Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET. The remaining 4 PNETs were composed entirely of undifferentiated small round blue cells and were classified as Ewing sarcoma/peripheral PNET. Eight PNETs were associated with another tumor type, including 5 ovarian mature cystic teratomas, 2 endometrial low-grade endometrioid carcinomas and a uterine carcinosarcoma. By immunohistochemistry, 17 PNETs expressed at least 1 marker of neuronal differentiation, including synaptophysin, NSE, CD56, S100, and chromogranin in 10, 8, 14, 8, and 1 tumors, respectively. GFAP was positive in 4 PNETs, all of which were of central type. Membranous CD99 and nuclear Fli-1 staining was seen in 10 and 16 tumors, respectively, and concurrent expression of both markers was seen in both central and Ewing sarcoma/peripheral PNETs. All tumors expressed vimentin; while keratin cocktail (CAM5.2, AE1/AE3) staining was only focally present in 4 PNETs. Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression. In conclusion, central and Ewing sarcoma/peripheral PNETs may be encountered in the female genital tract with central PNETs being more common. Central PNETs show a spectrum of morphologic features that overlaps with CNS tumors but lack EWSR1 rearrangements. GFAP expression supports a morphologic impression of central PNET and is absent in Ewing sarcoma/peripheral PNET. Ewing sarcoma/peripheral PNETs lack morphologic features of CNS tumors.
The term "MELF-pattern myometrial invasion" (MELF pattern) denotes an unusual morphology of myometrial invasion in endometrioid carcinomas, and is associated with frequent lymphovascular invasion and lymph node metastasis. In this study, tumor cells were directly collected from a MELF pattern site, using laser microdissection. Comprehensive microarray analysis of the genes was conducted, and based on the results, expression of a metastasis progression gene, CXCR4, and its ligands CXCL14 and CXCL12, was further investigated. In vitro studies of endometrioid carcinoma cell lines revealed elevated invasion activity in a manner dependent on the CXCL14-CXCR4 or CXCL12-CXCR4 axis. Immunohistochemical analysis of 93 (MELF group, 46; non-MELF group, 47) cases illustrated CXCR4 was expressed in all endometrioid carcinomas, while based on CXCL14 and CXCL12 expression score, high proportions of cells were positive at the sites of the MELF pattern (P<0.01). There was no significant difference in progression-free survival or overall survival between MELF group and non-MELF group by Kaplan-Meier analysis. These findings suggest a possibility that cells at the sites of MELF pattern had acquired increased invasiveness through the function of the CXCL14-CXCR4 and CXCL12-CXCR4 axes.
We present a case of a 34‐year‐old pregnant woman with a prior cesarean delivery presenting with placenta previa. Placenta previa accreta was diagnosed from missing decidual flow signals using superb microvascular imaging (SMI). At 31 weeks' gestation, B‐mode ultrasonography showed that the placenta was attached to the anterior uterine segment, extending over the internal cervical os. In normally appearing myometrium, SMI demonstrated double layers of flow signals underneath the placental basal plate, corresponding to myometrial and decidual flows. The thin myometrium located on the bladder where sonolucent zones were not visible revealed three different flow patterns in the Doppler signals underneath the basal plate as follows: double layers (both myometrium and decidual tissues present); a single layer (myometrium alone) or no layers (decidual tissues missing). A cesarean hysterectomy was performed at 37 weeks, and histology confirmed the presence of placenta accreta.
We present a patient with systemic lupus erythematosus receiving long-term steroid therapy, who had myometrial thinning, markedly thickened placenta, and fetal growth restriction (FGR). Blood flow profiles of the myometrium, decidua and placental villous vessels (VV) were described using superb microvascular imaging (SMI) at 35 weeks' gestation. Images showed no decidual blood flow underneath the placenta sitting on a thin myometrium and sparse VV distribution and non-visualization of peripheral VV flow. Emergency cesarean hysterectomy was performed at 36 weeks. Histological findings showed missing decidua on the thin myometrium, which indicated placenta accreta spectrum, and massive perivillous fibrin deposition and increased numbers of syncytial knots in the placenta. We speculated that the thick placenta and peculiar VV flow profiles resulted from congestion of the intervillous space and intervillous underperfusion/low intraplacental oxygenation, respectively, resulting in FGR. Superb microvascular imaging is useful for diagnosing placenta accreta spectrum and understanding the pathophysiology of thick placenta and FGR.
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