Vaginal epithelioid malignant peripheral nerve sheath tumor nearly misdiagnosed as advanced cervical cancer: A case report

Güzin, Kadir; Kinter, Alp Koray; Bozdağ, Halenur; Kır, Gözde; Sandal, Kemal

doi:10.1016/j.ijscr.2020.12.046

Cited by 4 publications

(5 citation statements)

References 14 publications

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“…MPNST of vaginal origin is particularly rare, with six cases reported to date. [5][6][7][8][9][10] A summary of the cases reported to date is given below in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…It has been reported in various sites but is rare in the field of gynecology. MPNST of vaginal origin is particularly rare, with six cases reported to date 5–10 . A summary of the cases reported to date is given below in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…MPNSTs commonly occur in association with peripheral nerves, 3 most often in the upper or proximal lower extremities 4 . Cases involving the vagina are exceedingly rare, with only six cases previously reported 5–10 . We describe a case of vaginal MPNST treated with postoperative adjuvant radiotherapy and complete surgical resection, and provide details on its diagnosis and treatment.…”

Section: Introductionmentioning

confidence: 98%

“…4 Cases involving the vagina are exceedingly rare, with only six cases previously reported. [5][6][7][8][9][10] We describe a case of vaginal MPNST treated with postoperative adjuvant radiotherapy and complete surgical resection, and provide details on its diagnosis and treatment.…”

Section: Introductionmentioning

confidence: 99%

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Vaginal malignant peripheral nerve sheath tumor treated with complete surgical resection and postoperative radiation therapy

Mizuta,

Kozono,

Asai

et al. 2023

J of Obstet and Gynaecol

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Malignant peripheral nerve sheath tumors (MPNSTs) are neoplasms originating from or differentiating into nerve sheaths of peripheral nerves. Vaginal origin is rare, with only six vaginal primary cases reported to date. A 55‐year‐old woman presented to our hospital with a 7 cm vulvar mass. Tumor biopsy results were suspicious of sarcoma, and pelvic magnetic resonance imaging and hysterofiberscopy showed that the tumor originated from the lower vagina. The mass was transvaginally excised, and histological examination confirmed the diagnosis of a vaginal MPNST with negative surgical margins. The patient underwent radiotherapy because the risk of recurrence was high, owing to the large tumor size and high mitotic index. The patient remained recurrence‐free for 1 year after the primary treatment. This is the first case of a high‐risk vaginal MPNST that avoided early disease recurrence with additional radiotherapy after complete tumor resection.

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“…MPNST of vaginal origin is particularly rare, with six cases reported to date. [5][6][7][8][9][10] A summary of the cases reported to date is given below in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Vaginal malignant peripheral nerve sheath tumor treated with complete surgical resection and postoperative radiation therapy

Mizuta,

Kozono,

Asai

et al. 2023

J of Obstet and Gynaecol

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“…MPNST arising in females' genital tract is extremely rare, with only approximately 20 cases reported, 15 and ovarian MPNST has never been documented. To investigate the clinical characteristics, biological behavior, and treatment options of this particular entity, we present a case of ovarian EMPNST (OEMPNST) with EWSR1‐CREM fusion in a 7‐year‐old girl.…”

Section: Introductionmentioning

confidence: 99%

Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1‐CREM fusion: A case report and literature review

Hong

Wang

et al. 2023

Intl J Gynecology & Obste

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Epithelioid malignant peripheral nerve sheath tumor (EMPNST) is a rare soft tissue sarcoma. The authors report the first case of EMPNST arising in the ovary (OEMPNST). A 7‐year‐old child underwent left salpingo‐oophorectomy due to tumor rupture and the pathology suggested a juvenile granulosa cell tumor (JGCT). Six cycles of bleomycin, etoposide, and carboplatin were administrated. A second surgery was applied due to relapse 4 months after the last cycle of chemotherapy, and the pathology revealed JGCT with extensive abdominopelvic seedings even after interinstitutional consultation in two hospitals. Next‐generation sequencing demonstrated EWSR1 exon12‐CREM exon6 fusion with neurofibromatosis‐2 gene deletion, and no mutation was detected in either FOXL2 or DICER1. However, pathology consultation in two other hospitals suggested the diagnosis of OEMPNST, and additional immunohistochemical (IHC) staining revealed positive H3K27me3. Nonetheless, she was treated with nine courses of chemotherapy but experienced a second recurrence of extensive abdominal metastases approximately 3 months after ceasing chemotherapy. Neither elevated tumor makers nor abnormal sex hormones level was noted since the initial presentation. Repeated cytoreductive surgery was conducted and IHC staining showed expression of SOX10, S‐100, INI‐1, and α‐inhibin in tumor tissue. A final diagnosis of OEMPNST with EWSR1‐CREM fusion was established, indicating that the probability of OEMPNST could not be excluded when treatment for JGCT showed poor response. A comprehensive evaluation including biological characteristics, morphology, IHC staining, and molecular features is vital in the differential diagnosis between JGCT and OEMPNST.

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Metastatic malignant peripheral nerve sheath tumor of the uterus and cervix: Diagnostic Challenges, prognostic determinants and treatment

Laird,

Diaz,

Gao

et al. 2024

Gynecologic Oncology Reports

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Vaginal epithelioid malignant peripheral nerve sheath tumor nearly misdiagnosed as advanced cervical cancer: A case report

Abstract: Highlights Vaginal schwannoma. Vaginal epithelioid MPNST. Malignant peripheral nerve sheath tumors. Vaginal cancer. Spindle-to-epithelioid tumor.

Cited by 4 publications

References 14 publications

Vaginal malignant peripheral nerve sheath tumor treated with complete surgical resection and postoperative radiation therapy

Vaginal malignant peripheral nerve sheath tumor treated with complete surgical resection and postoperative radiation therapy

Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1‐CREM fusion: A case report and literature review

Metastatic malignant peripheral nerve sheath tumor of the uterus and cervix: Diagnostic Challenges, prognostic determinants and treatment

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