Anaplastic lymphoma kinase (ALK) positive anaplastic large cell lymphoma (ALCL) of breast in a patient without a breast implant

Abstract

Non-Hodgkin lymphoma of the breast is an uncommon entity accounting for approximately 0.5% of malignant breast neoplasms and around 3% of extranodal lymphomas. Most cases of anaplastic large cell lymphoma (ALCL) of the breast have been associated with breast implants, and a few ALCL arising de novo in patients without breast implants have been reported. We report a case of a 19-year-old female who presented with a lump in the right breast of 3 months’ duration. Examination revealed an Eastern Cooperative Oncology Group performance status of 2 and a 6×5 cm2 lump in the right breast. Lumpectomy revealed large neoplastic cells positive for CD30, EMA, CD5, and anaplastic lymphoma kinase (ALK), suggestive of anaplastic large cell lymphoma. The patient underwent lumpectomy followed by 6 cycles of anthracycline-based chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone 3 weekly. On follow up, this patient had an event-free survival of 23 months. We are reporting this case of ALCL (ALK positive) in a patient with no breast implant previously, and, hence, it is of clinical importance.

Primary Lymphomas of the Breast: A Review

“… 33 , 37 Systemic/nodal ALCL, ALK+ mostly affects the paediatric population; this is also true for most cases of PB-ALCL, ALK+, although cases arising in pregnant women have also been reported. 38 Systemic/nodal ALCL, ALK+ primarily presents within the lymph nodes, 39 whilst PB-ALCL, ALK+ usually presents as an enlarging breast lump; 40 both often occur in combination with type-B symptoms and axillary lymph node and/or extra-nodal involvement. 39 , 41 …”

“…PB-ALCL, ALK+ has variable treatment outcomes. For example, one patient was successfully treated with surgery and Cyclophosphamise, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) chemotherapy, 40 and another case demonstrated an “excellent response” with cyclophosphamide, doxorubicin and prednisone (CHP) chemotherapy in combination with brentuximab vedotin (BV), even with antrum and bone marrow involvement. 41 BV is an antibody-drug conjugate consisting of a monoclonal antibody targeting CD30 linked to the cytotoxic compound monomethyl auristatin E (MMAE).…”

Anaplastic large cell lymphoma associated with breast implants. Importance for the surgeon

“…Anaplastic large cell lymphoma (ALCL) was first described in 1985 by Stein et al 3 as a non-Hodgkin lymphoma of anaplastic cytology and Ki-1 antigen (CD30) expression. A few years later, in 2008, the World Health Organization included ALCL in the classification of lymphomas, recognizing two main varieties depending on anaplastic lymphoma kinase (ALK) expression: ALK-positive ALCL and ALK-negative ALCL 4 .…”

Linfoma anaplásico de células grandes asociado a implantes mamarios. Importancia para el cirujano

“…El linfoma anaplásico de células grandes (ALCL) fue descrito por primera vez en 1985 por Stein, et al 3 como un linfoma no Hodgkin de citología anaplásica y expresión del antígeno Ki-1 (CD30). Unos años más tarde, en 2008, la Organización Mundial de la Salud incluyó el ALCL en la clasificación de los linfomas, reconociendo dos variedades principales en función de la expresión de ALK (anaplastic lymphoma kinase): ALCL-ALK positivos y ALCL-ALK negativos 4 .…”