Wang Chunsaier, Yang Yi, He Zhen, Wei Xuejing, Zhang Shutian, Song Jiugang
Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Transl Cancer Res. 2022 Dec;11(12):4441-4447. doi: 10.21037/tcr-22-1595.
Paraneoplastic cerebellar degeneration (PCD), which displays ataxia and other cerebellar symptoms, is the most common paraneoplastic neurological syndrome (PNS). PCD is more likely to occur in individuals with small cell lung cancer (SCLC), gynecological malignancies, and Hodgkin disease, but it is rarely associated with non-Hodgkin lymphoma (NHL).
We report a case of PCD accompanying high-grade B-cell lymphoma embedded in an individual's stomach and duodenum, who also presented with acute onset of gait ataxia and slurred speech. The results of the common laboratory tests for neurological disorders, including the paraneoplastic antibody test, were negative. The key to the accurate diagnosis was the positron emission tomography/computed tomography findings. The final diagnosis of high-grade B-cell lymphoma was unclear until the performance of repeated esophagogastroscopy with multipoint deep excavation biopsies. After standard chemotherapy, the patient's gastric tumor was significantly alleviated and cerebellar syndrome was significantly improved.
This case highlights the challenges of diagnosing PNS associated with occult malignancy. PNS patients may present with a variety of neurological disorders; Thus, if any unexplained neurological symptoms appear after a series of specific laboratory and imaging tests, a diagnosis of PNS should be taken into consideration in the differential diagnosis list, as it may help clinicians identify asymptomatic malignancies and ensure patients receive correct treatments in a timely manner. A high-quality endoscopic biopsy is essential, as it helps hematologists make an accurate diagnosis of lymphoma with gastroduodenal involvement based on pathology.
副肿瘤性小脑变性(PCD)表现为共济失调和其他小脑症状,是最常见的副肿瘤性神经综合征(PNS)。PCD更易发生于小细胞肺癌(SCLC)、妇科恶性肿瘤和霍奇金病患者,但很少与非霍奇金淋巴瘤(NHL)相关。
我们报告一例PCD病例,该病例伴有胃和十二指肠高级别B细胞淋巴瘤,患者还出现急性步态共济失调和言语不清。包括副肿瘤抗体检测在内的常见神经系统疾病实验室检查结果均为阴性。准确诊断的关键是正电子发射断层扫描/计算机断层扫描结果。在进行重复的食管胃镜多点深挖活检之前,高级别B细胞淋巴瘤的最终诊断并不明确。经过标准化化疗后,患者的胃部肿瘤明显缓解,小脑综合征显著改善。
该病例凸显了诊断隐匿性恶性肿瘤相关PNS的挑战。PNS患者可能出现多种神经系统疾病;因此,如果在一系列特定实验室和影像学检查后出现任何无法解释的神经症状,在鉴别诊断清单中应考虑PNS诊断,因为这可能有助于临床医生识别无症状恶性肿瘤,并确保患者及时接受正确治疗。高质量的内镜活检至关重要,因为它有助于血液科医生根据病理学准确诊断累及胃十二指肠的淋巴瘤。