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Primary Breast Lymphoma - An Overview

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Lymphomas of the breast is a condition caused due to the uncontrolled division of lymphocytes. Read more to know about the disease and its cure.

Medically reviewed by

Dr. Rajesh Gulati

Published At October 11, 2023
Reviewed AtOctober 11, 2023

Introduction

Primary breast lymphomas are uncommon conditions occurring in older women. It can also affect other age groups as well. It is usually seen as elastic, firm, well-defined, and multiple tumors. Lymphomas of the breast occur depending on the type of cell it affects. A primary lymphoma arises from a local site, whereas a secondary lymphoma arises from a secondary or distant body location. Primary lymphomas have a better prognosis than secondary lymphomas.

What Is a Primary Breast Lymphoma?

These are breast cancers affecting the lymphatic system. It usually presents as a painless lump in the outer quadrant of the breast. Sometimes, it can also be seen as a large mass at the neck region. It is rarely seen to be calcified. There are mainly two types of lymphoma in the breast.

  • Hodgkin’s Lymphoma: It affects a specific abnormal cell known as the Reed-Sternberg cell. These giant cells are seen as swelling of lymph nodes in the neck, under arms, groin, or stomach. Patients might experience fever, night sweats, weight loss, itchy skin, and generalized fatigue. It is seen in twenty to thirty-four age groups. This rare form of cancer accounts for five percent of all cancers. About eighty-six percent of patients diagnosed with this condition showed survival rates above five years.

  • Non-Hodgkin’s Lymphoma: It is developed from B or T cells. This is similar to Hodgkin’s lymphoma. In addition, the patient may show signs of skin rashes and pain in the chest, abdomen, or bones. It occurs in older patients above fifty-five years old. It is the seventh most diagnosed cancer. The survival rate is above five years.

What Are the Risk Factors Associated With Primary Breast Lymphomas?

There are no specific risk factors associated with breast lymphomas. However, it is known to be associated with the following:

  • Autoimmune disorders.

  • Older individuals.

  • Immunocompromised patients.

Other key factors which can cause risk are:

  • Family history of immune disease.

  • Smoking.

  • Exposure to radiation and industrial chemicals.

  • Chemotherapy.

  • X-rays or CT (computed tomography) exposure.

  • Professionals like radiologists, astronauts, pilots, and nuclear power plant workers.

What Is Breast Implant-Associated Lymphoma?

Studies have shown the link between breast implants and their association with developing breast cancers. The first ever case was reported in 1997. This is also known as breast implant-associated anaplastic large cell lymphoma (BIA-ALCL). Most breast lymphomas are developed from immune cells like B-cells or T-cells. But in BIA-ALCL, lymphomas arise from a T-cell. T-cell lymphomas are of three types:

  • Peripheral T-cell lymphomas.

  • Anaplastic large cell lymphomas.

  • T-cell leukemia or lymphomas.

However, breast implant lymphomas are anaplastic large cell-associated lymphomas. The anaplastic cell lymphoma is again classified into subtypes, such as:

  • Primary systemic ALCL, ALK (+) type.

  • Primary systemic ALCL, ALK (-) type.

  • Primary cutaneous ALCL.

  • Breast implant-associated with ALCL.

What Are the Causes of Breast Implant-Associated Lymphomas?

Most breast implant lymphomas develop after seven to ten years of implant fixation. The textured-surface implants are the most widely used implants in the market. About ninety-one percent of patients developed this condition using Allergan BIOCELL-manufactured textured breast implants compared to the other brands. These lymphomas arise from the boundaries of the implants, which are a fluid medium. About sixty percent of such cases complain of swelling and pain around the breast tissue. And twenty percent show mass and the other twenty percent of cases exhibit mass with an effusion. Such lymphomas are invasive in nature and have a tendency to spread to nearby lymph nodes. Not all enlarged lymph nodes are cancerous. It can also be due to an inflammatory reaction.

What Diagnostic Tests Are Conducted to Confirm the Condition?

Diagnosis of the condition is first made by a physical examination. Depending upon the history of illness and symptoms, doctors might conduct the following tests:

  • Ultrasound: This test evaluates the liquid around the breast implant.

  • Ultrasound Guided Fine Needle Aspiration Cytology (USG Guided FNAC): This is a biopsy test done with ultrasound assistance. The sample is collected and studied for the nature of cells.

  • Cell Block: This test is done by obtaining the centrifuged blood samples, adding more plasma, and preserving it for future studies.

  • Blood Tests: Blood tests like the HER2 protein gene, CA 125, and other tests are done to check for proteins in cancer cells. An overall complete blood count (CBC) is also evaluated.

  • Flow Cytometry: This is done to check for the presence of anaplastic lymphoma kinase (ALK).

  • CD30: The breast implant-associated lymphomas have ALK (-) and CD30 (+). If ALK is positive, it is not indicative of breast implant-associated lymphomas.

  • Magnetic Resonance Imaging (MRI): This is a more sensitive test to capture a better image of the soft tissues.

  • Positron Emission Tomography (PET) or Computed Tomography (CT): If the images obtained through ultrasound radiography are not clear enough, then these tests are conducted. This is mostly done to evaluate the staging of cancer. If the lymphoma is confined within the breast tissue involving eighty-five percent, then it is stage IE; if the lymphomas have spread to the local lymph nodes involving ten percent, then it is stage IIE; and if the lymphoma has spread to the distant organ or metastasized, then it is known to be stage IV. Most of the patients have stage one, two, or four cancers.

  • Mammogram: This is a less sensitive test than ultrasound and is done to detect effusion or a mass.

How Can Breast Lymphomas Be Treated?

The treatment includes the following procedures:

  • Surgery: A complete removal of the implant, the fibrous capsule, and any mass formed is done through surgery. A lumpectomy or mastectomy can remove the infected lump or breast tissue.

  • Chemotherapy: If the lymphoma is curable through surgical resection, there is no need for chemotherapy or radiation therapy. If the surgical resection is unsuccessful, chemotherapy (EPOCH or CHOP) or anti-CD30 antibody-drug conjugate (Brentuximab-vedotin; Adcetris) is given. Chemotherapy uses a combination of drugs and has many side effects, like infertility.

  • Radiation Therapy: This is done for advanced stages of cancer where the condition is beyond surgical resection. Radiation energy is used to destroy the DNA of cancer cells. The complications include damaging the normal growing cells.

After completion of treatment, regular check-ups and ultrasounds are done every six months for two years. Patients with silicone implants should have an MRI three years after placing the implant to check for leakage, then every two years after that. However, the prognosis is good, and the patients can survive an average of ten years. This also depends on age, as young individuals have a better recovery rate when compared to older individuals. A tumor occurring for the first time has a better prognosis when compared to recurring tumors. Treatment also depends on the response of the tumor growth resolving.

Conclusion:

The treatment for primary breast lymphoma depends on the overall health, age, and stage of cancer. A routine examination of breasts, swelling of lymph nodes in the axillary or armpit areas, and follow-up with the doctors should be done. The patient can take other supportive treatments like steroids, blood transfusions, anti-nausea medications, and antibiotics. Since lymphoma affects the immune system, patients are strictly kept isolated for better recovery. Altogether, lymphomas are slow-growing malignancies with a generally good prognosis.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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