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Malignant tumours of the salivary 
glands 
In great Britain, the incidence of malignant salivary neoplasm is 1.2% per 100000. 
1. Salivary malignancy increases with increased smoking & alcohol consumption. 
2. Ionizing radiation is also well-documented cause. 
3. Livestock feed processing has an increased risk of malignant salivary neoplasm( aflatoxin B1). 
4. Diet ; polyunsaturated fatty acids seems to exert beneficial effect. Decrease intake of 
polyunsaturated fatty acid inverse relationship with salivary malignancy. 
5. EBV infection. 
Molecular biology of salivary cancer 
Immunohistochemical markers of prognostic interest in salivary malignancy include the following 
1. Proliferating cell nuclear antigen(PCNA) immunoreativity is significantly increased in 
malignant salivary disease compared with benign disease. 
2. Ki-67(protein product of gene) is a useful marker in adenoid cystic carcinoma. 
3. Bcl-2 expression is relatively low in salivary carcinoma. 
4. Fibroblast growth factor 1 & 2 & fibroblast growth factor receptor 1 are overexpressed 
in human salivary malignancy. 
5. Oestrogen &/ or progestrone receptors are expressed in some malignant salivary 
tumours suggesting an opportunity for endocrine intervention. 
Molecular techniques are yielding a host of interesting data but as yet none of clinical relevance 
other than disproving viral theories of salivary oncogenesis especially EBV, HPV, Herpes virus & CMV. 
The classification &histology of malignant salivary tumours 
1. Acinic cell carcinoma; 
2. Mucoepidemoid carcinoma; 
3. Adenoid cystic carcinoma; 
4. Polymorphous low-grade adenocarcinoma; 
5. Papillary cystadenocarcinoma; 
6. Adenocarcinoma;
7. Carcinoma ex pleomorphic adenoma; 
8. Malignant mixed tumour; 
9. Squamous cell carcinoma; 
10. Undiffierentiated carcinoma. 
Acinic cell carcinoma(usually bilateral) 
Acinic cell carcinoma is often regarded as a low grade malignancy. Bilateral salivary malignancy is 
very rare but acinic cell carcinoma is the most common after Warthin’s tumour(benign) 
Clinically acinic cell carcinoma presents as a painless lump, usually in the parotid gland. It is 
commonly a solitary encapsulated lesion with well-defined margin. 
Histological diagnosis is difficult& the differential diagnosis may include thyroid, mucoepidermoid & 
myoepithelial carcinoma, oncocytoma & metastatic renal cell carcinoma. 
Although acinic cell carcinoma almost certainly has the best survival of any salivary cancers it has a 
long natural history with a 15- year survival rate of only 55%. 
Treatment should probably involve both total or near total parotidectomy with preservation of the 
facial nerve unless involved. Neck dissection & post-operative radiotherapy outcomes are limited. 
Mucoepidermoid carcinoma 
This tumour is the most common of the salivary malignancies, accounting for one-third of cases. 
Often presenting as a painless mass at the more benign end of the spectrum of this tumour to more 
aggressive cancers presenting with pain , swelling & facial palsy. 
In high-grade cancers, lymph node metastases occur in nearly three-quarters of patients at 
presentation. 
In case of mucoepidermod carcinoma, there are mucous secreting cells, epidermoid cells & 
intermediate cells. 
Mucoepidermoid carcinoma may be useful histological classification in term of high grade & low 
grade. 
Low grade tumours tend to be cystic whereas high grade tumour tend to be solid with area of 
necrosis & haemorrhage. 
1. For most favourable tumours a superficial parotidectomy with preservation of facial nerve. 
2. Radical excision is necessary for patients with large / high grade tumours. An associated 
elective neck dissection to include level 1, 2 & 3 for the N0 neck would be appropriate. 
Postoperative irradiation to the primary site & neck must be carried out in high grade 
tumour.
Adenoid cystic carcinoma 
Almost one-third of adenoid cystic carcinoma develop in major salivary glands. 
Forty percent are locally advanced at the time of presentation. 
11% have distant metastases at presentation. 
There are three histological subtypes; solid 25%, cribriform40%, & tubular 20%. 
Solid pattern is associated with poorer survival than other types. 
This disease has a very long history cure never achieved with 100 % recurrence at 30years. 
Adenoid cystic carcinoma have predisposition to invade & spread along peripheral nerves. 
