Department of Neurosurgery, Mitsui Memorial Hospital, 1 Kandaizumicho, Chiyoda-ku, Tokyo, Japan.
J Med Case Rep. 2021 Aug 18;15(1):431. doi: 10.1186/s13256-021-02979-z.
It is extremely rare for primary non-Hodgkin's lymphomas to occur singly in the cranial vault. One case diagnosed as primary diffuse large B-cell lymphoma is reported, initially misdiagnosed as metastatic skull tumor, complicated with Trousseau syndrome.
The patient was a 60-year-old Japanese woman with no particular previous medical history. In a head computed tomography examination for vertigo, bone destructive skull tumor covering the right frontal, parietal, and temporal bones was incidentally discovered. As positron emission tomography indicated an abnormal accumulation in the large intestine and multiple cerebral infarctions suspicious of Trousseau syndrome were observed on magnetic resonance images, a metastatic skull tumor due to colorectal cancer was first considered. However, various tumor markers were negative, and colonoscopic biopsy indicated no colorectal abnormality. After pathological examination of the resected tumor, it was diagnosed as diffuse large B-cell lymphoma. The tumor affected muscles and skin but did not develop in the brain or the dura mater. As further general examination revealed no other abnormalities, we considered that it was primary diffuse large B-cell lymphoma in the cranial vault associated with Trousseau syndrome. Treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone and high-dose methotrexate reduced the residual lesion; coagulation abnormalities, which are frequently associated with Trousseau syndrome, also improved.
Skull tumors can result from a variety of malignancies, and their diagnosis may be complicated with Trousseau syndrome. However, even in cases of a single lesion in the cranial vault without invasion of the central nervous system, diffuse large B-cell lymphoma should be considered as a differential diagnosis.
原发性非霍奇金淋巴瘤在颅腔中单独发生极为罕见。报告了一例最初误诊为转移性颅骨肿瘤、伴有 Trousseau 综合征的原发性弥漫性大 B 细胞淋巴瘤。
患者为 60 岁日本女性，无特殊既往病史。因眩晕行头部 CT 检查时偶然发现覆盖右额骨、顶骨和颞骨的骨质破坏颅骨肿瘤。由于正电子发射断层扫描显示大肠异常积聚，且磁共振成像上观察到多个疑似 Trousseau 综合征的脑梗死，首先考虑转移性颅骨肿瘤，可能由结直肠癌引起。然而，各种肿瘤标志物均为阴性，结肠镜活检也未发现结直肠异常。切除肿瘤的病理检查后，诊断为弥漫性大 B 细胞淋巴瘤。肿瘤累及肌肉和皮肤，但未累及大脑或硬脑膜。由于进一步的全身检查未发现其他异常，我们认为这是与 Trousseau 综合征相关的颅腔原发性弥漫性大 B 细胞淋巴瘤。利妥昔单抗、环磷酰胺、多柔比星、长春新碱和泼尼松龙以及大剂量甲氨蝶呤的治疗减少了残留病变；常与 Trousseau 综合征相关的凝血异常也得到了改善。
颅骨肿瘤可由多种恶性肿瘤引起，其诊断可能与 Trousseau 综合征有关。然而，即使在没有中枢神经系统侵犯的颅腔单个病变的情况下，也应考虑弥漫性大 B 细胞淋巴瘤作为鉴别诊断。
