Orbit, 2015; 34(1): 51–55 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.963879
RESEARCH REPORT
Esophageal Adenocarcinoma and Urothelial Carcinoma Orbital Metastases Masquerading as Infection George N. Magrath1, Charles M. Proctor1, Wade A. Reardon1, Krishna G. Patel2, Eric J. Lentsch2, and Andrew S. Eiseman1
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1
Storm Eye Institute, Charleston, South Carolina, USA and 2Department of Otolaryngology, Medical University of South Carolina, Charleston, South Carolina, USA
ABSTRACT Orbital metastases can masquerade as other orbital processes. We present two cases of orbital metastases, the first being the first reported adenocarcinoma of the esophagus presenting as an orbital metastasis prior to the primary being known, and the other as the first urothelial carcinoma to present as orbital cellulitis. The first patient presented with left upper eyelid pain. CT scan identified a superolateral subperiosteal fluid collection without concomitant sinus disease, which was drained in the operating room. Two weeks later repeat CT scan showed recurrent orbital subperiosteal fluid. It was drained and a biopsy showed necrotic adenocarcinoma. The second case presented with a painless right proptosis, decreased vision, and globally decreased ocular motility 3 days after bladder resection for urothelial carcinoma. CT scan demonstrated pan sinusitis with a soft tissue mass in the apex of the right orbit with extension through the superior orbital fissure. After no improvement on antibiotics endoscopic drainage was performed. Pathology revealed metastatic urothelial carcinoma within the orbital fat. Keywords: Adenocarcinoma, cellulitis, metastasis, oncology
INTRODUCTION
abnormalities and with swelling, erythema, chemosis, and pain. When acute inflammatory changes are the primary presenting signs, it can be difficult to initially differentiate these changes from orbital infection and other causes of inflammatory disease, especially when concomitant sinus disease is also present. Orbital cellulitis is the most common cause of acute inflammatory orbital disease and is commonly the result of spread of infection from the adjacent sinuses and can present with subperiosteal or intraorbital abscess as well.3,4 These patients are typically treated with broad spectrum antibiotics being sure to include
Cancer metastatic to the orbit is a well-known cause of a mass lesion in the orbit and a recent large series identified metastasis to the orbit as 3% of all space occupying lesions in the orbit.1 The three most common primary sites to metastasize to the orbit are prostate, breast, and lung.2 Metastatic lesions to the orbit can present in various ways including proptosis, globe dystopia, motility deficits, and vision loss. Metastases can also present as acute inflammatory changes with all of the previously mentioned
Received 17 March 2014; Revised 12 May 2014; Accepted 6 September 2014; Published online 15 October 2014 Correspondence: George N. Magrath, Storm Eye Institute Charleston, Medical University of South Carolina, 167 Ashley Ave., Charleston, SC 29425, USA. Tel: (843)-333-5241. Fax: (843)792-2262. E-mail: [email protected]
51
52 G. N. Magrath et al.
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
TABLE 1. A differential diagnosis for the acute orbit. Orbital cellulitis Subperiosteal abscess Orbital trauma, intraorbital foreign body Nonspecific orbital inflammatory syndrome Thyroid ophthalmopathy Wegener’s granulomatous Metastatic carcinoma Lymphangioma Lymphoma Ruptured dermoid cyst Extraocular extension of ocular melanoma Sarcoidosis Mucocele Fibrous histiocytoma Cavernous hemangioma Orbital varix Schwannoma Meningioma (sphenoid wing or optic nerve) Optic nerve glioma Dacryocystitis Herpes zoster
coverage for organisms found in the sinuses, and now increasingly, coverage for methicillin resistant Staphylococcus aureus is required.5 With appropriate medical and surgical management, these patients tend to do well and have stabilization and improvement in the disease process within the first few days of treatment.6 In patients with an acute orbit without concomitant sinus disease or cases which do not improve with antibiotics or surgical drainage alone, additional disease processes need to be investigated. One of the most common causes of acute orbital inflammation that does not improve with antibiotics the first few days is nonspecific orbital inflammation.3 These patients are typically treated with steroids with a quick improvement in symptomatology. However, other disease processes can also present with acute orbital inflammation like metastatic cancer and care must be taken to consider a wide differential diagnosis for patients with atypical orbital inflammation that does not respond as anticipated (Table 1). The authors present two recent cases of orbital metastases that both presented with acute inflammatory changes.The first case is an esophageal adenocarcinoma metastatic to the orbit that presented with a mass and a superolateral subperiosteal fluid collection before the primary site had been identified. The second case is a urothelial carcionoma metastatic to the apex of the orbit that presented with significant sinus mucosal thickening mimicking pansinusitis. Although esophageal and urothelial carcinomas have been previously reported to metastasize to the orbit,2,5 a review of the literature could not identify cases where these cancers presented in a similar fashion. The authors represent that this material was created in accordance with the provisions of the Health Insurance Portability and Accountability Act
(HIPAA) of 1996, and that this material does not include any protected health information as defined by HIPAA.