Carcinoma ex pleomorphic adenoma 
These tumours account for upto 10% of all malignant salivary cancer. It is very rarely develop within 
a pre-existing pleomorphic adenoma( 3%) although the risk of malignancy may increase to as much 
as 10% by 15 years. Malignant transformation most commonly took place 
1. in men over 40 years of age, 
2. in tumours of deep lobe, 
3. in solitary nodule greater than 2 cm & 
4. in those with history of a previous operation. 
Histological classification type 
1. true carcinoma in a pleomorphic adenoma; most common type 
2. carcinosarcoma in which both components of the tumour are malignant & can metastasize. 
3. Metastasizing mixed tumour( metastases contain malignant element & structure typical of a 
benign tumour). 
4. Noninvasive carcinoma or carcinoma in situ in a pre-existing benign pleomorphic adenoma. 
The prognosis of carcinoma ex pleomorphic adenoma is poor. With cause-specfic survival at 5 years 
of 40%, 10 years 24%, 15years 19%. 
During follow up,one- quarter of patients develop lymph node disease & one-third develop distant 
metastases. 
Undifferentiated carcinoma 
Histologically identical to nasopharyngeal carcinoma, later needs to be excluded as primary site. 
It is very unusual to achieve cure of the neoplasm with median survival figures of 30 months. 
Treatment with radiation.
Lymphoma 
Three criteria for the diagnosis of this disease 
1. Extraglandular lymphoma must not be present. 
2. There is histological proof that the lymphoma involves the gland parenchyma & not the 
intraglandular lymph nodes; 
3. Immunohistochemical screening must confirm the presence of lymphoma markers. 
Most lymphoma involve the salivary gland as part of a regional disease. 
In general , low grade non-Hodgkin lymphoma is either not treated at all or if it is conservative 
monoclonal management is undertaken with drugs such as chlorambucil. 
High-grade lesion are usually treated aggressively with complex regimen such as VAPEC-B. 
Both have median survival of approximately eight year. 
Best clinical practice(adenoid cystic carcinoma) 
Diagnostic work up should be include MRI of the primary site& neck with CT of the lungs, liver & 
possibly the primary site if bone involvement is a likelyhood, as well as isotope bone scans for 
mestastases. 
Treatment is with radical surgery to the primary site without elective neck dissection & with post-operative 
radiotherapy to achieve the maximum duration of locoregional control. 
Large tumour are almost inevitably associated with nerve involvement. If gross nerve involvement or 
frozen section of nerve involvement is found at operation, the nerve should be sacrifice & 
immediate nerve grafting carried out. 
The role of chemotherapy appears limited , with some response possibly with Cisplatinum.

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Malignant tumours of the salivary glands

  • 1. Malignant tumours of the salivary glands In great Britain, the incidence of malignant salivary neoplasm is 1.2% per 100000. 1. Salivary malignancy increases with increased smoking & alcohol consumption. 2. Ionizing radiation is also well-documented cause. 3. Livestock feed processing has an increased risk of malignant salivary neoplasm( aflatoxin B1). 4. Diet ; polyunsaturated fatty acids seems to exert beneficial effect. Decrease intake of polyunsaturated fatty acid inverse relationship with salivary malignancy. 5. EBV infection. Molecular biology of salivary cancer Immunohistochemical markers of prognostic interest in salivary malignancy include the following 1. Proliferating cell nuclear antigen(PCNA) immunoreativity is significantly increased in malignant salivary disease compared with benign disease. 2. Ki-67(protein product of gene) is a useful marker in adenoid cystic carcinoma. 3. Bcl-2 expression is relatively low in salivary carcinoma. 4. Fibroblast growth factor 1 & 2 & fibroblast growth factor receptor 1 are overexpressed in human salivary malignancy. 5. Oestrogen &/ or progestrone receptors are expressed in some malignant salivary tumours suggesting an opportunity for endocrine intervention. Molecular techniques are yielding a host of interesting data but as yet none of clinical relevance other than disproving viral theories of salivary oncogenesis especially EBV, HPV, Herpes virus & CMV. The classification &histology of malignant salivary tumours 1. Acinic cell carcinoma; 2. Mucoepidemoid carcinoma; 3. Adenoid cystic carcinoma; 4. Polymorphous low-grade adenocarcinoma; 5. Papillary cystadenocarcinoma; 6. Adenocarcinoma;
