著者

仲宗根 敏幸 又吉 亮 宮本 昇 後藤 新平 平野 惣大 牧志 祥子 中村 博幸

出版者

一般社団法人 日本口腔腫瘍学会

雑誌

日本口腔腫瘍学会誌 (ISSN:09155988)

巻号頁・発行日

vol.33, no.3, pp.151-158, 2021

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神経節細胞腫(GN)は,交感神経系または副交感神経系の神経節から発生すると考えられているまれな良性神経腫瘍である。3歳の時,副腎と右眼窩周囲骨に腫瘍を認め,神経芽腫(NB)Stage Ⅳと診断された。化学療法後,腫瘍を切除し,副腎の神経節芽腫(GNB)と眼窩周囲骨のGNの病理組織学的診断を得た。腫瘍は初発から21年後に頭蓋内硬膜で再発し,生検組織からGNと診断された。顎骨内のGNは,24年後に左側下顎,27年後に右側下顎で明らかとなり,切除された。病理組織学的には,腫瘍は成熟した神経節細胞で構成されていた。さらに,免疫組織化学で腫瘍細胞は,vimentin, S-100,neurofilament,Anti-Glia Fibrillary Acidic Protein (GFAP)およびsynaptophysinに対して陽性であり,α-Smooth muscle actin(α-SMA)およびCytokeratin AE1/AE3に対して陰性であった。Ki-67 labeling index (LI)は1%であった。最終診断としてGNであった。本症例は,一連の臨床経過から副腎腫瘍であったNBが両側下顎骨に転移し,長期経過をたどってGNとして発生した非常にまれな症例である。

著者

牧志 祥子 又吉 亮 宮本 昇 平野 惣大 仲宗根 敏幸 中村 博幸

出版者

社団法人 日本口腔外科学会

雑誌

日本口腔外科学会雑誌 (ISSN:00215163)

巻号頁・発行日

vol.67, no.3, pp.194-199, 2021

被引用文献数

1

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<p>Malignant peripheral nerve sheath tumors (MPNSTs) are very rare malignant neoplasia that arise from the peripheral nervous system and show nerve sheath differentiation. We report a case of primary malignant peripheral nerve sheath tumor of the mandibular gingiva.</p><p> A 53-year-old male was referred to our hospital presenting with a mass and pain in the mandibular gingiva. He had no appreciable disease and paralysis of the lower lip.</p><p> Computed tomography showed resorption of the mandible but no obvious lymph node metastasis.</p><p> Biopsy revealed a possible invasive unusual neuroectodermal tumor. Surgical resection was performed and the frozen section showed negative margins.</p><p> Histology revealed atypical spindle cells with clear chromatin and clear nucleolus which had proliferated and loosely infiltrated, but the cell boundaries were unclear. Immunohistochemical staining showed positive results for S-100 protein, p53, and vimentin, but negative results for pan-cytokeratin (AE1 / AE3), Melan A, and desmin. The Ki-67 labeling index was 10%. Accordingly, we diagnosed a low-grade peripheral nerve sheath tumor of the mandibular gingiva.</p><p> There were no signs of recurrence or metastasis at two years postoperatively. MPNSTs have poor prognoses and require careful follow-up.</p>

著者

仲宗根 敏幸 又吉 亮 村橋 信 後藤 新平 丸山 修幸 新崎 章

出版者

社団法人 日本口腔外科学会

雑誌

日本口腔外科学会雑誌 (ISSN:00215163)

巻号頁・発行日

vol.65, no.12, pp.780-785, 2019

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<p>Clear cell odontogenic carcinoma (CCOC) was reclassified from a benign tumor to a malignant tumor by the WHO classification in 2005 because of its aggressive nature and potential for local recurrence and distant metastases. Only 81 well-documented cases have been reported to date to the best of our knowledge. A patient was referred to our clinic because of rapid swelling of the left side of the mandible. Computed tomography (CT) showed a bone defect in the mandible, but no cervical lymph node metastasis. We suspected a malignant tumor and performed a biopsy. We diagnosed CCOC of the left side of the mandible and performed segmental mandibulotomy. Histopathologically, the tumor consisted of clear cells containing diastase-digestive PAS-positive granules. Furthermore, immunohistochemistry showed that the tumor cells were immunoreactive for cytokeratins (CKs) 7, 17, 19, Ki-67 and vimentin, but non-reactive for CK20, smooth muscle actin (SMA), epithelial membrane antigen (EMA) and S-100 protein. The Ki-67 labeling index (LI) was 10%. We finally diagnosed CCOC. One year 5 months after surgery, the patient died of multiple organ failure caused by the multiple bone metastases. We discuss this rare case of multiple bone metastases developing from CCOC of the mandible and review the literature.</p>