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The signs and symptoms are similar to other malignant salivary gland tumours; however, it may have been preceded by an appreciable mass that was long-standing and did not appear to be growing.
Findings that suggest a malignant salivary gland tumour include rapid growth, facial weakness (due to facial nerve compression), pain, skin ulceration, fixation of the mastoid tip
and parasthesias.
Due to the diverse nature of salivary gland tumours, many different terms and classification systems have been used. Perhaps the most widely used currently is that system proposed by the World Health Organization in 2004, which classifies salivary neoplasms as primary or secondary, benign or malignant, and also by tissue of origin. This system defines five broad categories of salivary gland neoplasms:
Benign epithelial tumors
- Pleomorphic adenoma
- Warthin's tumor
- Myoepithelioma
- Basal cell adenoma
- Oncocytoma
- Canalicular adenoma
- Lymphadenoma
- "Sebaceous lymphadenoma"
- "Nonsebaceous lymphadenoma"
- Ductal papilloma
- "Inverted ductal papilloma"
- "Intraductal papilloma"
- "Sialadenoma papilliferum"
- Cystadenoma
- Malignant epithelial tumors
- Acinic cell carcinoma
- Mucoepidermoid carcinoma
- Adenoid cystic carcinoma
- Polymorphous low-grade adenocarcinoma
- Epithelial-myoepithelial carcinoma
- Clear cell carcinoma, not otherwise specified
- Basal cell adenocarcinoma
- Sebaceous carcinoma
- Sebaceous lymphadenocarcinoma
- Cystadenocarcinoma
- Low-grade cribriform cystadenocarcinoma
- Mucinous adenocarcinoma
- Oncocytic carcinoma
- Salivary duct carcinoma
- Salivary duct carcinoma, not otherwise specified
- Adenocarcinoma, not otherwise specified
- Myoepithelial carcinoma
- Carcinoma ex pleomorphic adenoma
- Mammary analogue secretory carcinoma
- Carcinosarcoma
- Metastasizing pleomorphic adenoma
- Squamous cell carcinoma
- Large cell carcinoma
- Lymphoepithelial carcinoma
- Sialoblastoma
- Soft tissue tumors
- Hemangioma
- Hematolymphoid tumors
- Hodgkin lymphoma
- Diffuse large B-cell lymphoma
- Extranodal marginal zone B cell lymphoma
- Secondary tumors (i.e. a tumor which has metastasized to the salivary gland from a distant location)
Others, not included in the WHO classification above, include:
- Intraosseous (central) salivary gland tumors
- Hybrid tumors (i.e. a tumor displaying combined forms of histologic tumor types)
- Hybrid carcinoma
- Others
- Others
- Keratocystoma
- Sialolipoma
Salivary gland tumours usually present as a lump or swelling in the affected gland which may or may not have been present for a long time. The lump may be accompanied by symptoms of duct blockage (e.g. xerostomia). Usually, in their early stages it is not possible to distinguish a benign tumour from a malignant one. One of the key differentiating symptoms of a malignant growth is nerve involvement. For example signs of facial nerve damage (e.g facial palsy) are associated with malignant parotid tumours. Facial pain, and paraesthesia are also very often associated with a malignant tumours. Other red flag symptoms which may suggest malignancy and warrant further investigation are fixation of the lump to the overlying skin, ulceration and induration of the mucosa.
Carcinoma ex pleomorphic adenoma, abbreviated ca ex PA, is a type of cancer typically found in the parotid gland. It arises from the benign tumour pleomorphic adenoma.
Its prognosis depends on the stage. Early tumour have essentially a benign behaviour.
Pain is the most common symptom, followed by either sensorineural or conductive hearing loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow growing.
This tumor only affects the outer 1/3 to 1/2 of the external auditory canal as a primary site. If this area is not involved, the diagnosis should be questioned. The most common tumor type is ceruminous adenoid cystic carcinoma and ceruminous adenocarcinoma, NOS.
Bartholin gland can be differentiated by histology to determine whether the malignancy is due to squamous cell carcinoma, adenoid cystic carcinoma, or adenocarcinomas.
Adenoid cystic carcinoma (sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC or AdCC.) is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses.
It is the third most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma and polymorphous low grade adenocarcinoma). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years, reflecting deaths from late-occurring metastatic disease.
