Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report

Abe, Ricardo Y.; Pinto, Roberto Damian Pacheco; Bonfitto, João Felipe Leite; Lira, Rodrigo Pessoa Cavalcanti; Arieta, Carlos Eduardo Leite

doi:10.1590/s0004-27492012000600013

Cited by 10 publications

(13 citation statements)

References 10 publications

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“…It commonly presents in adult men, especially in Asia and Central America, but there are infrequent reports of cases in Europe and the USA [17]. Seventy to ninety percent of ENKTCLs originate in the nasopharynx, although disseminated and extranasal disease also occurs [2, 3, 12, 18], as it did in our patient. This malignancy is characterized by an aggressive angiodestructive growth pattern and tumor necrosis [18] as well as a pathogenic link to EBV [19-22].…”

Section: Discussionsupporting

confidence: 57%

“…In one published case of nasal ENKTCL, vitritis improved following intrathecal chemotherapy and intravitreal methotrexate; however, there were no uveoretinal lesions noted on the dilated fun dus examination [15]. Another patient with intraocular ENKTCL was treated with methylprednisolone for presumed optic neuritis due to optic nerve edema; autopsy subsequently revealed posterior optic nerve invasion by the tumor [3]. Cimino et al [4] reported a patient with nasal type ENKTCL who developed vitritis and retinochoroidal lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Like other non-Hodgkin lymphomas, it can masquerade as an intraocular inflammatory or infectious process [3], which can lead to delayed diagnosis and vision loss. The prognosis for ENKTCL is substantially worse than that of other T-cell or B-cell lymphomas [4], especially if patients present late or if there is orbital or ocular adnexal involvement [2, 5].…”

Section: Introductionmentioning

confidence: 99%

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Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

McCall

Proia

2018

Ocul Oncol Pathol

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Purpose: To report a case of extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type metastatic to the space beneath the retinal pigment epithelium (RPE) with coincident paraneoplastic lymphoma-associated retinopathy. Methods: Findings of clinical and histopathologic examination are presented with differential diagnoses and a literature review. Case Report: A 53-year-old man presented with bilateral blindness and had exudative retinal detachments overlying subretinal masses in both eyes. Flow cytometry of pericardial fluid revealed malignant T lymphocytes. After two cycles of chemotherapy, the patient was hospitalized and quickly expired. Autopsy revealed lymphoma involving the eyes, heart, right lung, and two subcarinal lymph nodes focally. Histopathologic examination of the eyes revealed intraocular metastases from ENKTCL, nasal type. Expression of CD3 and CD56, along with expression of Epstein-Barr virus by in situ hybridization, confirmed the diagnosis. Lymphomatous infiltrates were confined to the space beneath the neurosensory retina and between the RPE and the Bruch membrane, sparing the uveal tissue, similar to other metastatic T-cell lymphomas. Extensive RPE and photoreceptor loss in regions with and without underlying tumor was typical of a concurrent paraneoplastic lymphoma-associated retinopathy. Conclusion: Patients diagnosed with ENKTCL should be evaluated by an ophthalmologist, as ophthalmic involvement portends a poor prognosis.

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Section: Discussionsupporting

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

McCall

Proia

2018

Ocul Oncol Pathol

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“…NKTL especially that involves the ocular is generally a rapidly progressing disease, with short survival times from diagnosis despite standard therapy [10–13]. Persistent EBV infection of T or NK cells defines a distinct disease entity.…”

Section: Discussionmentioning

confidence: 99%

Ocular manifestations as first signs of systemic T cell lymphoma in two cases

et al. 2017

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BackgroundIntraocular involvement of systemic T-cell lymphomas are uncommon and have been broadly regarded as markers of poor prognosis. We reported two cases of uveitis patients finally diagnosed as systemic T cell lymphoma.Case presentationCase one is a 19-year-old female presented with fever and liver dysfunction, and was diagnosed as EBV-associated chronic active hepatitis. Fourteen months later, she suffered from recurrent granulomatous anterior uveitis in both eyes, which failed to respond to steroid and immunosuppressant therapy. A mass on the left side of pharynx was found and the final diagnosis was pharynx T cell non-Hodgkin’s lymphoma. After 13 cycles of chemotherapy, her systematic symptoms and uveitis relieved a lot, and eye condition is stable after cataract surgery. Case two is a 37-year-old male complaining bilateral blurred vision and recurrent abdominal pain. Panuveitis was diagnosed and anterior inflammation did not release after topical steroid. During the following days, the patient complained intermittent abdominal pain and fever, with rapidly progressive bilateral visual decrease. Final diagnosis was gallbladder type II enteropathy-associated T-cell lymphoma. The patient died of multiple organ failure 4 days after operation that was only 26 days after presenting to our hospital.ConclusionsOcular manifestations as first signs of systemic T cell lymphoma were rare. Diagnosis of lymphoma has to be suspected when patients have systemic manifestations including fever, fatigue, abdominal pain, EBV-associated liver disease, et al., and uveitis fails to respond to steroid therapy.

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“…A recent review that included 24 patients with primary nasal or nasopharyngeal NK/T-cell lymphoma identifi ed six patients (25% of the study population) who suffered vision-threatening complications that stemmed from uveitis/vitritis and/or orbital infl ammation (5). Intraocular and orbital presentations of the malignancy have been confused with a range of diagnoses, including orbital cellulitis (9, 10), nonspecifi c orbital infl ammation (6), conjunctivitis, anterior uveitis, and optic neuritis (11).…”

Section: Natural Killer/t-cell Lymphoma Invading the Orbit And Globementioning

confidence: 99%

Natural Killer/T-Cell Lymphoma Invading the Orbit and Globe

Lyons¹,

Vrcek²,

Somogyi³

et al. 2017

Baylor University Medical Center Proceedings

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Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report

Cited by 10 publications

References 10 publications

Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

Ocular manifestations as first signs of systemic T cell lymphoma in two cases

Natural Killer/T-Cell Lymphoma Invading the Orbit and Globe

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