J Pathol Transl Med.  2016 May;50(3):238-242. 10.4132/jptm.2016.02.19.

Sclerosing Perivascular Epithelioid Cell Tumor of the Lung: A Case Report with Cytologic Findings

• Kim HY
• Choi JH
• Lee HS
• Choi YJ
• Kim A
• Kim HK

• KMID: 2164602
• DOI: http://doi.org/10.4132/jptm.2016.02.19

Abstract

Benign perivascular epithelioid cell tumor (PEComa) of the lung is a rare benign neoplasm, a sclerosing variant of which is even rarer. We present a case of 51-year-old man who was diagnosed with benign sclerosing PEComa by percutaneous fine needle aspiration cytology and biopsy. The aspirate revealed a few cell clusters composed of bland-looking polygonal or spindle cells with fine granular or clear cytoplasm. Occasional fine vessel-like structures with surrounding hyalinized materials were seen. The patient later underwent wedge resection of the lung. The histopathological study of the resected specimen revealed sheets of polygonal cells with clear vacuolated cytoplasm, variably sized thin blood vessels, and densely hyalinized stroma. In immunohistochemical studies, reactivity of tumor cells for human melanoma black 45 and Melan-A further supported the diagnosis of benign sclerosing PEComa. To the best of our knowledge, this is the first case of benign sclerosing PEComa described in lung.

Keyword

Lung neoplasms; Solitary pulmonary nodule; Perivascular epithelioid cell neoplasms

MeSH Terms

Biopsy
Biopsy, Fine-Needle
Blood Vessels
Cytoplasm
Diagnosis
Epithelioid Cells*
Humans
Hyalin
Lung Neoplasms
Lung*
MART-1 Antigen
Melanoma
Middle Aged
Perivascular Epithelioid Cell Neoplasms
Solitary Pulmonary Nodule
MART-1 Antigen

Figure

• 

  Fig. 1. Liquid-based aspiration cytology of benign sclerosing perivascular epithelioid cell tumor. (A) A few cohesive clusters of polygonal to spindle-shaped bland cells in a clean background (Papanicolaou staining). (B) The tumor cells show oval nuclei, small distinct nucleoli and abundant basophilic and granular cytoplasm. Most cells contain clear intracytoplasmic vacuoles (Papanicolaou staining). (C) Thin-walled blood vessels are occasionally seen. Semitranslucent hyalinizing material was noted around the vascular structure (Papanicolaou staining).

• 

  Fig. 2. Histologic section of benign sclerosing perivascular epithelioid tumor. (A) Dilated vessels surrounded by collagenous stroma and polygonal cells with clear cytoplasm. (B) Sheet-like arrangement of tumor cells around various-sized blood vessels and a focus of microcalcification.

• 

  Fig. 3. Special stain and immunohistochemical stains (A, B) Periodic acid–Schiff (PAS) (A) stain and diastase-PAS (B) stain show positive reaction of the clear intracytoplasmic vacuoles in tumor cells. (C, D) Tumor cells show positive reaction to human melanoma black 45 (C) and to Melan-A (D).

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