Signet ring cell-rich microcystic stromal tumor of the ovary: A poorly recognized variant

Yoshikawa, Yutaka; Nakazono, Yuichi; Hirotani, Kenichiro; Kawanaka, Hirofumi

doi:10.1016/j.ehpc.2020.200451

Cited by 3 publications

(7 citation statements)

References 28 publications

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“…The distinction between SRST and MCST is clinically important given the potential for MCST to represent a rare extracolonic manifestation of familial adenomatous polyposis (FAP) and for occasional MCST to exhibit malignant behavior, although this is rare. [35][36][37] Interestingly, the MCST with prominent signet-ring cell change described by Yoshikawa et al 29 arose in a patient with FAP. The overall findings in the above 3 tumors (cases 5, 6 and 7) led us to reclassify them as MCST with signet-ring cell morphology.…”

Section: Discussionmentioning

confidence: 99%

Ovarian Signet-ring Stromal Tumor

Tchrakian

Oliva

Chong

et al. 2022

American Journal of Surgical Pathology

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Signet-ring stromal tumor (SRST) is a rare ovarian stromal neoplasm characterized by a population of bland signet-ring cells, devoid of mucin or lipid, in a generally cellular fibromatous stroma. Previous reports have described heterogenous immunohistochemical and molecular genetic findings, including occasional nuclear β-catenin expression and/or CTNNB1 mutations. We report 10 ovarian stromal neoplasms originally diagnosed as SRST. All but 1 tumor underwent detailed immunohistochemical analysis (including β-catenin) and 5 of 10 had CTNNB1 mutation analysis performed. All tumors contained a population of morphologically bland signet-ring cells that ranged from 15% to 95% of the neoplasm, characterized by a single large empty intracytoplasmic vacuole, mostly with nuclear indentation. Six of the 10 tumors contained cellular fibroma-like areas, comprising from 10% to 85% of the neoplasm. Three of the 10 tumors were reclassified as microcystic stromal tumor with signet-ring cells on the basis of the microcyst formation and hyalinized stroma, beta-catenin and cyclin D1 nuclear expression and/or CTNNB1 mutation, CD10 staining and largely absent expression of inhibin and calretinin. In the remaining 7 tumors, the diagnosis of SRST remained, constituting the largest series of SRST reported in the literature to date. The results of our study suggest that a subset of tumors diagnosed as ovarian SRST, especially those which show β-catenin nuclear positivity and/or CTNNB1 mutation, likely represent microcystic stromal tumor with variant morphology. We also suggest that at least a subset of SRSTs without evidence of Wnt/β-catenin pathway abnormalities may be related to ovarian fibromas. We discuss the differential diagnosis of ovarian neoplasms containing signet-ring cells.

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Section: Discussionmentioning

confidence: 99%

Ovarian Signet-ring Stromal Tumor

Tchrakian

Oliva

Chong

et al. 2022

American Journal of Surgical Pathology

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“…The intracytoplasmic vacuoles are considered degenerative since they do not contain any mucin, lipid or glycogen. Some intracytoplasmic vacuoles can be calcified, giving a psammoma body-like appearance [ 51 ]. Tumor cells have no to minimal nuclear atypia with low mitotic activity.…”

Section: The Family Of Low-grade Ovarian Stromal Tumors With β-Cateni...mentioning

confidence: 99%

“…Immunohistochemically, tumor cells are positive for CD10, cyclin D1 and vimentin and variably positive for WT1, FOXL2, and SF1. Tumor cells are usually negative for epithelial membrane antigen, pan-cytokeratin, smooth muscle actin, inhibin, and calretinin [ 51 , 52 , 53 ]. The pathogenesis of SRSCT of the ovary is largely unknown.…”

Section: The Family Of Low-grade Ovarian Stromal Tumors With β-Cateni...mentioning

confidence: 99%

“…However, some authors speculated that SRSCT might be derived from ovarian fibroma [ 54 ]. However, a recent study failed to identify reticulin fibers individually wrapping around tumor cells, the histochemical staining pattern typically seen in ovarian fibroma [ 51 ]. In another recently reported case, the aberrant nuclear β-catenin expression resulted from the oncogenic mutation of the CTNNB1 gene at exon 3, along with a germline missense mutation in Janus kinase 3 ( JAK3 ) gene [ 55 , 56 ].…”

