Vaginal endometrial stromal sarcoma: A literature review

Wu, Horng-Huei; Luo, Yi; Yin, Ning; Gui, Wenwu; Cao, Yi

doi:10.1016/j.eurox.2019.100094

Cited by 2 publications

(2 citation statements)

References 21 publications

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“…6 Hormonal therapy is reported to have a favorable survival outcome in endometrial stromal sarcomas if they express ER/PR. 11 The 5-year survival of vaginal sarcoma varies from 35% to 70%. 6 The factors that may predict recurrence and affect prognosis are tumor dimension greater than 5 cm, significant cytologic atypia, mitotic index > 10 figures per 10 HPFs, presence of atypical mitotic figures, presence of tumor cell necrosis, and pattern of tumor interface.…”

Section: Discussionmentioning

confidence: 99%

Adult Vaginal Sarcoma: When a Rarity Becomes Reality

Shanmugam¹,

Susikar²,

Sandhya³

2023

Journal of South Asian Federation of Obstetrics and Gynaecology

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Primary malignant diseases of the vagina are quite uncommon, particularly in adults. They constitute about 2% of gynecologic malignancies, the least common among them being sarcomas (3%). Very few cases have been reported in the literature due to the rarity of the disease. A 36-year-old lady presented with complaints of irregular and excessive bleeding per vaginum of 3-months duration. There were no specific findings on general examination of the patient. The per-speculum examination revealed an ulceroproliferative growth arising predominantly from the anterior vaginal wall extending from the anterior and right lateral fornices till introitus, filling the vaginal cavity. The cervix and all other fornices were free, so was the rectum. The MRI of the local part showed a 6 × 4 cm lesion arising from upper anterior vaginal wall causing obliteration of the vaginal cavity with extra serosal extension and abutment of base of bladder. The biopsy and the immunohistochemistry of the lesion were suggestive of high-grade sarcoma (Vimentin+, CD10+, p53+, ki 67-80%, SMA-, Desmin-, myogenin-, and Pan CK-). The patient received 3 cycles of neoadjuvant chemotherapy (AIM regimen -Doxorubicin, Ifosfamide, and Mesna) due to the bulky nature of the disease. The response assessment MRI revealed 25% decrease in the size of the lesion with infiltration of the posterior wall of the bladder. The patient underwent laparoscopic anterior pelvic exenteration. The postoperative histopathology revealed the growth to be high-grade fibrosarcoma with focal undifferentiated pleomorphic sarcoma-like areas with bladder wall infiltration (pT3). Sarcomas of the vagina are exceedingly rare. Surgery plays a major role in management. Postoperative radiation therapy may help in decreasing local recurrence. The benefits of chemotherapy, with its morbidity and possible mortality, remain unproven. Prognosis may be associated with tumor grade and stage. Owing to the rarity of the disease, treatment guidelines are still unclear and need further exploration.

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Section: Discussionmentioning

confidence: 99%

Adult Vaginal Sarcoma: When a Rarity Becomes Reality

Shanmugam¹,

Susikar²,

Sandhya³

2023

Journal of South Asian Federation of Obstetrics and Gynaecology

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“…Extra-genital ESS in the absence of a genital primary tumor is an exceptionally rare occurrence; isolated cases are documented in intestine, 5 omentum, 6 pelvis, 7 liver, 8 retroperitoneum, 9 vagina, 10 and sciatic nerve. 11 Only 11 cases of ESS arising from intestinal endometriosis are reported in English literature.…”

Section: Discussionmentioning

confidence: 99%

Extragenital endometrial stromal sarcoma of transverse mesocolon: A diagnostic conundrum

2021

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Endometrial stromal sarcoma (ESS) is a rare uterine neoplasm infrequently arising in extra-genital sites. Herein, we report an extremely rare case of primary extra-genital ESS of transverse mesocolon occurring in a 51-year-old female presenting with gradually increasing abdominal mass. The clinical diagnosis considered was a gastrointestinal stromal tumor. Intra-operatively, the mass was confined exclusively to the transverse mesocolon. Microscopy revealed a cellular tumor composed of oval to elongate neoplastic cells with hyperchromatic nuclei, inconspicuous nucleoli and were immunoreactive for CD10, progesterone receptor (PR), estrogen receptor (ER), and PAX8; negative for KIT, CD34, SMA, S100, synaptophysin, chromogranin, WT-1, and calretinin. A distinct arborizing network of arterioles along with foci of endometriosis was also seen. We present this case for its extreme rarity and the challenges entailed in its diagnosis.

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Vaginal endometrial stromal sarcoma: A literature review

Cited by 2 publications

References 21 publications

Adult Vaginal Sarcoma: When a Rarity Becomes Reality

Adult Vaginal Sarcoma: When a Rarity Becomes Reality

Extragenital endometrial stromal sarcoma of transverse mesocolon: A diagnostic conundrum

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