Saul S H, Kapadia S B
Cancer. 1985 Jul 1;56(1):157-66. doi: 10.1002/1097-0142(19850701)56:1<157::aid-cncr2820560125>3.0.co;2-x.
The clinical and pathologic findings were studied in 68 patients with primary lymphoma of Waldeyer's ring (WR). The initial sites included: tonsil (51%; 9% bilateral), nasopharynx (35%), base of tongue (9%), and multiple areas (4%). The mean age was 58 years, with a male to female ratio of 1.1:1.0. Tonsillar and base of tongue lesions presented with sore throat and dysphagia, whereas nasopharyngeal lymphomas most commonly presented with nasal, auditory, and cranial nerve sympatomatology. A neck mass was the sole presenting symptom in 12% of patients. Work-up showed 42% Stage I, 47% Stage II, 4% Stage III, and 7% Stage IV. All were non-Hodgkin's lymphomas. Diffuse architecture predominated (71%) and 70% had a histiocytic or large cell morphology (diffuse histiocytic 51%, nodular histiocytic 19%). The National Cancer Institute (NCI) Working Formulation grades were 13% low, 81% intermediate, and 6% high. In difficult cases, detection of monoclonal immunoglobulin, absence of keratin staining, and lack of epithelial features by electron microscopy were useful adjuncts aiding in diagnosis. Following complete remission, 28 patients relapsed, 75% of these within 2 years (median, 10 months). Thirty-seven (54%) patients died with disease (median survival, 27 months). Of these, 89% had disseminated lymphoma. Local-regional disease was the direct cause of death in 13%. Recurrence or persistence of disease in WR occurred in 9% of cases, most with initial clinical evidence of soft tissue extension. Stage I patients had a significantly better median survival (67 months) than Stage II patients (20 months) (P = 0.03). Prognostically favorable histologic parameters included lower NCI Working Formulation grade and follicular architecture. Ten patients (15%) developed extranodal disease, eight involving the gastrointestinal (GI) tract, and all died within 1 year of its occurrence. The findings indicate that stage and certain histologic parameters are important prognostic factors in WR lymphoma. The study confirms the association of primary WR lymphoma with other extranodal disease, particularly involving the GI tract, and emphasizes the poor prognosis of patients in whom this occurs.
对68例Waldeyer环（WR）原发性淋巴瘤患者的临床和病理表现进行了研究。初始发病部位包括：扁桃体（51%；9%为双侧）、鼻咽（35%）、舌根（9%）和多个部位（4%）。平均年龄为58岁，男女比例为1.1:1.0。扁桃体和舌根病变表现为咽痛和吞咽困难，而鼻咽淋巴瘤最常见的表现为鼻部、耳部和颅神经症状。12%的患者唯一的首发症状是颈部肿块。检查显示42%为I期，47%为II期，4%为III期，7%为IV期。所有均为非霍奇金淋巴瘤。弥漫性结构占主导（71%），70%具有组织细胞或大细胞形态（弥漫性组织细胞51%，结节性组织细胞19%）。美国国立癌症研究所（NCI）工作分类分级为低级别13%，中级81%，高级6%。在疑难病例中，检测单克隆免疫球蛋白、角蛋白染色阴性以及电镜下缺乏上皮特征有助于辅助诊断。完全缓解后，28例患者复发，其中75%在2年内复发（中位时间，10个月）。37例（54%）患者死于该疾病（中位生存期，27个月）。其中，89%患有播散性淋巴瘤。局部区域疾病是13%患者的直接死亡原因。WR疾病复发或持续存在于9%的病例中，大多数最初有软组织扩展的临床证据。I期患者的中位生存期（67个月）明显优于II期患者（20个月）（P = 0.03）。预后良好的组织学参数包括较低的NCI工作分类分级和滤泡结构。10例患者（15%）发生结外疾病，8例累及胃肠道（GI），且均在发病后1年内死亡。研究结果表明，分期和某些组织学参数是WR淋巴瘤重要的预后因素。该研究证实了原发性WR淋巴瘤与其他结外疾病的关联，尤其是累及胃肠道，并强调了发生这种情况的患者预后不良。
