MedPix Medical Image Database COW - Case of the Week Case Contributor: Caitlin M Nickens Affiliation: Uniformed Services University

MedPix No: History Pt Demographics: Age = 70 y.o. Gender = Woman 70-year-old female with swollen eyelids and pain on abduction of right eye x 4 days.Remote history of breast (age 46) and ovarian (age 28) cancer.1999: Mantle cell lymphoma, periaortic lymph nodes/ileocecal valve/bone marrow: CHOP, Hyper CVAD. Autologous stem cell transplant No chemotherapy since January 2004 Downloaded by (-1)

MedPix No: EXAM & LABS External examination: Bilateral superotemporal firm, non-mobile subconjunctival masses with dilated vasculature; 3.5 mm right proptosis; full ductions, no diplopiaInternal examination: 2-3+ Nuclear sclerotic cataracts O.U. resulting in 20/40 20/50 best corrected VA; Choroidal nevus, unchanged for 15 years

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands CT scan of a patient with mantle cell lymphoma of the lacrimal glands. Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands CT scan of a patient with mantle cell lymphoma of the lacrimal glands. Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Touch preparation: H&E x Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Lacrimal gland: H&E x 25 - Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Lacrimal gland: H&E x Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Lacrimal gland: PAS x 400 Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Flow cytometry Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Flow cytometry Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Flow cytometry Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Flow cytometry Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Flow cytometry Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Cytogenetics Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands A patient with mantle cell lymphoma of the lacrimal glands. - - Mid-treatment. Downloaded by (-1)

Mantle Cell Lymphoma of the Lacrimal Glands X-Ray of a patient with mantle cell lymphoma of the lacrimal glands. - - Post-treatment (Nov 2005) Downloaded by (-1)

FINDINGS Biopsies of right orbital lobe of lacrimal gland and right subconjunctival mass.Histopathology: Monomorphous centrocyte-like small lymphocytes, Cyclin D1 + [IHC]Flow cytometry: CD5/CD20 +,CD10, CD23, FMC7 +Cytogenetics: t(11;14)(q ;q32)

DIFFERENTIAL DIAGNOSIS What is your Differential Diagnosis?

Diagnosis: Mantle Cell Lymphoma Dx Confirmed by:

DISCUSSION Recently defined category of B-cell lymphomas (3-10%). - - Clinical features: - Late adulthood (median age 60) - Male predominance - Multifocal lymphadenopathy, hepatosplenomegaly, bone marrow involvement - GI tract, Waldeyers ring also common extranodal sites - Median survival 3-5 years - - Cell of origin: mantle cell immediately surrounding follicle: - Centrocyte-like morphology - Proliferative in monotonous sheets of small cells with irregular nuclear contours, inconspicuous nucleoli - Hyalinized capillaries - - Immunophenotype: - B-cell lymphocyte - CD19+, CD20+ - Coexpression of CD5 (CD5+/CD20+) - DDx: SLL/CLL - Negative for: - CD23 (CLL/SLL CD5+,CD23+) - CD10 (FL CD5-,CD10+); (MALT CD5-,CD10-) - Cyclin D1+, FMC Cytogenetics: - t(11;14) (q13;q32) - Juxtaposition of Ig heavy chain promoter and Cyclin D1 gene - Overexpression of Cyclin D1 gene, increased mRNA, gene product - Promotes phosphorylation of pRB, passage of cell into S phase - - Ocular adnexal involvement: - Rare: eyelid, conjunctiva, orbit, lacrimal gland - 3-5% of all ocular adnexal lymphomas - Demographics - Age > 60 - M >> F - Aggressive behavior - Median survival < 5 years - Largest series: White, Rootman et al - 10 cases: - 9 with multiple periocular sites - 6 bilateral - 9 orbital, 5 lacrimal gland, 5 eyelid - 8 periocular presenting site - Unusual findings - 3 cases CD5 - 2 cases composite lymphomas - Mantle cell/follicular lymphoma - Mantle cell/plasma cell neoplasm - -