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Bilateral orbital lymphoma presenting as recurrence of orbital fat pad after blepharoplasty
CORRESPONDENCE Bilateral orbital lymphoma presenting as recurrence of orbital fat pad after blepharoplasty Lymphoma is the most common orbital malignancy found in adults aged 450 years, representing approximately 10% of all orbital masses.1–3 Diagnosis may be delayed as these tumours are often slow-growing and malleable. Low-grade, small-cell lymphomas conform to the globe and other orbital structures, thus not exerting orbital effects until proptosis or other mass effects such as diplopia, ptosis, or subcutaneous bulging ensue. Tissue biopsy is required for diagnosis, and treatment is most often local radiation. Comparatively, orbital fat prolapse and dermatochalasis are common. The treatment of choice is elective cosmetic blepharoplasty, which is one of the most common cosmetic surgeries performed in North America. This case study demonstrates the need for maintaining a high index of suspicion for orbital lymphoma if tissue excised during a cosmetic blepharoplasty appears abnormal. In 2011, a 69-year-old Caucasian female was referred for slowly progressive bilateral bulging in her upper eyelids, which she stated had gradually enlarged over a 1–2-year period. She had no ophthalmological symptoms and was chiefly concerned with its cosmesis. Her medical history included osteoporosis, and her only medication was Fosavance, Merck. Her ocular history included dry eyes treated with artificial tears as needed. Her ophthalmological examination findings were normal with normal pupils, visual acuity, eye movements and lid function and no proptosis or strabismus. She was offered upper lid blepharoplasty for presumed dermatochalasis and medial fat pad prolapse, which she declined; however, she returned 1 year later wishing to pursue surgery (Fig. 1). Uneventful bilateral upper lid blepharoplasty was performed and medial fat pads were excised using Colorado needle cutting cautery. She was satisfied with her postoperative appearance (Fig. 2). The medial fat pads were noted to have a tough consistency and to be not pink or fleshy, but distinctly grey in colour. Specimens were not sent to pathology as the surgeon (R.A.) believed that the similar appearance bilaterally was indicative of a normal variant and perhaps a result of cautery-induced thermal changes. She was seen in follow-up 1 month postsurgery and discharged.
Fig. 1 — Preoperative appearance before bilateral upper lid blepharoplasty.
Fig. 2 — Postoperative appearance 1 month after blepharoplasty.
Five months after initial surgery, she returned for a small-suture cyst along her right eyelid scar. She had a moderate recurrence of the medial fat prolapse on her left and very subtle recurrence on the right. Upon palpation, this presumptive recurrent fat had a rubbery consistency. No nodes were palpated in the head and neck. The suture cyst was removed from the right scar (Fig. 3). A complete blood count and thyroid profile, including antibodies, were normal. Bilateral orbital masses of intermediate signal intensity were seen on T1- and T2-weighted magnetic resonance sequences. The left orbital mass in the superior orbit, measuring 3.2 cm 1.8 cm in transverse and anteroposterior dimensions, encircled the medial and superior globe and extended beneath the superior rectus into the posterior orbit. The right orbital mass was significantly smaller and confined to the anterior extraconal space (Fig. 4). As these findings were highly suspicious for orbital lymphoma, an anterior orbitotomy through the left lid crease was performed, and tissue biopsy confirmed extranodal marginal zone B-cell lymphoma of the mucosal-associated lymphoid tissue type. Staging computed tomography scans of the head, chest, abdomen, and pelvis to rule out metastasis were negative. She was treated with local low-dose radiotherapy using 400 cGy in 2 fractions leading to rapid tumour resolution. Her eyelids also returned to their initial postoperative appearance. Since treatment 2 years ago, she has shown no evidence of recurrence. Orbital lymphoma presenting as orbital fat prolapse has been reported in the literature only a few times. Two case reports presented lower lid fat prolapse,3,4 and 1 case series described 3 patients presenting with bilateral upper lid
Fig. 3 — Postoperative appearance with recurrence after 5 months. The left medial bulge is most pronounced. The small lesion along the right lid crease is from removal of a suture cyst that day. CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
1
Correspondence
Fig. 4 — Coronal T1-weighted magnetic resonance imaging through the anterior orbit showing bilateral orbital masses (marked by asterisk bilaterally) conforming to the globes of medium signal intensity.
