Moiyadi A V, Moiyadi A A, Sampath S, Kalpana S R, Mahadevan A, Shankar S K, Srikanth S G
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India.
Acta Neurochir (Wien). 2007 Nov;149(11):1157-62. doi: 10.1007/s00701-007-1291-1. Epub 2007 Oct 1.
Intracranial metastases from atrial myxoma producing symptomatic mass lesions are very rare with only ten examples reported in the literature. We report a patient with multiple metastases from a cardiac myxoma which had an unusual histopathology mimicking an adenocarcinoma.
A 35 year old man presented with left facio-brachial focal motor seizures unresponsive to antiepileptic drugs and these episodes preceded the symptoms of cardiac myxoma. The seizures worsened a year following resection of the cardiac myxoma. The MRI of the brain revealed multiple lesions of heterogeneous intensity, partly solid and cystic situated in the right fronto-parietal, left temporal and occipital lobes.
Right fronto-parietal craniotomy revealed lesions with haemorrhagic, calcified areas and a large cystic component was decompressed. Histological examination of the lesions in the brain demonstrated prominent glandular differentiation, identical in morphology to the primary cardiac lesion of a glandular variant of atrial myxoma.
This report highlights the rare presentation of atrial myxoma with intracranial metastases and reviews previously reported examples. This is only the second case report of a glandular variant of atrial myxoma with metastases to the brain. A pathologist, unaware of this unusual variant of primary atrial myxoma, may mistake the intracranial lesion for a metastatic adenocarcinoma.
心房黏液瘤颅内转移并产生有症状的占位性病变非常罕见,文献中仅报道过10例。我们报告1例患有心脏黏液瘤多处转移的患者,其具有模仿腺癌的不寻常组织病理学表现。
一名35岁男性出现左面部 - 臂部局灶性运动性癫痫发作,对抗癫痫药物无反应,且这些发作先于心脏黏液瘤症状出现。心脏黏液瘤切除术后一年,癫痫发作加重。脑部MRI显示右额顶叶、左颞叶和枕叶有多个强度不均的病变,部分为实性和囊性。
右额顶叶开颅手术显示病变有出血、钙化区域,一个大的囊性成分被减压。对脑部病变的组织学检查显示出明显的腺性分化,形态与心房黏液瘤腺性变体的原发性心脏病变相同。
本报告强调了心房黏液瘤伴颅内转移的罕见表现,并回顾了先前报道的病例。这是第二例关于心房黏液瘤腺性变体转移至脑部的病例报告。不了解原发性心房黏液瘤这种不寻常变体的病理学家可能会将颅内病变误诊为转移性腺癌。