CASE SERIES Case 1 A 51-year-old white male presented with a 5-day history of worsening left upper eyelid pain, redness, and swelling despite a 3-day course of broad spectrum oral antibiotics. Patient also had proptosis and decreased abduction. CT scan identified a superolateral subperiosteal fluid collection without concomitant sinus disease. The fluid collection was drained in the operating room and the patient was discharged on oral antibiotics. Two weeks later he presented with recurrent swelling, proptosis, and diplopia. An MRI scan showed recurrent orbital subperiosteal fluid with a soft tissue component, a subtemporalis muscle fluid collection, and bone changes of the lateral and superior orbital walls with dural enhancement (Figure 1, see Video, Supplemental Digital Content 1, which shows full MRI image series). Both were drained in the operating room and a biopsy of the tissue within the orbit showed necrotic adenocarcinoma. A full-body PET/CT demonstrated a large esophageal mass and liver masses. Biopsy of the esophageal mass confirmed this as the primary site. The patient received orbital and esophageal radiation therapy and 2 months later presented with a small bowel obstruction. He was subsequently admitted to inpatient hospice care for palliative care.
Case 2 A 57-year-old African American female developed painless right proptosis, decreased vision, and globally decreased ocular motility 3 days after bladder resection for urothelial carcinoma. CT scan demonstrated mucosal thickening in the adjacent sinuses consistent with pan sinusitis with a soft tissue mass in the apex of the right orbit with extension through the superior orbital fissure, anteromedial middle cranial fossa, and cavernous sinus (Figure 2, see Video, Supplemental Digital Content 2, which shows full CT image series). The patient was placed on broad spectrum intravenous antibiotics. Two days after a failure to improve, the patient was taken to the operating room for an endoscopic exploration and drainage of the ethmoid, maxillary, and sphenoid sinuses and orbital biopsy. Pathology revealed metastatic urothelial carcinoma within the orbital fat. Three weeks later the patient was readmitted obtunded Orbit
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Orbital Metastasis Masquerading as Infection
53
FIGURE 2. CT brain and orbits with and without contrast reveals ethmoid sinusitis with an ipsilateral soft tissue mass in the orbital apex extending posterior into the cavernous sinus and middle cranial fossa.
FIGURE 1. MRI brain and orbits with and without contrast shows a large superolateral fluid collection (A) with extension through the superior and lateral orbital walls into the epidural and subtemporalis space (B).
without evidence of stroke or meningitis. She was discharged to home hospice care for palliative care.
DISCUSSION Metastasis to the orbit is a well-known complication of primary malignancy. In one the largest reported series of orbital tumors, metastases accounted for 7% of the total orbital tumors in adults.4 Of these the most common primary sites were breast and prostate, accounting for 48% and 12% of the total orbital metastases, respectively. Esophageal and urothelial carcinoma metastases to the orbit have been previously reported but they occur at a much lower !
