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18 November 2010 – Case of the Week #191

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Case of the Week #191

Clinical History

A 54 year old man had a large posterior nasal septal polypoid mass protruding through his right nares, which was excised.

Micro images:

           

           
CK7                       CK20                     p63                       p63

What is your diagnosis?































Diagnosis:

Seromucinous hamartoma

Discussion:

Seromucinous hamartoma is a rare tumor resembling microglandular adenosis of the breast. The tumor is composed of a slightly disordered pattern of small serous tubules, ducts and glands with eosinophilic cytoplasm. There is no prominent mucinous component. The stroma is cellular with variable fibrosis and edema. The tubules are lined by one layer of cuboidal cells, without a myoepithelial or basal cell layer. There is no prominent mitotic activity or necrosis. The glandular cells are usually positive for CK7, CK19, S100, EMA and lysosyme. There is usually no or only focal p63 staining, due to the lack of myoepithelial cells (Virchows Arch 2010 Oct 5 [Epub ahead of print], Histopathology 2009;54:205.).

The differential diagnosis includes inflammatory nasal polyp, respiratory epithelial adenomatoid hamartoma and low grade sinonasal adenocarcinoma (Head Neck Pathol 2010;4:77). Inflammatory nasal polyp is composed of edematous lamina propria with a variable inflammatory infiltrate including eosinophils. Respiratory epithelial adenomatoid hamartoma (REAH) is composed of submucosal glands lined by respiratory epithelium, with periglandular hyalinization, and a variable connection to the surface. REAH may form a continuum with seromucinous hamartoma, as many cases have features of both entities. Low grade sinonasal adenocarcinoma is a diverse group of bland tubular or papillary tumors which have a potential to recur, and an uncertain potential for metastasis. These adenocarcinomas are divided into salivary and non-salivary types. The salivary types (adenoid cystic carcinoma, basal cell adenocarcinoma and epithelial-myoepithelial carcinoma) have a well defined myoepithelial component. The low grade non-salivary types are either intestinal (resemble intestinal epithelium, CK20+, CDX2+) or non-intestinal (back to back glands that are infiltrative).

Excision of a seromucinous hamartoma is usually curative, although these tumors may recur locally.

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