Fanconi 貧血に合併した進行下顎歯肉癌の1例

Fanconi anemia is an inherited genetic disorder characterized by progressive pancytopenia, transition to myelodysplastic syndrome, acute myeloid leukemia, body malformation, and increased risk of certain solid cancers. Among the solid tumors, there is a high incidence of squamous cell carcinoma of t...

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Published in日本口腔外科学会雑誌 Vol. 66; no. 8; pp. 360 - 365
Main Authors 山田, 慎一, 栗田, 浩, 柴田, 哲伸, 近藤, 英司, 横井, 啓, 太田, 亮輔
Format Journal Article
LanguageJapanese
Published 社団法人 日本口腔外科学会 20.08.2020
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ISSN0021-5163
2186-1579
DOI10.5794/jjoms.66.360

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Summary:Fanconi anemia is an inherited genetic disorder characterized by progressive pancytopenia, transition to myelodysplastic syndrome, acute myeloid leukemia, body malformation, and increased risk of certain solid cancers. Among the solid tumors, there is a high incidence of squamous cell carcinoma of the head and neck. Here, we report a case of advanced lower gingival cancer in a patient with Fanconi anemia. A 30-year-old man was referred to our hospital because he was aware of a painless mass arising in the right mandibular gingiva. In his medical history, the patient was given a diagnosis of Fanconi anemia at the age of 6 years because of pancytopenia. The patient achieved hematological recovery after allogenic hematopoietic stem cell transplantation following a preparative regimen consisting of total body irradiation and cyclophosphamide. The right mandibular tumor (36×27 mm) was diagnosed as lower gingival cancer. The patient underwent bilateral neck dissection, segmental mandibular resection, and total resection of the lower lip (squamous cell carcinoma, pT4aN2cM0). Reconstructive surgery comprised placement of a reconstructive titanium plate, a pectoralis major musculocutaneous flap, and local rotational flaps. Postoperative adjuvant treatment was not administered. 30 months postoperatively, there was no new lesion, recurrence, or metastasis.
ISSN:0021-5163
2186-1579
DOI:10.5794/jjoms.66.360