Broad ligament perivascular epithelioid tumour presenting with haemoperitoneum

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.…”

“…Patients usually experience vaginal bleeding, abdominal pain or discomfort or enlargement and palpable abdominal mass, ad rarely rupture of the uterus and hemoperitoneum ( Liu et al, 2019 , Musella et al, 2015 , Kovac et al, 2018 , Cho et al, 2014 , Staley et al, 2015 , Rothenberger et al, 2019 ). Rothenberger et al ( Rothenberger et al, 2019 ) reported a case of uterine PEComa in a 61-year old woman presenting with diffuse petechaie, mucosal and vaginal bleeding, severe thrombocytopenia and disseminated intravascular coagulation.…”

“…PEComas arising from the vagina, vulva, broad ligament and round ligament have been reported in 8 cases ( Folpe et al, 2005 , Schoolmeester et al, 2014 , Kalyanasundaram et al, 2005 , Ong et al, 2007 , Schoolmeester et al, 2015 , Cho et al, 2014 ), one case ( Tazelaar et al, 2001 ), 5 cases ( Rao et al, 2013 , Folpe et al, 2005 , Fink et al, 2004 , Kim et al, 2006 , Staley et al, 2015 ) and 2 cases ( Phongnarisorn et al, 2009 , Nitahara et al, 2019 ), respectively.…”

“…As for the patients with PEComas of the broad ligament, a 51-year-old woman treated with total hysterectomy, bilateral salpingo-oophorectomy, omentectomy and pelvic radiotherapy was free of disease 15 months after diagnosis ( Fink et al, 2004 ), a 33-year old woman with a mass compressing / infiltrating distal left ureter was free of disease 24 months after tumor excision and ureteric reimplantation ( Nitahara et al, 2019 ), whereas a 12- year old girl recurred in the form of multiple masses in the right iliac fossa within 12 months from surgery ( Kim et al, 2006 ). A 44-year-old patient presenting with hemoperitoneum underwent subtotal hysterectomy, bilateral salpingo-oophorectomy and excision of the broad ligament mass, but no follow-up data were given ( Staley et al, 2015 ). A 45-year-old woman was free of disease 18 months after laparoscopic radical excision of primary round ligament PEComa mimicking a leiomyoma ( Phongnarisorn et al, 2009 )…”

Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

“…The uterine corpus is the most common site for gynecologic PEComas; however, they have also been reported in the cervix, 4‐18 vagina, 5,19‐26 broad ligament, 5,27‐33 ovary, 23,34‐38 and rarely, the vulva 39 . Overall, most gynecologic PEComas present in patients in their fifth to sixth decades (range 6‐79 years), 5,17,23,24,40‐42 but those in the vagina, broad ligament, and vulva may occur at an earlier age (mean 28, 25, and 20 years, respectively) 43 .…”