Lymphoproliferative disorders (LDs) are an heterogeneous group of both malignant and benign diseases,
characterized by an abnormal proliferation of lymphoid cells,
that lead to lymphocytosis,
lymphadenopathy or involvement of extranodal sites.
The breast is an uncommon location of involvement,
one of the assumed reasons is the paucity of lymphoid tissue in the mammary gland.
Breast manifestations are usually secondary to systemic LDs,
though they may rarely present as primary neoplasms.
Chemotherapy,
radiation and immunotherapy are possible choices for local and systemic treatment.
Unlike most breast malignancies, surgery is not routinely performed.
In this poster,
we will review cases of the more common and the rarer LDs that may present with breast involvement:
LYMPHOMAS
Lymphomas are a large group of malignancies that arise from lymphoid cells and as such may develop anywhere in the body (fig 1).
They also represent the most frequent LD that may manifest in the breast,
presenting either secondary to systemic disease or more rarely as a primary neoplasm.
The majority of primary breast lymphomas are non-Hodgkin lymphomas,
most commonly the diffuse large B cell subtype,
though they only account for approximately 0,04 to 0,7 % of breast malignancies.
Other possible etiologies include Burkitt lymphoma,
follicular lymphoma and T cell lymphomas.
LEUKEMIAS
Leukemias comprise several hematopoietic neoplasms that manifest as clonal cells maturation defects,
usually with bone marrow infiltration.
Extramedullary disease is rare,
with few reported cases of breast involvement.
Acute myeloid leukemia is the most frequent subtype,
followed by chronic lymphatic leukemia and chronic myeloid leukemia.
MULTIPLE MYELOMA
Multiple myeloma is a disseminated malignant B-cell lineage neoplasm,
characterized by clonal proliferation of plasm cells in the bone marrow with an excessive production of monoclonal immunoglobulins by malignant cells (fig 3).
Involvement of the breast,
as disseminanted disease or extramedullary plasmocitoma is very rare,
usually associated with more agressive behavior,
increased resistance to treatment and shorter survival.
CASTLEMAN DISEASE
Castleman disease is a rare nonneoplastic cause of lymph node hyperplasia,
either localized (unicentric) or,
less commonly,
multicentric (fig 2).
Chest,
abdomen and neck are the most frequent sites of involvement with very few cases of localized breast disease.
Hyaline vascular is the most common subtype (90%),
followed by plasma cells (10%).
Though not considered a malignant neoplasm,
the multicentric varieties have been associated with an increased risk of developing non-Hodgkin lymphomas,
POEMS syndrome and Kaposi's sarcoma.
Unlike most other LDs in the breast,
surgery is recommended for treatment of unicentric disease.
