Lee Vanessa, Gessler Dominic, Cataltepe Oguz
University of Massachusetts Medical School, Worcester, MA, USA.
Horae Gene Therapy Center, University of Massachusetts Medical School, Worcester, MA, USA.
Childs Nerv Syst. 2020 Sep;36(9):2103-2107. doi: 10.1007/s00381-020-04568-9. Epub 2020 Mar 10.
Angiosarcoma is a rare malignant tumor with an aggressive course and poor prognosis. It is typically seen in adults but very rarely seen in children. Angiosarcoma of the skull with brain metastasis is exceptionally rare. Due to the rare nature of these tumors, much is left unknown about clinical progression and treatment guidelines are not well established.
A 14-year-old male patient presented with an enlarging mass on the parietal region of the head. Further investigations revealed a mass lesion involving scalp and skull tissue. Biopsy result showed angiosarcoma and the patient underwent multiple surgical interventions including scalp excision, craniectomy on tumor site, and excision of brain metastases. He also received chemotherapy and radiation therapy. Despite aggressive treatment, disease progression could not be controlled.
Here we report a pediatric patient with intracranially invasive angiosarcoma of the scalp and skull, with recurrent hemorrhagic metastases to the brain. This is a very rare case in pediatric age group with very poor prognosis. Our patient had impressively longer survival than those reported in the literature despite multiple hemorrhagic brain metastasis and this is most likely related to our aggressive treatment strategy that includes multiple craniotomies for metastatic tumor resection in addition to neoadjuvant chemotherapy and radiation therapy. We believe optimal treatment of skull angiosarcoma in children should aim gross total resection of the skull tumor including involving scalp and dura as well as neoadjuvant chemotherapy and radiation therapy, and the patient should be followed closely with repeated brain MRI studies to pursue additional surgeries to remove brain metastasis if feasible.
血管肉瘤是一种罕见的恶性肿瘤，病程侵袭性强，预后较差。它通常见于成人，在儿童中极为罕见。伴有脑转移的颅骨血管肉瘤异常罕见。由于这些肿瘤的罕见性，关于其临床进展仍有许多未知之处，治疗指南也尚未完善。
一名14岁男性患者头部顶叶出现逐渐增大的肿块。进一步检查发现肿块累及头皮和颅骨组织。活检结果显示为血管肉瘤，患者接受了多次手术干预，包括头皮切除、肿瘤部位颅骨切除术以及脑转移灶切除。他还接受了化疗和放疗。尽管进行了积极治疗，但疾病进展仍无法得到控制。
在此我们报告一例小儿头皮和颅骨颅内侵袭性血管肉瘤患者，伴有复发性脑内出血性转移。这在小儿年龄组中是非常罕见的病例，预后很差。尽管存在多次脑内出血性转移，我们的患者生存期比文献报道的患者明显更长，这很可能与我们积极的治疗策略有关，该策略包括除新辅助化疗和放疗外，多次开颅切除转移瘤。我们认为儿童颅骨血管肉瘤的最佳治疗应旨在对包括累及头皮和硬脑膜的颅骨肿瘤进行全切除，以及新辅助化疗和放疗，并且应密切随访患者，反复进行脑部MRI检查，以便在可行时进行额外手术切除脑转移灶。
