Bilateral Sclerosing Stromal Ovarian Tumor in an Adolescent

Abstract

Sclerosing stromal tumor of the ovary is a rare, benign, sex cord stromal tumor occurring predominantly in younger women in the 2nd and 3rd decades of life. It typically presents unilaterally with only 2 previously reported cases of bilateral presentation. Common clinical presentations include pelvic or abdominal pain, a mass, or menstrual changes. Although occasionally presenting with hormonal manifestations, virilization as a result of androgen production by the tumor is rare. Here we present an extremely rare case of a sclerosing stromal ovarian tumor in a 14-year-old patient with bilateral presentation and with clinical and biochemical evidence of hyperandrogenemia.

Imaging characteristics of ovarian sclerosing stromal tumor

“…However, in the delayed phase, the area of the prolonged and progressive enhancement observed in the inner zone of mass was considered to be associated with the collagenized hypocellular area, which showed hyperintensity on T2WI [6]. The present imaging findings were in line with the previous studies [6,8,[22][23][24]. Thus, the occurrence of early or prolonged enhancing was dependent primarily on whether the vascular component or collagen component was dominant in the tumor.…”

Sclerosing stromal tumour: a clinicopathological study of 100 cases of a distinctive benign ovarian stromal tumour typically occurring in the young

“…5,6 There has, however, been relatively scant literature in the last few decades on SST itself. Sporadic case reports have described examples with associated hormonal manifestations [7][8][9][10] or Meigs' syndrome, 11,12 variant morphology in pregnant patients, 13 bilaterality, [14][15][16] increased mitoses 17 and the possibility that ovarian myxomas may actually represent markedly myxoid SSTs. 18,19 Most recently, the genetic underpinnings of SST were elucidated when they were discovered to harbour recurrent GLI2 rearrangements, with FHL2 being the most common partner.…”

Bilateral ovarian fibromas in a 15-year-old primary amenorrhea patient: a case report

“…When interpreting the radiological picture based on literary sources [ 7 , 20 ], a sclerosing multifocal ovarian tumor was initially suspected. Both types of tumors—sclerosing stromal tumor and thecoma-fibroma, belong to sex cord stromal tumors and consist of fibrous stroma, but are not synonymous [21] .…”

“…Radiologically, fibroma is described to typically show low signal intensity on T1-weighted MR images, and marked hypointensity on T2-weighted images [3] ; the contrast medium enhancement of fibromas is heterogeneous and mild-to moderate [ 22 , 23 ]. The sclerosing stromal tumor also shows hypo- or isointensity, compared to normal muscles in T1-weighted images, and intermediate to low signal intensity in T2-weighted images; however, on postcontrast images, a typical early intensive peripheral enhancement with centripetal progression and hypovascularized central area is present [ 3 , 7 ]. In the case of our patient, the T1 post-contrast image showed intensive contrast enhancement, along with several hypovascularized regions, the largest of which was 5 cm in diameter.…”

“…In the T1 gadolinium enhanced series, the tumors showed intensive enhancement with several avascular regions within the largest mass. It was concluded that the pelvic masses were fibrotic in nature, most likely, a bilateral sclerosing multifocal ovarian tumor—a rare stromal tumor presenting with bilateral solid masses [7] . Further abdominal MRI and native chest CT scans excluded tumor dissemination.…”