• Users Online: 352
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 605-607

Paraneoplastic orbital myositis in a patient with natural killer/T-cell lymphoma

1 Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, South Korea
2 Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, South Korea
3 Department of Pathology, Yeungnam University College of Medicine, Daegu, South Korea

Date of Submission02-Sep-2020
Date of Acceptance02-Mar-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Prof. Won Jae Kim
170, Hyeonchung-ro, Nam-gu, Daegu, 42415
South Korea
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2850_20

Rights and Permissions

Paraneoplastic orbital myositis can occur in patients with natural killer/T-cell lymphoma (NKTL). A discrepancy between ocular motility examinations and imaging findings may suggest paraneoplastic orbital myositis in patients with malignancy. Extraocular muscle biopsy should be performed for definite diagnosis and appropriate treatment, especially in patients with atypical clinical manifestations.

Keywords: Diplopia, paraneoplastic syndromes, orbital myositis

How to cite this article:
Jeong A, Kim MK, Choi JH, Kim WJ. Paraneoplastic orbital myositis in a patient with natural killer/T-cell lymphoma. Indian J Ophthalmol Case Rep 2021;1:605-7

How to cite this URL:
Jeong A, Kim MK, Choi JH, Kim WJ. Paraneoplastic orbital myositis in a patient with natural killer/T-cell lymphoma. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 29];1:605-7. Available from: https://www.ijoreports.in/text.asp?2021/1/3/605/320017

The extraocular muscle enlargement is usually associated with thyroid eye disease.[1],[2] However, it can also be caused by other conditions including inflammatory, vascular, neoplastic, infectious, and metabolic disorders.[3],[4] The neoplastic causes include primary tumor, metastasis, and paraneoplastic syndrome.[3],[4],[5] When patients with a history of lymphoma show enlargement of the extraocular muscles in neuroimages, direct involvement of lymphoma may be the first consideration.[6],[7] The orbital myositis caused by paraneoplastic syndrome has rarely been reported throughout the literatures.[3],[5],[8],[9] We report a case of paraneoplastic orbital myositis in a patient with natural killer/T-cell lymphoma (NKTL).

  Case Report Top

A 57-year-old man visited our department presenting with an insidious onset of vertical diplopia 4 months ago. He was diagnosed with NKTL 4 years ago and treated with VIDL (etoposide, ifosfamide, dexamethasone, and L-asparaginase) and radiotherapy. The cancer recurred 1 year after complete remission and was treated with SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide). The lymphoma involved nasal cavity, nasopharynx, and neck. No other systemic disease was noted. The symptoms did not have a diurnal variation or any relationship with fatigue. There was mild chemosis and injection of the left eye without ocular pain. The best-corrected visual acuity was 20/20 in both eyes. The pupils showed a normal response to both light and near stimulation. There was no proptosis or abnormal lid problem in both eyes. Extraocular examination revealed 14 prism diopters (PD) left hypertropia in a primary gaze with moderate elevation limitation of the left eye [Figure 1]. Serologic examinations, including a thyroid function and anti-acetylcholine receptor antibody tests, revealed normal results. Magnetic resonance imaging (MRI) of the brain and orbit revealed enlargement and contrast enhancement of the medial rectus muscle (MR), inferior rectus muscle (IR), and superior oblique muscle (SO) of the left eye [Figure 2]a and [Figure 2]b. The lesion of the nasal cavity and nasopharynx were improved compared with the previous study. Position-emission tomography (PET) showed increased FDG (2-(F-18) fluoro-2deoxy-d-glucose) uptake by the MR and IR of the left eye. The whole-body PET-computed tomography (CT) did not show any abnormal uptake in other sites of body.
Figure 1: A 57-year-old man was consulted in our department with a complaint of binocular vertical diplopia. There was no proptosis or lid retraction in both eyes. Extraocular examination revealed the 14 prism diopters of hypertropia with moderate elevation limitation of the left eye

