- Department of Neurosurgery, LSU HSC Shreveport, Shreveport, LA, USA
- Department of Pathology, LSU HSC Shreveport, Shreveport, LA, USA
- Department of Plastic Surgery, LSU HSC Shreveport, Shreveport, LA, USA
- Department of Pediatric Medicine, LSU HSC Shreveport, Shreveport, LA, USA
Department of Neurosurgery, LSU HSC Shreveport, Shreveport, LA, USA
DOI:10.4103/2152-7806.102952Copyright: © 2012 Khan IS. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Khan IS, Thakur JD, Ahmed O, Shorter CD, Thomas-Ogunniyl J, Kim MT, Jeroudi MA, Guthikonda B. Primary calvarial angiosarcoma: A case report and review of the literature. Surg Neurol Int 29-Oct-2012;3:134
How to cite this URL: Khan IS, Thakur JD, Ahmed O, Shorter CD, Thomas-Ogunniyl J, Kim MT, Jeroudi MA, Guthikonda B. Primary calvarial angiosarcoma: A case report and review of the literature. Surg Neurol Int 29-Oct-2012;3:134. Available from: http://sni.wpengine.com/surgicalint_articles/primary-calvarial-angiosarcoma-a-case-report-and-review-of-the-literature/
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.
Keywords: Angiosarcoma, cranium, hemangioendothelioma, pediatric
Angiosarcomas are high-grade endothelial tumors remarkable for their rarity and malignant behavior. Their incidence is approximately 2–3 cases per 1,000,000.[
Primary calvarial angiosarcoma (PCA) is an extremely rare entity and its aggressive behavior usually sets it apart from other angiosarcoma types. To our knowledge 5 histologically confirmed cases of primary skull angiosarcomas have been described in the pediatric population;[
A 14-year-old African-American male was admitted to our institution in October 2008 with a single episode of subjective left leg numbness and weakness. His past medical history was unremarkable and this was the first time he had experienced such an episode. General physical examination was normal and neurologic exam only revealed mild left arm numbness. Magnetic resonance imaging (MRI) scans revealed a 3 cm extra-axial, heterogeneously enhancing mass in the right parieto-occipital region with edema of the adjacent parenchyma [
The patient was treated with monotherapy of vincristine. Initially, the tumor responded well to this treatment, but subsequently showed progression approximately 10 months after diagnosis. Due to the progression of the mass, the accuracy of the pathologic diagnosis was revisited and the initial diagnosis was changed to the more malignant angiosarcoma.
In January 2010, the patient had an acute onset of left-sided hemiparesis and headaches. Computed tomography (CT) scan revealed an intraparenchymal hematoma in the frontoparietal region, extending toward the lateral ventricle. An increase in the tumor size was also noticed [
Tumor progression initially stabilized with multidrug therapy; however, after 8 months, a significant calvarial deformity was revealed on routine surveillance neuroimaging. In January 2011, repeat MRI brain again revealed progression of the tumor, which now encompassed both hemispheres and was accompanied by a marked calvarial deformity. On physical examination, it was noted that the patient's left-sided hemiparesis was worse. Given that there was significant tumor progression in spite of aggressive chemotherapy and clinical deterioration, surgical intervention was reconsidered. After considering the size, vascular nature, and lack of response to chemotherapy, the patient and family wished to proceed with surgery.
Twenty-four hours prior to surgery, the patient underwent preoperative tumor embolization. Arterial supply was noted from the superficial temporal artery, middle meningeal artery, and branches of the occipital artery [
Microscopic sections of the tumor demonstrated variable cellularity and growth pattern [
Microscopic sections of the tumor showing variable cellularity and growth pattern. (a) Cellular spindle cell area ×100 (b) High-grade, neoplastic spindle cells with mitosis showing rudimentary small vessel differentiation ×400. (c) Epithelioid foci showing round-to-oval cells with large, prominent nucleoli ×200. (d) Congested dilated vessels lined by neoplastic endothelium with intraluminal budding ×200. (e) Irregular, anastomosing vascular channels lined by flat neoplastic cells ×100. (f) Vascular tumor invading and destroying adjacent bone trabeculae ×100
Angiosarcomas are malignant neoplasms derived from the endothelial cells of arteries, veins, and lymphatics. They are classified as WHO grade 3 (or malignant) hemangioendotheliomas and belong to the larger class of soft tissue sarcomas that make up 1% of the overall malignancies in adults.[
When bone is the source of origin, long bones of the extremities are most commonly affected.[
Diagnosis depends on imaging and pathologic findings. A plain skull X-ray typically shows a well-demarcated, expansile, lytic mass.[
Histologic diagnosis relies on the architecture of the tumoral tissue and the features of the neoplastic cells. The hemangioendotheliomas family of tumors is summarized in
The prognosis of angiosarcoma in general is very poor. For angiosarcoma involving the central nervous system, a mean overall survival of 8 months is reported.[
Treatment of cranial angiosarcoma has not been standardized thus far due to the paucity of patients. Some authors suggest that these tumors should be treated similar to osteosarcomas, with wide surgical excision, adjuvant chemotherapy, and radiotherapy.[
The role of adjuvant or neoadjuvant chemotherapy and radiotherapy is less well defined. The results of radiotherapy (combined with surgery in some instances) have been vague.[
Lack of sufficient patient data on the efficacy of chemotherapy in patients with cranial angiosarcoma makes it difficult to establish standard guidelines.[
Recently, there has been increased interest in treatment of angiosarcoma with monoclonal antibodies, in particular, bevacizumab. The inhibition of the vascular endothelial growth factor-vascular endothelial growth factor receptor (VEGF–VEGFR) pathway in angiosarcoma has shown some promise.[
Angiosarcomas are rare and highly aggressive tumors of the endothelial cells. Primary cranial involvement may be rarely encountered and usually portends a poor prognosis. Treatment is ill-defined due to the rarity of the pathology and generally consists of a combination of aggressive surgical excision, chemotherapy, and/or radiotherapy. Further studies need to be undertaken to clarify the best therapeutic route to take in these patients and to establish guidelines. Our case highlights the fact that with an aggressive multidisciplinary management protocol, survival greater than 3 years can be seen in pediatric patients with PCA.
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