Bilateral plasmacytoma of extraocular muscles presenting with orbital inflammation

Vijitha S Vempuluru; Anasua G Kapoor; Dilip K Mishra; Swathi Kaliki

doi:10.4103/ijo.IJO_195_21

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Year : 2022 | Volume : 2 | Issue : 1 | Page : 298-299

Bilateral plasmacytoma of extraocular muscles presenting with orbital inflammation

Vijitha S Vempuluru¹, Anasua G Kapoor², Dilip K Mishra³, Swathi Kaliki⁴
¹ Fellow Oculoplasty and Ocular Oncology, Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Vijayawada, Andhra Pradesh, India
² Consultant Oculoplasty and Ocular Oncology, Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Vijayawada, Andhra Pradesh, India
³ Consultant Pathologist, Ophthalmic Pathology Laboratory, Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, Telangana, India
⁴ Consultant Oculoplasty and Ocular Oncology, Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, Telangana, India

Date of Submission	13-Mar-2021
Date of Acceptance	07-Jul-2021
Date of Web Publication	07-Jan-2022

Correspondence Address:
Dr. Anasua G Kapoor
Consultant Oculoplasty and Ocular Oncology, The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Vijayawada, Andhra Pradesh
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_195_21

Abstract

Keywords: Extraocular muscle, eye, metastasis, multiple myeloma, ocular oncology, orbit, orbital inflammation, tumor

How to cite this article:
Vempuluru VS, Kapoor AG, Mishra DK, Kaliki S. Bilateral plasmacytoma of extraocular muscles presenting with orbital inflammation. Indian J Ophthalmol Case Rep 2022;2:298-9

How to cite this URL:
Vempuluru VS, Kapoor AG, Mishra DK, Kaliki S. Bilateral plasmacytoma of extraocular muscles presenting with orbital inflammation. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:298-9. Available from: https://www.ijoreports.in/text.asp?2022/2/1/298/334923

A 51-year-old gentleman presented to his ophthalmologist with sudden onset of bilateral painful proptosis. Bilateral orbital inflammation with eyelid signs had prompted the diagnosis of thyroid eye disease (TED), and the patient was treated with a course of intravenous methylprednisolone and right eye (RE) tarsorrhaphy. When the patient presented to us, both eyes showed limited ocular motility, eyelid retraction with temporal flare, chemosis, and RE relative afferent pupillary defect [Figure 1]a. Visual acuity was no light perception RE, 20/60 left eye [Figure 1]b. Magnetic resonance imaging (MRI) orbits showed bilateral tendon, involving massive thickening of the medial rectus and right lateral rectus causing globe indentation [Figure 1]c and [Figure 1]d. Further questioning revealed a pathological fracture of the femoral bone secondary to multiple myeloma (MM), treated with bortezomib chemotherapy and radiotherapy 5 years back. Bone marrow biopsy showed increased plasma cells [Figure 1]e, monoclonality of IgG (immunoglobulin G) lambda light chain [Figure 1]f, and positive urine Bence–Jones protein confirming orbital relapse of MM. The patient underwent external beam radiotherapy to both orbits, which controlled the disease process; however, visual acuity was no perception of light in both eyes at his last follow-up at 1 year with bilateral optic atrophy. The cause of loss of vision was a combination of compressive and radiation optic neuropathy.

Figure 1: (a) Clinical photograph of a patient showing right conjunctival chemosis with congestion status post tarsorrhaphy. Left eye shows upper eyelid retraction with temporal flare with mild chemosis. (b) Worsened orbital signs despite intravenous methylprednisolone therapy manifest as bilateral increased chemosis and proptosis. (c and d) MRI orbit (T1- and T2-weighted axial cut) showing isointense bilateral tendon-involving ovoid massive thickening of both medial recti and right lateral rectus muscle causing indentation of globe and proptosis. (e) Bone marrow trephine biopsy showing sheets of plasma cells (hematoxylin and eosin, 40×). (f) Bone marrow trephine biopsy showing IgG lambda light chain restriction in plasma cell (immune stain, 40×)

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Discussion

MM is a malignant plasma cell proliferation with orbit affected in <5% cases.^[1],[2] Isolated soft tissue lesions are uncommon^[2] and discrete lesions of extraocular muscles (EOMs; intramuscular plasmacytomas) are extremely rare.^[3] Typical intramuscular plasmacytomas are nonfusiform, confined to one EOM, isointense on T1 and hyperintense on T2, enhancing with contrast.^[3] Orbital signs may herald relapse of treated MM. Muscle enlargement in TED is usually bilateral, symmetrical, fusiform, and tendon-sparing enlargement of >2 muscles,^[4],[5] and metastases usually show isolated, tendon-involving, massive thickening of EOM. A high index of suspicion aids prompt diagnosis and institution of early therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This study was aided by a financial support provided by the Operation Eyesight Universal Institute for Eye Cancer and Hyderabad Eye Research Foundation. The funders have no role in the preparation of the manuscript.

Conflicts of interest

There are no conflicts of interest.

References

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