Paediatric Ophthalmology Strabismus Optics and Refraction Intraocular and Orbital Tumors Retina and Vitreous Neuro-Ophthalmology Cornea and External Disease Uveitis Glaucoma Orbit and Oculoplastics

This rare case uploaded by Mr Armin Moradi . Case generously contributed by Dr. Richard Judelson, Dr. Jefferey Judelson, and Dr. Ron Angeles. This case was presented at COS Pathology Meeting Ottawa June 17, 2016.

Clinical History

79 year old female presented with a three week history of an inflamed, painful left eye with reduced vision. Initially treated by referring ophthalmologist with Predforte for iritis. Previous history of bilateral pseudophakia. No prior iritis or glaucoma. Medical Hx: NIDDM, Hypertension, Hypercholesterolemia and arthritis. No history of malignancy. Previous smoker since 15 years of age (one pack per day). Stopped 4 years previously.


Q What is the differential diagnosis? (one per line)

The differential diagnosis is as follows:

  • Metastatic tumour from primary lung small cell carcinoma
  • Iris stromal cyst
  • Iris granuloma (sarcoidosis)
  • Tumor metastasis to iris
  • Primary amelanotic melanoma

Q What further investigations would you order? (one per line)

The following investigation results are available:

Anterior chamber aspiration biopsy

27g needle through a limbal paracentesis. Aspirated fluid from AC, pseudohypopyon and edge of iris. Fluid sent to cytology. * Hypercellular specimen with many single and groups of atypical cells. * Cells have a high nuclear to cytoplasmic ratio, coarse to fine chromatin, irregular nuclear borders and mitotic figures. * A few cells contain cytoplasmic pigmentation. * Immunocytochemical stains: positive for keratin CAM 5.2 and negative for S100. Impression: consistent with a poorly differentiated metastatic carcinoma.

CT chest

Lymphadenopathy in the mediastinum and right bronchopulmonary area. Multiple pulmonary nodules in right upper and lower lobe.

MRI head

Enhancing 6mm dural nodule along right greater wing of sphenoid.

CT abdomen and pelvis

Normal.

Lung needle biopsy - right upper lobe

* Few isolated clusters and single atypical cells with enlarged pleomorphic nuclei and eosinophilic cytoplasm. * These nests and cells are located in a fibrotic stroma. * The tumour is positive for P63 and CK5/6 which indicates a squamous origin. * Negative for TTF-1 and Napsin A, ruling out adenocarcinoma origin. Impression: Poorly differentiated squamous cell carcinoma


Q What is the most likely or definitive diagnosis?

The accepted diagnosis is Metastatic tumour from primary lung small cell carcinoma.


Q What is the management plan for this condition? (one per line)

The management of this condition is as follows:

  • External beam palliative radiotherapy
  • Chemotherapy

Primary iris tumors are not usually associated with an acutely inflamed eye with anterior chamber activity, pseudohypopyon or SOAG. Clinical presentation of metastatic iris lesions: 1. Pain with an inflamed eye compared to primary iris tumors. 2. Blurred vision. 3. Secondary glaucoma 37%. Sheilds et al reviewed 104 cases of iris metastasis - Of these, 28 originated for lung primaries - 26 lung carcinoma, 2 lung carcinoid - The type of lung carcinoma’s were not differentiated There have been various other cases reported of lung primaries metastasising to the iris. However, all these have been either adenocarcinoma or small cell carcinoma. Uveal metastasis most common malignant intraocular tumor. - Median age of presentation 60 years. - Primary sites: Breast 33%, Lung 27%, Skin (melanoma) 12%, Kidney 7%, Esophagus 3% and others 19%. - Breast most common in women and lung in men. - Vast majority unilateral involvement 98%. - Metastasis unifocal 78% and multifocal 22%. - Tumor location: Iris root 69%, midzone 21% and pupillary margin 10%. - Uveal metastasis – iris involved < 10% - Coexistent conjunctival or ciliary body/choroidal metastases 38% patients. Investigations – Metastatic Iris lesions: - FNAB of iris lesion or tumor excision - Systemic survey with imaging – chest/abdomen/brain - Consultation with medical oncologist Treatment - Difficult to manage depending on stage of disease and health of patient - External beam palliative radiotherapy - Chemotherapy - Prognosis generally very poor. Others sites for metastasis include: carcinoid tumor, laryngeal and prostate.

1. Iris metastatis from systemic cancer in 104 patients: the 2014 Jerry A. Shields lecture. Cornea 2015 Jan 34(1) 42-8 Shields JA . 2. Metastatic tumors to the iris in 40 patients. Am J Ophthalmol 1995 April 119(4) 422-30. Shields JA. 3. Iris metastasis from small cell lung cancer. Journal of thoracic oncology. Oct 2014 Vol 9 Issue 10 p1584-1585.