2015 Informa Healthcare USA, Inc.
frequency.7,8 In the recent large retrospective series the rate of metastasis in patients with space occupying orbital lesions was 3%.1 Although orbital metastasis from a known primary has been well reported, the typical presentation is with a mass lesion causing proptosis or an ocular motility defect.2 Orbital metastases from a known primary presenting as either acute inflammatory changes, orbital cellulitis, and/or subperiosteal abscess is much less common although previously reported.8–12 However, a literature search could find no previous cases of esophageal adenocarcinoma presenting with acute inflammatory changes with a subperiosteal fluid collection mimicking an abscess or urothelial carcinoma presenting as an inflammatory orbital apex syndrome with sinus mucosal thickening mimicking pansinusitis. Orbital metastases masquerading as orbital infection with acute inflammatory changes as the initial presentation of malignancy in an adult is rare. A literature search revealed only four prior cases of presumed orbital cellulitis or subperiosteal abscess presenting with an unknown primary malignancy, as in case 1.8,13,14 One of these cases involved an esophageal squamous cell carcinoma metastasis masquerading as an orbital abscess without concomitant sinus disease in a patient without a known history of cancer.8 The two cases in this manuscript presented as cases of acute orbital inflammation. In case 1 the patient presented with lid swelling and erythema, double vision, globe dystopia and pain. The initial CT scan showed only a subperiosteal fluid collection in the superolateral orbit without concomitant sinus disease. Subsequent magnetic resonance imaging
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
54 G. N. Magrath et al. identified bone changes with dural enhancement and subperiosteal fluid with a soft tissue component more consistent with metastatic disease. Without an external nidus for infection, the most common cause of orbital cellulitis is ipsilateral concurrent sinusitis and subperiosteal abscesses are therefore typically adjacent to the infected sinus as well.15,16 Although this case presented with aucte orbital inflammation and a subperiosteal fluid collection mimicking orbital infection, the lack of concomitant and adjacent sinus disease caused expansion of the differential diagnosis, especially after initial drainage and treatment with broad spectrum antibiotics failed to result in the anticipated improvement. It is important to consider all causes of acute orbital inflammatory changes when evaluating such patients since significant systemic disease can be the cause. In this case, the orbital inflammation was the initial presentation of a primary esophageal adenocarcinoma with metastases. Case 2 presented as an acute painless orbital inflammatory apex syndrome with concurrent, ipsilateral sinus mucosal inflammatory thickening. Although there have been several case reports of patients with orbital metastasis masquerading as orbital cellulitis with ipsilateral sinus disease, ours is the first urothelial carcinoma to present in a similar fashion.11–13 Patients with systemic malignancies may undergo surgical procedures that increase the risk of bacteremia and systemic infection and chemotherapy which may induce immunosuppression, also increasing susceptibility. This may make the differentiation between orbital cellulitis and orbital metastasis difficult. In case 2, the patient presented with acute orbital inflammatory changes in the immediate post-operative period after initial resection of the urothelial carcinoma. The lack of pain and the failure of the process to respond to broad spectrum antibiotics precipitated the decision to surgically explore the sinuses and orbit and this confirmed the diagnosis of metastatic spread to the orbit. Cancer metastatic to the orbit can lead to ophthalmic manifestations by several mechanisms. The first is a mechanical force in the orbit as the tumor expands. This can lead to proptosis, limitation of ocular motility, and compression of structures in the orbit. If metastases are also in the adjacent sinuses, tumor can obstruct the outflow system leading to acute inflammatory changes and even secondary infection. This is the likely explanation for the presentation in case 2. The second is the inflammatory microenvironment referred to as cancer related inflammation. This includes the infiltration of tumor associated macrophages and the presence of cytokines.17 This cancer related inflammation is the likely cause of the subperiosteal fluid collection, bone change, and dural enhancement in case 1.
