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Mantle zone lymphoma: an immunohistologic study of skin lesions
M Bertero1, M Novelli, M T Fierro
1Clinica Dermatologica I, Università degli Studi di Torino, Italy.
Insights
Mantle zone lymphoma (MZL) can initially present as skin lesions, mimicking pseudolymphoma. Immunohistochemistry is crucial for diagnosing MZL, distinguishing it from other lymphomas and confirming its presence.
Area of Science:
- Hematology
- Oncology
- Dermatopathology
Background:
- Mantle zone lymphoma (MZL) is a B-cell neoplasm, often considered a variant of follicular lymphoma.
- It is characterized by small lymphoid cells forming mantles around benign-appearing germinal centers.
Observation:
- Four cases of MZL presented with initial cutaneous lesions.
- Histopathology revealed neoplastic small lymphocytes in the dermis and subcutaneous tissue, with wide mantles around atrophic germinal centers.
- One case showed progression from pseudolymphoma to MZL in serial biopsies.
Findings:
- Immunohistochemistry of skin lesions mirrored lymph node MZL, with neoplastic cells being CD5+, CD20+, CD22+, CD25+, CD74+, Leu-8+, HLA-DR+, IgM+, IgD+, and lambda light chain restricted.
- Neoplastic cells were CD10- and CD71-.
- Germinal center cells were polyclonal, and many CD38+, PCA-1+ plasma cells were noted.
Implications:
- Cutaneous lesions can be the sole manifestation of MZL for prolonged periods.
- Distinguishing MZL from pseudolymphoma requires integrated analysis of histology, architecture, and immunohistochemistry.
- Early and accurate diagnosis is vital for appropriate patient management.
Background:
Mantle zone lymphoma (MZL) is a B-cell proliferation regarded as the follicular variant of intermediate lymphocytic lymphoma (ILL). Neoplastic small lymphoid cells proliferate as wide mantles around atrophic centers of benign appearance.
Objective:
The clinical, histologic, and immunohistochemical features of four cases of MZL, heralded by cutaneous lesions, are described and correlated with the lymph node pattern.
Results:
All specimens showed extensive nodules in the reticular dermis invading the subcutaneous tissue. They were mainly composed of a proliferation of small lymphocytes with slightly irregular nuclear contours and clumped chromatin, forming wide mantles around small atrophic germinal centers. Serial biopsy specimens in case 1 revealed evolution of the skin lesions from pseudolymphoma into MZL. Their immunohistochemistry was similar to that of lymph nodes and showed that the neoplastic cells were CD5+, CD20+, CD22+, CD25+, CD74+, Leu-8+, HLA-DR+, IgM+, IgD+ with restriction for the lambda light chain, CD10-, and CD71-, whereas the germinal center cells were polyclonal. In three cases many CD38+, PCA-1+ plasma cells were present both in the grenz zone and in bordering neoplastic nodules. The clinical course was chronic. The only death occurred from unrelated causes; one patient is still alive 17 years after onset.
Conclusion:
Skin lesions may be the only manifestation of MZL for an extended period. The differentiation between pseudolymphoma and other lymphoma subtypes is based not only on the histologic and cytologic features but also on the architecture, followed by immunohistochemical confirmation.