鼻窦非霍奇金淋巴瘤。120例临床病理及免疫表型研究。
Non-Hodgkin's lymphoma of the sinonasal tract. A clinicopathologic and immunophenotypic study of 120 cases.
作者信息
Abbondanzo S L, Wenig B M
机构信息
Department of Hematologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.
出版信息
Cancer. 1995 Mar 15;75(6):1281-91. doi: 10.1002/1097-0142(19950315)75:6<1281::aid-cncr2820750610>3.0.co;2-i.
BACKGROUND
Non-Hodgkin's lymphomas (NHLs) of the sinonasal tract are uncommon neoplasms that can be morphologically difficult to distinguish from destructive nonneoplastic processes or other malignant neoplasms in this site.
METHODS
From the files of the Otolaryngic Tumor Registry-Armed Forces Institute of Pathology from 1965 to 1992, 120 cases of NHL involving the sinonasal tract were selected for which clinical records and paraffin-embedded tissue blocks were available. The histologic features and immunophenotypic findings of each patient were examined, and follow-up data were obtained for 66 (55%).
RESULTS
The ratio of males to females was 1.35:1, and the ages ranged from 3 to 94 years (median, 59 years). Sixty percent of the cases of NHL occurred in the patients' sixth to eighth decades of life. Clinical presentations varied according to histologic type. The low grade lymphomas presented with a nasal cavity or paranasal sinus mass associated with obstructive symptoms. The high grade lymphomas were more likely to present with aggressive signs and symptoms including nonhealing ulcer, cranial nerve manifestations, facial swelling, epistaxis, or pain. Of note, the high grade B-cell lymphomas tended to present with soft tissue or osseous destruction, particularly of the orbit with associated proptosis, whereas the T-cell lymphomas were associated with nasal septal perforation and/or destruction. Sites of disease included the nasal cavity, one or more paranasal sinuses, or multiple regions within the sinonasal tract. Of patients who received adequate follow-up, nodal and extranodal dissemination were identified in a limited number (n = 11). Nodal dissemination occurred in cervical and axillary lymph nodes. Extranodal sites of involvement included the larynx, skin, liver, uvula, kidney, breast, lacrimal gland, testis, and prostate gland. There was a wide spectrum of morphologic types of lymphoma, classified according to the Working Formulation. Immunophenotypic analysis on paraffin embedded tissue sections of all patients demonstrated a B-cell to T-cell ratio of 1.18:1. Treatment primarily included radiotherapy and chemotherapy. Follow-up information was available for 66 (55%) patients ranging from 1 to 16 years (median, 3 years). Of these 66 patients, 24 (36.4%) died of disease, 17 (25.7%) are alive without disease, 13 (19.7%) are alive with disease, and 12 (18.2%) are dead of unrelated or unknown causes.
CONCLUSIONS
Non-Hodgkin's lymphomas of the sinonasal tract are heterogeneous diseases that can be clinically aggressive. The frequency of these lymphomas in the United States cannot be estimated accurately because all of our cases were of histologic slides submitted for consultations. There appears, however, to be a slight B-cell predominance in this population that previously has been observed, unlike in South America and Asia where the majority of cases have a T-cell phenotype.
背景
鼻窦非霍奇金淋巴瘤(NHLs)是一种罕见肿瘤,在形态学上很难与该部位的破坏性非肿瘤性病变或其他恶性肿瘤相区分。
方法
从1965年至1992年武装部队病理研究所耳鼻喉肿瘤登记处的档案中,选取120例累及鼻窦的NHL病例,这些病例均有临床记录和石蜡包埋组织块。检查了每位患者的组织学特征和免疫表型结果,并获得了66例(55%)患者的随访数据。
结果
男女比例为1.35:1,年龄范围为3至94岁(中位数为59岁)。60%的NHL病例发生在患者60至80岁之间。临床表现因组织学类型而异。低级别淋巴瘤表现为鼻腔或鼻窦肿物并伴有阻塞症状。高级别淋巴瘤更易出现侵袭性体征和症状,包括不愈合溃疡、颅神经表现、面部肿胀、鼻出血或疼痛。值得注意的是,高级别B细胞淋巴瘤往往表现为软组织或骨质破坏,尤其是眼眶伴眼球突出,而T细胞淋巴瘤与鼻中隔穿孔和/或破坏有关。病变部位包括鼻腔、一个或多个鼻窦,或鼻窦内的多个区域。在接受充分随访的患者中,发现少数(n = 11)有淋巴结和结外播散。淋巴结播散发生在颈部和腋窝淋巴结。结外受累部位包括喉、皮肤、肝脏、悬雍垂、肾脏、乳腺、泪腺、睾丸和前列腺。淋巴瘤的形态学类型广泛,根据工作分类法进行分类。对所有患者石蜡包埋组织切片的免疫表型分析显示,B细胞与T细胞的比例为1.18:1。治疗主要包括放疗和化疗。66例(55%)患者有1至16年(中位数为3年)的随访信息。在这66例患者中,24例(36.4%)死于疾病,17例(25.7%)无病存活,13例(19.7%)带病存活,12例(18.2%)死于无关或不明原因。
结论
鼻窦非霍奇金淋巴瘤是异质性疾病,临床上可能具有侵袭性。在美国,这些淋巴瘤的发病率无法准确估计,因为我们所有的病例都是提交会诊的组织学切片。然而,与南美和亚洲大多数病例为T细胞表型不同,在这一人群中似乎有轻微的B细胞优势,这一点此前已有观察。