Cancers, Vol. 15, Pages 5864: Relevance of Molecular Pathology for the Diagnosis of Sex Cord & ndash;Stromal Tumors of the Ovary: A Narrative Review

Cancers, Vol. 15, Pages 5864: Relevance of Molecular Pathology for the Diagnosis of Sex Cord–Stromal Tumors of the Ovary: A Narrative Review Cancers doi: 10.3390/cancers15245864 Authors: Alexis Trecourt Marie Donzel Nadjla Alsadoun Fabienne Allias Mojgan Devouassoux-Shisheboran Ovarian sex cord–stromal tumors (SCSTs) account for 8% of all primary ovarian neo-plasms. Accurate diagnosis is crucial since each subtype has a specific prognostic and treatment. Apart from fibrosarcomas, stromal tumors are benign while sex cord tumors may recur, sometimes with a significant time to relapse. Although the diagnosis based on morphology is straightforward, in some cases the distinction between stromal tumors and sex cord tumors may be tricky. Indeed, the immunophenotype is usually nonspecific between stromal tumors and sex cord tumors. Therefore, molecular pathology plays an important role in the diagnosis of such entities, with pathognomonic or recurrent alterations, such as FOXL2 variants in adult granulosa cell tumors. In addition, these neoplasms may be associated with genetic syndromes, such as Peutz–Jeghers syndrome for sex cord tumors with annular tubules, and DICER1 syndrome for Sertoli–Leydig cell tumors (SLCTs), for which the pathologist may be in the front line of syndromic suspicion. Molecular pathology of SCST is also relevant for patient prognosis and management. For instance, the DICER1 variant is associat...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research