  • 2. 7. Carcinoma ex pleomorphic adenoma; 8. Malignant mixed tumour; 9. Squamous cell carcinoma; 10. Undiffierentiated carcinoma. Acinic cell carcinoma(usually bilateral) Acinic cell carcinoma is often regarded as a low grade malignancy. Bilateral salivary malignancy is very rare but acinic cell carcinoma is the most common after Warthin’s tumour(benign) Clinically acinic cell carcinoma presents as a painless lump, usually in the parotid gland. It is commonly a solitary encapsulated lesion with well-defined margin. Histological diagnosis is difficult& the differential diagnosis may include thyroid, mucoepidermoid & myoepithelial carcinoma, oncocytoma & metastatic renal cell carcinoma. Although acinic cell carcinoma almost certainly has the best survival of any salivary cancers it has a long natural history with a 15- year survival rate of only 55%. Treatment should probably involve both total or near total parotidectomy with preservation of the facial nerve unless involved. Neck dissection & post-operative radiotherapy outcomes are limited. Mucoepidermoid carcinoma This tumour is the most common of the salivary malignancies, accounting for one-third of cases. Often presenting as a painless mass at the more benign end of the spectrum of this tumour to more aggressive cancers presenting with pain , swelling & facial palsy. In high-grade cancers, lymph node metastases occur in nearly three-quarters of patients at presentation. In case of mucoepidermod carcinoma, there are mucous secreting cells, epidermoid cells & intermediate cells. Mucoepidermoid carcinoma may be useful histological classification in term of high grade & low grade. Low grade tumours tend to be cystic whereas high grade tumour tend to be solid with area of necrosis & haemorrhage. 1. For most favourable tumours a superficial parotidectomy with preservation of facial nerve. 2. Radical excision is necessary for patients with large / high grade tumours. An associated elective neck dissection to include level 1, 2 & 3 for the N0 neck would be appropriate. Postoperative irradiation to the primary site & neck must be carried out in high grade tumour.
  • 3. Adenoid cystic carcinoma Almost one-third of adenoid cystic carcinoma develop in major salivary glands. Forty percent are locally advanced at the time of presentation. 11% have distant metastases at presentation. There are three histological subtypes; solid 25%, cribriform40%, & tubular 20%. Solid pattern is associated with poorer survival than other types. This disease has a very long history cure never achieved with 100 % recurrence at 30years. Adenoid cystic carcinoma have predisposition to invade & spread along peripheral nerves. Carcinoma ex pleomorphic adenoma These tumours account for upto 10% of all malignant salivary cancer. It is very rarely develop within a pre-existing pleomorphic adenoma( 3%) although the risk of malignancy may increase to as much as 10% by 15 years. Malignant transformation most commonly took place 1. in men over 40 years of age, 2. in tumours of deep lobe, 3. in solitary nodule greater than 2 cm & 4. in those with history of a previous operation. Histological classification type 1. true carcinoma in a pleomorphic adenoma; most common type 2. carcinosarcoma in which both components of the tumour are malignant & can metastasize. 3. Metastasizing mixed tumour( metastases contain malignant element & structure typical of a benign tumour). 4. Noninvasive carcinoma or carcinoma in situ in a pre-existing benign pleomorphic adenoma. The prognosis of carcinoma ex pleomorphic adenoma is poor. With cause-specfic survival at 5 years of 40%, 10 years 24%, 15years 19%. During follow up,one- quarter of patients develop lymph node disease & one-third develop distant metastases. Undifferentiated carcinoma Histologically identical to nasopharyngeal carcinoma, later needs to be excluded as primary site. It is very unusual to achieve cure of the neoplasm with median survival figures of 30 months. Treatment with radiation.
  • 4. Lymphoma Three criteria for the diagnosis of this disease 1. Extraglandular lymphoma must not be present. 2. There is histological proof that the lymphoma involves the gland parenchyma & not the intraglandular lymph nodes; 3. Immunohistochemical screening must confirm the presence of lymphoma markers. Most lymphoma involve the salivary gland as part of a regional disease. In general , low grade non-Hodgkin lymphoma is either not treated at all or if it is conservative monoclonal management is undertaken with drugs such as chlorambucil. High-grade lesion are usually treated aggressively with complex regimen such as VAPEC-B. Both have median survival of approximately eight year. Best clinical practice(adenoid cystic carcinoma) Diagnostic work up should be include MRI of the primary site& neck with CT of the lungs, liver & possibly the primary site if bone involvement is a likelyhood, as well as isotope bone scans for mestastases. Treatment is with radical surgery to the primary site without elective neck dissection & with post-operative radiotherapy to achieve the maximum duration of locoregional control. Large tumour are almost inevitably associated with nerve involvement. If gross nerve involvement or frozen section of nerve involvement is found at operation, the nerve should be sacrifice & immediate nerve grafting carried out. The role of chemotherapy appears limited , with some response possibly with Cisplatinum.