Plasma Cell Tumors of the Orbit and Periocular Region

Etiology

• Monoclonal or polyclonal proliferations of plasma cell or plasmacytoid lymphocytes
• IgG is most common, followed by IgA.
• 4 main variants (Burkat, Surv Ophthalmol. 2009)
• Polyclonal plasma cell tumor
• Heterogenous population of plasma cells and lymphocytes
• Typically affect conjunctiva, but can be found in the orbit
• Solitary plasmacytomas
• Isolated monoclonal plasma cells within bone
• No other skeletal bone involvement
• Extramedullary plasmacytoma
• Develops in soft tissue
• Commonly occur in upper respiratory tract and sinuses with orbital extension
• Plasma cell myeloma (multiple myeloma)
• Arise from bone marrow of skeletal bone; leukemic stage
• Lytic lesions of bone
• Necrobiotic xanthogranuloma (NXG)
• Histiocytic nodules that affect eyelids and periorbitum

Epidemiology

Solitary Plasmacytoma

• 6th–7th decades
• Male to female: 3:1

Extramedullary plasmacytoma

• 7th–8th decades
• Male to female: 2:1

Plasma cell myeloma

• 68–70 years median age

History

• Typically a manifestation of systemic disease; rarely a solitary focus in the orbit
• 65% of patients presenting with orbital involvement are carrying a diagnosis of multiple myeloma (MM) (Burkat, Surv Ophthalmol. 2009).
• Weight loss/fatigue
• Bone pain
• Particularly in multiple myeloma, whereas solitary plasmacytoma typically do not cause pain
• Anemia
• Hypercalcemia
• Lytic lesions of bone
• Peripheral neuropathy
• Cutaneous rash

Clinical features

(Burkat, Surv Ophthalmol. 2009)

• Slowly progressive proptosis (axial or abaxial) is the most common presenting sign.
• Periorbital swelling (Figure 1)
• Loss of vision
• Ptosis
• Diplopia
• Can be bilateral, however unilateral is more common (Malik, Indian J Ophthalmol. 2009)
• Location varies:
• Most commonly superotemporal, posterior, and extraconal
• Bone involvement is common.
• Solitary plasmayctoma
• Multiple myeloma
• Can also affect the lacrimal gland, optic nerve and extraocular muscles
• Amyloidosis develops in 8%–15% of patients.
• Should also be included in the differential diagnosis for a multiple myeloma patient with an orbital or subconjunctival mass
• Although the orbit is the most common location, conjunctival and anterior segment lesions have also been documented.
  • May manifest as a salmon patch conjunctival mass.
• Systemic myeloma can present with orbital muscle swelling and proptosis due to paraproteinemia (Rootman, Diseases of the Orbit: A multidisciplinary approach 2002).

Figure 1. Top: Periorbital swelling seen clinically. Bottom: Axial CT of the patient demonstrating periorbital and intracranial involvement with bony remodeling.

Testing

• Patients need to be evaluated for MM.
• Serum/urine protein electrophoresis for immunoglobulin components
• Light chain (Bence-Jones protein)
• Heavy chain (IgG, IgA, etc.)
• 15% of cases do not have immunoglobulinemia (Bataille, New Engl J Med 1997).
• Free light chain assay
• Serum calcium elevated in MM
• Erythrocyte sedimentation rate
• Alkaline phosphatase level elevated in MM
• Complete blood count with differential
• Bone marrow aspirate
• Skeletal bone imagery
• Scintigraphy of the head and neck
• Positron emission tomography (PET) scan
• Computed tomography
• Solitary plasmacytoma of bone: bone erosion
• Extramedullary plasmacytoma: homogeneous, nonencapsulated mass with mild enhancement; molds to surface of globe
• Plasma cell myeloma: soft tissue mass in retrobulbar space; may see lysis of walls of orbit; appears attached to underlying bone
• Magnetic resonance imaging (Jackson, Br J Radiol. 1993) (Figure 2)
• T1: Intensity similar to extraocular muscles
• T2: Intensity similar to orbital fat
• Useful in distinguishing from metastasis which generally are more intense that fat
• Tissue diagnosis critical in diagnosing the lesion and subsequent evaluation

Figure 2. MRI and CT of a patient with plasmacytoma involving brain and R lateral orbit. Note the relative paucity of bony changes on the CT and the variable densities of the mass on T1- and T2-weighted images.

• Age: The incidence of myeloma increases with age and peaks in individuals more than 80 years of age.
• Race: More common in blacks than whites
• Obesity: Body mass index (BMI) greater than 30
• Family history: More common in individuals with a family history of monoclonal gammopathy
• Gender: More common in men than women

• Lymphoma and other lymphoproliferative disorders
• Metastatic lesions

Natural history

• Mean survival (de Smet, Ophthalmology. 1987)
• Plasmacytoma: 28 months
• Compared to 8.3 years for plasmacytomas located in other areas of the body
• Extraosseous plasmacytoma: 8.3 years
• Multiple myeloma: 24 months
• Plasmacytoma (de Smet, Ophthalmology. 1987)

Treatment options

(Rootman, Diseases of the Orbit: A multidisciplinary approach 2002)

• Polyclonal plasmacytoma
• Excision
• Large or inaccessible lesions can be treated with low-dose radiation.
• Solitary plasmacytoma of orbital bone: radiotherapy
• Extraosseous plasmacytoma
• Radiotherapy as initial treatment
• Surgical excision and chemotherapy for persistent and recurrent disease
• Plasma cell myeloma
• Chemotherapy
• Bone marrow transplantation

Surgery

• Aimed at obtaining a tissue diagnosis
• Excisional biopsy
• Fine needle aspiration (Yakulis, A case report. Acta Cytol. 1995)

• Surgical complications
• Radiotherapy complications
• Chemotherapy complications

• Development of multiple myeloma
• Solitary plasmacytoma of bone: 50%
• Extraosseous plasmacytoma: 15%
• Necrobiotic xanthogranuloma: 10 %

Monitor for palpable mass in orbit following initial therapy to detect recurrence.

Follow osseous and extraosseous plasmacytoma patients for possible plasma cell myeloma.

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