Practice question answers Salivary gland pathology

Practice question answers
Salivary gland pathology
Updated: 02/13/2021
© Jun Wang, MD, PhD

1. A. Unilateral painful salivary gland enlargement with neutrophilic infiltration is most consistent with acute sialadenitis, usually bacterial infection. Mucinous carcinoma, regardless of location, has clusters of atypical malignant cells floating in mucin pools. Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Sjogren syndrome has dry mouth, dry eyes, and microscopically it has fibrosis, acinar atrophy and lymphocytic infiltrate. Squamous cell carcinoma has irregular growth, invasion, cytological atypia, squamous pearls and intercellular bridges.

2. B. Salivary gland mass with diffuse fibrosis, acinar atrophy and lymphocytic infiltrate, without involvement of other organs is most compatible with IgG4 related dacryoadenitis and sialadenitis. Acute sialadenitis usually has unilateral painful salivary gland enlargement with neutrophilic infiltration, but no fibrosis. Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Sjogren syndrome has dry mouth, dry eyes, and microscopically it has fibrosis, acinar atrophy and lymphocytic infiltrate. Squamous cell carcinoma has irregular growth, invasion, cytological atypia, squamous pearls and intercellular bridges.

3. C. IgG4 related dacryoadenitis and sialadenitis is one of the IgG4 related sclerosing diseases, like sclerosing retroperitoneal fibrosis. ANA and anti-SSA are seen in Sjogren syndrome and various connective tissue diseases, including SLE. Monoclonal IgG is seen in plasma cell disorders, including multiple myeloma, plasmacytoma and monoclonal gammopathy of undetermined significance (MGUS), etc. Rheumatoid factor is associated with rheumatoid arthritis, Sjogren syndrome, and other connective tissue diseases, including SLE.

4. D. Sjogren syndrome has dry mouth, dry eyes, and microscopically it has fibrosis, acinar atrophy and lymphocytic infiltrate. Acute sialadenitis usually has unilateral painful salivary gland enlargement with neutrophilic infiltration, but no fibrosis. IgG4 related dacryoadenitis and sialadenitis is one of the IgG4 related sclerosing diseases, usually without eye manifestation. Pleomorphic adenoma is a biphasic tumor with pleomorphic proliferation of both epithelial and stromal components. Squamous cell carcinoma has irregular growth, invasion, cytological atypia, squamous pearls and intercellular bridges.

5. A. ANA, anti-SSA, anti-SSB and rheumatic factor are seen in Sjogren syndrome and various connective tissue diseases, including SLE. IgG4 is associated with chronic sclerosing sialadenitis and other the IgG4 related sclerosing diseases, like sclerosing retroperitoneal fibrosis. IgE is associated with allergic reactions, including urticaria and atopic dermatitis. Immunofixation is useful for detecting monoclonal immunoglobulin, a sign for plasma cell neoplasia, including multiple myeloma, plasmacytoma and monoclonal gammopathy of undetermined significance (MGUS), etc. Rapid plasma reagin is a screening test for syphilis.

6. E. Benign collection of mucous in stroma with or without inflammatory changes is a mucocele.  Bullous pemphigoid is characterized by large tense blisters involving flexor surfaces, trunk and mucosa, and microscopically it has subepidermal blisters. Dermatitis herpetiformis has clusters of small pruritic vesicles or pustules, and microscopically it has subepidermal vesicle with neutrophilic aggregates. Herpes infection has cytological changes of multinucleation, nuclear molding and chromatin margining.

7. C. Pleomorphic adenoma is a biphasic tumor with pleomorphic proliferation of both epithelial and stromal components. IgG4 related dacryoadenitis and sialadenitis may presents as a salivary gland mass, but it usually has diffuse fibrosis, acinar atrophy and lymphocytic infiltrate.  Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Sjogren syndrome has dry mouth, dry eyes, and microscopically it has fibrosis, acinar atrophy and lymphocytic infiltrate. Warthin tumor is characterized papillary growth with dense lymphocytic infiltrate in stroma, and double layer of epithelial cells.

