VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature

被引:16
作者
Liapi, Aikaterini [1 ]
Mathevet, Patrice [2 ]
Herrera, Fernanda G. [1 ]
Hastir, Delfyne [3 ]
Sarivalasis, Apostolos [1 ]
机构
[1] Ctr Hosp Univ Vaudois CHUV, Dept Oncol, Lausanne, Switzerland
[2] Ctr Hosp Univ Vaudois CHUV, Dept Gynecol, Lausanne, Switzerland
[3] CHU Vaudois, Inst Univ Pathol, Lausanne, Switzerland
关键词
uterine perivascular epithelioid cell tumor; soft tissue sarcomas; mTOR; second line; VEGFR; PEComa;
D O I
10.3389/fonc.2021.641376
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms. PI3K/AKT/mTOR pathway upregulation is critical for their pathogenesis and is often associated with TSC1/TSC2 inactivation. Although first line mTOR inhibitors are an effective treatment, metastatic PEComas eventually progress. A 53-year-old woman presented a 4-month history of post-menopausal vaginal bleeding. Clinical and radiological examination detected a uterine mass and a single S1 bone lesion. The patient underwent a radical hysterectomy and bone biopsy. The anatomopathological evaluation concluded to an oligo-metastatic uterine PEComa. The tumor harbored a heterozygous deletion of 9q34 that contains the TSC1 gene. Concerning the primary lesion, the resection was complete and the single bone metastasis was treated with radiotherapy. Three months later, the patient presented bone, lung and subcutaneous metastatic progression. An everolimus and denosumab treatment was initiated. After 2 years of treatment, a clinically significant bone, lung and subcutaneous progression was detected. Following a literature review of the possible therapeutic options, we initiated a second line treatment by pazopanib. This treatment resulted in regression of the subcutaneous lesions and stability of lung and bone metastases. In this challenging, rare setting, our report suggests single agent, anti-angiogenic, tyrosine kinase inhibitor to be effective as second line treatment of metastatic uterine PEComa progressing on mTOR inhibitors.
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页数:7
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共 35 条
[1]   Metastatic Perivascular Epithelioid Cell Tumor Of The Kidney: A Case Report With Emphasis On Response To The Tyrosine-Kinase Inhibitor Sunitinib [J].
AlAzab, Rami S. ;
Alorjani, Mohammed S. ;
Sahawneh, Firas E. ;
Al-Sukhun, Sana .
RESEARCH AND REPORTS IN UROLOGY, 2019, 11 :311-317
[2]   High cumulative incidence of uterine leiomyoma in black and white women: Ultrasound evidence [J].
Baird, DD ;
Dunson, DB ;
Hill, MC ;
Cousins, D ;
Schectman, JM .
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 2003, 188 (01) :100-107
[3]   Uterine PEComas A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors [J].
Bennett, Jennifer A. ;
Braga, Ana C. ;
Pinto, Andre ;
Van de Vijver, Koen ;
Cornejo, Kristine ;
Pesci, Anna ;
Zhang, Lei ;
Morales-Oyarvide, Vicente ;
Kiyokawa, Takako ;
Zannoni, Gian Franco ;
Carlson, Joseph ;
Slavik, Tomas ;
Tornos, Carmen ;
Antonescu, Cristina R. ;
Oliva, Esther .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2018, 42 (10) :1370-1383
[4]   PEC AND SUGAR [J].
BONETTI, F ;
PEA, M ;
MARTIGNONI, G ;
ZAMBONI, G .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 1992, 16 (03) :307-308
[5]   Successful treatment with sirolimus for an angiomyolipoma mimicking renal cell carcinoma in a transplanted kidney [J].
Chiu, Hsien-Fu ;
Wen, Mei-Chin ;
Li, Jian-Ri ;
Ho, Hao-Chung ;
Shu, Kuo-Hsiung .
TRANSPLANT INTERNATIONAL, 2015, 28 (09) :1116-1120
[6]   Perivascular epithelioid tumours (PEComas) of the gynaecological tract [J].
Conlon, Niamh ;
Soslow, Robert A. ;
Murali, Rajmohan .
JOURNAL OF CLINICAL PATHOLOGY, 2015, 68 (06) :418-426
[7]   Phase II study of doxorubicin and bevacizumab for patients with metastatic soft-tissue sarcomas [J].
D'Adamo, DR ;
Anderson, SE ;
Albritton, K ;
Yamada, J ;
Riedel, E ;
Scheu, K ;
Schwartz, GK ;
Chen, H ;
Maki, RG .
JOURNAL OF CLINICAL ONCOLOGY, 2005, 23 (28) :7135-7142
[8]   Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin - A clinicoplathologic study of 26 cases and review of the literature [J].
Folpe, AL ;
Mentzel, T ;
Lehr, HA ;
Fisher, C ;
Balzer, BL ;
Weiss, SW .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2005, 29 (12) :1558-1575
[9]   Anthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma A Multi-institutional Case Series [J].
Frezza, Anna Maria ;
Jones, Robin L. ;
Lo Vullo, Salvatore ;
Asano, Naofumi ;
Lucibello, Francesca ;
Ben-Ami, Eytan ;
Ratan, Ravin ;
Teterycz, Pawel ;
Boye, Kjetil ;
Brahmi, Mehdi ;
Palmerini, Emanuela ;
Fedenko, Alexander ;
Vincenzi, Bruno ;
Brunello, Antonella ;
Desar, Ingrid M. E. ;
Benjamin, Robert S. ;
Blay, Jean Yves ;
Broto, Javier Martin ;
Casali, Paolo G. ;
Gelderblom, Hans ;
Grignani, Giovanni ;
Gronchi, Alessandro ;
Hall, Kirsten Sundby ;
Mir, Olivier ;
Rutkowski, Piotr ;
Wagner, Andrew J. ;
Anurova, Olga ;
Collini, Paola ;
Tos, Angelo P. Dei ;
Flucke, Uta ;
Hornick, Jason L. ;
Lobmaier, Ingvild ;
Philippe, Terrier ;
Picci, Piero ;
Ranchere, Dominique ;
Renne, Salvatore L. ;
Sbaraglia, Marta ;
Thway, Khin ;
Wagrodzki, Michal ;
Wang, Wei-Lien ;
Yoshida, Akihiko ;
Mariani, Luigi ;
Kawai, Akira ;
Stacchiotti, Silvia .
JAMA ONCOLOGY, 2018, 4 (09)
[10]   Combination targeted therapy of VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus induced a remakable response of rapid progressive Uterine PEComa [J].
Gao, Fang ;
Huang, Chengsuo ;
Zhang, Yiping ;
Sun, Ruirui ;
Zhang, Yujie ;
Wang, Huijun ;
Zhang, Shu .
CANCER BIOLOGY & THERAPY, 2016, 17 (06) :595-598