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复发性持久性加西综合征揭示颅底弥漫性B细胞淋巴瘤:经“哈特尔路径”诊断

Relapsing Long-Lasting Garcin Syndrome Revealing Skull Base Diffuse B Cell Lymphoma: The Diagnosis through the "Hartel's Route".

作者信息

Casolla Barbara, Candela Serena, Ciacciarelli Antonio, Ciolli Ludovico, Romano Andrea, Acqui Michele, Cox Maria Christina, Sette Giuliano, Orzi Francesco

机构信息

Department of NESMOS (Neurosciences Mental Health and Sensory Organs), School of Medicine and Psychology, "Sant'Andrea" Hospital, "Sapienza" University of Rome, Rome, Italy.

Department of Hematology, "Sant'Andrea" Hospital, "Sapienza" University of Rome, 00189, Rome, Italy.

出版信息

J Neurol Surg B Skull Base. 2019 Feb;80(1):105-108. doi: 10.1055/s-0038-1657794. Epub 2018 Jun 8.

Abstract

The Garcin syndrome is a rare condition characterized by multiple unilateral cranial nerve palsy, without neither long-tract involvement nor intracranial hypertension. Non-Hodgkin lymphoma is a systemic malignant disease that localizes in a minority of cases in the central nervous system. We report a case of Garcin syndrome that revealed a diffuse large B cell lymphoma (DLBCL) located in the skull base and in the right kidney. We reached the diagnosis by mean of a nonstandard, mini-invasive, transforamen biopsy of the intracranial lesion (Hartel's route). The nature of the renal mass was determined ex juvantibus. The patient responded to the polichemotherapy with a complete regression of the intracranial lesion and of the renal mass evaluated by computed tomography and total body positron emission tomography scans. We, therefore, confirmed the DLBCL location in the right kidney. Over 4 years of follow-up, the patient has showed a complete remission of the disease. In this report, we emphasize the importance of biopsy in case of Garcin syndrome.

摘要

加欣综合征是一种罕见疾病,其特征为多发性单侧颅神经麻痹,既无长束受累也无颅内高压。非霍奇金淋巴瘤是一种全身性恶性疾病,少数情况下定位于中枢神经系统。我们报告一例加欣综合征病例,该病例显示弥漫性大B细胞淋巴瘤(DLBCL)位于颅底和右肾。我们通过对颅内病变进行非标准的、微创的经孔活检(哈特尔路径)做出诊断。肾肿物的性质通过辅助诊断确定。患者对多药化疗有反应,颅内病变和肾肿物经计算机断层扫描和全身正电子发射断层扫描评估完全消退。因此,我们证实了DLBCL位于右肾。经过4年的随访,患者疾病完全缓解。在本报告中,我们强调了加欣综合征病例活检的重要性。

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