Primary epithelioid angiosarcoma originating from the mandibular gingiva: a case report of an extremely rare oral lesion
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(2020) 18:260
CASE REPORT
Open Access
Primary epithelioid angiosarcoma originating from the mandibular gingiva: a case report of an extremely rare oral lesion Yuko Komatsu1,2, Ikuya Miyamoto1,2* , Yu Ohashi1,2, Katsunori Katagiri2,3, Daisuke Saito2,3, Mizuki Obara1,2, Yasunori Takeda4, Kiyoto Shiga2,3 and Hiroyuki Yamada1,2
Abstract Background: Angiosarcoma occurs very rarely in the oral cavity, and the epithelioid type is even rarer. Here, we report a rare case involving an elderly man with a primary epithelioid angiosarcoma that originated from the mandibular gingiva and resembled a dentigerous cyst on radiographs. Case presentation: A 66-year-old Japanese man visited our hospital with a chief complaint of gingival swelling in right mandibular third molar region. A panoramic radiograph showed bone resorption around the crown of right mandibular third molar, which was impacted. Incisional biopsy confirmed a diagnosis of epithelioid angiosarcoma. The lesion exhibited aggressive proliferation after biopsy resulting in uncontrolled bleeding and difficulty in closing the mouth. Mandibular segmental resection including the tumor was performed without reconstruction. Because of the aggressive preoperative course of the tumor, the patient received adjuvant chemotherapy. There were no signs of recurrence during a 2-year follow-up period. Conclusions: A review of the literature yielded only four reported cases of epithelioid angiosarcoma in the jaw region, with the lesions occurring in the maxilla in three cases. To our knowledge, this is the second case of primary epithelioid angiosarcoma in the mandible. Keywords: Epithelioid angiosarcoma, Mandibular gingiva, Vascular tumor
Background Angiosarcomas account for only 2% of all soft tissue sarcomas [1–3]. More than 50% of all cases occur in the head and neck region, with the scalp and forehead being the most common sites [4]. However, primary angiosarcomas in the oral cavity represent only 1% of all angiosarcomas [5]. Angiosarcoma generally exhibits an aggressive clinical course and an unfavorable prognosis, * Correspondence: [email protected] 1 Division of Oral and Maxillofacial Surgery, Department of Oral and Maxillofacial Reconstructive Surgery, School of Dentistry, Iwate Medical University, 19-1 Uchimaru, Morioka, Iwate 020-8505, Japan 2 Head and Neck Cancer Center, Iwate Medical University, 2-1-1 Idaidori, Yahaba-cho, Shiwa-gun, Iwate 028-3694, Japan Full list of author information is available at the end of the article
even in cases where surgical removal of the tumor is possible. Affected patients usually do not benefit from chemoradiotherapy [6]. Approximately 50% patients die within 15 months after diagnosis, and only 12% survive for 5 years or longer [7]. Generally, well-differentiated tumors have been associated with better survival rates. Tumor-related death is usually due to uncontrolled local progression and/or distant metastases to the lungs, liver, and bone [1, 8]. Early detection and treatment are essential for controlling this highly malignant s
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