Reappraisal of Endometrial Stromal Sarcoma: Report of Four Cases with Review of Literature

Bn, Gayathri; Rao, Kadam Satyanarayan; Kr, Chatura

doi:10.5005/jp-journals-10006-1128

Cited by 1 publication

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References 6 publications

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“…The most common clinical presentation of vaginal sarcoma is similar to that of vaginal carcinomas, such as vaginal bleeding, mostly post coital or intermenstrual/postmenopausal. 3,4 The other symptoms like low back pain and urinary and fecal disturbances develop as the disease progresses to involve adjacent structures.…”

Section: Introductionmentioning

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: Introductionmentioning

confidence: 99%