A successful case of complete surgical resection via left upper and right lower lobectomy for bilateral lung metastases of a perivascular epithelioid cell tumor in the colon: a case report

Abstract Background Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with malignant potential. No effective treatment other than surgical resection has been established for lung metastases of PEComas. We describe a patient who underwent complete surgical resection via bilateral lobectomy involving a two-step procedure for lung metastases 8 years after undergoing radical surgery for a colonic PEComa. Case presentation A 53-year-old woman underwent partial colectomy for a PEComa in the transverse colon 8 years ago. She presented with an abnormal chest shadow during a health examination. Chest computed tomography (CT) revealed a solid nodule 2 cm in diameter located centrally in the right lower lobe and a solid nodule 3 cm in diameter located centrally in the left upper lobe. Positron emission tomography revealed 18F-fluorodeoxyglucose uptake in these nodules. These nodules were suspected to be metastatic tumors of the colonic PEComa and were considered for complete surgical resection. Segmentectomy could not be performed because of the anatomical location of the tumors straddling the segments; therefore, bilateral lobectomy was required for complete surgical resection. Therefore, we performed two-step lobectomy safely with the expectation of pulmonary function recovery. Microscopically, the tumors were diagnosed as lung metastases of the PEComa. One year after the last surgery, no recurrence was detected, and the patient’s pulmonary function improved. Conclusions This case indicates that even if multiple lung metastases of a PEComa require bilateral lobectomy, complete resection with a two-step surgery may be considered.

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Malignant Perivascular Epithelioid Cardiac Sarcomas: A Case Report and a Review of the Literature

Case Reports in Cardiology ◽

10.1155/2015/258140 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5

Author(s):

Candice Baldeo ◽

Abdul wahab Hritani ◽

Robert Ali ◽

Sana Chaudhry ◽

Fawad N. Khawaja

Keyword(s):

Gold Standard ◽

Epithelioid Cell ◽

Cell Tumor ◽

Cardiac Tumors ◽

Optimal Management ◽

Review Of The Literature ◽

Perivascular Epithelioid Cell Tumor ◽

Perivascular Epithelioid Cell ◽

Complete Surgical Resection ◽

Primary Cardiac Sarcoma

Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected.

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Malignant perivascular epithelioid cell neoplasm in the liver: report of a pediatric case

Surgical Case Reports ◽

10.1186/s40792-021-01300-w ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Tokuro Baba ◽

Takafumi Kawano ◽

Yusuke Saito ◽

Shun Onishi ◽

Koji Yamada ◽

...

Keyword(s):

Pediatric Patient ◽

Signal Intensity ◽

Lung Metastases ◽

Epithelioid Cell ◽

Unknown Etiology ◽

High Signal ◽

Thoracic Vertebrae ◽

Perivascular Epithelioid Cell ◽

Malignant Case ◽

Cell Neoplasm

Abstract Background Perivascular epithelioid cell neoplasm (PEComa) in a child is very rare. We herein report the first malignant case of PEComa developing in the liver of a pediatric patient. Case presentation A 10-year-old boy visited a private clinic with prolonged fever of unknown etiology. Abdominal ultrasonography was performed to evaluate the fever’s origin, revealing a large tumor in the liver. He was thus referred to a nearby hospital to investigate the tumor further. Enhanced computed tomography (CT) showed a 6.8 × 5.9 × 10.5-cm solid lesion on S4 and S5. On magnetic resonance imaging (MRI), the tumor had a low signal intensity on T1 imaging and high signal intensity on T2 imaging, with partial diffusion restriction. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a marked uptake in the mass lesion with no evidence of metastasis. The patient was negative for all tumor markers, including AFP, CEA and PIVKA-II. The results of a needle biopsy suggested hepatocellular carcinoma. The tumor’s rapid growth suggested malignancy. Hepatic segmentectomy (S4 + S5 + S8) was performed. The tumor was resected en bloc with a margin. Microscopically, the tumor showed atypical spindle, polygonal or oval-shaped cells with a high nuclear grade, and vascular invasion. Immunohistochemistry was positive for alpha-smooth muscle antigen (α-SMA), human melanin black-45 (HMB-45) and melan A. The pathological diagnosis was malignant PEComa. In the 6 months after surgery, the patient complained of shoulder pain. MRI showed a dumbbell-shaped tumor at the 2nd thoracic vertebrae, which was confirmed to be bone metastasis of PEComa. After chemotherapy, including ifosfamide and doxorubicin, vertebrectomy was performed. Two years later, thoracoabdominal CT showed a 10-cm solid mass occupying the pelvis and a 15-mm nodule in the middle lobe of the right lung. Under a diagnosis of peritoneal and lung metastases, they were surgically removed and metastasis of PEComa was pathologically confirmed. Four months after the 2nd relapse, pelvic metastasis appeared again and mTOR (mammalian target of rapamycin) inhibitor was initiated. To our knowledge, this is the first report of malignant hepatic PEComa in a pediatric patient. Conclusion Although extremely rare, malignant hepatic PEComa can develop in a child.

