Orbital Adnexal Lymphoma Diagnostic Misalignment

Lymphomas are known as neoplasm’s caused by clone proliferation of B and T lymphocytes. Extranodal lymphoma arises from tissues other than lymph nodes or even from sites which normally doesn’t contain lymph tissues. Orbital adnexal lymphoma arises from eyelid, orbit, lacrimal glands or conjunctivae and it is totally different from intraocular lymphoma. Wide range of differential diagnosis including infectious inflammatory orbital disease, preseptal and orbital cellulitis, orbital idiopathic inflammatory disease-pseudotumors, especially dacryoadenitis and myositis and thyroid associate orbit disease makes the diagnosis of orbital adnexal lymphoma even more difficult. We represent the case of diagnostic delay of very aggressive form orbital nonHodgkin lymphoma occurred because of the unspecific signs and symptoms as well as not indicative imaging investigation and laboratory tests.


Introduction
Lymphomas are known as neoplasm's caused by clone proliferation of B and T lymphocytes.Based on WHO Classification of Tumor of Hematopoietic and Lymphatic Tissues, there are 30 different subtypes [1].There are 4 categories: Hodgkin and non-Hodgkin lymphoma, myeloma multiple and immunoproliferative disease, but 90% of patients have non-Hodgkin lymphoma [2].Primary nodal lymphomas arise from lymph nodes and primary extranodal lymphomas arise from tissues other than lymph nodes or even from sites which normally doesn't contain lymph tissues [3].
Extranodal non-Hodgkin lymphomas appear more often in gastrointestine, skin, and but also in head and neck, nervous system and orbital lymphoma [4] B. Rama et al.DOI: 10.4236/ojoph.2017.74039304 Open Journal of Ophthalmology [5].Although intraocular and orbital adnexal NHL appears to be less than 5% -15% of all NHL and less than 1% of all NHL, it is the most common orbital malignancy representing up to 55% of all orbital tumors [6].
Sign and symptoms can vary depending on the site of the orbit.We represent the case of diagnostic delay of very aggressive form orbital non-Hodgkin lymphoma occurred because of the unspecific signs and symptoms as well as not indicative imaging investigation and laboratory tests.The purpose is to emphasize the importance of the early biopsy for the better and faster therapeutic and prognostic results.

Case Report
We represent the case study of unilateral non Hodgkin lymphoma.A 48 years old male patient was presented in our clinic with proptosis of the left eye and with palebral ptosis.Before coming in the clinic he was treated in the ambulances with antibiotics, but with no improvement (Figure 1).RTG pulmo had no significant changes.Cranio-orbital MRI revealed no intracranial involvement with suspicion of preseptal/orbital cellulitis and differential diagnosis for pseudotumor or lymphoma (Figure 3).We switched the treatment, adding to local antibiotics and corticosteroids; Prednison (20 mg 4 × 1), the Bactrim (480 mg 2 × 2).Although the visual acuity was still normal, the motility movement started to limit with no pupillary reaction and progression of the symptoms.We returned previous treatment and recommended to the patient the biopsy, which he refused.
In meanwhile, the proptosis advanced with dislocation down and nasal, with no motility movement, no pupillary reaction, cornea exposal and amaurotic eye (Figure 5).Second cranio-orbital MRI revealed still no intracranial involvement, but morbid progression of the diseases (Figure 6).
The biopsy was performed and the results revealed neoplastic-lymphoid non-Hodgkin lymphoma.The full body CT was made with result of mediastinal and hillar limphadenopathy.
After the immunohistochemical analysis the definitive diagnosis revealed neoplastic-lymphoid non-Hodgkin lymphoma, "high grade" diffuse large B-cell lymphoma.The patient was transferred in the Hematology department for the further treatment.The very fast progression of the diseases, from eyelid to lacrimal glands, at one point "S like proptosis" and posterior orbita more like IOD sympthoms [11],

Discussion
made the diagnosis ever harder.
Analyzing all the possible predisposing factors, there were no viral infections, immunosuppressive treatment, environmental factors or any other systemic involvement that could lead the present of other extranodal lymphomas [5] or other systemic diseases.
Although the duration of the symptoms at the presentation range can variety from 10 day with mean duration of 18 months [14], our patient refusing the biopsy, went from normal visual acuity to amaurotic eye in just few days.
The age manifestation was not typical for our patient.Knowing that the "high grade" aggressive type of orbital adnexal non-Hodgkin lymphomas, DLBCL and MCL represent 15% -20% of lymphomas, where DLBCL is most common subtype with male predominance, but with median age 70's [15].
After the DLBCL diagnosis, the cranio-ocular CT revealed no cranial in-

Figure 1 .
Figure 1.Patient before the admitting in hospital.

Figure 2 .
Figure 2. Patient after the admitting in hospital.

Figure 4 .
Figure 4. Progression of the disease.

Figure 5 .
Figure 5. Progression of the symptoms.
Primary orbital adnexal lymphoma is a rare disease.It involves eyelid, orbital lacrimal glands or conjunctivae.Non-Hodgkin lymphoma is a heterogeneous group of malignant lymphoma reflecting development stage of lymphocytes majority arising from B-cell origin [8].Owning unspecific clinical sign often causes delay in diagnosis, because of differential diagnosis with inflammatory orbital diseases and pseudotumors.Our patient, because of the initial sign's and symptoms was treated as preseptal and than orbital cellulitis, starting with antibiotics and corticosteroids.Additional to the treatment, laboratory test and Imaging investigation, as work up at diagnosing OAL [13], revealed initially suspicious of the presetal/orbital cellulitis.