Capone Gennaro, Della Pepa Giuseppe Maria, Sabatino Giovanni, Bartoccioni Elena, Albanese Alessio, Mannino Stefano, Maira Giulio
Institute of Neurosurgery, Catholic University, Rome, Italy.
J Clin Neurosci. 2009 Jul;16(7):977-80. doi: 10.1016/j.jocn.2008.10.013. Epub 2009 Apr 17.
We report a rare bone-leptomeningeal metastasis from an adrenal cortical carcinoma (ACC). ACC is a rare malignancy and represents one of the most unusual sources of intracranial metastases (0-0.2%); the localization to the skull bone and meninges is uncommon. A 45-year-old man underwent surgery for a non-functioning ACC; 4 months later he developed a soft left frontal mass. The CT scans and MRI showed a large tumor with bone and leptomeningeal involvement. Despite chemotherapy, the lesion increased in volume, which led to local pain and right hemiparesis. Thus, the patient underwent excision of the mass; histopathological diagnosis confirmed that it was an ACC metastasis. The patient underwent standard radiation therapy after surgery. At post-operative follow-up, the patient was in a good neurological condition with no radiological evidence of a cranial recurrence; however, there was a voluminous abdominal regrowth of the primary tumor. To our knowledge, this is the second case of bone and leptomeningeal metastasis arising from an ACC. This patient report confirms the effectiveness of aggressive surgery for management of large intracranial metastases, particularly those that arise from primary tumors that are resistant to radiotherapy and chemotherapy. In our opinion, surgery represents the most appropriate treatment for voluminous intracranial metastasis - even when there are no neurological signs.
我们报告了一例罕见的肾上腺皮质癌(ACC)骨-软脑膜转移。ACC是一种罕见的恶性肿瘤,是颅内转移最不常见的来源之一(0-0.2%);转移至颅骨和脑膜并不常见。一名45岁男性因无功能性ACC接受了手术;4个月后,他左侧额叶出现一个软组织肿块。CT扫描和MRI显示一个大肿瘤,伴有骨和软脑膜受累。尽管进行了化疗,病变体积仍增大,导致局部疼痛和右侧偏瘫。因此,患者接受了肿块切除术;组织病理学诊断证实为ACC转移。患者术后接受了标准放疗。术后随访时,患者神经状况良好,无颅内复发的影像学证据;然而,原发肿瘤在腹部大量复发。据我们所知,这是第二例由ACC引起的骨和软脑膜转移病例。该病例报告证实了积极手术治疗大型颅内转移瘤的有效性,特别是那些源于对放疗和化疗耐药的原发性肿瘤的转移瘤。我们认为,手术是治疗大型颅内转移瘤最合适的方法——即使没有神经症状。