My Journey into the World of Salivary Gland Sebaceous Neoplasms

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1 My Journey into the World of Salivary Gland Sebaceous Neoplasms Douglas R. Gnepp Warren Alpert Medical School at Brown University Rhode Island Hospital Pathology Department Providence RI

2 Asked to present the case from which I learned the most Decided to present an unusual salivary gland tumor that was involved in one of my earliest publications A year after finishing my residency, I came across an unusual cystic salivary gland tumor that led me to review the literature: Only 2 other similar tumors had been previously published.

3 57-year-old white male Mass posterior to and below the angle of the right ramus of the mandible Present for ~6 years; occasionally fluctuated in size; measured 3 x 2 cm. Non-tender, mobile, at tail of right parotid Clinical impression: Warthin s tumor A right superficial parotidectomy was done with preservation of facial nerve Postop course uneventful; free of 1 yr

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8 Diagnosis Cystic sebaceous lymphadenoma (lymphoepithelial cyst with sebaceous differentiation )

9 Literature review turned up only rare reports of salivary gland sebaceous tumors A 2nd salivary gland tumor with sebaceous differentiation came thru our service a yr later Warthin s Tumor with sebaceous differentiation; also contained prominent area with necrotizing sialometaplasia (had been recently described)

10 Case again required literature review going thru standard texts of period, finding minimal information Evan and Cruckshank s Book on Salivary Gland Tumors Armed Forces Institute of Pathology Salivary Gland Fascicle, 2 nd series 3rd case was sent to me several months later Was an unusual adenoid cystic carcinoma with areas of sebaceous differentiation

11 Decided to put together extensive literature review because of my experience with these 3 tumors & lack of info in literature Was going to AFIP to augment my experience in head & neck tumors several times a yr working with Dr. Vince Hyams (ENT chairman & one of fathers of H&N path) Because of interest in salivary gland tumors I also spent time in oral path AFIP Working with Dr. Bob Brannon we decided to review AFIP experience with sebaceous tumors

12 Resulted in largest series of major salivary gland sebaceous tumors published at that time: 21 cases 5 sebaceous adenomas 9 sebaceous lymphadenomas 5 sebaceous carcinomas 2 sebaceous lymphadenocarcinomas (Ca ex sebaceous lymphadenoma)

13 Became clear due to size, could not publish AFIP experience & literature review together So published this separately in Path Annual As part of review I contacted authors of previously published studies to obtain current follow-up

14 Entire process taught me how a single case report could provide the nidus & intellectual curiosity to: 1) Thoroughly review world literature 2) How to organize and write several complicated papers 3) About the generosity of other authors who went out of their way to obtain follow-up information on previously published cases

15 Sebaceous Glands: Normal Parotid: 10 42% Submandibular: 5 6% Sublingual: Rare Intraoral: Fordyce's granules - found in up to 80% of individuals, most frequently on buccal mucosa, vermilion border of upper lip; less frequently in retromolar & anterior tonsillar pillar region If 15 or more lobules considered hyperplastic

16 Normal Sebaceous Glands Parotid

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18 Sebaceous Neoplasms Sebaceous adenoma Sebaceous lymphadenoma Sebaceous carcinoma Sebaceous lymphadenocarcinoma Sebaceous differentiation in other tumors > 150 primary tumors described to date

19 Sebaceous Adenoma Rare, ~0.1% of salivary gland neoplasms Slightly > 30 reported to date Mean age at presentation: 59 yrs (22-90 yrs) 61% major glands (48% parotid, 13% submandibular gland) 39% minor glands, most commonly buccal mucosa & lower molar/retromolar regions

20 Sebaceous Adenoma Range in size: 0.4 to 6 cm Grayish white to yellow gray on gross exam Typically well circumscribed to encapsulated Composed of variably sized sebaceous cell nests, often with areas of squamous differentiation Do not invade local structures; don t see cytologic atypia, necrosis, and mitoses Rx: Complete excision; do not recur

21 Sebaceous Adenoma

22 Sebaceous Lymphadenoma Sebaceous lymphadenoma is a rare, benign, well circumscribed to encapsulated tumor composed of well-differentiated, variably shaped & sized nests of sebaceous glands & ducts within a background of lymphocytes and lymphoid follicles. Cytologic atypia is minimal No tendency to invade local structures. Most common salivary gland sebaceous tumor

