Salivary glands - Embryoma

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Salivary glands

Other tumors

Embryoma

Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 27 January 2013, last major update September 2012
Copyright: (c) 2003-2013, Pathol2gyOutlines.com, Inc.

General
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● Rare malignant tumor, diagnosed at or near birth, resembling epithelial anlage of salivary glands but in arrested state of differentiation (Ann Otol Rhinol Laryngol 1992;101:958, Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:109)
● Also called sialoblastoma

Clinical features
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● Childhood epithelial salivary gland tumor; common among the perinatal tumors
● Occurs in submandibular, parotid or minor salivary glands; also ectopic salivary gland tissue
● Uni- or multinodular, 2-7 cm, rapid growth
● May recur locally or involve regional lymph nodes; also lung metastases

Case reports
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● Female infant with tumor arising in ectopic salivary gland tissue in cheek (J Plast Reconstr Aesthet Surg 2009;62:e241)
● Male infant with congenital tumor arising in minor salivary gland of buccal mucosa (Fetal Pediatr Pathol 2011;30:32)
● 3 month old boy with submandibular swelling of insidious onset (J Pediatr Surg 2008;43:e11)
● 3 month old girl with recurrent and metastatic tumor (Rare Tumors 2011;3:e13)
● 18 month old girl with parotid gland tumor (Pediatr Blood Cancer 2010;55:1427)
● 2 year old old with 2 cm mass in parotid gland with increasing anaplasia (Am J Surg Pathol 1999;23:342)
● 6 year old girl with recurrent tumor (Acta Cytol 2003;47:1123)

Treatment
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● Excision; chemotherapy if cannot completely excise (Pediatr Blood Cancer 2010;55:374); possibly brachytherapy

Micro description
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● Ductules and solid organoid nests of basaloid cells with fine chromatin and cuboidal epithelial cells
● 2 histological patterns: favorable pattern with semiencapsulation of benign basaloid cells with intervening stroma; unfavorable pattern with anaplastic basaloid cells, broad pushing to infiltrative periphery and minimal stroma (Ann Diagn Pathol 2006;10:320)
● Variable necrosis, variable mitotic activity, variable nuclear atypia, no perineurial invasion

Micro images
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One year old boy with parotid tumor

Recurrent and metastatic tumor

Nests of basaloid cells with poorly demarcated eosinophilic cytoplasm and round, vesicular nuclei

Cytology description
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● Tight, solid clusters of atypical basaloid cells plus dispersed epithelial and myoepithelial cells with metachromatic magenta hyaline globular material

Positive stains
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● Cytokeratin (ductal structures), S100, smooth muscle actin, calponin, p63
● Also AFP, cytoplasmic HER2, p53; Ki67 index increases with recurrence and may predict behavior (Pediatr Dev Pathol 2010;13:32)

Differential diagnosis
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● Adenoid cystic carcinoma
● Basal cell adenoma
● Pleomorphic adenoma: may have embryonal structures
● Teratoma

End of Salivary glands > Other tumors > Embryoma

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