Malignant Salivary Gland Neoplasms Steven R Taylor, MD Robert P Zitsch, MD December 3, 2014
Outline
Overview
Anatomy
Pathogenesis
Select Primary Malignancies of the Salivary Glands
With quiz questions throughout
Overview
Primary intent of this lecture is to provide residents a good overview of many of the primary malignancies of the salivary glands
Inservice
13weeks, 3 days 57 minutes away
Overview Diagnostics and Work up
Pathophysiology and Differential diagnosis
Salivary Disease – Patient Care Level 1 Level 2 Obtains basic history and Obtains focused history and physical physical, including comprehensive head and neck exam, neck and cranial nerve exam; orders appropriate labs, fineneedle aspiration (FNA), and radiologic studies Understands normal Understands factors salivary gland function precipitating inflammatory salivary disease
Level 3 Interprets appropriate lab, pathologic, and radiologic studies
Level 4 Accurately tumor node metastasis (TNM) stages a specific patient
Level 5 Performs ultrasound guided FNA of salivary gland mass
Describes an accurate differential diagnosis of a salivary gland mass; able to clinically distinguish neoplastic from nonneoplastic etiologies
Makes correct diagnosis from clinical, radiologic, and pathologic information; knows histopathologic findings of common neoplastic processes
Teaches pathophysiology
Treatment and Surveillance
Knows treatment of sialadenitis
Discusses treatment modality options in general terms (including adjuvant treatment)
Discusses appropriate therapeutic options and understands implications of those options
Formulates appropriate treatment plan for a specific salivary gland cancer patient based on primary site, disease stage, and patient factors
Procedure and Surgery
Knows how to scrub; performs surgical time out; maintains sterile field
Performs intra-operative patient prep; raises skin flaps in appropriate plane; able to aesthetically close wound
Performs procedure with assistance; identifies neurovascular structures
Completes procedure with oversight
Lists some potential complications
Recognizes common complications; obtains appropriate consultations for patient management
Recognizes and is able to treat and/or develop treatment plan for common complications
Complications and Management
Comments:
Performs extended dissection of parotid bed neoplasm with preservation of neurovascular (NV) structures as appropriate; teaches procedure Treats complex complications
Anatomy
Netter Atlas. 4th Ed.
3 major glands
Parotid
Submandibular
Lingual
Minor Salivary Glands
Anatomy
http://flylib.com/books/en/2.953.1.23/1/
Anatomy
Parotid Gland
Superficial layer of the deep cervical fascia forms the parotid gland fascia
Serous secretions from the acinus glands
Empty’s via Stenson’s duct
2nd maxillary molar
90% Saliva production http://www.siumed.edu/~dking2/erg/GI121b.htm
Quiz Question
Which of the following features is unique to the parotid gland compared to the other major salivary glands? A. Lymphoid tissue (nodes) B. Serous and mucinous glands C. Innervation from the inferior salivatory nucleus D. A and C E. All of the above
Quiz Question
Which of the following features is unique to the parotid gland compared to the other major salivary glands? A. Lymphoid tissue (nodes) B. Serous and mucinous glands C. Innervation from the inferior salivatory nucleus D. A and C E. All of the above
Anatomy
Innervation of Parotid Gland
Para NS
Inferior salivatory nucleus
glossopharyngeal nerve(Jacobson’s)
lesser/superficial petrosal nerve
otic ganglion
postganglionic parasympathetic fibers
carried by auriculotemporal branch of CN V3
parotid gland
http://quizlet.com/3848921/gastro-intestinal-system-flash-cards/
Quiz Question
Which structure separates the parotid from the submandibular gland? A. Lingual nerve B. Greater auricular nerve C. Stylomandibular ligament D. Stylohyoid ligament
E. None. The two glands are integrated at the inferior anterior aspect of the parotid gland
Quiz Question
Which structure separates the parotid from the submandibular gland? A. Lingual nerve B. Greater auricular nerve C. Stylomandibular ligament D. Stylohyoid ligament
E. None. The two glands are integrated at the inferior anterior aspect of the parotid gland
Anatomy
Submandibular Gland
Superficial Layer of the deep cervical fascia forms the overlying fascia
2/3 serous, 1/3 mucus
5% Saliva production
Drains through Wharton’s Duct
No intraglandular nodes http://www.pathologyoutlines.com/images/salivary/01_17L.jpg
Quiz Question
Wharton’s Duct travels between the mylohyoid and Hyoglossus, and A. superior to XII and lingual nerve
B. inferior to XII and lingual nerve C. superior to XII and inferior to lingual nerve D. Inferior to XII and superior to lingual nerve
Quiz Question
Wharton’s Duct travels between the mylohyoid and Hyoglossus, and A. superior to XII and lingual nerve B. inferior to XII and lingual nerve
C. superior to XII and inferior to lingual nerve D. Inferior to XII and superior to lingual nerve
Anatomy
Sublingual Gland
Drain into FOM through Rivinus ducts
Other ducts may coalesce to form Bartholin’s duct, which drains into the Wharton’s duct.