23 Sebaceous Lymphadenoma SL diagnosed typically in 6th to 8th decade (range, yrs) Typically present with painless mass (up to 6 cm); may slowly enlarge; < 100 reported 95% in or around parotid; rarely intraoral; one case with bilateral buccal mucosa origin Composed of variably sized sebaceous glands mixed with salivary ducts in a benign lymphoid background; occasionally have prominent ductal component with rare sebaceous glands

24 Histiocytes and foreign body giant cell inflammatory reactions common; help with differential diagnosis of mucoepidermoid Ca May see foci of residual lymph node Rarely foci of Warthin tumor may be found (combined SL and Warthin tumor). Necrosis rare; been observed in 1 tumor Rx: Complete surgical excision; should not recur

25 Non-Sebaceous Lymphadenoma Rarely may see tumors that are similar to SL without sebaceous differentiation Called: Non-sebaceous Lymphadenoma 22 reported; mean age mid 50s (11-78 yr) Typically present with mass noted for up to 5yr; range up to 8 cm (mean ~2.5 cm) Arise in parotid & periparotid area? Two subtypes: One similar to SL without sebaceous differentiation One composed of solid nests & interconnecting trabeculae with frequent peripheral palisading, somewhat similar to basal cell adenoma Rx: Simple excision: should not recur

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32 Sebaceous Carcinoma Def n: Malignant tumor composed mostly of sebaceous cells of varying maturity arranged in sheets and/or nests, with varying degrees nuclear atypia, cytologic pleomorphism & invasiveness 44 patients; typically present with painful mass; varying degrees facial nerve paralysis; occasional fixation to the skin Parotid: 29 tumors 66% (2 were Ca in Ca ex PA) Oral cavity: 8 tumors; frequently buccal mucosa Misc other sites: Submandibular & sublingual glands, vallecula, epiglottis & hypopharynx

33 Sebaceous Carcinoma Ranged in size up to 9.5 cm Appears to be 2 age peaks 3 rd, 7 th & 8 th decades (range yr) Frequently well circumscribed or partially encapsulated, with pushing or locally infiltrating margins Tumor cells arranged in multiple, variably sized nests or sheets; hyperchromatic nuclei; abundant clear, vacuolated to eosinophilic cytoplasm Cellular pleomorphism/atypia varies: Mild - severe

34 Necrosis: Frequent Perineural invasion: ~20%; vascular invasion rare Rx: Wide surgical excision for low-grade/low-stage Ca s. Adjunctive radiation therapy for higher-stage and higher-grade tumors. Tumors may recur; rarely will metastasize. The overall 5-year survival rate is 62%; less than skin and orbit (84.5%) Oral tumors may have better prognosis: 0 of 6 tumors in recent review have recurred or metastasized

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37 Sebaceous LymphadenoCa Malignant counterpart of sebaceous lymphadenoma Represents a carcinoma arising in a sebaceous lymphadenoma Rarest salivary gland sebaceous tumor Only 7 patients reported to date All tumors arose in parotid gland or pariparotid lymph node

38 4 patients in 7 th decade (4 th to 8 th decades) Present with a mass; 1 month 20 yrs Ranged in size up to 6 cm Tumors focally encapsulated with areas of invasion Invasive component: Squamous Ca, sebaceous Ca to poorly differentiated Ca with adenoid cystic-like and epithelial myoepithelial-like Ca

39 Rx: Depends on type Ca and stage; typically wide excision frequently with adjuvant radiation Long term follow-up not available 4 patients alive (4 months 6 yrs) 2 patients died of other causes (8 & 18 months) 1 report had no follow-up

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43 Sebaceous Lesions Associated with other Salivary Gland Neoplasms > 75 tumors reported Warthin s tumor & mixed tumor most frequent Followed in incidence by mucoepidermoid Ca, epithelial myoepithelial Ca, Ca ex pleomorphic adenoma & oncocytoma Less common: lymphoepithelial sialadenitis, sialolipoma (lipoadenoma), oncocytic lipoadenoma, basal cell adenoca, adenoid cystic Ca, acinic cell Ca, basal cell adenoma, adenoid cystic Ca ex a basal cell adenoma & adenoca (NOS).