ptosis and dermatochalasis.5 In each case, unusual findings prompted imaging followed by tissue biopsy. These findings included abnormal fat texture and skin changes over the eyelid noted preoperatively or fat specimen abnormalities noted intraoperatively. In our case, suspicious findings included recurrence within 5 months and, in retrospect, abnormal fat texture and colour were noted intraoperatively. Orbital lymphomas typically present in the fifth to seventh decades of life.6 Although associated ocular symptoms are often present, they may present as a purely cosmetic concern for patients.3–5 Symptoms can include exophthalmos, conjunctivitis, ptosis, ophthalmoplegia, blurred vision, pain, and periorbital edema. Up to 10% of cases can be bilateral.6 In this case, the surgeon’s assumption that the medial fat pad appearances were a normal variant because of bilateral similarity was erroneous. As previously mentioned, orbital lymphoma can be bilateral in 10% of cases. Fortunately, the rapid recurrence was noted, surgical notes were reviewed, and further investigation was pursued. All patients with suspected orbital lymphomas should undergo radiographic imaging. Excisional biopsy and histological typing provides confirmatory diagnosis. Treatment for orbital lymphoma includes orbital radiotherapy for low-grade lymphoma.2 Although well tolerated, radiation toxicity effects include conjunctival irritation, cataract
2
CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
development, radiation retinopathy, secondary tumours, and optic neuropathy.6 High-grade lymphomas, even if localized, require systemic chemotherapy before orbital radiotherapy.2,6 Overall, 5-year ocular lymphoma survival rate is favourable at 75.9%, with no differences in subtypes when patients are aged 450 years. In conclusion, all patients presenting with dermatochalasis and orbital fat prolapse should receive a detailed ocular and orbital examination. Orbital lymphomas do not commonly present with lid bulging and excess skin, but this remains a diagnostic possibility as the cosmetic change may be the only symptom. In addition, if during blepharoplasty the excised orbital fat or contents appear unusual, even if similar bilaterally, the tissue must be sent for pathological examination.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Lili Tong, * Jenny Qian, * Robert Adam † University, Toronto, Ont; †University of Toronto, Toronto, Ont.
*McMaster
Correspondence to: Lili Tong, BSc, MD: [email protected] REFERENCES 1. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions. Ophthalmology. 2004;111: 997-1008. 2. Hussan WM, Alfaar AS, Bakry MS, Ezzat S. Orbital tumours in USA: difference in survival patterns. Can Epidemiol. 2014;38:515-22. 3. Hwang CS, Diaz-Marchan P, Mars DP. Mucosa-associated lymphoid tissue lymphoma masquerading as herniated orbital fat. Ophthal Plast Reconstr Surg. 2014;30:e45-7. 4. Marival T, Carpentier S, Vandaele S, De Fontaine S. Blepharoplasty revealing orbital lymphoma. Ann Plast Surg. 2013;70:261-3. 5. Arat YO, Boniuk M. Incidental diagnosis of orbital lymphoma during blepharoplasty. Ophthal Plast Reconstr Surg. 2003;19:316-9. 6. Woolf DK, Ahmed M, Plowman PN. Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma. Clin Oncol. 2012;24:339-44. Can J Ophthalmol 2016;]:]]]–]]] 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.08.006
Fig. 1 — Preoperative appearance before bilateral upper lid blepharoplasty.
Fig. 2 — Postoperative appearance 1 month after blepharoplasty.
