CASE PRESENTATION

1
CASE PRESENTATION

• A 77 year-old white male was sent to our
  department for complaints of right eye pain and
  foreign body sensation. On exam, his BCVA was
  20/30 OD, there were 2cells in the anterior
  chamber OD, and there was a small iris tumor in
  the angle at 700 (see images on next slide).
  There were no iris lesions in the left eye and
  examination of the fundus was negative for any
  choroidal tumors OU. The patient denied any
  other systemic symptoms except for some recent
  weight loss due to a decrease in appetite. His
  past medical history was significant for
  hypertension and diabetes. He had smoked for 10
  years, but quit approximately 25 years prior to
  presentation.

2
(No Transcript)
3

• The differential diagnosis at this point was an
  amelanotic melanoma versus a metastatic lesion.
  An anterior chamber tap was performed to see if
  the tumor could be identified by any possible
  tumor cells in the anterior chamber. However,
  cytology only showed lymphocytes. Thus, the next
  step was to do a fine needle biopsy of the
  lesion. However, after just 6 days, there was
  significant growth of the tumor (see image on
  next slide), leading us to feel that this was
  much more likely a metastatic lesion than a
  melanoma. Thus a systemic work-up was requested.
  
• CT scan of the chest and abdomen revealed diffuse
  thickening of the esophagus from just above the
  carina down to the gastroesophageal junction.
  There were also multiple bilateral pulmonary
  metastases (too many to count), ranging in size
  from 2mm to 2cm. An MRI of the brain and orbits
  was negative. PET scan was refused by insurance.
  Biopsy of the esophageal lesion revealed poorly
  differentiated squamous cell carcinoma. Thus,
  the patient was diagnosed with stage IV
  metastatic esophageal carcinoma.

4
(No Transcript)
5

• The iris tumor continued to grow larger in the
  right eye (4.7mm x 2.4mm), to the point that
  there was endothelial touch with associated focal
  corneal edema (see image 4). There was a
  subsequent worsening of anterior uveitis and
  ocular hypertension (28mm Hg). This was
  relatively controlled with topical medications.
• The patient underwent his first dose of
  chemotherapy (Cisplatin and Taxotere) 26 days
  after initial presentation. Ophthalmic exam ten
  days later already showed some reduction in the
  size of the tumor (See image 5). By thirty-two
  days out from the first chemotherapy treatment
  (eleven days after the second treatment), the
  iris tumor was down to 1.0mm x 1.5mm in size.
  After the third treatment, the iris lesion was
  barely visible.

6

• Image 4

7

• Image 5

8

• Unfortunately, about 6 weeks after his sixth and
  final chemotherapy treatment, follow-up
  examination showed that the iris tumor was
  definitely recurring and growing (see image on
  next slide). A hyphema developed, which slowly
  resolved, and the ocular hypertension
  significantly worsened. His IOP was 28 on
  maximum medical therapy.
• The Oncology department decided that further
  chemotherapy was not warranted. The option of
  palliative radiation therapy focally to the iris
  tumor was addressed, but the patient declined.
  The patients eye was kept relatively comfortable
  with medical management. Eight months after
  presentation, the patient passed away.

9
(No Transcript)
10
DISCUSSION

• Metastatic esophageal carcinoma to the iris is
  extremely rare. There are only three reported
  cases in the literature1-3. However, the case
  weve presented is the only reported case where
  the presenting signs and symptoms of the
  esophageal carcinoma occurred in the eye
  (secondary to metastasis).
• As was the case with our patient, the prognosis
  for metastatic esophageal carcinoma to the eye is
  quite poor. Our patient initially responded very
  well to chemotherapy. However, the disease
  process was too progressed by the time
  chemotherapy was initiated to be able to
  eradicate the disease, as is usually the
  situation in such cases.

11

• Fine-needle biopsy can be very helpful in the
  diagnosis of iris tumors when the type of tumor
  is unknown. This was not necessary in this case,
  as the diagnosis was evident after rapid growth
  of the iris tumor triggered an extensive systemic
  work-up, showing that an occult esophageal
  carcinoma was the focus of a metastatic disease.
• It is not uncommon for iritis, hyphemas, and
  ocular hypertension to occur secondary to iris
  tumors. The mechanism of ocular hypertension is
  secondary to inflammatory and red blood cells
  clogging the trabecular meshwork and tumor growth
  into the angle.

12

• In cases such as this one, if the tumor is not
  able to be controlled with chemotherapy, focal
  radiation therapy can be a good palliative option
  to control the tumor to keep the eye comfortable.
  If the patient does not agree to radiation
  treatment, if the prognosis is very poor, and if
  the patient is experiencing uncontrollable ocular
  pain, enucleation is another palliative option.
• In a series of metastatic iris tumors seen at
  Wills Eye Hospital2, sixty-eight percent of
  patients had a known primary cancer at the time
  of presentation for signs and symptoms of iris
  metastases. The case described above is very
  unique in that, not only was the primary cancer
  not known, but it is the only reported case of
  esophageal cancer where the presenting signs and
  symptoms were ocular in nature.

• REFERENCES
• 1. Segal A, Ducasse A, Mathot E, Jouhaud F.
  Metastase Irienne DUn Cancer De LOesophage.
  Bull Soc Ophtalmol Fr 1978 Jan87(1)137-9.
• 2. Shields JA, Shields CL, Kiratli H, de Potter
  P. Metastatic Tumors to the Iris in 40 Patients.
  Am J Ophthalmol. 1995 Apr119(4)422-30.
• 3. Shields CL, Shields JA, Gross NE, Schwartz GP,
  Lally SE. Survey of 520 Eyes with Uveal
  Metastases. Ophthalmology. 1997
  Aug104(8)1265-76.