Abstract：
OBJECTIVE: Angioleiomyoma, predominantly arising from the extremities, is a benign soft tissue tumor. Reports on its intracranial location are rare. We assessed clinical, radiological, and pathological features of intracranial angioleiomyoma (iALM) treated at our neurosurgical institution.
METHODS: We consecutively enrolled all patients with neuropathologically confirmed iALM treated at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were analyzed. A review of the literature on iALM was conducted.
RESULTS: Seven patients with iALM (four female) with a median age of 45 years (range: 32-76 years) were identified. In three cases, the lesion was found incidentally. In magnetic resonance imaging (MRI), all tumors were hypo- to isointense on T1-weighted, hyperintense on T2-weighted sequences, and gadolinium-enhancing. A strong FLAIR signal was seen in six patients. Surgery consisted of gross total resection in all cases without perioperative complications. Neuropathological staining was positive for smooth muscle actin (SMA) in all lesions. Mature smooth muscle cells arranged around blood vessels were typically observed. The Ki-67 index was ≤ 3%. The patients were discharged after a median of 6 days (range: 4-9 days). During a median follow-up time of 14 months (range: 4-41 months), no tumor recurrence occurred. In the current literature, 42 additional cases of iALM were identified.
CONCLUSIONS: Intracranial angioleiomyoma is a benign soft tissue tumor treated by gross total resection. Tumor morphology and positive staining for SMA lead to the neuropathological diagnosis.
METHODS: We consecutively enrolled all patients with neuropathologically confirmed iALM treated at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were analyzed. A review of the literature on iALM was conducted.
RESULTS: Seven patients with iALM (four female) with a median age of 45 years (range: 32-76 years) were identified. In three cases, the lesion was found incidentally. In magnetic resonance imaging (MRI), all tumors were hypo- to isointense on T1-weighted, hyperintense on T2-weighted sequences, and gadolinium-enhancing. A strong FLAIR signal was seen in six patients. Surgery consisted of gross total resection in all cases without perioperative complications. Neuropathological staining was positive for smooth muscle actin (SMA) in all lesions. Mature smooth muscle cells arranged around blood vessels were typically observed. The Ki-67 index was ≤ 3%. The patients were discharged after a median of 6 days (range: 4-9 days). During a median follow-up time of 14 months (range: 4-41 months), no tumor recurrence occurred. In the current literature, 42 additional cases of iALM were identified.
CONCLUSIONS: Intracranial angioleiomyoma is a benign soft tissue tumor treated by gross total resection. Tumor morphology and positive staining for SMA lead to the neuropathological diagnosis.
摘要：
目的：血管平滑肌瘤，主要来自四肢，是良性软组织肿瘤.关于其颅内位置的报道很少。我们评估了临床，放射学,和我们神经外科治疗的颅内血管平滑肌瘤（iALM）的病理特征。
方法：我们连续招募了所有在2013年至2021年间在单一神经外科机构接受神经病理学证实的iALM治疗的患者。收集临床和影像学资料,并对组织学组织切片进行分析。对有关iALM的文献进行了综述。
结果：确定了7名iALM患者（4名女性），中位年龄为45岁（范围：32-76岁）。在三种情况下，这个病变是偶然发现的。在磁共振成像(MRI)中，所有肿瘤在T1加权时都是低到等强度的，T2加权序列上的超强度，和钆增强。在6例患者中观察到强烈的FLAIR信号。在所有情况下，手术均包括全切，无围手术期并发症。所有病变的神经病理学染色均为平滑肌肌动蛋白（SMA）阳性。通常观察到排列在血管周围的成熟平滑肌细胞。Ki-67指数≤3%。患者在中位6天后出院（范围：4-9天）。在14个月的中位随访时间内（范围：4-41个月），无肿瘤复发。在目前的文献中,确定了42例额外的iALM病例。
结论：颅内血管平滑肌瘤是一种经全切除的良性软组织肿瘤。肿瘤形态学和SMA阳性染色导致神经病理学诊断。
方法：我们连续招募了所有在2013年至2021年间在单一神经外科机构接受神经病理学证实的iALM治疗的患者。收集临床和影像学资料,并对组织学组织切片进行分析。对有关iALM的文献进行了综述。
结果：确定了7名iALM患者（4名女性），中位年龄为45岁（范围：32-76岁）。在三种情况下，这个病变是偶然发现的。在磁共振成像(MRI)中，所有肿瘤在T1加权时都是低到等强度的，T2加权序列上的超强度，和钆增强。在6例患者中观察到强烈的FLAIR信号。在所有情况下，手术均包括全切，无围手术期并发症。所有病变的神经病理学染色均为平滑肌肌动蛋白（SMA）阳性。通常观察到排列在血管周围的成熟平滑肌细胞。Ki-67指数≤3%。患者在中位6天后出院（范围：4-9天）。在14个月的中位随访时间内（范围：4-41个月），无肿瘤复发。在目前的文献中,确定了42例额外的iALM病例。
结论：颅内血管平滑肌瘤是一种经全切除的良性软组织肿瘤。肿瘤形态学和SMA阳性染色导致神经病理学诊断。
