T-Cell-Rich B-Cell Lymphoma of the Skin

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Background

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.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/
Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/
Immunohistochemistry/
Electron Microscopy

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

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

GROSS APPEARANCE/
CLINICAL VARIANTS 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

SPECIAL STAINS/
IMMUNOPEROXIDASE
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

Survival
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.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
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.

Commonly Used Terms

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Commonly Used Terms
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Special Stains
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Last Updated 1/5/2004

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Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

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

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General	Usually a large nodule which may ulcerate
VARIANTS

SPECIAL STAINS/ IMMUNOPEROXIDASE	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
Survival	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.