Sakamoto N, Ichikizaki K, Komiyama M, Mikami K, Izumi C
No To Shinkei. 1983 Oct;35(10):989-99.
The authors experienced a case of histiocytosis X with a large intracranial mass resulting in a convulsive seizure. The patient showed left exophthalmos and a skin rash one year and two months after birth. Histiocytosis X was diagnosed from a skin biopsy, and predonine, endoxan and vincristine were administered. The rash disappeared, but the exophthalmos remained. At the age of two years and nine months, punched-out lesions appeared in the skull and 4,000 rads of radiation was applied. Thereafter, the exopthalmos persisted but there was no particular problem in the course. However, a convulsive seizure with fever suddenly appeared at nine years and ten months of age and the patient was hospitalized. At the time of admission, the general condition was good and there were no abnormalities in neurological tests. In neuroradiological examinations, a calcified and poorly vascularized mass 8 cm in maximum diameter was found to occupy the left middle cranial fossa. Chondrosarcoma was strongly suspected from these findings, but there was also symmetrical thickening of bone cortex in the peripheries of the long bones of the extremeties which appeared to be the recovery process from bone destruction caused by histiocytosis X. Therefore, the formation of an intracranial mass by histiocytosis X was diagnosed and surgery was performed. When left osteoplastic fronto-temporal craniotomy was performed, the mass was found to be raising the temporal lobe and it could be easily separated from the surrounding tissue. However, these was firm adherence to dura mater of the middle cranial fossa (especially that of the superior orbital fissure). Histologically, there were many cells with small nuclei, no polymorphism, abundant and clear cytoplasm which were darkly stained and slightly atypic. These findings matched those for histiocytosis X. Cases of histiocytosis X rarely show symptoms of the central nervous system or infiltration of the central nervous system. Only 31 such cases were seen in the literature investigated by the authors. Neurological symptoms include pyramidal symptoms such as hemiparesis and impairment of the cranial nerves, particularly paresis of the optic, trigeminal, facial and acoustic nerves. Convulsive seizures were seen in only five cases including the one reported here. It is also rare for intracranial masses to be formed in cases of histiocytosis X and only six cases, including the authors', have been found with masses of a maximum diameter of more than 5 cm.
作者遇到一例伴有巨大颅内肿块并导致惊厥发作的组织细胞增多症X。该患者在出生后一年零两个月出现左眼突出和皮疹。通过皮肤活检诊断为组织细胞增多症X，并给予强的松、环磷酰胺和长春新碱治疗。皮疹消失，但突眼仍然存在。在两岁零九个月时，颅骨出现穿凿样病变，并进行了4000拉德的放射治疗。此后，突眼持续存在，但病程中没有特别问题。然而，在九岁零十个月时突然出现伴有发热的惊厥发作，患者住院。入院时，一般情况良好，神经检查无异常。在神经放射学检查中，发现一个最大直径为8厘米的钙化且血运不佳的肿块占据左中颅窝。根据这些发现，强烈怀疑为软骨肉瘤，但四肢长骨周围骨皮质也有对称性增厚，这似乎是组织细胞增多症X所致骨破坏的恢复过程。因此，诊断为由组织细胞增多症X形成的颅内肿块并进行了手术。当进行左侧骨成形额颞开颅手术时，发现肿块抬高颞叶，且很容易与周围组织分离。然而，肿块与中颅窝硬脑膜（尤其是眶上裂处硬脑膜）紧密粘连。组织学上，有许多细胞核小、无多形性、胞质丰富且清晰、深染且稍有异型性的细胞。这些发现与组织细胞增多症X相符。组织细胞增多症X很少出现中枢神经系统症状或中枢神经系统浸润。作者查阅文献仅发现31例此类病例。神经症状包括锥体束征，如偏瘫和颅神经损害，尤其是视神经、三叉神经、面神经和听神经麻痹。惊厥发作仅在包括本文报道的这例在内的5例中出现。组织细胞增多症X病例中形成颅内肿块也很罕见，包括作者报道的病例在内，仅发现6例最大直径超过5厘米的肿块。
