Benign Spindel Cell Neoplasam: A Central Myofibroma of Maxilla in Male Child of Seven Years of Age: A Case Report

Hira Nisar, A. Zalan, Mehreen Ismail, Sabeen Masood, Ahsan Inayat
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Abstract

Myofibroma is a rare benign spindle cell neoplasm which occurs predominantly in infants and young children. It can occur as a solitary mass or as a multicentric lesion, consisting of myofibroblasts. In oral cavity, the sites most commonly involved are the mandible, tongue, lips and buccal mucosa however, it rarely involves the maxilla. It has aggressive clinical presentation mimicking malignancies. We report a case of a male child seven years of age presented with intraoral swelling on the left palatal aspect of the maxilla that extended from deciduous upper left second molar to the distal of upper permanent left first molar causing the mobility of these teeth. Radiologically, the lesion was osteolytic causing marked resorption of alveolar bone and displacement of permanent maxillary left second molar. A differential diagnosis of peripheral giant cell granuloma, central giant cell granuloma, fibroma, aggressive fibromatosis, nodular fasciitis, peripheral ossifying fibroma and spindle cell neoplasm was made. After the excluding malignant and vascular involvement, the surgical excision of the lesion was planned and was carried out under general anesthesia. Histologically, benign proliferation of spindle cells was seen giving the diagnosis of benign spindle cell neoplasm. Reports also showed positive immunoreactivity with vimentin and αSMA while non-responsive to desmin, validates the diagnosis of myofibroma. KEYWORDS: spindle cell neoplasm; myofibroma; maxilla
良性梭形细胞瘤:7岁男童上颌骨中枢性肌纤维瘤1例
肌纤维瘤是一种罕见的良性梭形细胞肿瘤,主要发生在婴幼儿。它可以是孤立的肿块,也可以是多中心病变,由肌成纤维细胞组成。在口腔中,最常见的受累部位是下颌骨、舌头、嘴唇和颊粘膜,但很少累及上颌骨。它具有模仿恶性肿瘤的侵袭性临床表现。我们报告一个病例的男性儿童七岁提出了口腔内肿胀的左腭方面的上颌骨,从乳牙左上第二磨牙延伸到上永久左第一磨牙远端导致这些牙齿的流动性。影像学表现为骨质溶解,导致牙槽骨明显吸收,上颌永久左第二磨牙移位。鉴别诊断为外周巨细胞肉芽肿、中央巨细胞肉芽肿、纤维瘤、侵袭性纤维瘤病、结节性筋膜炎、外周骨化纤维瘤和梭形细胞肿瘤。排除恶性及血管受累后,计划手术切除病变,并在全身麻醉下进行。组织学上可见梭形细胞良性增生,诊断为良性梭形细胞瘤。报告还显示对vimentin和αSMA的免疫反应阳性,而对desmin无反应,证实了肌纤维瘤的诊断。关键词:梭形细胞肿瘤;myofibroma;上颌骨
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