2/3 Mucus, 1/3 Serous
Innervation through the Superior salivatory nucleus through the chorda tympani-submandibular ganglion-- post ganglionic fibers
Ranulae most commonly form within this gland http://www.pathologyoutlines.com/images/salivary/01_18.jpg
Anatomy
Minor Salivary Glands
800-1000 glands
Not encapsulated by connective tissue
Highest concentration on the hard palate
Primarily Mucus secretion
http://www.pathologyoutlines.com/images/salivary/01_20.jpg
Anatomy
Minor Salivary Glands
Glands of Blandin- anterolingual
Van Ebner Glands
Under apex of tongue near lingual frenulum
Near circumvallate papillae
Serous secretion- aids in lipid hydrolysis
Increase perception of taste
Weber’s Glands
Soft palate/Superior tonsil pole
Theory of etiology of PTA
Pathogenesis
2 Theories
Multicellular Theory
Each type of neoplasm is thought to originate from a distinctive cell type within the salivary gland unit.
According to this theory, Warthin's and oncocytic tumors are thought to arise from striated ductal cells, acinic cell tumors from acinar cells, and mixed tumors from intercalated duct and myoepithelial cells.
Supported by the observation that all differentiated salivary cell types retain the ability to undergo mitosis and regenerate
http://flylib.com/books/en/2.953.1.23/1/
Pathogenesis
2 Theories
Bicellular reserve cell theory
Assumes that the origin of the various types of salivary neoplasms can be traced to the basal cells of either the excretory or the intercalated duct.
According to this theory, either of these two cells can act as a reserve cell with the potential for differentiation into a variety of epithelial cells. Hence, despite the seeming heterogeneity of salivary tumors, they are all thought to arise from one of two pluripotential cell populations.
In this model, adenomatoid tumors, including pleomorphic adenoma and oncocytic tumors, are derived from the reserve cell of the intercalated duct, whereas epidermoid tumors, such as squamous cell carcinoma and mucoepidermoid carcinomas, are derived from the reserve cell of the excretory duct
Salivary Gland Neoplasms
2.2-2.5/100,000 people
2% of Head and Neck Neoplasms
Approximately 80% of salivary gland neoplasms are benign
Parotid- 80% neoplasms
Submandibular- 10-15% neoplasm
80% benign (20% malignant)
Ca. 50% benign (50% malignant)
Sublingual/Minor- 5-10%
Ca 20-40% benign (60-80% malignant)
Quiz Question
What is the most common malignant neoplasm of the sublingual gland in adults? A. Mucoepidermoid caricinoma B. Adenoid cystic
C. Acinic cell carcinoma D. Adenocarcinoma E. Carcinoma ex-pleomorphic adenoma
Quiz Question
What is the most common malignant neoplasm of the sublingual gland in adults? A. Mucoepidermoid caricinoma B. Adenoid cystic
C. Acinic cell carcinoma D. Adenocarcinoma E. Carcinoma ex-pleomorphic adenoma
Salivary Gland Neoplasms
95% salivary gland tumors (benign/malignant) are in adults
50-65% of tumors in children are benign
Adults
Mucoepidermoid carcinoma most common malignancy in the parotid gland
Adenoid cystic more common in the SMG, Sublingual, minor salivary glands
Children
Mucoepidermoid carcinoma most common in all glands
Salivary Gland Neoplasms
Management of Salivary Gland Mass
Imaging- CT neck with contrast
Biopsy- FNA
86%(84-100%) sensitive, 92% specific (54-95%), 90% accurate compared to final path (84-97%)
Frozen Section
77% sensitive, 100% specific, 88% accurate
Seethala RR, LiVolsi VA, Baloch ZW. Relative accuracy of fine-needle aspiration and frozen section in the diagnosis of lesions of the parotid gland. Head Neck. 2005 Mar;27(3):217-23
Quiz Question
A 57 y M presents to your clinic with a painless, 3 cm parotid mass with normal facial function. No palpable neck adenopathy. A partial parotidectomy was performed. FNA and frozen section were indeterminate for type of malignancy, but final pathology showed a 3.4cm high grade mucoepidermoid carcinoma without perineural invasion. What is the next step in management? A. Elective Radiation B. Elective dissection alone C. Observation D. A or B E. All of the above
Quiz Question
A 57 y M presents to your clinic with a painless, 3 cm parotid mass with normal facial function. No palpable neck adenopathy. A partial parotidectomy was performed. FNA and frozen section were indeterminate for type of malignancy, but final pathology showed a 3.4cm high grade mucoepidermoid carcinoma without perineural invasion. What is the next step in management? A. Elective Radiation B. Elective dissection alone C. Observation D. A or B E. All of the above
Salivary Gland Neoplasms
Management of the neck?