Although orbital metastases are a rare cause of the acute orbit, it is important to exhaust a full differential diagnosis in patients who present with such changes. We have presented two cases of orbital metastases that presented with acute inflammatory changes. Case 1 is unique because it is the first time that a metastatic esophageal adenocarcinoma has presented as a subperiosteal fluid collection without a known primary. Case 2 is unique because it is the first time that metastatic urothelial carcinoma to the orbit presented as a painless apex syndrome with concurrent inflammatory sinus mucosal thickening. Clinicians must maintain a high index of suspicion when dealing with patients who present with acute orbital inflammation, especially if they are atypical or fail to respond as anticipated.
FUNDING This research is supported in part by an unrestricted grant from Research to Prevent Blindness.
DECLARATION OF INTEREST The authors do not report any conflicts of interest or financial interest in this manuscript.
REFERENCES 1. Bonavolonta` G, Strianese D, Grassi P, et al. An Analysis of 2,480 Space-Occupying Lesions of the Orbit From 1976 to 2011. Ophthal Plast Reconstruct Surg 2013;29(2):79–86. 2. Shields JA, Shields CL, Brotman HK, et al. Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture. Ophthal Plast Reconstruct Surg 2001;17(5):346–354. 3. Harris GJ. Idiopathic orbital inflammation: a pathogenetic construct and treatment strategy: The 2005 ASOPRS Foundation Lecture. Ophthal Plast Reconstr Surg 2006; 22(2):79–86. 4. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111(5): 997–1008. 5. Mathias MT, Horsley MB, Mawn LA, et al. Atypical presentations of orbital cellulitis caused by methicillinresistant Staphylococcus aureus. Ophthalmology 2012;119(6): 1238–1243. 6. Ferguson MP, McNab AA. Current treatment and outcome in orbital cellulitis. Aust N Z J Ophthalmol 1999;27(6): 375–379. 7. SooHoo JR, Gonzalez MO, Siomos VJ, Durairaj VD. Urothelial carcinoma with orbital metastasis. Urology 2012;80(4):e45–e46. 8. Lee JY, Lee HJ, Jung MS, Kim SY. Metastatic esophageal squamous cell carcinoma to the orbit and periorbit masquerading as periorbital abscess. Kor J Ophthal 2010; 24(2):123–125. 9. Barahimi B, Patel A, Bilyk JR. Orbital metastasis mimicking subperiosteal abscess. Orbit 2010;29(3):165–167. Orbit
Orbital Metastasis Masquerading as Infection
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
10. Gonsalves SR, Lobo GJ, Mendonca N. Truth under a masquerade! BMJ Case Rept 2013;1. 11. Kumar R, Issing W. Orbital cellulitis: A rare presentation of metastatic bronchial carcinoma. Case Rept Otolaryngol 2011; 2011:397–451. 12. Qureshi F, Maddula S, Hardman-Lea S. Recurrent metastatic breast carcinoma presenting with pansinusitis and choroidal metastases and subsequent bilateral simultaneous orbital cellulitis. Semin Ophthalmol 2012;27(1–2):33–34. 13. Fyrmpas G, Televantou D, Papageorgiou V, et al. Unsuspected breast carcinoma presenting as orbital complication of rhinosinusitis. Euro Arch Oto-Rhino-Laryngol Head Neck Surg 2008;265(8):979–982.
!
2015 Informa Healthcare USA, Inc.
55
14. Fortson JK, Bezmalinovic ZL, Moseley DL. Bilateral ethmoid sinusitis with unilateral proptosis as an initial manifestation of metastatic prostate carcinoma. J Natl Med Asso 1994;86(12):945–948. 15. Robinson A, Beech T, McDermott AL, Sinha A. Investigation and management of adult periorbital and orbital cellulitis. J Laryngol Otol 2007;121(6):545–547. 16. Dewan MA, Meyer DR, Wladis EJ. Orbital cellulitis with subperiosteal abscess: demographics and management outcomes. Ophthal Plast Reconstr Surg 2011;27(5):330–332. 17. Colotta F, Allavena P, Sica A, et al. Cancer-related inflammation, the seventh hallmark of cancer: links to genetic instability. Carcinogenesis 2009;30(7):1073–1081.
