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Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 45 year old man underwent partial mandibulectomy for a jaw mass.
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1. Question
A 45 year old man underwent partial mandibulectomy for a jaw mass. Choose the correct diagnosis.
Correct
Answer: PERIPHERAL MUCOEPIDERMOID CARCINOMA WITH BONE INVASION
Histology: The bony cortex is replaced by an expansile cystic mass lined by three intermixed cell types: mucinous cells with intracytoplasmic mucin vacuoles, epidermoid cells with abundant cytoplasm and focal keratinization, and intermediate cells with minimal cytoplasm. In areas, these cells demonstrate complex architecture with invasion into the surrounding stroma.
Discussion: Mucoepidermoid carcinoma is the most common malignant salivary gland tumor. The majority of these tumors harbor a t(11;9) CRTC1-MAML2 fusion, which has been associated with a better prognosis. However, the main prognostic determinant is grade, which is assessed by the extent of a cystic component, degree of invasive growth, cytologic atypia, and necrosis. Mucoepidermoid carcinoma can involve the jaw bones by two routes: invasion from a peripheral tumor based in the gingiva or growth as a central lesion originating within the bone itself. In central lesions, the main differential diagnosis includes a glandular odontogenic cyst, a simple cyst with duct-like spaces, variable amounts of mucous cells, and lack of architectural complexity or invasive growth. Although glandular odontogenic cysts were once thought to be the precursors to central mucoepidermoid carcinomas, they have recently been shown to lack the CRTC1-MAML2 fusion. Peripheral tumors must be distinguished from adenosquamous carcinomas, which generally show separate superficial squamous components and deep glandular components rather than the intermixed cell types seen in mucoepidermoid carcinomas.
Incorrect
Answer: PERIPHERAL MUCOEPIDERMOID CARCINOMA WITH BONE INVASION
Histology: The bony cortex is replaced by an expansile cystic mass lined by three intermixed cell types: mucinous cells with intracytoplasmic mucin vacuoles, epidermoid cells with abundant cytoplasm and focal keratinization, and intermediate cells with minimal cytoplasm. In areas, these cells demonstrate complex architecture with invasion into the surrounding stroma.
Discussion: Mucoepidermoid carcinoma is the most common malignant salivary gland tumor. The majority of these tumors harbor a t(11;9) CRTC1-MAML2 fusion, which has been associated with a better prognosis. However, the main prognostic determinant is grade, which is assessed by the extent of a cystic component, degree of invasive growth, cytologic atypia, and necrosis. Mucoepidermoid carcinoma can involve the jaw bones by two routes: invasion from a peripheral tumor based in the gingiva or growth as a central lesion originating within the bone itself. In central lesions, the main differential diagnosis includes a glandular odontogenic cyst, a simple cyst with duct-like spaces, variable amounts of mucous cells, and lack of architectural complexity or invasive growth. Although glandular odontogenic cysts were once thought to be the precursors to central mucoepidermoid carcinomas, they have recently been shown to lack the CRTC1-MAML2 fusion. Peripheral tumors must be distinguished from adenosquamous carcinomas, which generally show separate superficial squamous components and deep glandular components rather than the intermixed cell types seen in mucoepidermoid carcinomas.
