Korean J Radiol.  2013 Aug;14(4):692-696. 10.3348/kjr.2013.14.4.692.

Uncommon of the Uncommon: Malignant Perivascular Epithelioid Cell Tumor of the Lung

• Lim HJ
• Lee HY
• Han J
• Choi YS
• Lee KS

• KMID: 1715776
• DOI: http://doi.org/10.3348/kjr.2013.14.4.692

Abstract

A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant pulmonary PEC tumor with lung to lung metastases was established. Herein, the difficulties of diagnosis and management we confronted are described.

Keyword

Perivascular epithelioid cell; Pecoma; Clear cell sugar tumor; Lung tumor

MeSH Terms

Diagnosis, Differential
Follow-Up Studies
Humans
Lung Neoplasms/*diagnosis/surgery
Male
Middle Aged
Perivascular Epithelioid Cell Neoplasms/*diagnosis/surgery
Pneumonectomy
Positron-Emission Tomography/*methods
Thoracic Surgery, Video-Assisted/methods
Tomography, X-Ray Computed/*methods

Figure

• 

  Fig. 1 Imaging and pathologic findings of 63-year-old man with malignant pulmonary PEComa (A-G) and modulation of PEC (H) Chest radiography revealing huge mass in left mid to lower lung field, abutting mediastinal structures (A). Computed tomography demonstrating mass attached to posteromedial aspect of left lower lobe with air-bronchogram (arrow) in peripheral portion and without displacement of bronchovascular bundle adjacent lung parenchyma (B), which indicates lung parenchymal lesion. Several metastatic nodules (arrowheads) in both lungs (B, C). Well circumscribed, heterogeneously enhancing mass (D) containing curvilinear calcifications (arrows, E). Positron emission tomography scan (F) showing hypermetabolic mass with SUVmax of 4.4. Specimen showing rounded or oval shaped tumor cells with abundant clear cytoplasm (histological examination), H&E × 400. Immunoreactivity for SMA and immunonegative for HMB45 of tumor cells (histological examination), × 200 (G). Concept of modulation of perivascular epithelioid cell in morphology and immunophenotype (H).

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