Gold DR, Annino DJ. Management of the Neck in Salivary Gland Carcinoma. Otolaryngol Clin N Am 38 (2005) 99–105.
Salivary Gland Neoplasms
Gold DR, Annino DJ. Management of the Neck in Salivary Gland Carcinoma. Otolaryngol Clin N Am 38 (2005) 99–105.
Salivary Gland Neoplasm
Gold DR, Annino DJ. Management of the Neck in Salivary Gland Carcinoma. Otolaryngol Clin N Am 38 (2005) 99–105.
Salivary Gland Neoplasm
Staging of Salivary Gland Neoplasms
http://www.cancer.gov/cancertopics/pdq/treatment/salivarygland/HealthProfessional/Table1
Malignant Salivary Gland Neoplasms
Mucoepidermoid carcinoma
Polymorphous low grade adenocarcinoma
Adenoid cystic
Acinic Cell carcinoma
Clear cell carcinoma
Adenocarcinoma
Malignant Mixed
Epithelial-myoepithelial carcinoma
Undifferentiated carcinoma
Carcinoma ex pleomorphic adenoma
Carcinosarcoma
Metastatic Mixed
Squamous cell carcinoma
Quiz Question
A 67 y M referred to your head and neck clinic for a nontender mass of his right retromolar trigone. On physical exam, there is a 4-5 cm area of mucosal irregularity, with palate and anterior tonsillar pillar involvement. No neck disease. An biopsy was performed, which was consistent with SCC. However, final pathology was consistent with a parotid malignancy. Which parotid malignancy is most likely to be confused with SCC? A. Low grade mucoepidermoid carcinoma
B. Polymorphous low grade adenocarcinoma C. Adenoid cystic carcinoma D. High grade mucoepidermoid carcinoma
Quiz Question
A 67 y M referred to your head and neck clinic for a nontender mass of his right retromolar trigone. On physical exam, there is a 4-5 cm area of mucosal irregularity, with palate and anterior tonsillar pillar involvement. No neck disease. An biopsy was performed, which was consistent with SCC. However, final pathology was consistent with a parotid malignancy. Which parotid malignancy is most likely to be confused with SCC? A. Low grade mucoepidermoid carcinoma
B. Polymorphous low grade adenocarcinoma C. Adenoid cystic carcinoma D. High grade mucoepidermoid carcinoma
Mucoepidermoid Carcinoma
From the epithelial cells of the excretory ducts
Also from the goblet cells (hence the “muco” part)
Primarily present as painless mass
30% of salivary malignancies
5-9% of neoplasms
Mucoepidermoid Carcinoma
90% low grade MucoEp
High mucin content
10 % high grade
Aggressive clinically
70-90% 5 yr survival
45% 5 yr survival
PAS (periodic acid Schiff) + stain for mucin
Mucoepidermoid Carcinoma
Low grade Mucoepidermoid carcinoma
Mucus cells> epidermoid cells
Prominent cysts
http://cai.md.chula.ac.th/chulapatho/chu lapatho/systemic/ent/mslmeca3.html
http://upload.wikimedia.org/wikipedia/commons/8/86/Mu coepidermoid_carcinoma_%281%29_AB-PAS_stain.jpg
Mucoepidermoid Carcinoma
High grade mucoepidermoid
Epidermoid> mucus
Can resemble SCC
Mucin Stain
http://www.webpathology.com/image.asp?n=3&Case=119
Mucoepidermoid Carcinoma
Chan, RC, Chan JY. Head and Neck Mucoepidermoid Carcinoma: A Curious Association with Second Primary Malignancy. Otolaryngology–Head and Neck Surgery 2014, Vol. 151(5) 797–801.
Mucoepidermoid Carcinoma
Retrospective review of head and neck Mucoep from 01/03-12/13
57 cases
14/57 (24.6%) had second primary malignancy of the head and neck
MC- Nasopharyngeal carcinoma
2nd MC- Thyroid carcinoma
More common in patients with major salivary gland mucoep.
No longterm effect on survival
Chan, RC, Chan JY. Head and Neck Mucoepidermoid Carcinoma: A Curious Association with Second Primary Malignancy. Otolaryngology–Head and Neck Surgery 2014, Vol. 151(5) 797–801.
Mucoepidermoid Carcinoma
Chan, RC, Chan JY. Head and Neck Mucoepidermoid Carcinoma: A Curious Association with Second Primary Malignancy. Otolaryngology–Head and Neck Surgery 2014, Vol. 151(5) 797–801.
