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Salivary glands
Sarcomas
Hemangioendothelioma
Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 26 January 2013, last major update September 2012
Copyright: (c) 2003-2013, Pathol2gyOutlines.com, Inc.
General
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● Vascular tumor of anastomosing thin walled capillaries growing between salivary ducts and acini
● Also called juvenile hemangioma
● Most common salivary gland tumor of infants/children
Clinical features
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● Often congenital, involves parotid gland, submandibular gland; more common in females
● Not malignant; intermediate behavior between angioma and angiosarcoma
● May be associated with Kasabach-Merritt syndrome
● 70-95% regress spontaneously by age 7 years
● Fine needle aspiration may be useful for diagnosis (Arch Pathol Lab Med 2001;125:1340)
Case reports
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● 4 month old boy with growing lobulated mass (Pathol Int 1999;49:668)
● 3 year old boy with submandibular swelling due to lymphadenopathy (Virchows Arch 2006;448:843)
● 28 year old man with parotid tumor (Chir Ital 2004;56:457)
● 44 year old woman with submandibular mass (Auris Nasus Larynx 2008;35:157)
● 48 year old woman treated with superficial parotidectomy (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:730)
Treatment
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● Delay excision in hope of spontaneous regression; postoperative radiotherapy
Gross description
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● Diffuse soft mass uninvolved with overlying skin
● Periglandular muscular extension
Micro description
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● Anastomosing thin walled capillaries growing between salivary ducts and acini
● Variable mitotic figures, epithelioid or Kaposiform patterns, microcalcifications
Micro images
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Cytology description
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● Spindle shaped cells in sheets and clusters in bloody background
● Cell block shows ductal structures trapped in sheets of spindle cells
Cytology images
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Hypercellular clusters of bland spindle cells surrounding ductal structures in cell block, factor VIII+, CD34+
Positive stains
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● CD34 and factor VIII, CD31, vimentin, smooth muscle actin
● Ki-67: 6%
● May have CMV+ ducts
Differential diagnosis
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● Hemangioma
End of Salivary glands > Sarcomas > Hemangioendothelioma
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