Perivascular epithelioid cell tumors of the uterine cervix

Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist

“…PEComas of the female genital tract account for nearly 25% of all PEComas of all body sites e, and the original site of the tumor is the uterine corpus in most cases ( Folpe et al, 2005 , Fadare, 2008 , Conlon et al, 2015 , Acosta, 2017 , Bennett et al, 2018 , Liu et al, 2019 , Schoolmeester et al, 2014 , Fukunaga, 2005 , Liu et al, 2009 , Musella et al, 2015 , Choi et al, 2016 , Kwon et al, 2017 , Shan et al, 2019 , Nishio et al, 2019 ), the cervix rarely ( Wagner et al, 2010 , Folpe et al, 2005 , Conlon et al, 2015 , Liu et al, 2019 , Bradshaw et al, 2010 , Yamamoto et al, 2010 , Stone et al, 2013 , Natella et al, 2014 , Kudela et al, 2016 , Kovac et al, 2018 ), and adnexa ( Conlon et al, 2015 , Liu et al, 2019 , Schoolmeester et al, 2014 , Anderson et al, 2002 , Ramaiah et al, 2009 , Westaby et al, 2017 , Rampisela et al, 2016 ), vagina/vulva ( Tazelaar et al, 2001 , Folpe et al, 2005 , Conlon et al, 2015 , Liu et al, 2019 , Schoolmeester et al, 2014 , Kalyanasundaram et al, 2005 , Ong et al, 2007 , Schoolmeester et al, 2015 , Cho et al, 2014 ) or broad or round ligament ( Rao et al, 2013 , Folpe et al, 2005 , Conlon et al, 2015 , Fink et al, 2004 , Kim et al, 2006 , Phongnarisorn et al, 2009 , Staley et al, 2015 ) exceptionally.…”

“…Although there is no consensus regarding the management of gynecological PEComas owing to their rarity, complete surgical resection with a tumor-free margin is usually considered to be the standard treatment ( Liu et al, 2019 , Musella et al, 2015 , Kwon et al, 2017 , Shan et al, 2019 , Kudela et al, 2016 , Kovac et al, 2018 , Fink et al, 2004 ), whereas only anecdotal and inconclusive data are available as for adjuvant radiotherapy ( Fink et al, 2004 ) or chemotherapy ( Shan et al, 2019 ). These tumors tend to recur both locally and in distant sites, especially in the lung, even many years after initial surgery ( Greene et al, 2003 , Armah and Parwani, 2007 ).…”

“…Fifteen cases of cervical PEComas have described in the literature, and their surgical approach ranged from tumor resection to total hysterectomy or radical hysterectomy with bilateral salpingectomy-oophorectomy and pelvic lymphadenectomy to pelvic exenteration ( Wagner et al, 2010 , Folpe et al, 2005 , Conlon et al, 2015 , Liu et al, 2019 , Bradshaw et al, 2010 , Yamamoto et al, 2010 , Stone et al, 2013 , Natella et al, 2014 , Kudela et al, 2016 , Kovac et al, 2018 ). The clinical status is known for 11 of these.Two patients treated with tumor resection developed recurrent disease in the cervix after four months ( Yamamoto et al, 2010 ).…”

Perivascular epithelioid cell tumour and mesonephric adenocarcinoma of the uterine cervix: an unknown co-existence

“…The existence of a perivascular epithelioid cell tumour (PEComa) with a mesonephric adenocarcinoma (MNA) of the cervix is extremely exceptional. The first case of cervical PEComa was reported in 2004 and to the best of our knowledge there are only 15 cases of this tumour described in the published literature including the present case [1–6]. A recent literature review has reported that since 1962 there have been only 40 cases of MNA of the cervix described to date [7].…”

Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge

“…The vast majority of patients received a total hysterectomy with or without bilateral salpingo-oophorectomy. Radical hysterectomy with bilateral salpingo-oophorectomy should be considered in patients with PEComas spreading or localized in the uterine cervix [8]. …”