Case of perivascular epithelioid cell tumor of lung with malignant features

Q: What does this research mean for the field?

Primary lung perivascular epithelioid cell tumors (PEComas) can present with malignant features and strong cellular atypia, requiring careful pathological differentiation from other lung tumors such as carcinoids. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Tomoki Nakagawa; Hiroaki Harada; Koichi Akayama; Takuya Hattori; Satoshi Shibata

doi:10.2995/jacsurg.40.467

July 14, 2026The Journal of the Japanese Association for Chest SurgeryOpen Access

Case of perivascular epithelioid cell tumor of lung with malignant features

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Key Points

To investigate a case of perivascular epithelioid cell tumor in the lung with malignant characteristics.
Monitoring a lung nodule via CT for four years until surgical intervention was deemed necessary.
Robotic-assisted right middle lobe resection performed for diagnosis and treatment after observing tumor growth.
Intraoperative and final pathological analyses conducted to confirm diagnosis.
Initial CT showed an 8 mm nodule that grew to 16 mm with a maximum SUV of 3.9 in PET scan.
Final pathology confirmed diagnosis of perivascular epithelioid cell tumor with significant cell atypia.
After 14 months of follow-up post-surgery, no recurrence observed.

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Implication

Case of a 44-year-old male with a malignant perivascular epithelioid cell tumor, indicating careful observation after surgery.

Abstract

症例は44歳男性．4年前に健診の胸部単純X線写真で異常を指摘され，CTで右肺S4に辺縁整の径8 mm大の結節影が認められた．その後2年間CTにて経過観察されたが変化はなく，以後通院を自己中断したが健診で陰影の増大が認められた．CTで径16 mm大であり，PET検査ではSUVmax 3.9の集積を認めた．形状や増大速度より硬化性肺胞上皮腫やカルチノイドを疑い，診断と治療目的にロボット支援下右中葉切除術を行った．迅速病理検査で定型カルチノイドが疑われたが，最終的な病理組織学的検査で血管周囲類上皮細胞腫瘍（perivascular epithelioid cell tumor：PEComa）と診断された．本例は病理学的に細胞の異型性が強く肺原発として非典型的だったが，他部位に原発を疑う所見はなく，肺原発PEComaと診断した．現在慎重に経過観察しており，術後1年2ヵ月時点で再発なく経過している．

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Cite This Study

Nakagawa et al. (2026) studied this question.

synapsesocial.com/papers/6a59c5a7a58755010b47114f https://doi.org/10.2995/jacsurg.40.467

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Case of perivascular epithelioid cell tumor of lung with malignant features

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Discussion

Cite This Study

Case of perivascular epithelioid cell tumor of lung with malignant features

Key Points

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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