8. D. Pleomorphic adenoma is associated with PLAG1 rearrangement. APC mutation can be seen in various malignancies, including stomach and colon cancers. CDH-1 mutation is seen in diffuse type gastric adenocarcinoma, and lobular carcinoma of breast. P53 mutation can be seen in many malignancies. RET mutation can be seen in MEN2 and thyroid papillary carcinoma and medullary carcinoma.

9. B. Carcinoma ex pleomorphic adenoma is malignant transformation of pleomorphic adenoma, and most occurs in a patient with recurrent pleomorphic adenoma. Adenoid cystic carcinoma has basaloid cells with cribriform architecture. IgG4 related dacryoadenitis and sialadenitis may presents as a salivary gland mass, but it usually has diffuse fibrosis, acinar atrophy and lymphocytic infiltrate. Recurrent pleomorphic adenoma has the same morphology of previous tumor, without atypia. Warthin tumor is characterized papillary growth with dense lymphocytic infiltrate in stroma, and double layer of epithelial cells.

10. E. Warthin tumor is characterized papillary growth with dense lymphocytic infiltrate in stroma, and double layer of epithelial cells. Adenoid cystic carcinoma has basaloid cells with cribriform architecture. IgG4 related dacryoadenitis and sialadenitis may presents as a salivary gland mass, but it usually has diffuse fibrosis, acinar atrophy and lymphocytic infiltrate. Follicular lymphoma has packed lymphoid follicles. Hodgkin lymphoma has Reed Sternberg cells or LP cells.

11. C. Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Adenoid cystic carcinoma has basaloid cells with cribriform architecture. Metastatic colon adenocarcinoma usually has irregular glands, but rarely has squamous cells. Squamous cell carcinoma has irregular growth, invasion and cytological atypia, but not mucinous cells. Warthin tumor is characterized papillary growth with dense lymphocytic infiltrate in stroma, and double layer of epithelial cells.

12. B. Mucoepidermoid carcinoma is associated with MECT1-MAML2 fusion. APC mutation can be seen in various malignancies, including stomach and colon cancers. PLAG1 rearrangement is seen in pleomorphic adenoma. RB1 abnormalities may be seen in various tumors, including retinoblastoma, urothelial carcinoma, and HPV associated cervical or oral squamous cell carcinoma, etc. RET mutation can be seen in MEN2 and thyroid papillary carcinoma and medullary carcinoma.

13. C. See discussion of question 11.

14. C. Mucin stain will highlight mucin production in mucinous cell containing tumors, including mucoepidermoid carcinoma. E-cadherin may be used to differentiate ductal and lobular carcinoma of breast. P16 is usually overexpressed in HPV associated tumors. Silver stain can be used to detect microorgansims, such as helicobacter or dermatophytosis.

15. C. See discussion of question 8.

16. A. Adenoid cystic carcinoma has basaloid cells with cribriform architecture. Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Small cell carcinoma and small lymphocytic lymphoma have sheets of small to intermediate sized cells with scant cytoplasm, usually without cribriform architecture, regardless of the location. Squamous cell carcinoma has irregular growth, invasion, cytological atypia, squamous pearls and intercellular bridges.

17. D. Adenoid cystic carcinoma tends to have perineural invasion. In this case, it most likely has affected the mandibular branch of trigeminal nerve.

18. A. Acinic cell carcinoma has sheets of cells with acinar cell differentiation, including production of amylase and alpha-1-antichymotrypsin. Adenoid cystic carcinoma has basaloid cells with cribriform architecture. Carcinoid tumors express cytokeratin and neuroendocrine markers, but have decent amount cytoplasm. Mucoepidermoid carcinoma has mixed mucinous, intermediate, clear and squamous cells with cytological atypia. Small cell carcinoma has sheets of small to intermediate sized cells with scant cytoplasm, usually without cribriform architecture, regardless of the location. None of these expresses amylase or alpha-1-antichymotrypsin.

19. A. Acinic cell carcinoma has sheets of cells with acinar cell differentiation, including production of amylase and alpha-1-antichymotrypsin. Chromogranin is expressed by cells with neuroendocrine differentiation, including carcinoid and small cell carcinoma. CD20 is a B cell marker. IgG4 is seen in plasma cells of IgG4 related sclerosing diseases, including chronic sclerosing sialadenitis and sclerosing retroperitoneal fibrosis.

20. A. See discussion of question 18. Ranula is a type of mucocele.


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