RESEARCH REPORT
Esophageal Adenocarcinoma and Urothelial Carcinoma Orbital Metastases Masquerading as Infection George N. Magrath1, Charles M. Proctor1, Wade A. Reardon1, Krishna G. Patel2, Eric J. Lentsch2, and Andrew S. Eiseman1
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
1
Storm Eye Institute, Charleston, South Carolina, USA and 2Department of Otolaryngology, Medical University of South Carolina, Charleston, South Carolina, USA
ABSTRACT Orbital metastases can masquerade as other orbital processes. We present two cases of orbital metastases, the first being the first reported adenocarcinoma of the esophagus presenting as an orbital metastasis prior to the primary being known, and the other as the first urothelial carcinoma to present as orbital cellulitis. The first patient presented with left upper eyelid pain. CT scan identified a superolateral subperiosteal fluid collection without concomitant sinus disease, which was drained in the operating room. Two weeks later repeat CT scan showed recurrent orbital subperiosteal fluid. It was drained and a biopsy showed necrotic adenocarcinoma. The second case presented with a painless right proptosis, decreased vision, and globally decreased ocular motility 3 days after bladder resection for urothelial carcinoma. CT scan demonstrated pan sinusitis with a soft tissue mass in the apex of the right orbit with extension through the superior orbital fissure. After no improvement on antibiotics endoscopic drainage was performed. Pathology revealed metastatic urothelial carcinoma within the orbital fat. Keywords: Adenocarcinoma, cellulitis, metastasis, oncology
INTRODUCTION
abnormalities and with swelling, erythema, chemosis, and pain. When acute inflammatory changes are the primary presenting signs, it can be difficult to initially differentiate these changes from orbital infection and other causes of inflammatory disease, especially when concomitant sinus disease is also present. Orbital cellulitis is the most common cause of acute inflammatory orbital disease and is commonly the result of spread of infection from the adjacent sinuses and can present with subperiosteal or intraorbital abscess as well.3,4 These patients are typically treated with broad spectrum antibiotics being sure to include
Cancer metastatic to the orbit is a well-known cause of a mass lesion in the orbit and a recent large series identified metastasis to the orbit as 3% of all space occupying lesions in the orbit.1 The three most common primary sites to metastasize to the orbit are prostate, breast, and lung.2 Metastatic lesions to the orbit can present in various ways including proptosis, globe dystopia, motility deficits, and vision loss. Metastases can also present as acute inflammatory changes with all of the previously mentioned
Received 17 March 2014; Revised 12 May 2014; Accepted 6 September 2014; Published online 15 October 2014 Correspondence: George N. Magrath, Storm Eye Institute Charleston, Medical University of South Carolina, 167 Ashley Ave., Charleston, SC 29425, USA. Tel: (843)-333-5241. Fax: (843)792-2262. E-mail: [email protected]
51
52 G. N. Magrath et al.
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
TABLE 1. A differential diagnosis for the acute orbit. Orbital cellulitis Subperiosteal abscess Orbital trauma, intraorbital foreign body Nonspecific orbital inflammatory syndrome Thyroid ophthalmopathy Wegener’s granulomatous Metastatic carcinoma Lymphangioma Lymphoma Ruptured dermoid cyst Extraocular extension of ocular melanoma Sarcoidosis Mucocele Fibrous histiocytoma Cavernous hemangioma Orbital varix Schwannoma Meningioma (sphenoid wing or optic nerve) Optic nerve glioma Dacryocystitis Herpes zoster
coverage for organisms found in the sinuses, and now increasingly, coverage for methicillin resistant Staphylococcus aureus is required.5 With appropriate medical and surgical management, these patients tend to do well and have stabilization and improvement in the disease process within the first few days of treatment.6 In patients with an acute orbit without concomitant sinus disease or cases which do not improve with antibiotics or surgical drainage alone, additional disease processes need to be investigated. One of the most common causes of acute orbital inflammation that does not improve with antibiotics the first few days is nonspecific orbital inflammation.3 These patients are typically treated with steroids with a quick improvement in symptomatology. However, other disease processes can also present with acute orbital inflammation like metastatic cancer and care must be taken to consider a wide differential diagnosis for patients with atypical orbital inflammation that does not respond as anticipated (Table 1). The authors present two recent cases of orbital metastases that both presented with acute inflammatory changes.The first case is an esophageal adenocarcinoma metastatic to the orbit that presented with a mass and a superolateral subperiosteal fluid collection before the primary site had been identified. The second case is a urothelial carcionoma metastatic to the apex of the orbit that presented with significant sinus mucosal thickening mimicking pansinusitis. Although esophageal and urothelial carcinomas have been previously reported to metastasize to the orbit,2,5 a review of the literature could not identify cases where these cancers presented in a similar fashion. The authors represent that this material was created in accordance with the provisions of the Health Insurance Portability and Accountability Act
(HIPAA) of 1996, and that this material does not include any protected health information as defined by HIPAA.
