Soft Tissue Lesions Involving Female Reproductive Organs

Fetsch, John F.; Laskin, William B.

doi:10.1007/978-3-319-46334-6_22

Cited by 3 publications

(21 citation statements)

References 418 publications

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“…This aspect has been confirmed in our study, with 68% of all cases in which the HP features were mentioned, having had a central-type morphology. Although most authors claim that one can encounter these tumors in a wide range of age (between the second and the eight decade) [ 2 ], the average age in our study was 31.55 years, with most (79.41%) patients being situated in the 15–40 years interval.…”

Section: Clinical Aspectsmentioning

confidence: 65%

“…The former variant resembles CNS tumors from HP point of view, while pPNET is a small round blue cell tumor, which frequently features EWS breakpoint region 1 (EWSR1) gene rearrangements. The pathogenesis of these tumors is still unclear, but most authors agree that most of the cPNETs feature a germ cell origin [ 2 ]. Multiple authors consider that cPNETs stem from a preexistent teratoma, which features central-type nervous tissue [ 28 ].…”

Section: Discussionmentioning

confidence: 99%

“…The primitive type of cPNET has a more immature aspect and is usually characterized by hypercellularity and can feature different morphological aspects, ranging from medulloepithelioma, neuroblastoma and medulloblastoma to ependymoblastoma [ 28 ]. The common feature was the presence of small blue hyperchromatic cells with little cytoplasm ranging from clear to eosinophilic, which are arranged in a lobular configuration [ 2 ]. The neoplastic cells were accompanied by fine fibrillar process and various amounts of necrosis [ 7 ].…”

Section: Histopathological Aspectsmentioning

confidence: 99%

“…These entities have neuroectodermal origin and in exceptional cases may affect the gynecological tract [ 1 ]. To date, only a few cases have been reported in the scientific literature, affecting each of the following sites: vulva, vagina, uterine cervix, uterine body, ovaries, broad ligament, and rectovaginal septum [ 2 ]. Due to their exceptional appearance in the gynecological tract, their true incidence in the ovary is still unknown.…”

Section: Introductionmentioning

confidence: 99%

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Primitive neuroectodermal tumors of the ovary: a multidecade review of the scientific literature

Georgescu,

Voichiţoiu

et al. 2024

Rom J Morphol Embryol

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Primitive neuroectodermal tumor (PNET) is a general term used in scientific literature for a heterogeneous group of small round-cell malignant tumors primarily arising from neural crest cells. These are extremely aggressive neoplasms which usually occur within soft tissue or bone of young adults. Ovarian tumors composed of primitive neuroectodermal elements are extremely rare, with only few case reports in scientific literature. Due to being so exceedingly rare, PNETs are frequently misdiagnosed and there are no standard therapeutic guidelines. Young patients seem to have better prognoses and individualized strategy is recommended. Limited data suggests that various gene deletions as well as amplifications may be crucial factors for tumorigenesis and the aggressive behavior of PNET. In this paper, we performed a brief review of all cases of primary ovarian PNETs published in the scientific literature to date, in regard to their clinical, histopathological, and therapeutic aspects, with the aim to provide a more comprehensive understanding of this exceedingly rare pathology.

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Section: Clinical Aspectsmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Histopathological Aspectsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Primitive neuroectodermal tumors of the ovary: a multidecade review of the scientific literature

Georgescu,

Voichiţoiu

et al. 2024

Rom J Morphol Embryol

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“…Gynecologists may occasionally encounter soft-tissue tumors arising from nerve sheaths outside the central nervous system (i.e. peripheral-nerve-sheath tumors (PNSTs)), in the vicinity of reproductive organs in the pelvic retroperitoneum 1 . These are usually benign, although a very low risk of malignant transformation has been reported 2,3 .…”

Section: Introduction Aimmentioning

confidence: 99%

Imaging in gynecological disease (26): clinical and ultrasound characteristics of benign retroperitoneal pelvic peripheral‐nerve‐sheath tumors

Fischerová

Santos²,

Wong

et al. 2023

Ultrasound in Obstet & Gyne

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ObjectiveTo describe the clinical and sonographic characteristics of benign, retroperitoneal, pelvic peripheral nerve sheath tumors (PNST).MethodsThis was a retrospective, single, gynecologic oncology center study conducted between 1 January 2018 and 31 August 2022. All ultrasound images, clips, and final specimens of benign PNSTs were reviewed by the authors to describe (1) the ultrasound appearance of the tumors using the terminology of the International Ovarian Tumor Analysis (IOTA), Morphological Uterus Sonographic Assessment (MUSA) and Vulvar International Tumor Analysis (VITA) groups on a predefined ultrasound assessment form, (2) the origin of the tumors in relation to nerves and pelvic anatomy, and (3) the correlation between ultrasound features and histotopograms. A review of literature on benign, retroperitoneal, pelvic PNSTs with preoperative ultrasound examination was performed.ResultsFive women (mean age 53 years) with benign, retroperitoneal, pelvic PNSTs were identified: four with schwannomas and one with a neurofibroma, all of which were sporadic and solitary. All patients had good quality ultrasound images and clips and final biopsies of surgically excised tumors, except for one patient managed conservatively with a tru‐cut biopsy. In four of these cases, the findings were incidental. Size range for the five PNSTs was from 31‐50 mm. All five PNSTs were solid, moderately vascular tumors, with non‐uniform echogenicity, well‐circumscribed by hyperechogenic epineurium, and had no acoustic shadowing. Most of the masses were round (n = 4 (80%)), and contained small, irregular, anechoic, cystic spaces (n = 3 (60%)), and hyperechoic areas (n = 4 (80%)). A literature search identified 47 cases of retroperitoneal schwannomas and neurofibromas, the characteristics of which were compared with the cases in our series.ConclusionsOn ultrasound, benign PNSTs were solid, non‐uniform, moderately vascular tumors without acoustic shadowing. Most were round, containing small, irregular, anechoic, cystic spaces, and hyperechoic areas, consistent with degenerative changes on pathology. All tumors were well‐circumscribed by a hyperechogenic rim composed of epineurium. No imaging characteristics reliably differentiated between schwannomas and neurofibromas. In fact, they overlap with the ultrasound appearance of malignant tumors. Hence, ultrasound‐guided biopsy plays a pivotal role in diagnosis, and if confirmed as benign PNSTs, these tumors can undergo ultrasound surveillance.This article is protected by copyright. All rights reserved.

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2024

Rom J Morphol Embryol

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Soft Tissue Lesions Involving Female Reproductive Organs

Cited by 3 publications

References 418 publications

Primitive neuroectodermal tumors of the ovary: a multidecade review of the scientific literature

Primitive neuroectodermal tumors of the ovary: a multidecade review of the scientific literature

Imaging in gynecological disease (26): clinical and ultrasound characteristics of benign retroperitoneal pelvic peripheral‐nerve‐sheath tumors

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