Primary calvarial angiosarcoma: A case report and review of the literature

Abstract

Background:Angiosarcomas are high-grade endothelial tumors remarkable for their rarity and malignant behavior. Primary calvarial angiosarcoma is an extremely rare entity and its behavior usually sets it apart from other angiosarcoma types. We highlight the successful management of cranial angiosarcoma using a multidisciplinary approach.Case Description:We present a 16-year-old male who was first noted to have a right-sided parietal cranial mass that was biopsied in 2008. Pathology was initially thought to be Kaposiform hemangioendothelioma. The patient subsequently underwent chemotherapy with vincristine. The patient did well until early 2010, when he suffered a right-sided intraparenchymal intratumoral hemorrhage. At this time, the original pathologic diagnosis was revisited and the diagnosis was upgraded to an angiosarcoma. The patient underwent a second round of chemotherapy using vincristine, cyclophosphamide, and actinomycin. The tumor continued to progress despite this treatment and he developed extensive skull deformity. At this point more definitive surgical intervention was reconsidered. Preoperative embolization of the mass was performed followed by aggressive surgical resection of the bony disease. The patient tolerated the procedure well and was discharged 6 days postoperatively without any new deficits. The patient is currently in the process of completing radiation therapy to entire tumor bed. He has clinically done well with no neurologic deterioration and has demonstrated long-term survival (>3 years).Conclusion:With the combined efforts of pediatric oncology, radiation oncology, interventional neuroradiology, and neurosurgery, a survival of greater than 3 years is possible with this aggressive pathology.

Neoadjuvant Systemic Treatment of Primary Angiosarcoma

“…The remaining case reports describe four cases with a histologically proven angiosarcoma with rare sites of origin:, one in the spleen [ 35 ], one in the calvarial space [ 36 ], one in the seminal vesicle [ 37 ], and one in the thyroid [ 38 ]. These four patients received a variety of neoadjuvant therapies, which makes it difficult to interpret the impact of these separate cases for a general treatment advise.…”

“…These four patients received a variety of neoadjuvant therapies, which makes it difficult to interpret the impact of these separate cases for a general treatment advise. Almost all patients showed a response to chemotherapy and all patients showed long term disease control after surgery [ 35 , 36 , 37 , 38 ].…”

Pediatric cutaneous angiosarcoma of the scalp: Report of a rare case with literature review

“…It can be challenging to diagnose angiosarcoma in a patient under the age of 21, partly because the condition is uncommon in this age group [1] and in several instances, the morphology is misleading towards a benign lesion. Several benign and malignant tumors which are considered in its differential include kaposi sarcoma, epithelioid hemangioendothelioma, hemangiopericytoma, and spindle cell hemangioendothelioma and kaposiform hemangioendothelioma [6,7]. The literature has mostly documented pediatric angiosarcomas as case reports and short series, including descriptions of their emergence in the heart, liver, head and neck, spleen, abdomen/pelvis and bone [1].…”

Primary angiosarcoma of the skull: A rare case report

“…described a case of primary calvarial angiosarcoma who presented with a single episode of left limb numbness and weakness due to brain parenchymal compression. [ 12 ] Yamada et al . reported a case of epithelioid angiosarcoma causing subdural hematoma.…”

“…have also reported the use of adjuvant radiotherapy with good local control at the tumor site. [ 12 20 ]…”