A Rare Ovarian Tumor: The Sclerosing Stromal You Do Not Expect-A Case Series in the Adolescent Population and a Literature Review

Abstract

Sclerosing stromal tumor (SST) is a rare ovarian tumor arising from the sex cord-stromal cells that occurs mainly in young adults during the second and third decades of life and rarely in pediatric and adolescent populations. The objective of this study is to report three illustrative cases of SST in young girls who had undergone surgery at our clinic in or after 2009, and to perform a literature review of this rare ovarian tumor. A retrospective chart review of female patients aged <18 years with a diagnosis of SST treated in a tertiary pediatric hospital was performed. Furthermore, a 10-year review of the SST literature was completed. Three cases of SST at our institution were outlined. After reviewing the literature, 18 SST cases were identified. The mean age at diagnosis was 13.4 years, and the reported clinical presentations were abdominal or pelvic pain and menstrual irregularity. Seven patients had abnormal hormone tests or CA-125 levels. In approximately 30% of cases, conservative surgery was performed, preserving residual ovarian tissue. In conclusion, some preoperative findings may help in suggesting the presence of SST. However, definitive diagnosis can only be made by histopathological examination. It is important to consider this tumor because, given its benign behavior, a conservative approach is preferred, particularly in this age group.

Keywords: adolescent; fertility sparing; ovarian sex cord-stromal tumor; ovarian tumor; pediatric; sclerosing stromal tumor.

Figure 1

(A) Ultrasound image reveals a solid mass with focal hypoechoic components, possibly due to necrosis; (B) Doppler US image reveals prominent peripheral vasculature; (C) MR images show an uneven expansive mass in the right paramedian pelvic region, which dislocates the uterus. The arrow indicates the formation found on the MRI.

Figure 1

(A) Ultrasound image reveals a solid mass with focal hypoechoic components, possibly due to necrosis; (B) Doppler US image reveals prominent peripheral vasculature; (C) MR images show an uneven expansive mass in the right paramedian pelvic region, which dislocates the uterus. The arrow indicates the formation found on the MRI.

Figure 2

(A) Spindle and round cells intermingled together in a hyaline stroma (asterisk) (HE, 40×); (B) Fascicular spindle cell proliferation (HE, 63×); (C) Proliferation of round, epithelioid clear cells (arrowhead) (HE 63×); (D) A clear gelatinous area is observed (arrow) (HE, 5×); (E) A rim of residual ovarian parenchyma is shown in the upper part of the picture (rectangle) (HE, 10×); (F) Alternation of hyper- and hypocellular areas in the same tumor (“dark” and “clear” proliferation pattern) (HE, 5×).