CASE SERIES Case 1 A 51-year-old white male presented with a 5-day history of worsening left upper eyelid pain, redness, and swelling despite a 3-day course of broad spectrum oral antibiotics. Patient also had proptosis and decreased abduction. CT scan identified a superolateral subperiosteal fluid collection without concomitant sinus disease. The fluid collection was drained in the operating room and the patient was discharged on oral antibiotics. Two weeks later he presented with recurrent swelling, proptosis, and diplopia. An MRI scan showed recurrent orbital subperiosteal fluid with a soft tissue component, a subtemporalis muscle fluid collection, and bone changes of the lateral and superior orbital walls with dural enhancement (Figure 1, see Video, Supplemental Digital Content 1, which shows full MRI image series). Both were drained in the operating room and a biopsy of the tissue within the orbit showed necrotic adenocarcinoma. A full-body PET/CT demonstrated a large esophageal mass and liver masses. Biopsy of the esophageal mass confirmed this as the primary site. The patient received orbital and esophageal radiation therapy and 2 months later presented with a small bowel obstruction. He was subsequently admitted to inpatient hospice care for palliative care.
Case 2 A 57-year-old African American female developed painless right proptosis, decreased vision, and globally decreased ocular motility 3 days after bladder resection for urothelial carcinoma. CT scan demonstrated mucosal thickening in the adjacent sinuses consistent with pan sinusitis with a soft tissue mass in the apex of the right orbit with extension through the superior orbital fissure, anteromedial middle cranial fossa, and cavernous sinus (Figure 2, see Video, Supplemental Digital Content 2, which shows full CT image series). The patient was placed on broad spectrum intravenous antibiotics. Two days after a failure to improve, the patient was taken to the operating room for an endoscopic exploration and drainage of the ethmoid, maxillary, and sphenoid sinuses and orbital biopsy. Pathology revealed metastatic urothelial carcinoma within the orbital fat. Three weeks later the patient was readmitted obtunded Orbit
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
Orbital Metastasis Masquerading as Infection
53
FIGURE 2. CT brain and orbits with and without contrast reveals ethmoid sinusitis with an ipsilateral soft tissue mass in the orbital apex extending posterior into the cavernous sinus and middle cranial fossa.
FIGURE 1. MRI brain and orbits with and without contrast shows a large superolateral fluid collection (A) with extension through the superior and lateral orbital walls into the epidural and subtemporalis space (B).
without evidence of stroke or meningitis. She was discharged to home hospice care for palliative care.
DISCUSSION Metastasis to the orbit is a well-known complication of primary malignancy. In one the largest reported series of orbital tumors, metastases accounted for 7% of the total orbital tumors in adults.4 Of these the most common primary sites were breast and prostate, accounting for 48% and 12% of the total orbital metastases, respectively. Esophageal and urothelial carcinoma metastases to the orbit have been previously reported but they occur at a much lower !
