Bilateral Orbital Plasmacytomas With Orbital Compartment Syndrome

Abstract

Orbital plasmacytomas are uncommon soft-tissue plasma cell neoplasms that are associated with a monoclonal or myeloma protein. There are four types of plasma cell neoplasms: multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), amyloidosis, and plasmacytoma. Plasmacytomas may be classified as medullary, occurring only within the bone, or extramedullary, occurring in soft tissues. Orbital plasmacytomas are extramedullary manifestations associated with MM and they may present with signs and symptoms such as unilateral proptosis, conjunctival injection, ocular pain, diplopia, and vision changes. The diagnosis of orbital plasmacytomas is based on tissue biopsy and histologic and immunohistochemical confirmation of a homogenous infiltrate of monoclonal plasma cells.In this report, we present a case of a 60-year-old female patient with a prior diagnosis of MM and new-onset bilateral orbital plasmacytomas following an autologous peripheral blood stem cell transplant; her condition improved significantly following treatment with dexamethasone, cisplatin, doxorubicin, cyclophosphamide, and etoposide along with palliative radiation therapy (RT) of 2000 cGy in 10 fractions to the orbits. Unfortunately, three months later, she had progression of extramedullary disease with parotid gland involvement. She had multiple complicated hospitalizations and eventually expired. As patients with orbital plasmacytomas classically have lower remission and survival rates compared to those with extramedullary plasmacytomas involving other locations, they must be considered high-risk patients who require a multidisciplinary approach for early diagnosis and timely treatment in order to prevent disease progression and to alleviate symptoms related to the disease.

Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review

“…Although orbital plasmacytomas are usually unilateral, bilateral manifestations, as in our case, have been reported ( 16 – 18 ). Orbital plasmacytomas have been described as the first manifestation of systemic disease and also as the first sign of relapse, like in our case ( 12 – 15 ).…”

Bilaterale orbitale Affektion bei multiplem Myelom

“…Es gibt 4 verschiedene Typen einer Plasmazellneoplasie. Darunter fallen das multiple Myelom, die „monoclonal gammopathy of undetermined significance“ (MGUS), die Amyloidose und das Plasmozytom [ 3 ]. Das multiple Myelom mit orbitaler Beteiligung ist selten.…”

“…Der Wirkmechanismus liegt einerseits in der Inhibition der Phospholipase A2 und der Zyklooxygenase sowie andererseits in einem immunsuppressiven Effekt [ 12 ]. In den wenigen beschriebenen Fallberichten wird von spontanen Rückbildungen [ 7 ], einer Kombinationstherapie von Steroiden und Chemotherapie [ 3 , 13 ] sowie gar einem akuten operativen Vorgehen mittels lateraler Kanthotomie und Kantholyse berichtet [ 6 ].…”

Orbital involvement in long-standing multiple myeloma

“…However, out of 220 published case reports and series regarding orbital involvement in multiple myeloma, mostly including case reports and small case series, only few case reports have established coexisting orbital tumours such as T cell lymphoma, squamous cell carcinoma, amyloidosis and, very rarely, aspergilloma (other than plasmacytoma) in patients with prior history of multiple myeloma 9–12. Few authors advocate radiation therapy without waiting for the histopathology confirmation and it has shown good restoration of vision and most importantly globe salvage 13 14…”