Bartholin gland carcinoma is an uncommon type of malignancy in the Bartholin gland that accounts for 1% of all vulvar malignant neoplasms. It is most common in women in their mid-60s. The tumor can become large before a woman is aware of symptoms. One of the first symptoms can be dyspareunia. In other instances a woman may find a mass or ulcer in the vulva area. Many clincians assume that an enlarged Bartholin gland is malignant in postmenopausal woman until proven otherwise. The growth of the tumor can spread to nearby areas such as the ischiorectal fossa and inguinal lymph nodes. Approximately 50% of bartholin gland carcinomas originate from squamous cell carcinomas. Another uncommon characteristic of Bartholin gland malignancies is that the growth of a lesion originates from the three types of epithelial tissue present in the gland: mucinous, transitional, and squamous.
Parathyroid carcinoma is a rare malignant neoplasm resulting in parathyroid adenoma to carcinoma progression. It forms in tissues of one or more of the parathyroid glands (four pea-sized glands in the neck that make parathyroid hormone, which helps the body store and use calcium).
It is rare, and much less common than parathyroid adenoma.
It can be difficult to excise.
Thymic carcinoma is a rare type of thymus gland cancer. It usually spreads, has a high risk of recurrence, and has a poor survival rate. Thymic carcinoma is divided into subtypes, depending on the types of cells in which the cancer began. Also called type C thymoma.
Most patients experience moderate to severe hypercalcemia and high parathyroid hormone levels. A large mass in the neck is often seen, and renal and bone abnormalities are common.
Prostatic stromal tumour of uncertain malignant potential, abbreviated PSTUMP, is a rare tumour of the prostate gland stroma that may behave benign or like cancer, i.e. "malignant".
It can be abbreviated STUMP; an abbreviation used for a uterine lesion of uncertain malignant potential.
It is also known as prostatic stromal proliferation of uncertain malignant potential (abbreviated PSPUMP).
Basophilic, bland cells similar to acinar cells. Growth pattern: solid - acinar cells, microcytic - small systic spaces mucinous or eosinophilic, papillary-cystic - large cystic lined by epithelium, follicular - similar to thyroid tissue.
These tumors which resemble serous acinar cells vary in their behavior from locally aggressive to blatantly malignant.
It can also appear in the breast. The pancreatic form of acinic cell carcinoma is a rare subtype of exocrine pancreatic cancer. Exocrine pancreatic cancers are the most common form of pancreatic cancer when compared to endocrine pancreatic cancer.
Acinic cell carcinomas arise most frequently in the parotid gland. Other sites of primary tumors have included the submandibular gland and other major and minor salivary glands. There have been rare cases of primary tumors involving the parapharyngeal space and the sublingual gland.
Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. 90% of the time found in the parotid gland, 10% intraorally on buccal mucosa or palate. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Often appears pseudoencapsulated.
Epithelial-myoepithelial carcinoma of the lung (EMECL) is a very rare histologic form of malignant epithelial neoplasm ("carcinoma") arising from lung tissue.
Mammary analogue secretory carcinoma (MASC) (also termed MASC; the "SG" subscript indicates salivary gland)) is a salivary gland neoplasm that shares a genetic mutation with certain types of breast cancer. MASC was first described by Skálová et al. in 2010. The authors of this report found a chromosome translocation in certain salivary gland tumors that was identical to the (12;15)(p13;q25) fusion gene mutation found previously in secretory carcinoma, a subtype of invasive ductal carcinoma of the breast.
Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or both. Adenocarcinomas are part of the larger grouping of carcinomas, but are also sometimes called by more precise terms omitting the word, where these exist. Thus invasive ductal carcinoma, the most common form of breast cancer, is adenocarcinoma but does not use the term in its name—however, esophageal adenocarcinoma does to distinguish it from the other common type of esophageal cancer, esophageal squamous cell carcinoma. Several of the most common forms of cancer are adenocarcinomas, and the various sorts of adenocarcinoma vary greatly in all their aspects, so that few useful generalizations can be made about them.
In the most specific usage (narrowest sense), the glandular origin or traits are exocrine; endocrine gland tumors, such as a VIPoma, an insulinoma, or a pheochromocytoma, are typically not referred to as adenocarcinomas but rather are often called neuroendocrine tumors. Epithelial tissue sometimes includes, but is not limited to, the surface layer of skin, glands, and a variety of other tissue that lines the cavities and organs of the body. Epithelial tissue can be derived embryologically from any of the germ layers (ectoderm, endoderm, or mesoderm). To be classified as adenocarcinoma, the cells do not necessarily need to be part of a gland, as long as they have secretory properties. Adenocarcinoma is the malignant counterpart to adenoma, which is the benign form of such tumors. Sometimes adenomas transform into adenocarcinomas, but most do not.
