Article
Oncology
Andrew J. Wagner, Vinod Ravi, Richard F. Riedel, Kristen Ganjoo, Brian A. Van Tine, Rashmi Chugh, Lee Cranmer, Erlinda M. Gordon, Jason L. Hornick, Heng Du, Berta Grigorian, Anita N. Schmid, Shihe Hou, Katherine Harris, David J. Kwiatkowski, Neil P. Desai, Mark A. Dickson
Summary: The study evaluated the efficacy and safety of nab-sirolimus in patients with malignant PEComa, showing an overall response rate of 39% with rapid and durable responses. Patients with TSC2 mutations were more likely to achieve a positive response. nab-sirolimus was considered an important new treatment option for this disease based on response rate, disease control, and safety profile.
JOURNAL OF CLINICAL ONCOLOGY
(2021)
Review
Oncology
Aikaterini Liapi, Patrice Mathevet, Fernanda G. Herrera, Delfyne Hastir, Apostolos Sarivalasis
Summary: Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms often associated with abnormal activation of the PI3K/AKT/mTOR pathway. Treatment with surgical resection and radiotherapy was administered, but metastatic lesions eventually progressed. A second line treatment with pazopanib showed effectiveness in controlling the disease progression.
FRONTIERS IN ONCOLOGY
(2021)
Article
Medicine, General & Internal
Wenbi Yang, Quanlin Sun, Maocai Shang, Song Li, Xiao Hu, Xianwen Hu
Summary: Hepatic perivascular epithelioid cell tumors (PEComas) are rare interstitial tumors that are often misdiagnosed as hepatocellular carcinomas. This case report presents a 61-year-old male patient who had a lesion in the left medial segment of the liver incidentally found during a chest CT examination. Imaging revealed solid components and areas of low-density adipose tissue within the lesion. Magnetic resonance imaging showed areas with unevenly high signal intensity and areas with unevenly low signal intensity in different sequences, and contrast-enhanced T1WI displayed a fast in and fast out enhancement pattern. These distinct imaging features contribute to the diagnosis of hepatic PEComas and distinguish it from hepatocellular carcinoma.
FRONTIERS IN MEDICINE
(2023)
Article
Oncology
Hui Yan, Shuhui Zhang, Ying Ba, Kun Li, Guoling Gao, Yanmin Li, Yan Zhang, Chengxia Liu, Ning Shi
Summary: This article reports a case of sigmoid colon PEComa in a middle-aged female. Immunohistochemistry analysis showed the expression of HMB-45, a specific marker of PEComa. Surgical resection is the primary treatment, but close follow-up is necessary after surgery.
FRONTIERS IN ONCOLOGY
(2023)
Review
Oncology
Jennifer A. Bennett, Esther Oliva
Summary: PEComas of the female genital tract are rare mesenchymal neoplasms that closely resemble smooth muscle tumors, making their diagnosis and treatment challenging. Molecular analysis plays a key role in distinguishing between different molecular groups and guiding targeted therapy options.
GENES CHROMOSOMES & CANCER
(2021)
Article
Medicine, General & Internal
Gurneel K. Dhanesar, Harish Rengarajan, Baidarbhi Chakraborty
Summary: PEComa, a type of soft tissue tumor, is associated with TSC1 and TSC2 gene mutations, leading to hyperactivation of the mTOR signaling pathway. Clinical symptoms commonly include postmenopausal abnormal uterine bleeding and pelvic pain. Surgical resection is the mainstay of treatment.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Adrien Holzgreve, Matthias P. Fabritius, Thomas Knoesel, Bernhard W. Renz, Lars H. Lindner, Dorit Di Gioia, Peter Bartenstein, Johannes Ruebenthaler, Reinhold Tiling
Summary: This case study highlights the treatment process of a 38-year-old woman with a rare perivascular epithelioid cell tumor of the gastrointestinal tract, demonstrating both the role and limitations of F-18-FDG PET/CT in monitoring disease progression.
CLINICAL NUCLEAR MEDICINE
(2021)
Review
Oncology
Roli Purwar, Kishan Soni, Mridula Shukla, Ashish Verma, Tarun Kumar, Manoj Pandey
Summary: PEComas are a rare type of tumor characterized by expression of muscle and melanocytic markers. TFE3-associated PEComas are a new variant that lack response to targeted mTOR inhibitor therapy. The patient in the case showed excellent response to Everolimus therapy.
