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Adenoid Cystic Carcinoma Overview

Adenoid cystic carcinoma of the upper airway is a rare tumor, which is locally invasive and frequently amenable to resection. Although late local recurrence after resection is a feature of this tumor (up to 29 years), excellent long-term palliation is commonly achieved after both complete and incomplete resection. There was a small difference in survival between patients having complete and incomplete resection. Long periods of control can be obtained with radiotherapy alone. The best results, in this series of patients, were obtained by resection. Adjuvant radiotherapy is assumed to favorably influence survival.

Adenoid cystic carcinomas are not associated with cigarette smoking. These tumors have a propensity to spread along both submucosal and perineural planes. Regional lymph node metastases are reported in 10% of patients and remote metastases to lung, bone, and brain have been observed. Despite these malignant features, adenoid cystic carcinoma often follows a prolonged course. Slow and insidious progression, often over several years, is characteristic of even untreated cases.

This is the most common malignant tumor of the submandibular and minor salivary glands and comprises 4% of all salivary gland tumors. In the older literature, it is sometimes referred to as cylindroma. These tumors occur with a median age of 43 years. They commonly present as a slowly growing tumor with severe pain and occasionally with facial nerve paralysis as the tumor infiltrates into this nerve. These tumors have a history of relentless recurrence and becoming progressively more aggressive.

Adenoid Cystic Carcinoma Diagnosis

The following series of CT scan photos show a cross-section of my torso with the adenoid cystic carcinoma visible in the trachea. The first image shows the trachea clear from any obstructions. As the images progress at about 1.5 centimeters for each photo, the tumor (neoplasm) becomes visible, and almost completely blocks the airway in image 3. The trachea is labeled in the first photo with an . Click on any photo to enlarge.

1. Trachea is clear of obstruction	2. neoplasm becomes visible
3. tumor obstructing trachea	4. end of tumor

Surgical Procedure for Removing Adenoid Cystic Carcinoma Tumors

Trachea Resection Surgery Results and Photos

1. Internal scar from trachea resection: trachea resection is marked by the ringed white scar as noted in the photo; trachea branching to lungs visible at center. This photo was taken during my brachytherapy at The Cleveland Clinic, approximately 3 months after adenoid cystic carcinoma tumor was removed.
2. Following the resection of the adenoid cystic carcinoma tumor (compare this image with the images of the surgery above), my chin was stitched to my chest for two weeks to prevent extension of the trachea, which could have pulled the trachea apart. Generally, I did not have any problems with the stitches. There was some discomfort from being stationary for a long period, but in general it was not as bad as I expected it would be. This aspect of the surgery seemed more bizarre than any other part of the surgery.
3. X-ray of the adenoid cystic carcinoma resection region with wires visible from sternotomy. The sternum was cut because the tumor was lower in the trachea than originally thought. In most ACC cases, the sternum in not cut. Most of the discomfort I experienced post-surgery was caused by pain associated with the sternotomy.
4. External scar from trachea resection: transverse incision is nearly invisible, while the perpendicular incision is clearly visible. This photo of the trachea resection scar was taken 2 months after my surgery. Compare this photo with the adenoid cystic carcinoma surgical procedure shown above. Some blistering and redness is still visible from the external radiation treatments

Radiotherapy for Adenoid Cystic Carcinoma

I underwent five weeks of radiation therapy consisting of 5 radiation treatments per week for a total of 25 treatments. The treatments were localized on my upper thorax and lower neck. Although the field of radiation treatments varied over the course of the treatments, it was generally focused in the same region as highlighted in the cross-section view of my neck and torso:

	1. The radiation chart shows the projected plan for radiation exposure. The colored lines show varying degrees of radiation exposure. Note the spinal cord in blue. This image shows a cross-section of my upper thoracic region where the trachea resection occurred.
	2. Side view of me receiving radiation therapy; the radiation field marks are indicated by blue crosses taped to the mask. The mask ensures the patient does not move during radiotherapy.
	3. Another view of the radiation therapy room at the University of Virginia oncology center.
	4. This view of the radiation mask shows the projected radiation beam targets on the tape marked with blue. The bottom of the resection incision is marked by a blue cross on my sternum.
Back Front	5. The two photos to the left show the extent of blistering caused by radiation therapy on my back. While we expected blistering on the front where the beam was directed, we didn't expect blistering on the back. There was some surface pain associated with the blistering caused by the radiation.

Disease Course

Due to its slow growth, ACC has a relatively indolent but relentless course. Unlike most carcinomas, most patients with ACC survive for 5 years, only to have tumors recur and progress. In a recent study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years. Another unusual feature of ACC is that, unlike most carcinomas, it seldom metastasizes to regional lymph nodes. Distant metastasis is the most common presentation of treatment failure. The lung is by far the most common site of metastasis, with the liver being the second most common site. Bone metastases usually indicate a fulminant clinical course. Poor prognostic signs at the time of initial surgery are a solid growth pattern, perineural invasion of major nerves and/or positive margins after histopathologic examination.

Pathologic examination revealed local invasion beyond the wall of the trachea in all patients. In a majority, microscopic extension was found in submucosal and perineural lymphatics, well beyond the grossly visible or palpable limits of the tumor. Lymphatic metastases were relatively uncommon, occurring in only five of 32 (19%) patients undergoing resection. Metachronous hematogenous metastases occurred in 17 of 38 patients (44%). Thirteen of these 38 patients (33%) had pulmonary metastases. Sixteen of 32 resections were complete and potentially curative. There were two deaths within 30 days of operation. The mean survival in the 14 patients undergoing complete resection was 9.8 years (12 months to 29 years). Sixteen of 32 resections were incomplete (residual tumor at the airway margin on final pathologic examination), with one operative death occurring in this group. The mean survival in the 15 surviving patients was 7.5 years (4 months to 21 years). Six patients were treated with primary radiation only and had a mean survival of 6.2 years (2 months to 14.3 years). In the patients with pulmonary metastases, mean survival was 37 months (4 months to 7 years) from the time of diagnosis of the pulmonary metastasis until their death.

Additional Information

More details are listed at the University of Virginia Health System website.

For an actual image of a tumor attached to a trachea, click here [Cornell University Website].