T-cell rich B-cell lymphomas (TCRBCL) are more commonly known in the lymph nodes. However, there is increasing information of these tumors primarily occurring in the skin, without lymph node involvement. They are sometimes known as Angiocentric TCRBCLs because of their marked propensity to infiltrate pre-existing blood vessels. There are too few cases to predict the clinical behavior of these rare cases occurring in the skin.
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EPIDEMIOLOGY CHARACTERIZATIONS SYNONYMS Angiocentric T-cell rich B-cell lymphoma INCIDENCE Rare <20 cases reported
<1% of all non-Hodgkin's lymphomas
AGE RANGE-MEDIAN 18-61 years SEX (M:F) About equal
PATHOGENESIS CHARACTERIZATION Clonal proliferation of B cells
Arch Dermatol 1996;132:1464
Possible that these cases may represent lymphomatoid granulomatosis without lung involvement
There has been increasing evidence that LyG is really a B cell lymphoma associated with EBV infection
CHARACTERIZATION General Usually a large nodule which may ulcerate VARIANTS
HISTOLOGICAL TYPES CHARACTERIZATION General
J Cutan Pathol 1996;23:101-108
Am J Dermatopathol 1995;17:618-624
Atypical large cells occasionally with striking angiocentric/angiodestructive pattern-may have involvement of the subcutaneous fat
Increased plasma cells, histiocytes, and multinucleated giant cells
CHARACTERIZATION Special stains Immunoperoxidase Large atypical cells positive for B cell markers-surrounded by reactive T cells, usually accounting for about <15% of lymphocytes
TARC, a CC Chemokine, Is Frequently Expressed in Classic Hodgkin's Lymphoma But Not in NLP Hodgkin's Lymphoma, T-Cell-Rich B-Cell Lymphoma, and Most Cases of Anaplastic Large Cell Lymphoma
S. C. Peh, M.B.B.S., F.R.C.Path.; L. H. Kim, B.BMed.Sc.; S. Poppema, M.D., Ph.D., F.R.C.P.
From the Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur and Department of Pathology and Laboratory Medicine, University Hospital Groningen, The Netherlands.
Am J Surg Pathol 2001;25:925-929 Abstract quote
Thymus and activation-regulated chemokine (TARC) has been identified as a lymphocyte-directed CC chemokine that attracts activated T-helper type 2 (Th2) cells in humans. Recent studies showed that the T cells surrounding Reed-Sternberg cells in Hodgkin's lymphomas (HL) are Th2 type. Anaplastic large cell lymphomas (ALCL), T-cell-rich B-cell lymphoma (TCRBCL) can mimic HL in some instances.
This study aimed to establish the pattern of TARC expression in these diseases. Immunohistochemical stain using a polyclonal goat anti-human antibody to TARC was performed on 119 cases of confirmed HL; 99 were classical type (43 mixed cellularity, 43 nodular sclerosis, 5 lymphocyte depleted, 4 lymphocyte rich, 4 unclassifiable) and 20 lymphocyte predominant HL. Additional 27 ALCL (9 T-, 18 null-cell phenotype), 16 T-cell and 8 B-cell non-Hodgkin's lymphoma (NHL) were studied.
A total of 85.8% of the classical HL, one case of ALCL, and one case of large cell B-cell lymphoma with anaplastic morphology showed positive TARC expression in the tumor cells. The expression was paranuclear and/or diffuse in the cell cytoplasm. The tumor cells in all cases of lymphocyte predominant HL, TCRBCL, null ALCL, and T-NHL did not express TARC.
The high frequency of TARC expression in the Reed-Sternberg cells of classical HL may explain the characteristic T-cell infiltrate in this disease. The absence in other types that may be morphologically similar indicates that staining for TARC may aid in differential diagnosis.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Lymphomatoid granulomatosis
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors
Mod Pathol 2001;14:10-13
Too few to adequately define
One report of 2 primary cases found both patients disease free at 4 months and 5 years after therapy
May have better prognosis compared to its nodal counterpart
Of 8 patients with primary disease, 1 died after 8 months
Remission in 5/8
Recurrence 2/8 Treatment Combination of excision, excision and radiation, or excision and chemotherapy
Complete response of a primary cutaneous T-cell-rich B cell lymphoma treated with interferon alpha2a.
Wollina U. Department of Dermatology, Friedrich Schiller University of Jena, Germany.
J Cancer Res Clin Oncol 1998;124(2):127-9 Abstract quote
Primary B cell lymphomas of the skin are rare disorders and therapy is not yet standardized. A 45-year-old male patient presented 4 years after surgical removal of a subcutaneous tumour of the leg. On examination a reddish nodular tumour was found in the same region. The biopsy revealed a T-cell-rich primary B cell lymphoma of the skin. Interferon alpha2a was given perilesionally at a dosage of 9 MU three times a week.
The treatment was well tolerated, had no severe side-effects and induced a complete remission, both clinically and histologically, during a 12-months course.
Am J Surg Pathol 1988;12:433-443
Am J Surg Pathol 1989;13:335.
Am J Surg Pathol 1990;14:933-938
Br J Dermatol 1993;129:196-200
J Cutan Pathol 2000;27:516-525.
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Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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