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Aderxid Clstic

Cacinora I Doctor I Hiert,co.t*

Adenoid C stic Carcinoma
This patientplus article is written for healthcare professionals so the language may be more technical than the condition helPful. leaflets. You may find

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Adenoid cystic carcinoma (ACC) is an urrcommon and unusually indolent cancer arising within glands and occuning mainly in the head ard neck but also in the breast, trachea, lacrimal glands, skin and

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ACC arising in the head and neck often has a relenfless progress and poor prognosis but tumours arising in some sites (notably in the breast) may have a better prognrcsis. ACCs are often slowgrowing but locally aggressive and particularly prone to recunerce.

Epidemiology
o Adenoid cystic carcinoma (ACC) accounts for about 1o/o of all head and neck malignancies.[ ] o The age rElnge is unusually wide and it can occur in children. lt is most common between the ages of 40 and 70 years. o
It occurs more often in females in a 3:2 ratio. The p53 tumour suppressor gene may be inactirated -iq advranced disease but otherwise no particular or consistent genetic or enrironmental hctors hatebeen identifed.[ ] Fbwerer, a recent study identifies loss of.1p32-p36 as the most fequent genetic change which was also a marker for .t I poor prognosis in ACC

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Histopathology
It has a distinct histopathological appearance. There are three main growth pattems at
histology:
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Cribiform. Tubular.

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Solid.This fcllows a more aggressire course usually.

It may, howeter, be confused with basaloid squamous carcinoma.

Presentation
Presentation depends'on site, but it can present as:

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A painless slowgnowing mass in the face or mouth. ln one study using endoscopic. and anterigr-skult-base,ldenoid cystic resection of tumours in the carcinoma (ACC) represented 15% of the tumours found'[ I More adwnced tumours may inrade nenes, causing paralysis and pain. ACC is of interest to neurosurgeons and neurologists because of a tendency to infiltrate negqll[ I structures and spreid perineuratly. lntiacranial inrplr,ement can occur !u1 i5 rars.[ . ACC Tumours of the lacrimal gland may afiect rision and also may cause is the most common primary malignant epithelial neoplasm afiecting lacrimal glands'

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patients and is relatirely resistant to treatment.t t , and diffculty breathing.[ a ln the , , it may cause wice changes and presents with j,ft present in men.[ , representing less than a ACC may present as a breast tumour and can lq q ] 1% of breast carcinomas. Generally it has a farourable Prognosis.[ a ACC is unusual in not routinely metastasising to regional lymph nodes. I a ACC may present late or afteitreatment failuie, wit[ dista;t metastases (most commonly lung, followed by lir,er).t

ln the lung, ACC may present with respiratory symptoms. ACC is the second most common pnmary tumour of the trachea aii canies poor prognosis (38.5% Sygar suruiral). Relatire to squamous carcinomas it is found in younger

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