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.
~Maziak
DE, Todd TR, Keshavjee SH, Winton TL, Van Nostrand P, Pearson FG. Adenoid
cystic carcinoma of the airway: thirty-two-year experience. J Thorac Cardiovasc
Surg. 1996 Dec;112(6):1522-31; discussion 1531-2.
NCBI
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.
~Christopher G. Compeau, Shaf
Keshavjee. Management of Tracheal Neoplasms. The Oncologist, Vol. 1, No. 6,
347–353, December 1996.
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 Krysti's 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), Krysti's chin
was stitched to the chest for two weeks to prevent extension of the
trachea, which could have pulled the trachea apart. Generally, Krysti
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 we 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 Krysti 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 Krysti's
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
Krysti underwent five weeks of radiation therapy
consisting of 5 radiation treatments per week for a total of 25 treatments. The
treatments were localized on her 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
Krysti's 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 Krysti's upper thoracic region where the trachea
resection occurred.
2. Side view of Krysti 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 Krysti's
sternum.
5. The two photos to the left show the extent of blistering caused by
radiation therapy on Krysti. 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.
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.
~Excerpt from UVA website [
http://www.healthsystem.virginia.edu/internet/cancer/teampages/moskaluk/ACChome.cfm#cc]
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.
~Maziak DE, Todd TR, Keshavjee
SH, Winton TL, Van Nostrand P, Pearson FG. Adenoid cystic carcinoma of the
airway: thirty-two-year experience. J Thorac Cardiovasc Surg. 1996 Dec;112(6):1522-31;
discussion 1531-2.
NCBI
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becoming progressively more aggressive.
.
Click on any photo to enlarge.
