Medullary carcinoma of the breast | |
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Specialty | Oncology, pathology |
Medullary breast carcinoma is a rare type of breast cancer [1] that is characterized as a relatively circumscribed tumor [2] with pushing, rather than infiltrating, margins. It is histologically characterized as poorly differentiated cells with abundant cytoplasm and pleomorphic high grade vesicular nuclei. [3] It involves lymphocytic (a type of white blood cell) infiltration [4] in and around the tumor and can appear to be brown in appearance with necrosis and hemorrhage. [5] Prognosis is measured through staging but can often be treated successfully and has a better prognosis than other infiltrating breast carcinomas.
Medullary breast carcinoma is one of five types of epithelial breast cancer: ductal, lobular, medullary, colloid, and tubular.[ citation needed ] Very rare cases of it have been diagnosed in men (see male breast cancer). [6]
Ductal Carcinoma in situ (DCIS) is less commonly present, and medullary breast cancer presents as a soft, fleshy mass with a pushing border. Tumors commonly possess mutations of E-cadherin, which results in its overexpression. Strengthened adhesions between tumor cells reduce the frequency of metastasis.[ citation needed ]
It tends to occur more often in younger women and is more frequently found in those with BRCA1 gene mutations. Although this breast carcinoma is more frequently found in BRCA 1 gene mutations, most individuals with BRCA 1 gene mutations do not have medullary breast carcinoma. [7] Medullary breast carcinoma is rare and can be seen in about less than 5% of invasive breast cancers. Due to the complicated nature of classification, there are difficulties in subtyping this type of breast cancer. [5]
Criteria must be met through the Ridolfi criteria. Although there are other classifications for diagnosis, the Ridolfi criteria are the most commonly used. There must be histologic evidence of lymphoplasmacytic infiltration, noninvasive microscopic circumscription, greater than 75% syncytial growth pattern, and high-grade nuclei. [5] It is immunologically typically triple-negative, with negative estrogen receptors (ER), negative progesterone receptors (PR), and negative HER2/neu receptors. [3] There are also medullary breast carcinomas that are found to be estrogen receptors (ER) and/or progesterone receptor (PR) positive, making diagnosis less straightforward.
Ridolfi Criteria | |
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1. | Lymphoplasmacytic infiltration |
2. | Noninvasive microscopic circumscription |
3. | >75% syncytial growth pattern |
4. | High-grade nuclei |
TNM Staging is used to determine the extent of the disease and is used to guide the management and treatment of the cancer. It can be divided into Primary Tumor (T), Lymph Nodes (N), and Metastasis (M). The American Joint Committee on Cancer (AJCC) revised the staging system in 2018 to include the anatomic extent of the disease as well as prognostic biomarkers. [8]
Primary Tumor (T) | |
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T0 | No primary tumor |
Tis | Carcinoma in situ |
T1 | Tumor is ≤2 cm |
T2 | Tumor is >2 cm but ≤5 cm |
T3 | Tumor is >5 cm |
T4 | Tumor extends to chest wall or skin |
Lymph Nodes (N) | |
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N0 | No lymph node metastasis |
N1 | Metastasis to 1-3 axillary lymph nodes |
N2 | Metastasis to 4-9 axillary lymph nodes |
N3 | Metastasis to ≥10 axillary lymph nodes |
Metastasis (M) | |
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M0 | No metastasis |
M1 | Metastasis |
If breast cancer is suspected, imaging should be obtained through ultrasound, mammography, and/or MRI with appropriate biopsies.
Immunological and histological testing should also be obtained for receptor status, which influences the type of treatment required. Since medullary breast carcinoma typically presents as triple negative, it may be treated with a more intensive chemotherapy regimen as with other triple negative breast cancers. This cancer has been found to respond well to chemotherapy compared to other breast cancers. Despite this, some cases of medullary breast carcinoma do not require chemotherapy for successful treatment. Depending on immunologic status, endocrine therapy can be utilized as well. [9]
Medullary breast carcinoma has a lower propensity to metastasize compared to other types of breast cancers. Compared to infiltrating ductal carcinomas, medullary breast carcinoma has a better prognosis and a significantly higher survival rate. The best measure of prognosis is through staging and axillary lymph node involvement in the absence of metastatic disease. The higher the involvement of lymph nodes, the worse the prognosis. [9] There is also an association between higher survival rates and chemotherapy response with the presence of lymphocytic infiltration. [10]
Carcinoma is a malignancy that develops from epithelial cells. Specifically, a carcinoma is a cancer that begins in a tissue that lines the inner or outer surfaces of the body, and that arises from cells originating in the endodermal, mesodermal or ectodermal germ layer during embryogenesis.
Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.
Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different cell types including epithelial cells, germ cells, and stromal cells. When these cells become abnormal, they have the ability to divide and form tumors. These cells can also invade or spread to other parts of the body. When this process begins, there may be no or only vague symptoms. Symptoms become more noticeable as the cancer progresses. These symptoms may include bloating, vaginal bleeding, pelvic pain, abdominal swelling, constipation, and loss of appetite, among others. Common areas to which the cancer may spread include the lining of the abdomen, lymph nodes, lungs, and liver.
Inflammatory breast cancer (IBC) is one of the most aggressive types of breast cancer. It can occur in women of any age. It is referred to as "inflammatory" due to its frequent presentation with symptoms resembling a skin inflammation, such as erysipelas.
This is a list of terms related to oncology. The original source for this list was the US National Cancer Institute's public domain Dictionary of Cancer Terms.
Ovarian clear-cell carcinoma, or clear-cell carcinoma of the ovary, also called ovarian clear-cell adenocarcinoma, is one of several subtypes of ovarian carcinoma – a subtype of epithelial ovarian cancer, in contrast to non-epithelial cancers. According to research, most ovarian cancers start at the epithelial layer which is the lining of the ovary. Within this epithelial group ovarian clear-cell carcinoma makes up 5–10%.
Lumpectomy is a surgical removal of a discrete portion or "lump" of breast tissue, usually in the treatment of a malignant tumor or breast cancer. It is considered a viable breast conservation therapy, as the amount of tissue removed is limited compared to a full-breast mastectomy, and thus may have physical and emotional advantages over more disfiguring treatment. Sometimes a lumpectomy may be used to either confirm or rule out that cancer has actually been detected. A lumpectomy is usually recommended to patients whose cancer has been detected early and who do not have enlarged tumors. Although a lumpectomy is used to allow for most of the breast to remain intact, the procedure may result in adverse affects that can include sensitivity and result in scar tissue, pain, and possible disfiguration of the breast if the lump taken out is significant. According to National Comprehensive Cancer Network guidelines, lumpectomy may be performed for ductal carcinoma in situ (DCIS), invasive ductal carcinoma, or other conditions.
Invasive carcinoma of no special type, invasive breast carcinoma of no special type (IBC-NST), invasive ductal carcinoma (IDC), infiltrating ductal carcinoma (IDC) or invasive ductal carcinoma, not otherwise specified (NOS) is a disease. For international audiences this article will use "invasive carcinoma NST" because it is the preferred term of the World Health Organization (WHO).
Triple-negative breast cancer (TNBC) is any breast cancer that either lacks or shows low levels of estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) overexpression and/or gene amplification. Triple-negative is sometimes used as a surrogate term for basal-like.
Breast cancer management takes different approaches depending on physical and biological characteristics of the disease, as well as the age, over-all health and personal preferences of the patient. Treatment types can be classified into local therapy and systemic treatment. Local therapy is most efficacious in early stage breast cancer, while systemic therapy is generally justified in advanced and metastatic disease, or in diseases with specific phenotypes.
Metaplastic carcinoma, otherwise known as metaplastic carcinoma of the breast (MCB), is a heterogeneous group of cancers that exhibit varied patterns of metaplasia and differentiation along multiple cell lines. This rare and aggressive form of breast cancer is characterized as being composed of a mixed group of neoplasms containing both glandular and non-glandular patterns with epithelial and/or mesenchymal components. It accounts for fewer than 1% of all breast cancer diagnoses. It is most closely associated with invasive ductal carcinoma of no special type. (IDC), and shares similar treatment approaches. Relative to IDC, MCB generally has higher histological grade and larger tumor size at time of diagnosis, with a lower incidence of axillary lymph node involvement. MCB tumors are typically estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor-2 (HER-2) negative, meaning hormone therapy is generally not an effective treatment option, which correlates to a relatively poor prognosis. MCB was first recognized as a distinct pathological entity in 2000 by the World Health Organization.
Medullary thyroid cancer is a form of thyroid carcinoma which originates from the parafollicular cells, which produce the hormone calcitonin. Medullary tumors are the third most common of all thyroid cancers and together make up about 3% of all thyroid cancer cases. MTC was first characterized in 1959.
