Koch M, Blatterspiel G J, Niedobitek G, Constantinidis J
Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universität Erlangen-Nürnberg.
Laryngorhinootologie. 2001 Jul;80(7):410-5. doi: 10.1055/s-2001-15713.
The term "midline granuloma syndrome" (MGS) is a clinical description of a broad spectrum of diseases, which are characterised by aggressive and progressive destruction of mucosa and adjacent structures of the midface and upper aerodigestive tract. After exclusion of granulomatous infections, rare granulomatous diseases and epithelial neoplasias, the differential diagnosis includes the following entities: Wegener's granulomatosis (WG), malignant lymphoma and idiopathic midline destructive disease (IMDD). Today there are doubts about the existence of IMDD. After exclusion of WG nearly all remaining cases presenting as MGS are peripheral sinonasal angiocentric T- and/or NK-cell lymphomas, which show a close association to Epstein-Barr virus infection and now are recognised as a special clinicopathological entity. The natural history of these lymphomas is characterised through a rapidly progressive course with a poor prognosis.
A case of a 35-year-old male patient with an angiocentric nasal T/NK-cell lymphoma, which involved the left lacrimal cyst, the left maxillar and ethmoid sinus as well as the soft and hard palates, is presented. First clinical signs and symptoms were similar to chronic-recurrent sinusitis. For almost two years the patient was treated with systemic corticoids for suspected limited Wegener's granulomatosis. The patient underwent sinus surgery for pansinusitis three times. After development of midline destructive disease the diagnosis of angiocentric lymphoma was established.
Soon after the diagnosis a combination high-dose radiochemotherapy was performed. The patient died only 3 months later because of multiorgan failure.
Because of its poor prognosis the angiocentric nasal NK/T-cell lymphoma should included early into the differential diagnosis of the midline granuloma syndrome. Correct biopsy technic and in situ hybridization of EBV can be important for an early diagnosis. Therapy should be aggressive and consists of high-dose radiotherapy, which is most important to reach local tumor control, and combination chemotherapy, the use of which is presently in discussion.
“中线肉芽肿综合征”（MGS）一词是对一系列广泛疾病的临床描述，其特征是面部中部和上呼吸道黏膜及相邻结构的侵袭性和进行性破坏。在排除肉芽肿性感染、罕见的肉芽肿性疾病和上皮性肿瘤后，鉴别诊断包括以下几种疾病：韦格纳肉芽肿（WG）、恶性淋巴瘤和特发性中线破坏性疾病（IMDD）。如今，人们对IMDD的存在存在疑问。在排除WG后，几乎所有表现为MGS的剩余病例都是外周鼻窦血管中心性T细胞和/或NK细胞淋巴瘤，它们与爱泼斯坦-巴尔病毒感染密切相关，现在被认为是一种特殊的临床病理实体。这些淋巴瘤的自然病程以快速进展和预后不良为特征。
本文报告一例35岁男性血管中心性鼻T/NK细胞淋巴瘤患者，病变累及左泪囊、左上颌窦和筛窦以及软硬腭。最初的临床症状与慢性复发性鼻窦炎相似。近两年来，该患者因疑似局限性韦格纳肉芽肿接受全身皮质类固醇治疗。患者因全鼻窦炎接受了三次鼻窦手术。在出现中线破坏性疾病后，确诊为血管中心性淋巴瘤。
确诊后不久即进行了高剂量联合放化疗。患者仅3个月后因多器官功能衰竭死亡。
由于血管中心性鼻NK/T细胞淋巴瘤预后较差，应尽早纳入中线肉芽肿综合征的鉴别诊断。正确的活检技术和EBV原位杂交对早期诊断可能很重要。治疗应积极，包括高剂量放疗（这对实现局部肿瘤控制最为重要）和联合化疗（目前对联合化疗的应用仍在讨论中）。
