Liu Kai-Yuan, Wu Sheng-Mao, Chen Wei-Yu, Chang Chia-Lun
Division of Colorectal Surgery, Department of Surgery, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.
Division of Trauma and Emergency Surgery, Department of Surgery, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.
Int J Surg Case Rep. 2022 Apr;93:106933. doi: 10.1016/j.ijscr.2022.106933. Epub 2022 Mar 10.
Diffuse large B-cell lymphoma (DLBCL) is the most common histologic subtype of non-Hodgkin lymphoma (NHL), accounting for approximately 25% of all NHL cases. Primary appendiceal lymphomas (PAL) presenting as acute appendicitis are very rare, occurring in only 0.015% of all cases of gastrointestinal lymphoma.
A 57-year-old man who was initially presented as acute appendicitis and subsequently underwent interval laparoscopic appendectomy. Pathological examination revealed diffuse large B cell lymphoma with cut end involvement. Whole-body positron emission tomography (PET) scan revealed enlarged right palatine tonsil and raised a suspicion of lymphoma involvement in two right cervical lymph nodes (level II and III); biopsy, however, showed that the lymph nodes were benign, with non-specific cellular changes. Bone marrow biopsy of the iliac crest also did not show lymphoma involvement. Subsequently, a diagnosis of primary appendiceal diffuse large B cell lymphoma (Ann Arbor Stage II) was established. After six courses of definite chemotherapy with cyclophosphamide, doxorubicin HCl, vincristine, and rituximab (R-CHOP), PET/CT showed complete remission of the prior FDG-avid malignancy of appendiceal DLBCL. The patient continued to be stable with no recurrence for fifteen months of regular outpatient department follow-ups.
PAL is rare, and it clinically manifests the signs and symptoms of acute appendicitis. Specific characteristics of lymphoma in CT scans may lead to a more confirmative diagnosis. PET/CT is important for staging the lymphoma. Patients with PAL should be managed with surgical resection followed by R-CHOP-21 for 6 cycles regardless of whether they have localized disease or disseminated disease.
弥漫性大B细胞淋巴瘤（DLBCL）是非霍奇金淋巴瘤（NHL）最常见的组织学亚型，约占所有NHL病例的25%。表现为急性阑尾炎的原发性阑尾淋巴瘤（PAL）非常罕见，仅占所有胃肠道淋巴瘤病例的0.015%。
一名57岁男性最初表现为急性阑尾炎，随后接受了间隔期腹腔镜阑尾切除术。病理检查显示为弥漫性大B细胞淋巴瘤伴切端受累。全身正电子发射断层扫描（PET）显示右侧腭扁桃体肿大，怀疑右侧两个颈部淋巴结（II级和III级）有淋巴瘤累及；然而，活检显示淋巴结为良性，有非特异性细胞改变。髂嵴骨髓活检也未显示有淋巴瘤累及。随后，确诊为原发性阑尾弥漫性大B细胞淋巴瘤（Ann Arbor分期II期）。在用环磷酰胺、盐酸阿霉素、长春新碱和利妥昔单抗（R-CHOP）进行六个疗程的确定性化疗后，PET/CT显示阑尾DLBCL先前的FDG摄取性恶性肿瘤完全缓解。在定期门诊随访的十五个月中，患者持续稳定，无复发。
PAL罕见，临床上表现为急性阑尾炎的体征和症状。CT扫描中淋巴瘤的特异性特征可能有助于做出更确切的诊断。PET/CT对淋巴瘤分期很重要。无论PAL患者是局限性疾病还是播散性疾病，均应先进行手术切除，随后进行6个周期的R-CHOP-21治疗。
