PDF(Pubmed)
Abstract：
UNASSIGNED: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region.
UNASSIGNED: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER + PR + HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored.
UNASSIGNED: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer.
UNASSIGNED: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma.
UNASSIGNED: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.
UNASSIGNED: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER + PR + HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored.
UNASSIGNED: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer.
UNASSIGNED: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma.
UNASSIGNED: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.
摘要：
未经证实：颅咽管瘤是良性肿瘤，主要局限于鞍上区域的颅腔。
未经证实：我们介绍了一例罕见的侵袭性乳头状颅咽管瘤伴播散性脊髓硬膜内疾病。一名67岁的女性，有4个月的头痛史，视觉障碍,急性意识错乱和神经根腿部疼痛。注意到乳腺癌病史（ER+PR+HER2-）。探讨了在治疗具有非典型或侵袭性特征的鞍区或鞍上病变之前进行组织学诊断的重要性。
UNASSIGNED：MRI显示鞍区和鞍上区部分实性和部分囊性垂体肿块，伴有交叉压迫和下丘脑受累。蝶鞍轻度增大，无钙化。全神经轴MRI显示硬膜内沉积累及心室系统，脊髓和圆锥.一个月内,病变的大小迅速增加。患者接受了开颅手术和鞍区和鞍区上肿块的经脑室切除术。颅骨病变组织学有利于乳头状颅咽管瘤，经BRAFV600突变证实。腰椎穿刺脑脊液细胞学检查证实颅咽管瘤有BRAF突变，无转移性乳腺癌的证据。
UNASSIGNED：患者术后仍然困惑，没有局灶性神经功能缺损，并接受了姑息性全脑放疗。她4个月后去世。对文献的回顾确定了29例颅咽管瘤破裂的报道。
UNASSIGNED：颅咽管瘤破裂表现为鞍上肿块和椎管内的跌落病变，放射学特征与转移性疾病难以区分。强调了组织学诊断在指导这些病例治疗中的重要性。
未经证实：我们介绍了一例罕见的侵袭性乳头状颅咽管瘤伴播散性脊髓硬膜内疾病。一名67岁的女性，有4个月的头痛史，视觉障碍,急性意识错乱和神经根腿部疼痛。注意到乳腺癌病史（ER+PR+HER2-）。探讨了在治疗具有非典型或侵袭性特征的鞍区或鞍上病变之前进行组织学诊断的重要性。
UNASSIGNED：MRI显示鞍区和鞍上区部分实性和部分囊性垂体肿块，伴有交叉压迫和下丘脑受累。蝶鞍轻度增大，无钙化。全神经轴MRI显示硬膜内沉积累及心室系统，脊髓和圆锥.一个月内,病变的大小迅速增加。患者接受了开颅手术和鞍区和鞍区上肿块的经脑室切除术。颅骨病变组织学有利于乳头状颅咽管瘤，经BRAFV600突变证实。腰椎穿刺脑脊液细胞学检查证实颅咽管瘤有BRAF突变，无转移性乳腺癌的证据。
UNASSIGNED：患者术后仍然困惑，没有局灶性神经功能缺损，并接受了姑息性全脑放疗。她4个月后去世。对文献的回顾确定了29例颅咽管瘤破裂的报道。
UNASSIGNED：颅咽管瘤破裂表现为鞍上肿块和椎管内的跌落病变，放射学特征与转移性疾病难以区分。强调了组织学诊断在指导这些病例治疗中的重要性。
