Hemangiopericytoma of the breast
Hemangiopericytoma is a rare, soft tissue tumor which has in extremely rare instances been known to develop within the breast.
It is a soft tissue tumor which arises in the ‘pericytes‘ surrounding the walls of blood vessels. It that sense a hemangiopericytoma can be thought of as a ‘vascular‘ tumor, occurring within blood vessels of the breast. A hemangiopericytoma is is characterized by the proliferation of pericytes above the basement membrane of a blood vessel or organ lining. These lesions have been found in various locations in the body, and are considered to be mostly benign. Breast hemangiopericytomas occur almost exclusively in women. The age of breast hemangiopericytoma development is quite variable, ranging from the early twenties right up to late 60’s and later.
Hemangiopericytomas of the breast are so rare, there is still controversy as to whether or not they constitute a distinct category of breast tumor. They are difficult to evaluate and most diagnostic methods fail to produce a definitive diagnose. Pathologists will tend to classify this breast lesion as an ‘anaplastic mesenchymal neoplasm‘, and the kinds of neoplastic cells associated with hemangiopericytomas tend to lack specific ‘markers‘ for easy identification.
Just letting you know that this page is a little bit outdated, but still fairly useful. However, I have created a newer and more up-to-date version of this page involving Breast Hemangiopericytoma.
Pericytes or Rouget cells
A pericyte or “Rouget” cell is a connective tissue cell that usually occurs around blood vessels. They are also sometimes called adventitial cells, or mural cells. Pericytes are relatively ‘immature‘ or undifferentiated cells, that can also form into fibroblasts, smooth muscle cells, or macrophages. They are thought to be important in the proper regulation of blood flow at the capillary level, and they frequently express smooth muscle actin (SMA) and desmin proteins.
Is a hemangiopericytoma a type of breast cancer, or associated with increased breast cancer risk?
Hemangiopericytoma is a neoplastic cell growth, but the cells involved are not the bizarre, genetically mutated cells that are generally associated with cancer. When hemangiopericytoma occurs in the breast, the only real issue is that it not be misdiagnosed as cystosarcoma phyllodes (phylloides’ tumor). However, hemangiopericytomas have been known to grow aggressively in very rare cases to metastize to distant body sites. Hemangiopericytoma does not bring any increased risk for breast cancer development either at the same site or in other areas of the breast, but it is nonetheless an abnormal growth, and it should be treated (surgically removed).
How is Breast Hemangiopericytoma discovered?
The clinical presentation of breast hemangiopericytoma can vary. One typical feature of hemangiopericytoma of the breast is that it is a very slow growing tumor. Where a palpable lump is discovered, they tend to be painless, firm and not attached to the skin. Breast hemangiopericytomas tend to be round, well-circumscribed, and often of a pinky-red color. The size of the tumors can be quite variable, ranging from about 1.5 cm all the way up to 19 cm in some cases. Hemangiopericytomas generally do not contain microcalcifications. On the whole, their mammographic profile might be described as ‘non-specific‘.
Breast hemangiopericytomas tend to be round to oval in shape, somewhat radiopaque (somewhat dense, blocking light) with smooth, well-circumscribed borders. However, in some cases the borders might be less well-defined. Ultrasound imaging of breast hemangiopericytomas will tend to show a well-circumscribed, hypoechoic solid mass, usually with homogeneous distribution of internal echoes and posterior enhancement. Sometimes a mammogram will be able to identify large veins surrounding the lesion, which is a useful clue as to the ‘vascular‘ nature of the tumor.
Histological features characteristic of hemangiopericytoma of the breast
Microscopic aspects of hemangiopericytomas can often be enhanced by reticulin staining. Typical histological features of breast hemangiopericytoma include multiple layers of spindle-shaped neoplastic cells surrounding thin-walled blood vessels. Normal endothelial cells tend to line these blood vessels, which often appear in ‘stag-horn‘ shapes. Tumor cells grow outside the capillaries and preserve the basement membrane. Sometimes an electron microscope is utilized to help diagnose breast hemangiopericytoma, which might then reveal myofibrils, basal laminae, pinocytic granules, elongated cytoplasmic processes, and sparse
Histological features suggesting a more malignant presentation of breast hemangiopericytoma
Hemangiopericytomas, including breast hemangiopericytomas, are generally thought to be benign, but certain histological features tend to be associated with a more aggressive, potentially metastasizing form of the disease. These may include: increased cellular density, cytological pleomorphism (such as multiple irregular vascular luminal formations), focal calcification, hemorrhage, necrosis, an increased number of mitoses, and perivascular lamellar growth of atypical mesenchymal cells with vimentin-positive cytoplasm. However, it is generally agreed that hemangiopericytoma develops as a primary breast lesion (and not as a metastasis of a tumor somewhere else) it will almost always behave as a benign tumor, irrespective of its histological features.
Treatments for hemangiopericytoma of the breast
Breast hemangiopericytomas are usually treated by a wide local excision, and careful clinical follow-up. Generally speaking, if the lesion is larger, there is a higher probability of potential malignancy, in which case mastectomy might be recommended.
Lymph node dissection is generally not felt to be necessary. Attempts have been made at incorporating chemotherapy and radiotherapy, with inconclusive results. The recurrence rate of breast hemangiopericytoma is estimated at between 25% and 50%, but there are no conclusive reports of morbidity directly associated with the disease.
Here are a few very helpful Q&A…
- What are the hemangiopericytoma symptoms? It depends on its location in the body. These may include sweating, a severe cough, persistent chest pain, headaches, and difficulty breathing. However, most of these tumors are painless masses, often without any associated symptoms.
- What are the hemangiopericytoma treatments? Someone on the internet wrote “Minimally invasive therapy, radiotherapy, radiation therapy and chemotherapy”, but I think that’s only for brain tumors. For the breast, start with surgical removal alone, and wait to see if it recurs, would be my suggestion.
- What are hemangiopericytoma diagnosis? A biopsy can provide a definite diagnosis, and imaging scans such as MRI or CT scan are sometimes used.
- What about the hemangiopericytoma prognosis? The internet says, a patient’s prognosis varies depending on the stage, location, and size of the tumor. I simply say, excellent prognosis.
- What is the hemangiopericytoma survival rate? If you read the internet, you might stumble accidentally into writings about when the tumor is in the brain, cerebellum or brainstem. Those are completely different. Ignore that. The breast hemangiopericytoma has an excellent prognosis.
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