Five months after initial surgery, she returned for a small-suture cyst along her right eyelid scar. She had a moderate recurrence of the medial fat prolapse on her left and very subtle recurrence on the right. Upon palpation, this presumptive recurrent fat had a rubbery consistency. No nodes were palpated in the head and neck. The suture cyst was removed from the right scar (Fig. 3). A complete blood count and thyroid profile, including antibodies, were normal. Bilateral orbital masses of intermediate signal intensity were seen on T1- and T2-weighted magnetic resonance sequences. The left orbital mass in the superior orbit, measuring 3.2 cm 1.8 cm in transverse and anteroposterior dimensions, encircled the medial and superior globe and extended beneath the superior rectus into the posterior orbit. The right orbital mass was significantly smaller and confined to the anterior extraconal space (Fig. 4). As these findings were highly suspicious for orbital lymphoma, an anterior orbitotomy through the left lid crease was performed, and tissue biopsy confirmed extranodal marginal zone B-cell lymphoma of the mucosal-associated lymphoid tissue type. Staging computed tomography scans of the head, chest, abdomen, and pelvis to rule out metastasis were negative. She was treated with local low-dose radiotherapy using 400 cGy in 2 fractions leading to rapid tumour resolution. Her eyelids also returned to their initial postoperative appearance. Since treatment 2 years ago, she has shown no evidence of recurrence. Orbital lymphoma presenting as orbital fat prolapse has been reported in the literature only a few times. Two case reports presented lower lid fat prolapse,3,4 and 1 case series described 3 patients presenting with bilateral upper lid
Fig. 3 — Postoperative appearance with recurrence after 5 months. The left medial bulge is most pronounced. The small lesion along the right lid crease is from removal of a suture cyst that day. CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
1
Correspondence
Fig. 4 — Coronal T1-weighted magnetic resonance imaging through the anterior orbit showing bilateral orbital masses (marked by asterisk bilaterally) conforming to the globes of medium signal intensity.
ptosis and dermatochalasis.5 In each case, unusual findings prompted imaging followed by tissue biopsy. These findings included abnormal fat texture and skin changes over the eyelid noted preoperatively or fat specimen abnormalities noted intraoperatively. In our case, suspicious findings included recurrence within 5 months and, in retrospect, abnormal fat texture and colour were noted intraoperatively. Orbital lymphomas typically present in the fifth to seventh decades of life.6 Although associated ocular symptoms are often present, they may present as a purely cosmetic concern for patients.3–5 Symptoms can include exophthalmos, conjunctivitis, ptosis, ophthalmoplegia, blurred vision, pain, and periorbital edema. Up to 10% of cases can be bilateral.6 In this case, the surgeon’s assumption that the medial fat pad appearances were a normal variant because of bilateral similarity was erroneous. As previously mentioned, orbital lymphoma can be bilateral in 10% of cases. Fortunately, the rapid recurrence was noted, surgical notes were reviewed, and further investigation was pursued. All patients with suspected orbital lymphomas should undergo radiographic imaging. Excisional biopsy and histological typing provides confirmatory diagnosis. Treatment for orbital lymphoma includes orbital radiotherapy for low-grade lymphoma.2 Although well tolerated, radiation toxicity effects include conjunctival irritation, cataract
2
CAN J OPHTHALMOL — VOL. ], NO. ], ] 2016
development, radiation retinopathy, secondary tumours, and optic neuropathy.6 High-grade lymphomas, even if localized, require systemic chemotherapy before orbital radiotherapy.2,6 Overall, 5-year ocular lymphoma survival rate is favourable at 75.9%, with no differences in subtypes when patients are aged 450 years. In conclusion, all patients presenting with dermatochalasis and orbital fat prolapse should receive a detailed ocular and orbital examination. Orbital lymphomas do not commonly present with lid bulging and excess skin, but this remains a diagnostic possibility as the cosmetic change may be the only symptom. In addition, if during blepharoplasty the excised orbital fat or contents appear unusual, even if similar bilaterally, the tissue must be sent for pathological examination.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Lili Tong, * Jenny Qian, * Robert Adam † University, Toronto, Ont; †University of Toronto, Toronto, Ont.
*McMaster
Correspondence to: Lili Tong, BSc, MD: [email protected] REFERENCES 1. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions. Ophthalmology. 2004;111: 997-1008. 2. Hussan WM, Alfaar AS, Bakry MS, Ezzat S. Orbital tumours in USA: difference in survival patterns. Can Epidemiol. 2014;38:515-22. 3. Hwang CS, Diaz-Marchan P, Mars DP. Mucosa-associated lymphoid tissue lymphoma masquerading as herniated orbital fat. Ophthal Plast Reconstr Surg. 2014;30:e45-7. 4. Marival T, Carpentier S, Vandaele S, De Fontaine S. Blepharoplasty revealing orbital lymphoma. Ann Plast Surg. 2013;70:261-3. 5. Arat YO, Boniuk M. Incidental diagnosis of orbital lymphoma during blepharoplasty. Ophthal Plast Reconstr Surg. 2003;19:316-9. 6. Woolf DK, Ahmed M, Plowman PN. Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma. Clin Oncol. 2012;24:339-44. Can J Ophthalmol 2016;]:]]]–]]] 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.08.006