Click here to view
Figure 2: (a and b). Initial magnetic resonance imaging (MRI) of the brain and the orbit in our patient who had a history of NKTL and presented with binocular vertical diplopia. The MRI showed enlargement and contrast enhancement of medial rectus muscle (MR), inferior rectus muscle (IR), and superior oblique muscle (SO) of the left eye ((a) Coronal view, (b) Sagittal view, arrow). (c and d). The follow-up MRI showed that the enlargement of MR, IR, and SO was improved after steroid treatment compared with initial examination (C: Coronal view, D: Sagittal view, arrowhead)

Click here to view

The extraocular muscle biopsy was performed to make a definitive diagnosis and confirm effectiveness of chemotherapy. The forced duction test was performed under general anesthesia and revealed a mild tightness of the MR and IR. The biopsy of both MR and IR was performed throughout the limbal approach. The muscle was divided into longitudinal thirds using the Stevens hook parallel to the fibers of the muscle. The central segment of muscle was biopsied. The specimen shows focally mild infiltration of mature lymphocytes in the muscle fiber without malignant cells. The pathologic report confirmed myositis of the extraocular muscles [Figure 3]a and [Figure 3]b. He was diagnosed with orbital myositis caused by paraneoplastic syndrome, and steroid treatment was initiated (Dexamethasone 40 mg/D, IV) for 4 days. The additional oral steroids were prescribed. One month later, the follow-up MRI showed that the enlargement of the extraocular muscle had improved compared with that at the initial examination [Figure 2]c and [Figure 2]d. Extraocular examination showed 2 PD left hypertropia in the primary gaze without ocular movement limitation. However, hypertropia and diplopia relapsed after discontinuation of steroids. At 8 months after the biopsy, the ocular motility findings deteriorated to 35 PD left hypertropia and 20 PD exotropia in a primary gaze with moderate elevation and adduction limitation of the left eye. The follow-up MRI demonstrated the enlargement of MR, IR, and SO without other orbital involvement. He is being followed-up with monocular occlusion for diplopia and currently tolerating chemotherapy (GDP, Gemcitabine, Dexamethasone, and Cisplatin). The surgical treatment for strabismus is considering.
Figure 3: (a and b) Histologic findings. (a) Infiltrated lymphocytes in the orbital muscle fibers (hematoxylin and eosin stain, original magnification 200×). (b) CD3-positive T lymphocytes are present (CD3 immunohistochemical stain, original magnification 200×)

Click here to view

  Discussion Top

Paraneoplastic myositis is considered to involve autoantibodies directed against the tumor that cross-react with extraocular muscle antigens and induced autoimmune inflammation.[5],[9],[10] In Shafi et al.'s study of 16 patients with non-thyroid-related extraocular muscle enlargement, 4 patients were paraneoplastic myositis.[3] Paraneoplastic myositis has been reported to associate with breast cancer, thyroid cancer, lymphoma, and seminoma.[3],[5],[8],[9]

In this case, the patient had an insidious onset of diplopia without definitive ocular pain, which is different from the typical clinical features of orbital myositis.[1],[5] Orbital myositis is usually idiopathic with an acute onset, presents with painful diplopia, and responds dramatically to corticosteroid therapy.[1],[3],[4],[5] However, previous studies showed that the clinical manifestations of paraneoplastic myositis are not associated with acute painful diplopia.[5],[8],[9] There are many atypical cases occurring in the setting of systemic inflammatory or infective conditions.[5]

Because this patient had a history of lymphoma, direct involvement to the extraocular muscles was considered. Clinical findings of our patients were different from previous reports with patients with orbital involvement of NKTL. Orbital involvement usually rapidly progressive and occur in multiple orbital structures in patient with NKTL.[6],[7] The inferior oblique muscles were most commonly affected extraocular muscles in orbital involvement of NKTL.[7] Our patient showed the enlargement of MR, IR, and SO without other orbital involvement. The pre-existing lesions including nasal cavity and nasopharynx improved during the follow-ups. The pathologic examination did not show any direct involvement of NKTL. It is therefore likely that the extraocular enlargement in our patient was caused by a paraneoplastic myositis.