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Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa)

Respiratory Medicine Case Reports ◽

10.1016/j.rmcr.2017.06.011 ◽

2017 ◽

Vol 22 ◽

pp. 77-82 ◽

Cited By ~ 1

Author(s):

Shouichi Okamoto ◽

Moegi Komura ◽

Yasuhisa Terao ◽

Aiko Kurisaki-Arakawa ◽

Takuo Hayashi ◽

...

Keyword(s):

Lung Metastases ◽

Epithelioid Cell ◽

Cell Tumor ◽

Perivascular Epithelioid Cell Tumor ◽

Perivascular Epithelioid Cell

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Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review

Journal of International Medical Research ◽

10.1177/03000605211041509 ◽

2021 ◽

Vol 49 (9) ◽

pp. 030006052110415

Author(s):

Hsiu-Chung Cheng ◽

Chia-Yu Kuo ◽

Ching-Wen Huang ◽

Hsiang-Hung Shih ◽

Chih-Hung Lin ◽

...

Keyword(s):

Lymph Node ◽

Lymph Node Metastasis ◽

Surgical Resection ◽

Regional Lymph Node ◽

Epithelioid Cell ◽

Cytotoxic Chemotherapy ◽

Regional Lymph Node Metastasis ◽

Cell Tumour ◽

Node Metastasis ◽

Perivascular Epithelioid Cell

Perivascular epithelioid cell tumour (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, with few reports of paediatric cases, and there is no standardized treatment for the tumour type. Here, the case of a 17-year-old female, who presented with painless haematochezia for 2 days and was diagnosed with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis after serial examination, is presented. She was treated by surgical resection of the tumour and cytotoxic chemotherapy comprising 900 mg/m2 gemcitabine and 100 mg/m2 docetaxel every 3 weeks for six cycles. Haematochezia did not recur, and complete response was achieved, with progression-free survival at the 24-month follow-up examination. Surgical resection with adjuvant conventional cytotoxic chemotherapy may be considered as an option for treating gastrointestinal PEComa.

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Complete Surgical Resection of Gastric Cancer with Metachronous Liver and Lung Metastases

The Showa University Journal of Medical Sciences ◽

10.15369/sujms1989.17.169 ◽

2005 ◽

Vol 17 (3) ◽

pp. 169-176 ◽

Cited By ~ 1

Author(s):

Koji MOROHARA ◽

Satoshi SUZUKI ◽

Shigeo AOKI ◽

Masanori KATO ◽

Kimiyasu YAMAZAKI ◽

...

Keyword(s):

Gastric Cancer ◽

Surgical Resection ◽

Lung Metastases ◽

Complete Surgical Resection

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Perivascular epithelioid cell tumour of the uterus: what do we know?

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20205255 ◽

2020 ◽

Vol 9 (12) ◽

pp. 5110

Author(s):

Yvonne W. Y. Wong ◽

Julian H. L. Kang

Keyword(s):

Multidisciplinary Approach ◽

Clinical Presentation ◽

Uterine Fibroids ◽

Mtor Inhibitors ◽

Epithelioid Cell ◽

Unexpected Finding ◽

Mesenchymal Tumour ◽

Perivascular Epithelioid Cell ◽

Complete Surgical Resection ◽

Cell Neoplasm

Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumour characterized by distinctive histological and immunohistochemical perivascular epithelioid cells. These tumours can be found in various anatomic sites, with gynaecologic PEComas accounting for nearly one-fourth of reported cases in the literature. However, due to its non-specific clinical presentation and a lack of definitive radiological appearance, the diagnosis of PEComas remains challenging. In this case report, we describe a 45-year old lady suffering from urinary retention secondary to large uterine fibroids, who then underwent a total hysterectomy. Postoperative histopathology with immunohistochemical stains confirmed an unexpected finding of uterine PEComa. Although the treatment of gynaecologic PEComas remains controversial, complete surgical resection with negative margins is recommended. A multidisciplinary approach will be beneficial in determining the necessity of adjuvant therapy such as targeted therapy with mTOR inhibitors, especially for PEComas which exhibit aggressive and high-risk features.

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