invaginations (C) HMB45+ staining

Tumor with malignant histiology

Cytology images
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(A) Large, irregular, cohesive clusters of columnar and spindle-shaped cells in background of single naked nuclei (Pap); (B) several large clusters with open cribriform-like spaces (Pap); (C) spindle-shaped and polygonal cells with round to oval nuclei and vacuolated ill-defined cytoplasm(Pap); (D) cell block shows neoplastic cells arranged in a nested and insular pattern surrounding sinusoid-like vessels; inset shows sinusoid-like vessel amidst tumor

Positive stains
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● HMB45, S100 (focal)

Electron microscopy description
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● Membrane bound glycogen in lysosome-like organelles, intracytoplasmic filaments

Cytogenetics description
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● TFE3 gene fusions in some cases (Am J Surg Pathol 2010;34:1395)

Differential diagnosis
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● Carcinoma with clear cell pattern
● Langerhans cell histiocytosis: cleaved nuclei, CD1a+ (see Pathol Int 2006;56:453-unclear how to best classify this tumor)
● Metastatic renal cell carcinoma

End of Lung tumor > Benign tumors > Clear cell (sugar) tumor

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