2015 Informa Healthcare USA, Inc.
frequency.7,8 In the recent large retrospective series the rate of metastasis in patients with space occupying orbital lesions was 3%.1 Although orbital metastasis from a known primary has been well reported, the typical presentation is with a mass lesion causing proptosis or an ocular motility defect.2 Orbital metastases from a known primary presenting as either acute inflammatory changes, orbital cellulitis, and/or subperiosteal abscess is much less common although previously reported.8–12 However, a literature search could find no previous cases of esophageal adenocarcinoma presenting with acute inflammatory changes with a subperiosteal fluid collection mimicking an abscess or urothelial carcinoma presenting as an inflammatory orbital apex syndrome with sinus mucosal thickening mimicking pansinusitis. Orbital metastases masquerading as orbital infection with acute inflammatory changes as the initial presentation of malignancy in an adult is rare. A literature search revealed only four prior cases of presumed orbital cellulitis or subperiosteal abscess presenting with an unknown primary malignancy, as in case 1.8,13,14 One of these cases involved an esophageal squamous cell carcinoma metastasis masquerading as an orbital abscess without concomitant sinus disease in a patient without a known history of cancer.8 The two cases in this manuscript presented as cases of acute orbital inflammation. In case 1 the patient presented with lid swelling and erythema, double vision, globe dystopia and pain. The initial CT scan showed only a subperiosteal fluid collection in the superolateral orbit without concomitant sinus disease. Subsequent magnetic resonance imaging
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
54 G. N. Magrath et al. identified bone changes with dural enhancement and subperiosteal fluid with a soft tissue component more consistent with metastatic disease. Without an external nidus for infection, the most common cause of orbital cellulitis is ipsilateral concurrent sinusitis and subperiosteal abscesses are therefore typically adjacent to the infected sinus as well.15,16 Although this case presented with aucte orbital inflammation and a subperiosteal fluid collection mimicking orbital infection, the lack of concomitant and adjacent sinus disease caused expansion of the differential diagnosis, especially after initial drainage and treatment with broad spectrum antibiotics failed to result in the anticipated improvement. It is important to consider all causes of acute orbital inflammatory changes when evaluating such patients since significant systemic disease can be the cause. In this case, the orbital inflammation was the initial presentation of a primary esophageal adenocarcinoma with metastases. Case 2 presented as an acute painless orbital inflammatory apex syndrome with concurrent, ipsilateral sinus mucosal inflammatory thickening. Although there have been several case reports of patients with orbital metastasis masquerading as orbital cellulitis with ipsilateral sinus disease, ours is the first urothelial carcinoma to present in a similar fashion.11–13 Patients with systemic malignancies may undergo surgical procedures that increase the risk of bacteremia and systemic infection and chemotherapy which may induce immunosuppression, also increasing susceptibility. This may make the differentiation between orbital cellulitis and orbital metastasis difficult. In case 2, the patient presented with acute orbital inflammatory changes in the immediate post-operative period after initial resection of the urothelial carcinoma. The lack of pain and the failure of the process to respond to broad spectrum antibiotics precipitated the decision to surgically explore the sinuses and orbit and this confirmed the diagnosis of metastatic spread to the orbit. Cancer metastatic to the orbit can lead to ophthalmic manifestations by several mechanisms. The first is a mechanical force in the orbit as the tumor expands. This can lead to proptosis, limitation of ocular motility, and compression of structures in the orbit. If metastases are also in the adjacent sinuses, tumor can obstruct the outflow system leading to acute inflammatory changes and even secondary infection. This is the likely explanation for the presentation in case 2. The second is the inflammatory microenvironment referred to as cancer related inflammation. This includes the infiltration of tumor associated macrophages and the presence of cytokines.17 This cancer related inflammation is the likely cause of the subperiosteal fluid collection, bone change, and dural enhancement in case 1.
Although orbital metastases are a rare cause of the acute orbit, it is important to exhaust a full differential diagnosis in patients who present with such changes. We have presented two cases of orbital metastases that presented with acute inflammatory changes. Case 1 is unique because it is the first time that a metastatic esophageal adenocarcinoma has presented as a subperiosteal fluid collection without a known primary. Case 2 is unique because it is the first time that metastatic urothelial carcinoma to the orbit presented as a painless apex syndrome with concurrent inflammatory sinus mucosal thickening. Clinicians must maintain a high index of suspicion when dealing with patients who present with acute orbital inflammation, especially if they are atypical or fail to respond as anticipated.
