PDF(Pubmed)
Abstract：
BACKGROUND: A malignant myoepithelioma is a rare tumor, mostly arising from the salivary glands. Myoepitheliomas of the ear have rarely been reported. The manuscript reports myoepithelial carcinoma of the external auditory canal (EAC) spreading to the infratemporal fossa. A clinician must be aware of anatomical variation of the bony EAC wall, such as the foramen of Huschke. This rare defect may be a pathway for spreading pathologies between these two anatomical regions.
METHODS: We present a case of osteoma-like stenosis of the EAC, which turned out to be an extremely rare malignant tumor. The preoperative MRI and PET/CT revealed that two parts of the tumor communicated through a defect in the antero-inferior portion of the bony ear canal. No distant metastases were detected. Subsequently, the tumor was resected from the ear canal and the infratemporal fossa en bloc. Perioperatively the defect in the EAC wall was suspected of the foramen of Huschke. After the surgery, the older scans of the patient from the past showed no presence of a congenital EAC wall defect. Therefore, the authors concluded that the tumor aggressively grew through the bone due to its biological nature.
CONCLUSIONS: Malignant myoepithelioma of the external auditory canal is an extremely rare condition and could be misdiagnosed as other benign lesions. In cases of suspicious lesions, it is advisable to do a probatory biopsy from the EAC. Surgery is the treatment of choice in malignant myoepitheliomas, and regular follow-ups are essential to monitor for recurrence or metastatic disease. Any mass located at the antero-inferior portion of the EAC wall warrants close evaluation due to its potential for expansion from the EAC.
METHODS: We present a case of osteoma-like stenosis of the EAC, which turned out to be an extremely rare malignant tumor. The preoperative MRI and PET/CT revealed that two parts of the tumor communicated through a defect in the antero-inferior portion of the bony ear canal. No distant metastases were detected. Subsequently, the tumor was resected from the ear canal and the infratemporal fossa en bloc. Perioperatively the defect in the EAC wall was suspected of the foramen of Huschke. After the surgery, the older scans of the patient from the past showed no presence of a congenital EAC wall defect. Therefore, the authors concluded that the tumor aggressively grew through the bone due to its biological nature.
CONCLUSIONS: Malignant myoepithelioma of the external auditory canal is an extremely rare condition and could be misdiagnosed as other benign lesions. In cases of suspicious lesions, it is advisable to do a probatory biopsy from the EAC. Surgery is the treatment of choice in malignant myoepitheliomas, and regular follow-ups are essential to monitor for recurrence or metastatic disease. Any mass located at the antero-inferior portion of the EAC wall warrants close evaluation due to its potential for expansion from the EAC.
摘要：
背景：恶性肌上皮瘤是一种罕见的肿瘤，主要来自唾液腺。很少报道耳部肌上皮瘤。手稿报道了外耳道（EAC）的肌上皮癌扩散到颞下窝。临床医生必须意识到骨EAC壁的解剖变化，比如Huschke的孔.这种罕见的缺陷可能是在这两个解剖区域之间传播病理的途径。
方法：我们介绍一例EAC骨瘤样狭窄，这是一种极为罕见的恶性肿瘤.术前MRI和PET/CT显示，肿瘤的两个部分通过骨耳道的前下部分的缺损连通。未检测到远处转移。随后，肿瘤从耳道和颞下窝切除。围手术期怀疑EAC壁的缺损是Huschke孔。手术后,过去对患者进行的较早扫描显示不存在先天性EAC壁缺损.因此,作者得出的结论是，由于其生物学特性，肿瘤在骨骼中迅速生长。
结论：外耳道恶性肌上皮瘤极为罕见，可误诊为其他良性病变。在可疑病变的情况下，建议从EAC进行探查活检。手术是恶性肌上皮瘤的首选治疗方法，定期随访对于监测复发或转移性疾病至关重要。位于EAC壁前下部分的任何质量都需要进行密切评估，因为它有可能从EAC扩展。
方法：我们介绍一例EAC骨瘤样狭窄，这是一种极为罕见的恶性肿瘤.术前MRI和PET/CT显示，肿瘤的两个部分通过骨耳道的前下部分的缺损连通。未检测到远处转移。随后，肿瘤从耳道和颞下窝切除。围手术期怀疑EAC壁的缺损是Huschke孔。手术后,过去对患者进行的较早扫描显示不存在先天性EAC壁缺损.因此,作者得出的结论是，由于其生物学特性，肿瘤在骨骼中迅速生长。
结论：外耳道恶性肌上皮瘤极为罕见，可误诊为其他良性病变。在可疑病变的情况下，建议从EAC进行探查活检。手术是恶性肌上皮瘤的首选治疗方法，定期随访对于监测复发或转移性疾病至关重要。位于EAC壁前下部分的任何质量都需要进行密切评估，因为它有可能从EAC扩展。
