Posters

Ocular and orbital onset in an extended malignant non-Hodgkin lymphoma

Poster Details

First Author: R. Pirvulescu ROMANIA

Co Author(s):    D. Buciumeanu   V. Dide   G. Simion   A. Vladareanu   A. Popa Cherecheanu        

Abstract Details

Purpose:

To show a particular case of a clinical unapparent extended malignant lymphoma, with bilateral orbital and adnexal onset in a 56 years old female patient who presented in the Ophthalmology Department of the Emergency University Hospital Bucharest, with right eye (RE) depression and ptosis, and painless proptosis; the onset was insidious, starting 2 months before hospital presentation. Patient is known to have hypothyroidia, treated with Euthyrox 0.25 mg/daily. The patient mentions no other simptomatology. The case highlights the importance of a complete assessment in patients with ocular and orbital tumours, which could be due to secondary determination of systemic diseases.

Setting:

Ocular lymphoma can be either intraocular or orbital and adnexal; it is considered to be the most common malignant orbital tumor (55% of cases in adults and 10% of cases in older patients). Orbital lymphoma usually presents around 6th decade of life and could be associated with systemic lymphoma.

Methods:

Patient exam showed painless proptosis, downward deviation of the RE and ptosis, gradually installed and no other ocular symptoms. A detailed exam of the RE showed a purple hard tumoral mass, adherent, weak delimitated in the supero-temporal angle of the orbit, with no ocular/orbital inflammation. RE was deviated infero-nasal and its motility was limited on upward gaze. On the left eye (LE) we noticed a well confined salmon-pink tumoral mass of the upper bulbar conjunctiva. Patient underwent functional exams (normal in both eyes), ultrasound, blood work, hematological exam, and CT scan of head, chest and abdomen.

Results:

Ocular ultrasound of the RE and orbit showed a hypoechoic, well limited upper orbital mass that doesn’t modify the bone structure. CT revealed an expansive orbital process on the RE and many nodular hyperechoic images in the epigastrum and around the splenic hilum, hepatosplenomegaly, nodular masses in the lombar region fat, bilateral. Blood work revealed monocitosis, high fibrinogen, LDH and TSH, modified hepatic profile and coagulation tests. Hematological exam recomanded biopsy. Biopsy on both tumors showed non-Hodgkin lymphoma (NLH) with small B cells (MALT), with C20 marker positive and CD5 positive, Cyclin 2 negative and BCL 6 negative.

Conclusions:

NLH is the most common type of ocular lymphoma; it presents later in life and causes symptoms due to gradually increasing mass effect. Prognosis for ocular lymphoma depends on the tumor’s histological type and stage, and on the treatment employed. Modern treatment increases survival rate at 5 years to approximately 60%. Particular in this case is the association between orbital and adnexal lymphoma with extended systemic NLH (association encountered in 30% of the patients). The patient had a good general condition, and the presence of an old time neglected hypotiroidia, which could have triggered the disease.

Financial Disclosure:

NONE