Well differentiated adenocarcinomas tend to resemble the glandular tissue that they are derived from, while poorly differentiated adenocarcinomas may not. By staining the cells from a biopsy, a pathologist can determine whether the tumor is an adenocarcinoma or some other type of cancer. Adenocarcinomas can arise in many tissues of the body owing to the ubiquitous nature of glands within the body, and, more fundamentally, to the potency of epithelial cells. While each gland may not be secreting the same substance, as long as there is an exocrine function to the cell, it is considered glandular and its malignant form is therefore named adenocarcinoma.
An endocrine gland neoplasm is a neoplasm affecting one or more glands of the endocrine system.
Examples include:
- Adrenal tumor
- Pituitary adenoma
The most common form is thyroid cancer.
Condition such as pancreatic cancer or ovarian cancer can be considered endocrine tumors, or classified under other systems.
Pinealoma is often grouped with brain tumors because of its location.
Papillary thyroid cancer or papillary thyroid carcinoma is the most common type of thyroid cancer, representing 75 percent to 85 percent of all thyroid cancer cases. It occurs more frequently in women and presents in the 20–55 year age group. It is also the predominant cancer type in children with thyroid cancer, and in patients with thyroid cancer who have had previous radiation to the head and neck. It is often well-differentiated, slow-growing, and localized, although it can metastasize.
Sebaceous carcinoma is an uncommon and aggressive malignant cutaneous tumor. Most are typically about 10 mm in size at presentation. This neoplasm is thought to arise from sebaceous glands in the skin and, therefore, may originate anywhere in the body where these glands are found. Because the periocular region is rich in this type of gland, this region is a common site of origin. The cause of these lesions are, in the vast majority of cases, unknown. Occasional cases may be associated with Muir-Torre syndrome.
This type of cancer usually has a poor prognosis because of a high rate of metastasis.
A Hürthle cell () or Askanazy cell () is a cell in the thyroid that is often associated with Hashimoto's thyroiditis as well as benign and malignant tumors (Hürthle cell adenoma and Hürthle cell carcinoma, a subtype of follicular thyroid cancer). This version is a relatively rare form of differentiated thyroid cancer, accounting for only 3-10% of all differentiated thyroid cancers. Oncocytes in the thyroid are often called Hürthle cells. Although the terms oncocyte, oxyphilic cell, and Hürthle cell are used interchangeably, Hürthle cell is used only to indicate cells of thyroid follicular origin.
Salivary gland–like carcinomas of the lung generally refers a class of rare cancers that arise from the uncontrolled cell division (mitosis) of mutated cancer stem cells in lung tissue. They take their name partly from the appearance of their abnormal cells, whose structure and features closely resemble those of cancers that form in the major salivary glands (parotid glands, submandibular glands and sublingual glands) of the head and neck. Carcinoma is a term for malignant neoplasms derived from cells of epithelial lineage, and/or that exhibit cytological or tissue architectural features characteristically found in epithelial cells.
This class of primary lung cancers contains several histological variants, including mucoepidermoid carcinoma of the lung, adenoid cystic carcinoma of the lung, epithelial-myoepithelial carcinoma of the lung, and other (even more rare) variants. .
Lung cancer is an extremely heterogeneous family of malignant neoplasms, with well over 50 different histological variants recognized under the 4th revision of the World Health Organization (WHO) typing system ("WHO-2004"), currently the most widely used lung cancer classification scheme. Because these variants have differing genetic, biological, and clinical properties, including response to treatment, correct classification of lung cancer cases are necessary to assure that lung cancer patients receive optimum management.
The WHO-2004 scheme groups lung carcinomas into 8 major types:
- Squamous cell carcinoma
- Small cell carcinoma
- Adenocarcinoma
- Large cell carcinoma
- Adenosquamous carcinoma
- Sarcomatoid carcinoma
- Carcinoid tumor
- Salivary gland-like carcinoma
EMECL is considered a subtype of salivary gland-like carcinoma, tumors so named because their histological appearance and characteristics closely resemble malignant neoplasms arising in the major and minor salivary glands.
Acinic cell carcinoma of the lung is a very rare malignant neoplasm originating from bronchial glands. It is classified as a salivary gland-like carcinoma under the most widely used lung cancer classification system.