Rodrigo Juan Pablo, Suárez Carlos, Rinaldo Alessandra, Devaney Kenneth O, Carbone Antonino, Barnes Leon, Heffner Dennis K, Ferlito Alfio
Department of Otolaryngology, Hospital Universitario Central de Asturias, Oviedo, Spain.
Oral Oncol. 2005 Apr;41(4):340-8. doi: 10.1016/j.oraloncology.2004.10.007.
The differential diagnosis of a progressive destructive lesion of the midface and upper airway region includes both neoplastic and non-neoplastic entities; of these, the majority of cases prove to be either Wegener's granulomatosis or lymphoma. Historically, these sorts of necrotizing midfacial lesions were diagnosed clinically, and as a consequence a variety of overlapping categories of disease sprang up. As pathologic examination of biopsy material became both more widespread and (particularly in the last several years) more sophisticated, many lesions previously thought to be of mysterious origins have proven to be examples of lymphoma (in particular, sinonasal natural killer cell or T cell [NK/T] lymphomas). At present, the evaluation of a patient with a progressive destructive process involving the midface region should include imaging studies (to delineate the extent of disease) as well as biopsy (with sampling of lesional tissue for application of sophisticated testing--including immunohistochemical studies, flow cytometry, or molecular studies as necessary--to exclude the possibility of a NK/T cell lymphoma). There remain occasional patients whose necrotizing midfacial lesions continue to be difficult to classify despite the application of extensive testing; such patients are sometimes described as suffering from the nebulous entity of "idiopathic midline destructive disease". While it remains to be seen whether such patients will ultimately be assigned to other diagnostic groups (as, for example, occult toxic injuries--as in the case of cocaine abusers who are not forthcoming with regard to their drug usage), it seems likely that "idiopathic midline destructive disease" is a diagnostic term of questionable validity which should be used only with extreme reticence in modern practice.
中面部和上呼吸道区域进行性破坏性病变的鉴别诊断包括肿瘤性和非肿瘤性疾病;其中,大多数病例被证明是韦格纳肉芽肿或淋巴瘤。从历史上看,这类坏死性中面部病变是通过临床诊断的,因此出现了各种重叠的疾病类别。随着活检材料的病理检查变得更加普遍,并且(特别是在过去几年中)更加精细,许多以前被认为起源不明的病变已被证明是淋巴瘤的例子(特别是鼻窦自然杀伤细胞或T细胞[NK/T]淋巴瘤)。目前,对患有涉及中面部区域进行性破坏过程的患者的评估应包括影像学检查(以确定疾病范围)以及活检(采集病变组织样本以进行精细检测——必要时包括免疫组织化学研究、流式细胞术或分子研究——以排除NK/T细胞淋巴瘤的可能性)。尽管进行了广泛的检测,但仍有少数患者的坏死性中面部病变难以分类;这类患者有时被描述为患有“特发性中线破坏性疾病”这一模糊的病症。虽然这类患者最终是否会被归入其他诊断组还有待观察(例如,隐匿性毒性损伤——如可卡因滥用者隐瞒其药物使用情况时),但“特发性中线破坏性疾病”似乎是一个有效性存疑的诊断术语,在现代实践中应极其谨慎地使用。