Ovarian microcystic stromal tumor with omental metastasis: the first case report and literature review

Abstract

Background Microcystic stromal tumor (MCST) of the ovary is an extremely rare subtype of sex cord-stromal neoplasm first described by Irving and Young in 2009. Tumors from all previously reported cases (fewer than 40 total) were benign, but one was a case of ovarian MCST that reoccurred. Case presentation Herein, we present a unique single case of ovarian MCST with omental metastasis in a 47-year-old Chinese female along with its histologic and immunohistochemical profile and genetic alterations. The tumor exhibited the previously described classic microscopic features and immunoprofiles of MCST. The tumorlet in the omentum presented the same histological structures and characteristically expressed β-catenin protein (localized in the nucleus). Molecular analysis identified a point mutation (c.98C > G) in exon 3 of CTNNB1. Conclusions To the best of our knowledge, no such report has been documented for ovarian MCST with omental metastasis. The study may provide new insights into the tumor biology of MCST and provide a better understanding of this rare entity.

Microcystic Stromal Tumor with Predominant Bizarre Nuclei of Ovary in a Pregnant Woman

“…Although the majority of MST patients had a benign clinical course, the understanding of the clinical behavior of this tumor may be limited by its rarity and the lack of follow-up in some cases [ 2 ]. Recently, two cases of MST with recurrence or extraovarian spread have been reported [ 13 , 14 ]. Tumors in both cases were found in the reproductive age group and exhibited classical morphologic and immunophenotypic features.…”

“…In one patient, recurrence was detected in bilateral ovaries and bilateral iliac fossa 9 years after cystectomy for MST [ 13 ]. In the other patient, microscopic spread in the omentum was detected at the diagnosis of MST [ 14 ]. These findings may raise the possibility for the uncertain malignant potential of this tumor [ 13 , 14 ].…”

“…In the other patient, microscopic spread in the omentum was detected at the diagnosis of MST [ 14 ]. These findings may raise the possibility for the uncertain malignant potential of this tumor [ 13 , 14 ]. Further molecular study of additional cases is required to warrant the biologic and clinical behavior of MST.…”

Ovarian microcystic stromal tumour: from morphological observations to syndromic associations

“…The second was a 47‐year‐old patient, who presented with a complex left ovarian mass and underwent hysterectomy, bilateral salpingo‐oophorectomy and peritoneal staging biopsies. Pathological examination revealed a 9.5‐cm left ovarian MST and a microscopic (2 mm) metastatic tumour deposit in the omentum; the ovarian tumour had a c.98C>G missense mutation in exon 3 of CTNNB1 15 . When reporting these tumours, it can be stated that while these are usually benign neoplasms, this is not invariably so.…”

“…Pathological examination revealed a 9.5-cm left ovarian MST and a microscopic (2 mm) metastatic tumour deposit in the omentum; the ovarian tumour had a c.98C>G missense mutation in exon 3 of CTNNB1. 15 When reporting these tumours, it can be stated that while these are usually benign neoplasms, this is not invariably so.…”