Case # 3Slide Seminar
Los Angeles Society of Pathologists
March 13, 2007Randa Alsabeh, M.D.
Cedars-Sinai Medical Center
Clinical History
• 41 year old white male• Fevers, shaking chills, cough, shortness
of breath• Chest X-ray: innumerable bilateral
pulmonary nodules• Physical exam: right testicular mass
Differential Diagnosis
• Low grade lymphoma with transformation to large cell lymphoma
• NK/T-cell lymphoma
CD20
CD3
CD30
CD45
Bob-1
Oct-2
KAPPA
LAMBDA
Differential Diagnosis
• Low grade lymphoma with transformation to large cell lymphoma
• NK/T-cell lymphoma• T-cell rich large B-cell lymphoma with
areas of Diffuse large B-cell lymphoma!
X
X
EBER
EBER
EBV positive large B-cell lymphoma consistent with lymphomatoid granulomatosis, grade 2 and 3
Lymphoma of the Male Genital Tract
• Majority arise in the testes• Children: Acute leukemia (ALL)• Adults: Primary versus secondary spread
of systemic lymphoma• Prostate, epididymis and penis: reflect
widespread disease
Primary Testicular Lymphoma• 5% of all testicular malignancies• 7th to 8th decades, single most common primary
testicular neoplasm• Painless testicular enlargement, more bilateral
then germ cell tumors (50% of bilateral testicular neoplasms are lymphomas)
• Patients with B symptoms are younger, bilateral disease (Burkitt lymphoma)
• No association with previous trauma, inflammation, malformation or undescended testicle
Testicular Lymphoma
• Most common diffuse large B-cell lymphoma
• Low grade lymphomas, NK/T cell lymphoma, ALCL and angiotropic lymphoma
• 50% have systemic disease• Relapse occurs in CNS and contralateral
testicle
Lymphomatoid Granulomatosis (LYG)
• Angiocentric and angiodestructive lymphoproliferative disorder involving extranodal sites: lung (100%), brain (26%), kidney (32%), liver (29%) and skin (25-50%)
• Lymph nodes and spleen are rarely involved• Also known as angiocentric
immunoproliferative lesion (AIL)• Must be distinguished from extranodal NK/T-
cell lymphoma, nasal type
Lymphomatoid Granulomatosis (LYG)
• First description: unclear whether it a neoplastic or inflammatory process, 1972 by Liebow
• Initially misclassified as T-cell lymphoma• Clonal EBV sequences in B-cells
Lymphomatoid Granulomatosis (LYG)
• Adults, M/F: 2/1• Patients with immunodeficiency
disorders are at increased risk: HIV, allogeneic BMT, Wiskott-Aldrich syndrome, x-linked lymphoproliferative disorder
Lymphomatoid Granulomatosis (LYG)
• Mixed infiltrate of small lymphocytes, histiocytes, plasma cells, and variable numbers of large atypical lymphoid cells
• Angiocentric and angiodestructive infiltrates• EBV positive B-cells, CD30 positive in a
background of reactive T-cells-Grade I: EBV positive cells (
Morphology• D.D. includes benign angiocentric
lymphohistiocytic processes (mycobacterial or fungal infections,necrotizing sarcoidosis, and Wegener's granulomatosis)
• True granulomas are not usually present in lymphomatoid granulomatosis
Why is it important to make the diagnosis?
• A. Because hematopathologist love toclassify to justify their jobs
• B. Because it is clinically relevant• C. All of the above
“The urge to classify is a fundamental human instinct; like a predisposition to sin, it accompanies us into the world at birth and stays with us to the end”Hopwood A.T.Proceedings of the Linnean of London1957;171:230-234
Clinically Relevant
• Grade 1 and 2, uncertain malignant potential, waxing and waning, and may regress with interferon alfa-2b therapy and should be differentiated from T-cell rich B-cell lymphoma
• Grade 3 considered a variant large B-cell lymphoma, treated as aggressively but clinician should be alerted to the possibility of an underlying immune deficiency status
Prognosis and Management
• 27% complete remission without therapy• Majority with progressive disease with a
median survival of 14 months• CHOP 47% response with a mean
duration of 5.2 years• Interferon alfa-2b 67% response for
grade 1 and 2• Rituxan
• Our patient was treated with CHOP/Rituxan, underwent autologous stem cell transplantation and is in complete remission with complete resolution of his lung masses
Thank You