Valenzuela Fabiola, Desai Sohum
Department of Surgery, University of Texas Rio Grande Valley School of Medicine, Edinburg, USA.
Department of Neurological Surgery, BHS Physicians Network, Harlingen, USA.
Cureus. 2020 Sep 30;12(9):e10728. doi: 10.7759/cureus.10728.
Neuroendocrine carcinoma of the cervix (NECC) accounts for 2% of all cervical cancers. Brain metastasis is rare, with few cases described in the literature, and is usually associated with preceding pulmonary metastasis. We describe an additional case of isolated brain metastasis without pulmonary metastasis from NECC and reflect on unique management. A 37-year-old woman with a history of NECC presented with severe headache post-total hysterectomy with pelvic lymph node dissection. The computed tomography (CT) scan demonstrated obstructive hydrocephalus with several intra-axial lesions located in the pineal region, left cerebellar hemisphere, and left frontal operculum. A right frontal ventriculostomy was initially placed to relieve the hydrocephalus. CSF was sent for cytology but was unrevealing. Due to the degree of brainstem compression and the need to obtain a pathologic diagnosis, a posterior fossa craniotomy for the removal of the lesion was performed. Histopathology demonstrated small blue cell tumors positive for neuroendocrine markers consistent with neuroendocrine carcinoma of the cervix. Resection of additional metastasis was not recommended. An endoscopic third ventriculostomy (ETV) was then performed in order to remove the ventriculostomy with success. The patient was then referred to radiation oncology and received whole-brain radiotherapy (WBRT) for a total of 30 Grays (3000 cGy) over 10 fractions. Interval imaging demonstrated complete resolution of the pineal and left frontal lesions. The patient was symptom-free for approximately three months. She then presented with paraplegia consistent with follow-up imaging of her neuraxis, demonstrating drop metastasis in her cervical, thoracic, and lumbar spine. Spinal radiation was given with partial recovery in upper extremity function, however, lower extremity function did not recover. The patient was then transferred to palliative care. There are no guidelines on NECC brain metastasis management. Brain metastasis is associated with reduced longevity. NECC has a propensity for early dissemination and treatment failure. ETV is preferred over ventriculoperitoneal shunting in cases with obstructive hydrocephalus, as it may reduce the risk of tumor seeding. Retrospectively, our patient may have benefitted from upfront craniospinal radiation.
宫颈神经内分泌癌(NECC)占所有宫颈癌的2%。脑转移罕见,文献中报道的病例较少,且通常与先前的肺转移相关。我们描述了一例来自NECC的孤立性脑转移且无肺转移的病例,并对独特的治疗方法进行反思。一名有NECC病史的37岁女性在全子宫切除加盆腔淋巴结清扫术后出现严重头痛。计算机断层扫描(CT)显示梗阻性脑积水,松果体区、左小脑半球和左额盖有多个脑内病变。最初进行了右额脑室造瘘术以缓解脑积水。脑脊液送检细胞学检查,但未发现异常。由于脑干受压程度以及获得病理诊断的需要,进行了后颅窝开颅手术以切除病变。组织病理学显示小蓝细胞肿瘤神经内分泌标志物呈阳性,与宫颈神经内分泌癌一致。不建议切除其他转移灶。然后进行了内镜下第三脑室造瘘术(ETV),成功移除了脑室造瘘管。患者随后被转诊至放射肿瘤学部门,接受了全脑放疗(WBRT)共30格雷(3000厘戈瑞),分10次进行。间隔期影像学检查显示松果体区和左额叶病变完全消退。患者无症状约三个月。随后她出现截瘫,神经轴随访影像学检查显示颈椎、胸椎和腰椎有转移性肿瘤播散。给予脊柱放疗后上肢功能部分恢复,但下肢功能未恢复。患者随后转入姑息治疗。目前尚无关于NECC脑转移治疗的指南。脑转移与生存期缩短相关。NECC有早期播散和治疗失败的倾向。在梗阻性脑积水病例中,ETV优于脑室腹腔分流术,因为它可能降低肿瘤种植的风险。回顾来看,我们的患者可能从前期的全颅脊柱放疗中获益。