Adenoid cystic carcinoma is a rare type of
cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
that can exist in many different body sites. This tumor most often occurs in the
salivary gland
The salivary glands in mammals are exocrine glands that produce saliva through a system of ducts. Humans have three paired major salivary glands ( parotid, submandibular, and sublingual), as well as hundreds of minor salivary glands. Salivary ...
s, but it can also be found in many anatomic sites, including the
breast
The breast is one of two prominences located on the upper ventral region of a primate's torso. Both females and males develop breasts from the same embryological tissues.
In females, it serves as the mammary gland, which produces and sec ...
,
lacrimal gland
The lacrimal glands are paired exocrine glands, one for each eye, found in most terrestrial vertebrates and some marine mammals, that secrete the aqueous layer of the tear film. In humans, they are situated in the upper lateral region of each or ...
,
lung,
brain
A brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It is located in the head, usually close to the sensory organs for senses such as vision. It is the most complex organ in a ve ...
,
bartholin gland
The Bartholin's glands (named after Caspar Bartholin the Younger; also called Bartholin glands or greater vestibular glands) are two pea sized compound alveolar glandsManual of Obstetrics. (3rd ed.). Elsevier. pp. 1-16. . located slightly poster ...
,
trachea
The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air- breathing animals with lungs. The trachea extends from the ...
, and the
paranasal sinuses
Paranasal sinuses are a group of four paired air-filled spaces that surround the nasal cavity. The maxillary sinuses are located under the eyes; the frontal sinuses are above the eyes; the ethmoidal sinuses are between the eyes and the sphenoi ...
.
It is the third-most common malignant salivary gland tumor overall (after
mucoepidermoid carcinoma
Mucoepidermoid carcinoma (MEC) is the most common type of minor salivary gland malignancy in adults. Mucoepidermoid carcinoma can also be found in other organs, such as bronchi, lacrimal sac, and thyroid gland.
Mucicarmine staining is one stain us ...
and
polymorphous 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.
Cause
Activation of the oncogenic transcription factor gene ''
MYB
MyB (Hangul: 마이비) was a South Korean girl group formed by Maroo Entertainment. The group debuted on August 25, 2015 with ''My Oh My'' and made a comeback on November 13, 2015 with ''Ddoddo'', before the group officially disbanded on December ...
'' is the key genomic event of ACC and seen in the vast majority of cases.
Most commonly, ''MYB'' is activated through gene fusion with the transcription factor encoding ''
NFIB'' gene as a result of a t(6;9) translocation.
[ Alternatively, ''MYB'' is activated by copy number gain or by juxtaposition of enhancer elements in the vicinity of the ''MYB'' gene.]
In a subset of ACCs, the closely related ''MYBL1'' gene is fused to ''NFIB'' or to other fusion partners.
MYB drives proliferation of ACC cells and regulates genes involved in cell cycle control, DNA replication and repair, and RNA processing. Thus, the ''MYB'' oncogene is a potential diagnostic and therapeutic target in ACC.
ACC has a relatively quiet genome with few recurrent copy number alterations or point mutations,[ consistent with the view that MYB and MYBL1 are the main oncogenic drivers of the disease.
]
Treatment
Primary treatment for this cancer, regardless of body site, is surgical removal with clean margins. This surgery can prove challenging in the head and neck region due to this tumor's tendency to show a perineural discontinuous growth, meaning that it follows nerves and different "nests" of the tumor can exist without a connection to the original tumor. Therefore, MRI-images should be analysed following nerve tracts up to the brainstem. Adjuvant In pharmacology, an adjuvant is a drug or other substance, or a combination of substances, that is used to increase the efficacy or potency of certain drugs. Specifically, the term can refer to:
* Adjuvant therapy in cancer management
* Analgesi ...
or palliative radiotherapy is commonly given following surgery. For advanced major and minor salivary gland tumors that are inoperable, recurrent, or exhibit gross residual disease after surgery, fast neutron therapy
Fast neutron therapy utilizes high energy neutrons typically between 50 and 70 MeV to treat cancer. Most fast neutron therapy beams are produced by reactors, cyclotrons (d+Be) and linear accelerators. Neutron therapy is currently available in ...
is widely regarded as the most effective form of treatment.
Chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemothe ...
is used for metastatic
Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, ...
disease. Chemotherapy is considered on a case-by-case basis, as data on the positive effects of chemotherapy are limited. Clinical studies are ongoing, however.
Images
File:Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor.jpg, Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor: The tumor originates in the right parotid gland and spreads along the trigeminal nerve via the auricuotemporal branch extending intracranially through the foramen ovale at the skull base towards Meckel's cave.
File:MRI showing perineural spread of adenoid cystic carcioma along the facial nerve.jpg, Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor along the facial nerve extending to the stylomastoid foramen
Image:Adenoid cystic carcinoma (2).jpg, Histopathological image of adenoid cystic carcinoma of the salivary gland infiltrating a nerve (center), H&E stain
Image:Adenoid cystic carcinoma (3) S-100.jpg, Histopathological image of adenoid cystic carcinoma of the salivary gland, immunostain for S-100 protein
Image:Adenoid cystic carcinoma cytology.jpg, Micrograph of adenoid cystic carcinoma, fine needle aspiration
Fine-needle aspiration (FNA) is a diagnostic procedure used to investigate lumps or masses. In this technique, a thin (23–25 gauge (0.52 to 0.64 mm outer diameter)), hollow needle is inserted into the mass for sampling of cells that, aft ...
specimen, Pap stain
Papanicolaou stain (also Papanicolaou's stain and Pap stain) is a multichromatic (multicolored) cytological staining technique developed by George Papanicolaou in 1942. The Papanicolaou stain is one of the most widely used stains in cytology, wh ...
File:Adenoid cystic carcinoma with comedonecrosis.jpg, Adenoid cystic carcinoma with comedonecrosis
File:Relative incidence of submandibular tumors.png, Relative incidence of submandibular tumors, with adenoid cystic carcinoma being the second most common.[
File:Relative incidence of parotid tumors.png, Relative incidence of parotid tumors, with adenoid cystic carcinoma being relatively rare.][ Updated: Jan 13, 2021]
Diagrams by Mikael Häggström, MD
See also
* Primary cutaneous adenoid cystic carcinoma
Primary or primaries may refer to:
Arts, entertainment, and media Music Groups and labels
* Primary (band), from Australia
* Primary (musician), hip hop musician and record producer from South Korea
* Primary Music, Israeli record label
Works
...
* Collagenous spherulosis
Collagenous spherulosis, or simple spherulosis, is a benign finding in breast pathology. It is almost always an incidental finding, though it is occasionally associated with calcifications, which may lead to a biopsy.
Significance
It is important ...
References
Further reading
* Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.
External links
Search for clinical trials at ClinicalTrials.gov
Search for research literature at PubMed.gov
Search for consumer health information at Medlineplus.gov
{{Tumors of lip, oral cavity and pharynx
Salivary gland neoplasia
Rare cancers