Systemic non-Hodgkin’s lymphoma involving the orbit and leptomeninges

Abstract

SummaryWe report a case of diffuse large B-cell lymphoma in a 46-year-old female presenting in an unusual manner with stage IVB disease including concurrent orbital and leptomeningeal involvement. The cytologic features and cytogenetics of the malignancy are noted, and the management and progression of the disease, with attention to orbital involvement, is recorded for a period of over 2.5 years, until the patient's death. Case ReportA 46-year-old woman presented to our clinic with a complaint of intermittent proptosis, diplopia, and ptosis in her right eye for 4 months. During this period, she also noted a loss of balance, gait difficulties, and lower extremity weakness, numbness, and tingling. For the 6 months prior to presentation, she had also experienced weight loss and fatigue.Ophthalmologic examination revealed visual acuities of 20/20 in both eyes. External examination demonstrated proptosis, soft tissue swelling in the upper and lower lids, and scleral "show" with mild conjunctival injection and chemosis in the right eye (Figure 1). Enlarged, matted, and non-tender cervical lymphadenopathy was also found. There was marked restriction of the right eye on adduction. The rest of the anterior and posterior segment examinations were unremarkable in both eyes. Neurological examination was pertinent for bilateral distal lower extremity motor and sensory deficits, diminished reflexes in the knees, and absent reflexes in the ankles. A Romberg test was positive.Computed tomography of the head showed an enhancing soft tissue mass in the medial aspect of the right orbital fossa. MRI demonstrated an enhancing right orbital mass with intraconal and extraconal involvement, which communicated with the right ethmoid sinus. An MRI of the spine showed diffuse enlargement and enhancement of the lumbar and sacral nerve roots.Biopsy of the left cervical lymph node (Figure 2) demonstrated diffuse large B-cell lymphoma (DLBCL). Diagnosis was confirmed with immunohistochemical stains that showed positive staining for B-cell markers CD19, CD20 (Figure 3) and lambda light chain restriction. Staining for CD3 and CD5 was negative, ruling out a T-cell lymphoma, small lymphocytic lymphoma/ chronic lymphocytic leukemia, and mantle cell lymphoma; negative staining for CD10 ruled out a follicular lymphoma. 1 Furthermore, the BCL-1 stain showed heavy background staining, and definitive positivity was not ascertained, while fluorescence in situ hybridization of 100 cells did not show a [t(11;14)] rearrangement-a typical hallmark of mantle-cell lymphoma. 2 The Ki-67 proliferation index was 20% to 30%. Cytogenetic stud-

Non-Hodgkin lymphoma with relapses in the lacrimal glands

“…The differential diagnosis of an orbital mass lesion is vast [ 7 ] and although the orbit is rarely a secondary site of lymphoma dissemination, patients with NHL diagnosis should be immediately investigated if ophthalmic or orbital symptoms develop [ 1 ].…”