In the present case, the extraocular motility findings did not correlate with those of MRI. The MRI showed the enlargement of MR, IR, and SO of the left eye. The forced duction test confirmed mild mechanical restriction of MR and IR. These findings may suggest left hypotropia or esotropia in primary gaze with an up-gaze limitation. However, he showed left hypertropia in primary gaze with an up-gaze limitation of the left eye. This orbital myositis probably affected multiple extraocular muscles and was in a combined paretic and restrictive clinical phase.[5] Ocular motility findings of myositis vary according to the clinical course. Orbital myositis may exhibit the following clinical course over time: normal motility for several days, followed by a paretic phase, then combined paretic and restrictive phase, and finally a restrictive phase.[5] Another study showed that idiopathic orbital myositis showed variable degrees of ocular motility limitation, and limitations in the same direction as that of the action of the affected muscle were more frequent.[11] Therefore, the discrepancy between ocular motility findings and neuroimaging results may indicate orbital myositis.

In addition, orbital imaging alone is not diagnostic, and extraocular muscle measurement can vary between modalities.[12] Extraocular muscle biopsy may be necessary for patients with atypical clinical features accompanying extraocular muscle enlargement.

  Conclusion Top

The orbital myositis can occur due to paraneoplastic syndrome, and extraocular muscle biopsy can be considered for definite diagnosis and appropriate treatment when the findings of imaging are not correlated with ocular movement in patients with tumors.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Miller NR, Subramanian PS, Patel VR. Myopathies of neuro-ophthalmologic significance. In: Walsh and Hoyt's Clinical Neuro-Ophthalmology the Essentials. 3rd ed. Philadelphia: Wolters Kluwer; 2016. p. 386-401.  Back to cited text no. 1
Chung SM, Kim WJ. Acquired strabismus and diplopia as initial presentation signs of hyperthyroidism and thyroid eye disease. J Korean Ophthalmol Soc 2020;61:288-93.  Back to cited text no. 2
Shafi F, Mathewson P, Mehta P, Ahluwalia HS. The enlarged extraocular muscle: To relax, reflect or refer? Eye (Lond) 2017;31:537-44.  Back to cited text no. 3
Mombaerts I, Rose GE, Verity DH. Diagnosis of enlarged extraocular muscles: When and how to biopsy. Curr Opin Ophthalmol 2017;28:514-21.  Back to cited text no. 4
McNab AA. Orbital myositis: A comprehensive review and reclassification. Ophthalmic Plast Reconstr Surg 2020;36:109-17.  Back to cited text no. 5
Woog JJ, Kim YD, Yeatts RP, Kim S, Esmaeli B, Kikkawa D, et al. Natural killer/T-cell lymphoma with ocular and adnexal involvement. Ophthalmology 2006;113:140-7.  Back to cited text no. 6
Cruz AA, Valera FC, Carenzi L, Chahud F, Barros GE, Elias J Jr. Orbital and central nervous system extension of nasal natural killer/T-cell lymphoma. Ophthalmic Plast Reconstr Surg 2014;30:20-3.  Back to cited text no. 7
Harris GJ, Murphy ML, Schmidt EW, Hanson GA, Dotson RM. Orbital myositis as a paraneoplastic syndrome. Arch Ophthalmol 1994;112:380-6.  Back to cited text no. 8
S Kumar, T Diamond. Paraneoplastic syndrome-a rare but treatable cause of non-thyroid-related extraocular muscle enlargement. Orbit 2019;38:468-73.  Back to cited text no. 9
Gordon L, Dinkin M. Paraneoplastic syndromes in neuro-ophthalmology. Continuum (Minneap Minn) 2019;25:1401-21.  Back to cited text no. 10
Kang MS, Yang HK, Kim N, Hwang JM. Clinical features of ocular motility in idiopathic orbital myositis. J Clin Med 2020;18;9:1165.  Back to cited text no. 11
Assam JH, Miller AM, Chevez-Barrios P, Lee AG. Extraocular muscle biopsy during surgery for strabismus of unknown etiology. J AAPOS 2019;23:356-9.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded14    
    Comments [Add]    

Recommend this journal