Nasal cavity, paranasal sinuses, nasopharynx
Other tumors
Angiosarcoma

Author: Abul Ala Syed Rifat Mannan, M.D. (see Authors page)
Editor: Songyang Yuan, M.D., Ph.D.

Revised: 22 May 2018, last major update July 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Angiosarcoma[TI] nasal

Cite this page: Mannan, A.A.S.R. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalangiosarcoma.html. Accessed August 20th, 2018.
Definition / general
  • Malignant vascular neoplasm uncommon in the sinonasal region
Epidemiology
Sites
  • Maxillary sinus is most common location
  • Other paranasal sinuses and the nasal cavity can also be affected
Etiology
  • Reported risk factors are radiation exposure, Thorotrast, arsenic, vinyl chloride
Clinical features
  • Common presentations are recurrent epistaxis, mass lesion, regional pain, nasal obstruction, foul smelling / blood tinged nasal discharge, paresthesia
  • Lymph node and distant metastasis are uncommon at presentation
Prognostic factors
  • Tumor recurrence is common, often due to incomplete excision
  • Metastasis is uncommon; favored sites are lung, liver, spleen, bone marrow
  • Overall survival approximately 61%, more favorable than angiosarcoma of soft tissue and skin (Head Neck Pathol 2007;1:1)
Case reports
Treatment
  • Surgical resection with radiation or chemotherapy
Gross description
  • Mean tumor size of 4 cm; may be as large as 8 cm
  • Usually nodular, polypoid, soft, friable
  • Often associated with hemorrhage or necrosis
Microscopic (histologic) description
  • Usually infiltrative, invading surrounding bone and soft tissue
  • Characterized by anastomosing vascular channels that dissect through stroma
  • Channels are lined by atypical endothelial cells with nuclear pleomorphism, hyperchromasia, increased mitotic activity
  • Endothelial cells may pile up along the lumen, creating papillations
  • Cells can be flat to plump, spindly to epitheliod
  • Hemorrhage and necrosis are common
  • Epithelioid variant is characterized by formation of intracytoplasmic neolumen, containing erythrocytes
  • Either low grade or high grade
Microscopic (histologic) images

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Vascular neoplasm

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Blood with degeneration

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Moderately pleomorphic nuclei

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Solid pattern of growth

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Atypical mitotic figure

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CD34, Ki67

Positive stains
Negative stains