Pelvic Ependymoma With Clinical Response to GnRH Analog Therapy

Zhou, Fang; Song, Joon Young; Mikolaenko, Irina; Rosenblum, Marc K.; Shukla, Pratibha S.

doi:10.1097/pgp.0000000000000156

Cited by 16 publications

(16 citation statements)

References 37 publications

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“…CD99 and S100 are more commonly positive in CNS ependymomas than in their extraneural counterparts 6 . This immunophenotype stated by Idowu, is present in all published cases of EnE 3,4,5 , and it confirms the hypothesis that EnE arise by different mechanisms from their CNS counterparts 6 . However, it appears that EnE arising in different place or mechanism, show different immunoprofile.…”

Section: Discussionsupporting

confidence: 78%

“…While sacrococcygeal ependymomas are equally distributed among males and females, paraovarian pelvic and extra pelvic ependymomas have been exclusively reported in women, mainly of child bearing age 1,3 . However, there was a case of a 75-year-old patient with pelvic EnE 5 .…”

Section: Discussionmentioning

confidence: 99%

“…Ependimomas of CNS have distinct histology characterized by perivascular pseudorosettes, true ependymal rosettes, and fibrillary areas. In contrast, primary EnE have been described as having a wider range of microscopic architecture: perivascular pseudorosettes and occasionally true ependymal rosettes, along with mixtures of solid, macrocystic, microcystic, pseudopapillary, papillary, trabecular, and cribriform architectures 3 .…”

Section: Discussionmentioning

confidence: 99%

“…Rarely, they occur outside the CNS (extraneural ependymomas, EnE), and even than mostly in close relationship with neural axis. The majority of primary EnE are seen in the sacrococcygeal subcutaneous tissue and paraovarian area, but they have also been reported in the extraovarian pelvic region and more infrequently in other sites 1,2,3,4 .…”

Section: Introductionmentioning

confidence: 99%

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Mediastinal metastasis of primary extraneural ependymoma: Case report

et al. 2020

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Introduction. The rarity of primary extraneural ependymomas, its great variations in morphology and rare occurrence of metastasis, increase chances of misdiagnosis, particulary if found in paraovarian localization. Case report. In presented case, onset of the disease was 14 years ago, when after right salpingo-oophorectomy, patient was diagnosed with malignant mesothelioma. In following years patient had multiple and extensive surgical procedures, resulting in different pathohistological diagnosis, and after seven years, a diagnosis of extraneural ependymoma was established. Later on, patient was surgically treated in several medical centers across the region, again with different pathohistological diagnosis. At present, tumor metastasized to mediastinum, presenting as grey to brown, multicystic formation, with cysts filed with clear serous fluid or red-brown hemorrhagic fluid. Inner surface of the cysts had smooth to partly papillary appearance. Tumor cells exhibited several architectural paterns (solid, pseudorosette or rosette formations, papillary and pseudopapilary structures), and immunophenotype specific for extraneural ependymoma (GFAP, ER, PR positive; calretinin, WT-1, S100, synaptophysin, chromogranin, CK7 and pan-cytokeratin negative). Conclusion. This case demonstrates not only specific and diagnostic immunophenotype of extraneural ependymoma, but above all an important principle in tumor pathology. Rare neoplasms may occur in unusual and unexpected primary and metastatic sites. Pathologists need to be familiar with histologic features of a wide range of neoplasms and not just the appearance of neoplasms within their own limited subspecialty area.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Mediastinal metastasis of primary extraneural ependymoma: Case report

et al. 2020

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“…In addtion, a case of ovarian ependymoma responding to gonadotropin-releasing hormone(GnRH) therapy was reported by Fang et al in 2015, in which a 30-yr-old female with a large pelvic ependymoma who was treated with presurgical GnRH analogue therapy and fertility-sparing surgical resection remained disease-free after over 30 months without post-surgical treatment [ 17 ]. Similarly, some studies have demonstrated the role of anti-oestrogens in the treatment of ovarian ependymoma.…”

Section: Discussionmentioning

confidence: 99%

Ovarian ependymoma presenting in pregnancy: a case report and literature review

Jin

Jiang

Peng

2020

BMC Pregnancy Childbirth

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Background Ovarian ependymoma is a rare malignancy. Because of the extreme rarity, certain features of the neoplasm, including its clinical behaviour and optimal therapy, are incompletely characterized. Case presentation A 32-year-old pregnant woman at term presented with a left ovarian neoplasm that occurred in the early stage of pregnancy. She underwent left adnexectomy during the caesarean section, and the neoplasm was histologically and immunohistochemically identified to be ovarian ependymoma. Immunohistochemical staining with oestrogen receptors and progesterone receptors showed strong positive staining. According to reports in the literature, the pathological type of ovarian ependymoma in our patient was the extra-axial type. Interestingly, the foetus was also found to have bilateral ependymal cysts during pregnancy. The patient received no further surgical treatment or adjuvant therapy. She and her 14-month-old baby both have no evidence of disease at present. The follow-up of both mother and child is still continuing. Conclusions The case presented here illustrates high levels of oestrogen during pregnancy may stimulate viable malignant ependymal cells to proliferate. Hence, young women with extra-axial-type ependymomas may not be suitable for fertility preservation. Moreover, hormone-based therapies can be a potentially effective treatment for women with extra-axial ependymomas.

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Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments

Alvarado-Cabrero

2023

Encyclopedia of Pathology

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Pelvic Ependymoma With Clinical Response to GnRH Analog Therapy

Cited by 16 publications

References 37 publications

Mediastinal metastasis of primary extraneural ependymoma: Case report

Mediastinal metastasis of primary extraneural ependymoma: Case report

Ovarian ependymoma presenting in pregnancy: a case report and literature review

Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments

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