Undifferentiated Pleomorphic Sarcoma of Vulva
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CASE REPORT
Undifferentiated Pleomorphic Sarcoma of Vulva Amita Naithani1 • Parul Sharma1 • R. Pratima1 • Priyanka Makkar1 • Sunil Pasricha1 • Rishu Sangal1 Rupinder Sekhon1
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Received: 27 May 2020 / Revised: 5 July 2020 / Accepted: 11 July 2020 Ó Association of Gynecologic Oncologists of India 2020
Introduction Undifferentiated pleomorphic sarcoma (UPS) of vulva previously known as malignant fibrous histiocytoma (MFH) is an exceedingly rare tumor of the female genital tract [1]. Early stage diagnosis is key to successful management of such a rare entity. The accurate and conclusive diagnosis is by histopathological examination combined with immunohistochemistry (IHC) markers. Magnetic resonance imaging (MRI) is the imaging method of choice in addition to positron emission tomography-computed tomography (PET CT) scan to complete the metastatic work up. Treatment modalities include chemotherapy and radiotherapy or a combination of these with surgery [2]. A rare and aggressive case of vulvar sarcoma in a young and unmarried girl is being reported.
Case Report A 27-year-old unmarried lady presented to Rajiv Gandhi Cancer Institute & Research Centre with complaints of progressively increasing perineal mass associated with pain and sanguinous discharge vaginally. She gave history of increased frequency and burning micturition and constipation for five-to-six months accompanied by history of loss of appetite and weight loss of over 5 kg in over past 6 months. On physical examination patient had severe pallor though was clinically stable Eastern Cooperative Oncology Group-2 (ECOG-2). There was no significant palpable lymphadenopathy. On abdominal examination, no mass or & Amita Naithani [email protected] Rupinder Sekhon [email protected] 1
Rajiv Gandhi Cancer Institute and Research Center, New Delhi, Delhi, India
viscera palpable. Local examination revealed fleshy mass (approximately 14 9 11 9 8 cm) in perineum with necrotic debris and pus, reaching till perianal region (Fig. 1a, b). On digital rectal examination, a hard mass occupying pelvis and compressing the rectum was noted. The growth did not seem to invade the rectal mucosa on per rectal examination. Vulvar growth excision was performed at another healthcare facility, and histopathology examination (HPE) report was suggestive of malignant small round cell tumor of vulva. IHC on tumor cells was suggestive of metastatic high-grade sarcoma. Patient received 4 cycles of Vincristine, Adriamycin and Cisplatin/Cyclophosphamide and Etoposide-based chemotherapy. Evaluation postchemotherapy was suggestive of progressive disease, and chemotherapy regimen was changed to Gemcitabine and Docetaxel. Due to poor tolerance, the chemotherapy was stopped after first cycle and patient was put on supportive care. After admission at our center, the vulvar mass was managed locally by betadine douching and application of Sumag ointment to reduce the edema and induration. Systemic intravenous antiobiotics were given to prevent superadded secondary bacterial infection. His
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