Adenoid Cystic CarcinomaMaxilla

Adenoid Cystic Carcinoma Most common malignant tumor of the

submandibular and minor salivary glands Parotid: 3% Submandibular: 15% Minor salivary gland: 30%

Comprises 6% of all salivary gland tumors

it is sometimes referred to as cylindroma

Commonly present as a slowly growing tumor

Adenoid Cystic Carcinoma5th most common malignant epithelial tumor of

the salivary gland

7.5% of all epithelial malignancies

Presentation in childhood is unusual and the mean age at the time of clinical diagnosis is in midforties.

Lymphogenous spread

Adenoid Cystic CarcinomaThese tumors have a relentless recurrence and

becoming progressively more aggressive

Severe pain and spontaneous paralysis of the facial nerve occur in nearly 1/3 of the patients.

Hallmark of the tumor is peripheral nerve invasion

Both sexes are affected with about equal frequency

CausesOccupational exposure

NickelWood dustIsopropyl oilVolatile hydrocarbonsOrganic fibers found on wood, shoes

SymptomsDental painLoose teeth Oropalatal fistula/palatal massOcular symptoms

EpiphoraProptosisDiplopiaVisual loss

SymptomsFacial manifestation

Cheek swelling Pain Nasal complaints: epistaxis, anosmia, and

discharge

Staging TNM (AJCC) 2002 TX: Indicates the primary tumor cannot be evaluated. T0: No evidence of a tumor is found. T1: Describes a small noninvasive tumor that is 2 centimeters

(cm) at its greatest dimension. T2: Describes a larger noninvasive tumor, between 2 cm to 4 cm. T3: Describes a tumor that is larger than 4 cm, but not larger

than 6 cm, that has spread beyond the salivary gland. However, the tumor does not affect the seventh nerve, which is the facial nerve that controls such expressions as smiles or frowns.

T4a: The tumor invades the skin, jawbone, ear canal, and/or facial nerve.

T4b: The tumor invades the skull base and/or the nearby bones and/or encases the arteries.

Staging TNM (AJCC) 2002NX: Indicates the regional lymph nodes cannot be evaluated.N0: There is no evidence of cancer in the regional nodes.N1: Indicates that cancer has spread to a single node on the same

side as the primary tumor and the cancer found in the node is 3 cm or smaller.

N2: Describes any of these conditions:N2a: Cancer has spread to a single lymph node on the same side

as the primary tumor, and is larger than 3 cm, but not larger than 6 cm.

N2b: Cancer has spread to more than one lymph node on the same side as the primary tumor, and no tumor measures larger than 6 cm.

N2c: Cancer has spread to more than one lymph node on either side of the body, and no tumor measures larger than 6 cm.

N3: Cancer found in lymph nodes is larger than 6 cm.

Staging TNM (AJCC) 2002MX: Indicates distant metastasis cannot be

evaluated.M0: Indicates the cancer has not spread to

other parts of the body.M1: Describes cancer that has spread to

other parts of the body.

Adenoid Cystic CarcinomaPrognosis: (after first histological verification)

5 year survival rate 75%

10 year survival rate 30%

20 year survival rate 13%Local recurrence: 42%Distant metastasis: Lungs

Prognostic significance of metastases is difficult to ascertain since this is usually a late finding.

Horizontal lines of SebileauOldest classification

Horizontal line TOP: floors of the orbits BOTTOM: floors of the antra

Suprastructure: ethmoid, sphenoid, frontal sinuses

Mesostructure: maxillary sinus, respiratory portion of the nose

Infrastructure: alveolar process

1

23

Line of LedermanUtilized the horizontal line of Sebileu and added a vertical line on each side of the nose

Vertical lines separates the ethmoid sinuses and nasal fossa from the maxillary sinus

1

23

Triple S Lines of BacleseFormed by:

Lesser wing of the sphenoidPosterolateral wall of the maxillary antrumPosterior wall orbit

For assessment of superoposterior extent of maxillary Carcinoma

Triple S-lines of Baclese

Ohngren’s LineExtends from the medial canthus to the angle of the mandible.

Divides the maxillary sinus into superoposterior (suprastructure) and Inferoanterior (infrastructure)

Plane of Malignancy

Adenoid Cystic Carcinoma

Fairly high recurrence rate may well be due to:

Unsatisfactory primary surgical treatment and failure to recognize the neural extension.

Insidious infiltration of adenoid cystic carcinomas

Relation of site of primary to Survival rates of ACC

Follow-up time

(Years)

PERCENTAGE OF DETERMINATE SURVIVAL

Parotid Gland Submandibular Gland

Palate

5 73 50 80

10 39 25 44

15 21 0 38

20 13 0 36Determination survival are from the data presented by Eneroth et al.

Adenoid Cystic CarcinomaTreatment:

Complete local excisionTendency for perineural invasion: facial nerve

sacrificePostoperative XRT

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