Ovarian Microcystic Stromal Tumor with Intraovarian Recurrence and Peritoneal and Omental Spread: A Case Report with Morphological, Immunohistochemical, and Molecular Analysis

Microcystic stromal tumors (MCSTs) are rare ovarian stromal tumors. They harbor CTNNB1 or APC mutations, resulting in β-catenin nuclear expression. To date, all MCST cases treated with oophorectomy or more extensive surgery have followed benign clinical courses. However, 1 of the 3 cases treated with ovarian cystectomy/tumor resection recurred in the residual ovary and iliac fossa 9 years after ovarian cystectomy. Here, we report a case of recurrent MCST in a 38-year-old woman. The patient underwent ovarian cystectomy for a 7.5 cm solid-cystic right ovarian mass, which showed classic morphological and immunophenotypical features of MCST. Four years later, the tumor recurred in the residual right ovary as a 21 cm mass, involving the pelvic peritoneum and omentum. Molecular analysis using next-generation sequencing revealed a single CTNNB1 exon 3 S37A mutation in the recurrent tumor. To the best of our knowledge, this is the second case of recurrent MCST, which presents more evidence that MCST has the potential to recur and spread locally. Rather than ovarian cystectomy/tumor resection, more aggressive surgery, such as unilateral oophorectomy, may be necessary to decrease the risk of recurrence. Long-Term postsurgery follow up is needed, especially after simple ovarian cystectomy/tumor resection.