FUNDING This research is supported in part by an unrestricted grant from Research to Prevent Blindness.
DECLARATION OF INTEREST The authors do not report any conflicts of interest or financial interest in this manuscript.
REFERENCES 1. Bonavolonta` G, Strianese D, Grassi P, et al. An Analysis of 2,480 Space-Occupying Lesions of the Orbit From 1976 to 2011. Ophthal Plast Reconstruct Surg 2013;29(2):79–86. 2. Shields JA, Shields CL, Brotman HK, et al. Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture. Ophthal Plast Reconstruct Surg 2001;17(5):346–354. 3. Harris GJ. Idiopathic orbital inflammation: a pathogenetic construct and treatment strategy: The 2005 ASOPRS Foundation Lecture. Ophthal Plast Reconstr Surg 2006; 22(2):79–86. 4. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111(5): 997–1008. 5. Mathias MT, Horsley MB, Mawn LA, et al. Atypical presentations of orbital cellulitis caused by methicillinresistant Staphylococcus aureus. Ophthalmology 2012;119(6): 1238–1243. 6. Ferguson MP, McNab AA. Current treatment and outcome in orbital cellulitis. Aust N Z J Ophthalmol 1999;27(6): 375–379. 7. SooHoo JR, Gonzalez MO, Siomos VJ, Durairaj VD. Urothelial carcinoma with orbital metastasis. Urology 2012;80(4):e45–e46. 8. Lee JY, Lee HJ, Jung MS, Kim SY. Metastatic esophageal squamous cell carcinoma to the orbit and periorbit masquerading as periorbital abscess. Kor J Ophthal 2010; 24(2):123–125. 9. Barahimi B, Patel A, Bilyk JR. Orbital metastasis mimicking subperiosteal abscess. Orbit 2010;29(3):165–167. Orbit
Orbital Metastasis Masquerading as Infection
Orbit Downloaded from informahealthcare.com by Nyu Medical Center on 05/28/15 For personal use only.
10. Gonsalves SR, Lobo GJ, Mendonca N. Truth under a masquerade! BMJ Case Rept 2013;1. 11. Kumar R, Issing W. Orbital cellulitis: A rare presentation of metastatic bronchial carcinoma. Case Rept Otolaryngol 2011; 2011:397–451. 12. Qureshi F, Maddula S, Hardman-Lea S. Recurrent metastatic breast carcinoma presenting with pansinusitis and choroidal metastases and subsequent bilateral simultaneous orbital cellulitis. Semin Ophthalmol 2012;27(1–2):33–34. 13. Fyrmpas G, Televantou D, Papageorgiou V, et al. Unsuspected breast carcinoma presenting as orbital complication of rhinosinusitis. Euro Arch Oto-Rhino-Laryngol Head Neck Surg 2008;265(8):979–982.
!
2015 Informa Healthcare USA, Inc.
55
14. Fortson JK, Bezmalinovic ZL, Moseley DL. Bilateral ethmoid sinusitis with unilateral proptosis as an initial manifestation of metastatic prostate carcinoma. J Natl Med Asso 1994;86(12):945–948. 15. Robinson A, Beech T, McDermott AL, Sinha A. Investigation and management of adult periorbital and orbital cellulitis. J Laryngol Otol 2007;121(6):545–547. 16. Dewan MA, Meyer DR, Wladis EJ. Orbital cellulitis with subperiosteal abscess: demographics and management outcomes. Ophthal Plast Reconstr Surg 2011;27(5):330–332. 17. Colotta F, Allavena P, Sica A, et al. Cancer-related inflammation, the seventh hallmark of cancer: links to genetic instability. Carcinogenesis 2009;30(7):1073–1081.