Quiz Question
A 51 y F is referred to your clinic for a h/o a painful right sided facial mass. On examination, you notice facial paralysis. The patient states that it has been that way for over 5 years, but that it slowly progressed from superior to inferior. The patient has a fixed mass of the lateral face, with an aspect draining behind her ear. Outside core biopsy showed “infiltrating atypical glandular proliferation.” What is the most likely diagnosis? A. Adenoid cystic carcinoma
B. High grade mucoepidermoid carcinoma C. Adenocarcinoma D. Squamous cell carcinoma
Quiz Question
A 51 y F is referred to your clinic for a h/o a painful right sided facial mass. On examination, you notice facial paralysis. The patient states that it has been that way for over 5 years, but that it slowly progressed from superior to inferior. The patient has a fixed mass of the lateral face, with an aspect draining behind her ear. Outside core biopsy showed “infiltrating atypical glandular proliferation.” What is the most likely diagnosis? A. Adenoid cystic carcinoma
B. High grade mucoepidermoid carcinoma C. Adenocarcinoma D. Squamous cell carcinoma
Adenoid Cystic Carcinoma
Most common malignancy of the submandibular, sublingual and minor salivary glands in adults
From intercalated cells
High rate of perineural invasion-- Neutropism
20% present with facial paralysis
25% present with pain
Propensity for invasion
Adenoid Cystic Carcinoma
3 types
Cribriform- Most common
Swiss cheese
http://commons.wikimedia.org/wiki/File:Adenoid_cystic_carcinoma_-_intermed_mag.jpg
Adenoid Cystic Carcinoma
3 types
Cribriform- Most common
Swiss cheese
Tubular- Best prognosis
http://www.indianjcancer.com/viewimage.asp?img=IndianJournalofCancer_2010_47_4_424_73571_u3.jpg
Adenoid Cystic Carcinoma
3 types
Cribriform- Most common
Swiss cheese
Tubular- Best prognosis
Solid- Poor prognosis
http://www.indianjcancer.com/viewimage.asp?img=IndianJournalofCancer_2010_47_4_424_73571_u5.jpg
Adenoid Cystic Carcinoma
Do not perform elective neck dissection
Post op radiotherapy is advocated to minimize risk of local recurrence
Propensity for slow growth, metastases (lung)
Quiz Question
What is the second most common pediatric salivary malignancy? A. Mucoepidermoid carcinoma B. Adenoid Cystic Carcinoma C. Carcinoma ex-pleomorphic adenoma D. Adenosarcoma
E. Acinic Cell Carcinoma
Quiz Question
What is the second most common pediatric salivary malignancy? A. Mucoepidermoid carcinoma B. Adenoid Cystic Carcinoma C. Carcinoma ex-pleomorphic adenoma D. Adenosarcoma
E. Acinic Cell Carcinoma
Acinic Cell Carcinoma
2nd most common pediatric and parotid malignancy
Bilateral parotid disease in 3%
Most common
Pathology
Amyloid stroma on histo
PAS postiive
http://en.wikipedia.org/wiki/Acinic_cell_carcinoma
Acinic Cell Carcinoma
No elective neck required, but approximately 15% present with clinically pathologic nodes
Good prognosis
75% 5 year
50% 25 yr
Quiz Question
72y Male presents with a rapidly growing mass of the left lateral face. He states that 20 years ago, he had a biopsy of a salivary gland mass, which was “benign.” It had been stable until 3 months ago. What is the most likely diagnosis? A. Clear cell carcinoma B. Carcinoma ex-pleomorphic adenoma
C. Lymphoma D. Squamous cell carcinoma
Quiz Question
72y Male presents with a rapidly growing mass of the left lateral face. He states that 20 years ago, he had a biopsy of a salivary gland mass, which was “benign.” It had been stable until 3 months ago. What is the most likely diagnosis? A. Clear cell carcinoma B. Carcinoma ex-pleomorphic adenoma
C. Lymphoma D. Squamous cell carcinoma
Carcinoma ex-pleomorphic Adenoma
99% of all mixed malignant tumors
Develops in the existing epithelial component of a pleomorphic adenoma
Parotid > SMG> palate
Presentation
Longstanding painless mass with rapid degeneration and growth
Facial nerve weakness in 25-40%
Carcinoma ex-pleomorphic Adenoma
Metastases to lymph nodes, bones (vertebral bodies), brain
Tx
Resection, with neck dissection
Post op XRT
http://emedicine.medscape.com/article/1652374-overview#showall
Conclusion
Salivary gland malignancy is rare, but is seen
FNA and frozen can aid in diagnosis, but both can be nondiagnostic and final pathology may be needed
Do not sacrifice the facial nerve unless necessary
Elective neck dissection and/or radiation depending on the type of salivary gland malignancy
