Retrobulbar secondary plasmacytoma:a case report and systematic review of literature

Soodeh Enayati,1,* Afshin karami,2 Mehrdad payandeh,3 Noorodin karami,4



Retrobulbar secondary plasmacytoma: a case report and systematic review of the literature multiple myeloma is a tumor of malignant plasma cells that consider as the second most common hematologic malignancy. this disease has an age-adjusted incidence of 4.7 cases per 100,000 populations. it occurs more often in adults, and the mean age at diagnosis is 70 years. one of the most prominent features of this disorder is the presence of more than 10% clonal plasma cells in the bone marrow or biopsy associated with end-organ damage. also, in 1846, dalrymple and bence jones discovered an uncommon plasma cell tumor known as plasmacytoma. the etiology of plasmacytoma remains largely unknown, but factors such as viral pathogenesis have been noted and genetic agents may also play a role. the purpose of this paper is to report a 62-year-old man with a clinical and pathological history of mm, who presented evidence of a mass in the retrobulbar area of the eye.


In january 2018 a 62-year-old man was referred to our clinic of hematology and oncology, with one week of back pain history, loss of consciousness in neurological exam. further analysis was done that founded a sign of kidney failure. initial laboratory investigations he had normocytic normochromic anemia with hb: 9.6g/dl, normal wbc and a decrease in platelet count (wbc: 8,300/mm3; platelet count: 79,000/mm3). renal function tests (rft) and serum electrolytes were done usually in multiple myeloma. the results of these tests showed hypergammaglobulinemia (5g/dl) and an increase in blood urea nitrogen (bun) and serum creatinine (cr) levels (bun 64 mg/dl, cr 8.1 mg /dl). magnetic resonance imaging of the lumbar spine shows vertebral collapse at t12 and l2. examination of a bone marrow aspirate and bone marrow biopsy revealed a high percentage of plasma cells (>20% plasma cells) were negative for cd19, and positive for cd38, cd138.


We made a diagnosis of multiple myeloma and immediately started treatment with bortezomib, cyclophosphamide, and dexamethasone (the approved vcd is preferable as induction therapy for newly diagnosed multiple myeloma especially with renal failure). also, zoledronic acid was used to reduce bone pain. after 6 cycles, the initial response to treatment was relatively good. five months later, the patient referred to an ophthalmologist with symptoms such as swelling of the right eyelid with ptosis, diplopia and blurred vision. in fundus examination, few scattered hemorrhages were seen in the left eye and results of his left eye examination were as follows visual acuity (cc): od: 6/10, os: 4/10. the results of orbit computed tomography (ct) demonstrated of a 26 × 12 mm soft tissue mass in the retrobulbar of the left eye in fine needle aspiration cytology (fnac) of the mass, mature and immature plasma cells were seen, which indicate diagnosis of secondary extramedullary plasmacytoma. so, the patient underwent surgical debulking, the lesion was removed from the retrobulbar area and the result was successful. currently, condition of patient condition is good and he is continuing next chemotherapy cycles with bortezomib and dexamethasone.


Eventually, the important point in these cases is that multiple myeloma should be investigated during differential diagnosis.


Plasmacytoma, neoplasm, multiple myeloma, extramedullary