MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases

Ectopic pregnancy of the fundal myometrium in a patient with tuberous sclerosis

“…These benign growths may infiltrate extensively into the myometrium causing consequent loss of myometrial contraction strength and, thus promote large hemorrhagic cavities which may disrupt into the endometrial cavity. 11 We speculate that these findings alone may have predisposed our patient to a myometrial pregnancy.…”

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.…”

“…At ultrasound examination an uterine PEComa can show either a heterogeneous echotexture with no cystic areas or significant vascularity on Doppler examination and with well defined margins, similarly to a fibroid, or a hyperechogeneous aspect with no clear separation from the adjacent uterus and an extremely rich central vascular network, similarly to a leiomyosarcoma ( Socolov et al, 2016 ). Magnetic resonance [MR] imaging can better define the internal structure of the lesion, but a wide range of imaging characteristics have been reported ( Kwon et al, 2017 , Nishio et al, 2019 , Yoo-Bee et al, 2016 ). MR can display a heterogeneous mass which is hypointense on T1-weighted image and isointense or hyperintense on T2- weighted image, with significant enhancement uptake after gadolinium.Pre-contrast non-fat-suppressed and post-gadolinium fat-suppressed T1-weighted MR images can demonstrate homogeneous isointense signal of mass with heterogeneous enhancement.…”

“…MR can display a heterogeneous mass which is hypointense on T1-weighted image and isointense or hyperintense on T2- weighted image, with significant enhancement uptake after gadolinium.Pre-contrast non-fat-suppressed and post-gadolinium fat-suppressed T1-weighted MR images can demonstrate homogeneous isointense signal of mass with heterogeneous enhancement. At MR examination an uterine PEComa can appear as a well- circumscribed homogeneous submucosal mass with signal intensity or enhancement similar to that of the myometrium ( Kwon et al, 2017 ), or as a well- defined uterine wall tumor mimicking a swornstorm and avidly capturing gadolinium, or as single or multiple, irregularly shaped or lobulated hemorrhagic lesions within the myometrium that differ from typical adenomyotic cysts in their larger size and irregular margins ( Nishio et al, 2019 ). Malignant PEComas are hypodense or isodense on unenhanced CT scan, but usually present significant homogeneous or heterogeneous enhancement after contrast medium.…”

“…After a median follow-up of 19 months, 47 (69.1%) were alive with no evidence of disease, 11 (16.2%) died of disease, and the remaining were alive with disease. Additional 11 cases of uterine PECOmas were reported between 2018 and 2019, and the clinical status was known for 8 of these: 7 were free of disease after a median interval of 22 months (range, 3 to 71 months) and one died of disease 10 months after surgery ( Shan et al, 2019 , Nishio et al, 2019 , Rothenberger et al, 2019 ).…”