Orbital Pseudotumor Case 3 - ECN Tumor Board Presentation

A Recent External Photograph of the 72 year old male - May 2001

Subject: Sclerosing Pseudotumor Management Case
Date: Sunday, May 27th 2001
From: Paul T. Finger, MD, FACS

Dear List Members

We have a difficult case and would like to get your comments on treatment.

Case 1

A 72-year old male presented with a 9-year history of a chronic remitting relapsing orbital inflammation involving his right eye. He notes that it originally occurred shortly after sinus surgery. He has undergone 3 orbital surgeries, two followed by oral steroids and the last by both steroid and methotrexate.

Histopathology from his Most Recent Biopsy

Upon presentation to The New York Eye Cancer Center, he had discontinued his oral medications (steroid and methotrexate) and suffered an exacerbation of his orbital inflammation. A review of his records revealed that Wegener's granulomatosis was suspected. A rheumatologic evaluation was negative.

Laboratory Evaluations Revealed

ANCA - Negative
Urinalysis - trace blood, crystals, protein
Chest x-ray - old lingular scar
Thyroid function tests - normal
Complete blood count with differential - normal
SMA Profile - normal

A Recent Computed Tomogram - Note the optic nerve on stretch (white arrow)

Findings of Steven A McCormick, MD - Ocular Pathologist
Description: "Idiopathic orbital inflammation with sub-acute vasculitis, focal dense fibrosis and rare foci of necrosis with abcess."

Nine months after 80% of the mass was resected (above) followed by 2000 cGy of external beam radiation therapy to the medial orbit. His most recent ophthalmic examination revealed a vision of 20/25 OD (20/20 OS), he has returned to 9 mm of relative proptosis OD, no color defect, no afferent pupillary defect, and asymmetric intraocular pressures 17 OD, 13 OS. Ophthalmoscopy revealed no optic neuropathy and mild macular degeneration.

Question: What will be your approach

  1. More Laboratory Evaluations? 
  2. More Surgery? 
  3. More Radiation Therapy? 
  4. More or Different Chemotherapy? 
  5. All of the above?

Responses From ECN Members

I think I would proceed with more radiotherapy 2000 cGy under cover of steroid therapy at high dosage. After all he only had 2000cGy on the first treatment so he can take an additional tx. I also think it will be necessary to remove somme of the tumor (as it is so big) then proceed with the proposed therapy.

Alain Rousseau, MD Montreal, Canada

I think it will be needed to find supplementary inflammatory focus in nasalis sinusis. Pay attention on sinus sphenoidalis. About treatment: maybe it will be resonable to add non specific non steroid antiinflammetory medicine and antibacterial therapy. Best regards,

Alexander Bouiko, MD

I will favor additional surgery for this case. In my experience sclerosing pseudotumor is extremely resistant to radiotheray, and in some cases may even worsen the orbital disease inducing more fibrosis. Moreover additional surgery will provide a large specimen for an extensive histopathologic evaluation.

Gonzalo Blanco MD PhD
Instituto Universitario Oftalmobiolog Aplicada (IOBA)
Oculoplastics and orbit unit

From reviewing the clinical data from the most recent exam, it seems that at the moment, the patient's vision and visual function is stable. Is there much pain that he is experiencing at the present time? IF not, and if his vision is stable at the stated 20/25 level, I would probably consider another observation along with another form of chemotherapy or anti-metabolite treatment to prevent future recurrence. Given that he has already had 20Gy of radiation (I assume with good response), additional radiation will be associated with more ocular morbidity and should be reserved for when all else fails.

Bita Esmaeli, MD
Assistant Professor and Chief, Ophthalmology Section
M. D. Anderson Cancer Center Houston, TX

DISCLAIMER: Postings on The ECN Mailing List are strictly the opinions of the authors. The ECN and its sponsors assume no responsibility for the accuracy of the information, nor do they assure the safety or effectiveness of any clinical recommendations in these postings.

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