WORLD JOURNAL OF SURGICAL ONCOLOGY
(2022)
Review
Obstetrics & Gynecology
Yanping Jiang, Xiaoqin Liu, Sai Zhang, Qian Wang, Qian Xu, Kulsoom Ghias, Lanqin Cao
Summary: This study aims to understand the risk stratification and outcomes of gynecologic PEComas. The results showed that tumor size, growth pattern, mitotic rate, nuclear grade and cellularity were significantly related to recurrence and/or metastasis of gynecologic PEComas. Nuclear grade and cellularity were identified as independent risk factors for R/M.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
Article
Medicine, General & Internal
Rafey Rehman, Mariam Aoun, Ronald Levitin, Thomas Quinn, Peyman Kabolizadeh
Summary: PEComas are rare mesenchymal tumors that typically occur in the uterus but can also arise in other locations such as the buttock region.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Medicine, General & Internal
Muhammad Haseeb, Priyanka Sachdev, Mary Sravani, Chandana Tadigotla, Naga Anjani Bhaskar Srinivas Sunkara, Nikhil Gadyalpatil
Summary: Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor that is challenging to diagnose clinically. This article reports a peculiar case of PEComa treated with mTOR inhibitors, and the tumor completely regressed after treatment.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Medicine, Research & Experimental
Jacques Greenberg, Jessica Limberg, Akanksha Verma, David Kim, Xiang Chen, Yeon J. Lee, Maureen D. Moore, Timothy M. Ullmann, Jessica W. Thiesmeyer, Zachary Loewenstein, Kevin J. Chen, Caitlin E. Egan, Dessislava Stefanova, Rohan Bareja, Rasa Zarnegar, Brendan M. Finnerty, Theresa Scognamiglio, Yi-Chieh Nancy Du, Olivier Elemento, Thomas J. Fahey III, Irene M. Min
Summary: This study investigates the therapeutic options for metastatic pancreatic neuroendocrine tumors (PNETs). The results suggest that remodeling the tumor microenvironment (TME) may enhance susceptibility to immunotherapy. The TMEs of localized and metastatic PNETs were analyzed, revealing activation of inflammatory and immune-related pathways in metastatic tumors. Additionally, a histone deacetylase inhibitor, vorinostat, was shown to increase chemokine expression and enhance recruitment of T cells in PNET tissues.
Article
Medicine, General & Internal
Yan-Qi Kou, Yu-Ping Yang, Wei-Xiang Ye, Wei-Nan Yuan, Shen-Shen Du, Biao Nie
Summary: This paper reports three cases of hepatic perivascular epithelioid cell neoplasms (PEComas), including one case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. These cases were initially misdiagnosed as hepatocellular carcinoma (HCC) but were confirmed as hepatic PEComa postoperatively. Hepatic PEComa is often misdiagnosed as HCC, especially when combined with tumors in other organs. The patients eventually underwent partial hepatectomy and experienced no recurrence or metastases during follow-up of 1-4 years.
WORLD JOURNAL OF CLINICAL CASES
(2023)
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Rui Sun, Guangping Zeng, XiaoLiang Chen
Summary: We present the Ga-68-DOTATATE PET/CT findings of a 55-year-old female with pancreatic perivascular epithelioid cell tumor. Ga-68-DOTATATE PET/CT showed increased radioactivity in the pancreatic body region, suggesting a malignant tumor. However, postoperative pathological findings confirmed the presence of perivascular epithelioid cell tumor. This case highlights the importance of considering this tumor in the differential diagnosis of pancreatic nodules with moderate DOTATATE activity.
CLINICAL NUCLEAR MEDICINE
(2023)
Article
Oncology
Yuya Izubuchi, Shunsuke Hamada, Yoshikazu Tanzawa, Ikuo Fujita, Jungo Imanishi, Hirotaka Koyanagi, Akiyoshi Shimatani, Tadashi Komatsubara, Takaaki Tanaka, Mana Fukushima, Yoshiaki Imamura, Takafumi Ueda, Hirotaka Kawano, Akihiko Matsumine
Summary: This study investigated the clinical and pathological characteristics of PEComas in bone and soft tissues by leveraging information from the Japanese Musculoskeletal Oncology Group. It found that bone and soft tissue PEComas may have a higher malignancy potential than other visceral PEComas and are more likely to develop as TFE3-rearranged PEComas.
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
(2023)