Lobular carcinoma in situ (LCIS) is an incidental microscopic finding with characteristic cellular morphology and multifocal tissue patterns. The condition is a laboratory diagnosis and refers to unusual cells in the lobules of the breast. The lobules and acini of the terminal duct-lobular unit (TDLU), the basic functional unit of the breast, may become distorted and undergo expansion due to the abnormal proliferation of cells comprising the structure. These changes represent a spectrum of atypical epithelial lesions that are broadly referred to as lobular neoplasia (LN).
Breast cancer classification divides breast cancer into categories according to different schemes criteria and serving a different purpose. The major categories are the histopathological type, the grade of the tumor, the stage of the tumor, and the expression of proteins and genes. As knowledge of cancer cell biology develops these classifications are updated.
Male breast cancer (MBC) is a cancer in males that originates in their breasts. Males account for less than 1% of new breast cancers with about 20,000 new cases being diagnosed worldwide every year. Its incidence rates in males vs. females are, respectively, 0.4 and 66.7 per 100,000 person-years. The worldwide incidences of male as well as female breast cancers have been increasing over the last few decades. Currently, one of every 800 men are estimated to develop this cancer during their lifetimes.
Carcinoma of the tonsil is a type of squamous cell carcinoma. The tonsil is the most common site of squamous cell carcinoma in the oropharynx. It comprises 23.1% of all malignancies of the oropharynx. The tumors frequently present at advanced stages, and around 70% of patients present with metastasis to the cervical lymph nodes. . The most reported complaints include sore throat, otalgia or dysphagia. Some patients may complain of feeling the presence of a lump in the throat. Approximately 20% patients present with a node in the neck as the only symptom.
Mammary secretory carcinoma (MSC), also termed secretory carcinoma of the breast, is a rare form of the breast cancers. MSC usually affects women but in a significant percentage of cases also occurs in men and children. Indeed, McDvitt and Stewart first described MSC in 1966 and termed it juvenile breast carcinoma because an increased number of cases were at that time diagnosed in juvenile females. MSC is the most common form of breast cancer in children, representing 80% of childhood breast cancers, although it accounts for less than 0.15% of all breast cancers.
Papillary carcinomas of the breast (PCB), also termed malignant papillary carcinomas of the breast, are rare forms of the breast cancers. The World Health Organization (2019) classified papillary neoplasms of the breast into 5 types: intraductal papilloma, papillary ductal carcinoma in situ (PDCIS), encapsulated papillary carcinoma (EPC), solid-papillary carcinoma (SPC), and invasive papillary carcinoma (IPC). The latter four carcinomas are considered here; intraductal papilloma is a benign neoplasm. The World Health Organization regarded solid papillary carcinoma as having two subtypes: in situ and invasive SPC.
Invasive cribriform carcinoma of the breast (ICCB), also termed invasive cribriform carcinoma, is a rare type of breast cancer that accounts for 0.3% to 0.6% of all carcinomas in the breast. It originates in a lactiferous duct as opposed to the lobules that form the alveoli in the breasts' mammary glands. ICCB was first described by Dixon and colleagues in 1983 as a tumor that on microscopic histopathological inspection had a cribriform pattern, i.e. a tissue pattern consisting of numerous "Swiss cheese"-like open spaces and/or sieve-like small holes. The latest edition (2019) of the World Health Organization (2019) termed these lesions invasive cribriform carcinomas indicating that by definition they must have a component that invades out of their ducts of origin into adjacent tissues. In situ ductal cancers that have a cribriform histopathology are regarded as belonging to the group of ductal carcinoma in situ tumors.
Pure apocrine carcinoma of the breast (PACB) is a rare carcinoma derived from the epithelial cells in the lactiferous ducts of the mammary gland. The mammary gland is an apocrine gland. Its lactiferous ducts have two layers of epithelial cells, a luminal layer which faces the duct's lumen and a basal layer which lies beneath the luminal layer. There are at least 4 subtypes of epithelial cells in these ducts: luminal progenitor cells and luminal mature cells which reside in the luminal layer and mammary stem cells and basal cells which reside in the basal layer. Examination of the genes expressed in PACB cancer cells indicate that most of these tumors consist of cells derived from luminal cells but a minority of these tumors consist of cells derived from basal cells.
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: CS1 maint: others (link) This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.