Section: The Family Of Low-grade Ovarian Stromal Tumors With β-Cateni...mentioning

confidence: 99%

“…As mentioned above, the aberrant nuclear and cytoplasmic immunostaining pattern of β-catenin is the most striking immunohistochemical phenotype found in ovarian SPPN, MCST, and SRSCT. Immunohistochemical detection of nuclear and cytoplasmic accumulation of β-catenin has much higher sensitivity compared to molecular testing because mutations in several Wnt signaling genes, including CTNNB1 and APC , can result in the same aberrant expression pattern of β-catenin protein [ 24 , 44 , 51 ].…”

Section: The Family Of Low-grade Ovarian Stromal Tumors With β-Cateni...mentioning

confidence: 99%

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Low-Grade Ovarian Stromal Tumors with Genetic Alterations of the Wnt/β-Catenin Pathway That Is Crucial in Ovarian Follicle Development and Regulation

Zhang

Michener

Yang

2022

Cancers

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The Wnt signaling pathway is important in the normal development and regulation of ovarian follicles throughout the lifecycle of females. Dysregulation of the Wnt signaling pathway, genetically or epigenetically, with subsequent activation of β-catenin has been implicated in tumorigenesis of a spectrum of ovarian neoplasms, from benign to malignant. We review the recent findings of the Wnt signaling pathway involved in regulating normal physiologic processes of the ovarian follicle cycle. We also review the β-catenin mutations in a family of low-grade ovarian stromal tumors, focusing on characterizing their shared morphological features and the utility of immunohistochemistry of β-catenin in facilitating the accurate diagnosis of these ovarian stromal tumors. The Wnt signaling pathway is one of the most critical mechanisms in regulating cell proliferation, differentiation, and morphogenesis. The Wnt signaling pathway comprises a diverse group of glycoproteins that serve as ligands and bind to transmembrane Frizzled family receptors. The ligand-receptor interactions activate the pathway and govern the downstream signaling cascades, ultimately affecting the transcriptional control of the cellular cytoskeleton, organelle dynamics, epithelial-mesenchymal interaction, and tissue remodeling in the ovary. Wnt signaling consists of two major pathways: a canonical pathway that is β-catenin-dependent and a non-canonical Wnt pathway that is β-catenin-independent. Canonical Wnt signaling is governed by the interaction of β-catenin with other molecules to regulate cellular decisions related to proliferation and differentiation. Recent studies have demonstrated that the Wnt signaling pathway plays important roles in the development and regulation of ovarian folliculogenesis and oogenesis.

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

Pongsuvareeyakul

Kingnate

Sukpan

et al. 2022

Case Reports in Pathology

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Microcystic stromal tumor (MST) is a rare type of pure stromal tumor in the category of ovarian sex cord-stromal tumors. It is characterized by a distinctive microcystic appearance with bland tumor cells. Although the pathological diagnosis can be straightforward based on the typical histomorphology in most MSTs, the cases with morphologic variation can pose a diagnostic challenge due to unfamiliarity of pathologists with the histologic spectrum of MST and its negativity for inhibin and calretinin, the commonly used sex cord-stromal markers. The coexistence between MST and mucinous epithelial tumor is extremely rare. We present the first case, to our knowledge, of ovarian MST with predominant bizarre nuclei coexisting with mucinous cystadenoma in a pregnant woman. The histomorphology in this case presents a diagnostic challenge and raises differential diagnosis for a wide variety of ovarian malignant neoplasms including nonneoplastic lesions.

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Signet ring cell-rich microcystic stromal tumor of the ovary: A poorly recognized variant

Cited by 3 publications

References 28 publications

Ovarian Signet-ring Stromal Tumor

Ovarian Signet-ring Stromal Tumor

Low-Grade Ovarian Stromal Tumors with Genetic Alterations of the Wnt/β-Catenin Pathway That Is Crucial in Ovarian Follicle Development and Regulation

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

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