FaCD Online Syndrome Fact Sheet

Last updated: 20 Mar 2008

Name: Salivary Gland Tumors, Familial Clustering of

Synonym: incl.: Familial Submandibular Gland Carcinoma, Familial Salivary Glands Cystadenolymphoma, Familial Pleomorphic Adenoma, Familial Parotid Gland Acinic Cell Carcinoma

Mode of Inheritance: multifact?

Tumor features

acinic cell carcinoma of the parotid glands
malignant lymphoepithelial lesion (MLEL) of the parotid glands
mucoepidermoid carcinoma of the submandibular glands
parotid gland cancer
parotid gland tumor, mixed (pleomorphic)
salivary gland (cyst)adenolymphoma (Warthin tumor)

Tumor features (possible)

nasopharyngeal cancer
pituitary adenoma


Russo et al.[1] reported a mother and son with a cystadenolymphoma (Wartin tumor) of the salivary glands at the age of 73 and 51 years, respectively. Other familial clusters of Wartin tumors (brother(65 yrs)-brother(69 yrs) and mother(76 yrs)-son(57 yrs)) had been reported previously by Skerlavay et al.[2] and Noyek et al.[3].

Familial clustering of pleomorphic adenoma of the parotid glands has been reported by Hayter and Robertson[4]: 2 brothers (diagnosed at 27 and 29 yrs, respectively), and Klausner and Handler[5]: brother-sister (11 and 15 yrs, respectively) and Ahn et al: 2 siblings[11]

Newman et al.[6] reported on a 33-year-old Bolivian male diagnosed with moderately differentiated adenocarcinoma of the submandibular gland. The patient’s sister was diagnosed with a low-grade mucoepidermoid carcinoma of the submandibular gland at the age of 32 years.

The occurrence of an acinic cell carcinoma of the parotid gland, diagnosed at the age of 35 years and a similar tumor diagnosed in his 16-year-old daughter was presented by Depowski et al.[7]. Other examples have been reported as well, including a case with a pituitary adenoma[10]

Merrick et al.[8] and Albeck et al.[9] have drawn attention to the familial clustering of salivary gland (anaplastic) carcinoma in Greenland natives. Some families showed clustering of nasopharyngeal cancer as well[9].


[1] Russo F, Coscarella G, Prisco LA, Spina C, Sica GS, Sileri P, Neri A, Gaspari AL. [Cystadenolymphoma of the salivary glands: is there a familial role?]. Ann Ital Chir 1999; 70(2):233-7.
[2] Skerlavay MA, Gardner B, Nicastri AD. Letter: Warthin tumor in two brothers. Arch Pathol Lab Med 1976; 100:508.
[3] Noyek AM, Pritzker KP, Greyson ND, Blackstein M, Chapnik JS, Shapiro BJ. Familial Warthin's tumor. 1. Its synchronous occurrence in mother and son. 2. Its association with cystic oncocytic metaplasia of the larynx. J Otolaryngol 1980; 9:90-96.
[4] Hayter JP, Robertson JM. Familial occurrence of pleomorphic adenoma of the parotid gland. Br J Oral Maxillofac Surg 1990; 28:333-334.
[5] Klausner RD, Handler SD. Familial occurrence of pleomorphic adenoma. Int J Pediatr Otorhinolaryngol 1994; 30:205-210.
[6] Newman AN, Calcaterra TC, Bhuta S. Familial carcinoma of the submandibular gland. Arch Otolaryngol 1981; 107:169-171.
[7] Depowski PL, Setzen G, Chui A, Koltai PJ, Dollar J, Ross JS. Familial occurrrence of acinic cell carcinoma of the parotid gland. Arch Pathol Lab Med 1999; 123:1118-1120.
[8] Merrick Y, Albeck H, Nielsen NH, Hansen HS. Familial clustering of salivary gland carcinoma in Greenland. Cancer 1986; 57:2097-2102.
[9] Albeck H, Bentzen J, Ockelmann HH, Nielsen NH, Bretlau P, Hansen HS. Familial clusters of nasopharyngeal carcinoma and salivary gland carcinomas in Greenland natives. Cancer 1993; 72(1):196-200.
[10] Delides A, Velegrakis G, Kontogeorgos G, Karagianni E, Nakas D, Helidonis E. Familial bilateral acinic cell carcinoma of the parotid synchronous with pituitary adenoma: case report. Head & neck 2005; 27(9):825-8.
[11] Ahn MS, Hayashi GM, Hilsinger RL, Lalwani AK. Familial mixed tumors of the parotid gland. Head & neck 1999; 21(8):772-5.