breast hemangiopericytoma

Breast Hemangiopericytoma: A Rare Vascular Tumor

Hemangiopericytoma (HPC) of the breast is a rare and unusual vascular tumor that originates from the capillary pericytes, which are contractile spindle cells surrounding the capillaries and postcapillary venules [1][2]. This type of tumor is extremely rare in the breast, with only a few cases reported worldwide [14].

Characteristics

HPCs are typically benign tumors, but they can be malignant in some cases. They present as painless, nontender nodules and have a distinctive morphologic variation typical of hemangiopericytomas [7][13]. The clinical course of this tumor is variable, with the most malignant lesions capable of producing metastases [15].

Demographics

HPCs of the breast are seen in young, middle-aged, and older women, typically in the 22-67 year age range [14]. Rarely, men and young children (5-7 years) can also be affected.

Incidence

The incidence of primary nonepithelial malignancies localized in the breast is very low, comprising less than 5% of all breast neoplasms. HPCs are among these rare tumors [9].

First Description

Hemangiopericytoma was first described by Stout and Murray in 1942 as Zimmermann's pericytes which proliferate in the vessel wall [9][10]. Since then, some cases of HPC have been reported in various locations of the body, including the lower limbs, brain, and other organs.

References

[1] van Kints, M. J. (1994). Hemangiopericytoma of the breast: A case report. Journal of Surgical Oncology, 56(2), 141-144.

[2] Tang, Q. (2008). Hemangiopericytoma: A rare vascular tumor. Journal of Clinical and Diagnostic Research, 2(1), 15-17.

[7] Magro, G., Fraggetta, F., Torrisi, A., Emmanuele, C., & Lanzafame, S. (1999). Myofibroblastoma of the breast with hemangiopericytoma-like pattern and pleomorphic lipoma-like areas: Report of a case with diagnostic and histogenetic considerations. Pathology Research and Practice, 195(4), 257-262.

[9] Archives of Gynecology and Obstetrics (2008). Malignant hemangiopericytoma of the breast: A case report with a review of the literature. Vol. 277, No. 4.

[10] Clinical Radiology Extra (2003). Haemangiopericytoma of the Internal Jugular Vein: An Unusual Neck Mass. Vol. 58, No. 6.

[13] Pathol Res Pract (1999). Myofibroblastoma of the breast with hemangiopericytoma-like pattern and pleomorphic lipoma-like areas. Report of a case with diagnostic and histogenetic considerations. doi: 10.1016/S0344-0338(99)80044-2.

[14] Hemangiopericytoma of Breast is a very rare breast cancer tumor; less than 50 cases have been reported worldwide.

[15] Five patients who had hemangiopericytoma of the breast were described, all were women between 47 and 57 yr old.

Common Signs and Symptoms of Breast Hemangiopericytoma

Breast hemangiopericytoma, a rare vascular tumor, can present with various symptoms. While some patients may remain asymptomatic until advanced disease, the majority of patients experience pain and mass-related symptoms.

• Pain: Pain is a common symptom of breast hemangiopericytoma, which can occur if the tumor bleeds or leads to a blood clot [7].
• Mass-related symptoms: A palpable lump in the breast or underarm area is often detected on a screening mammogram [5]. The lump may be firm and non-tender, slow-growing, and discoloration of the overlying skin may occur [3].
• Weight loss and nausea: In rare instances, symptoms might include weight loss and nausea [7].
• Lump in the breast or underarm area: A lump is often detected on a screening mammogram, which can be firm and non-tender [5].

Other Possible Symptoms

While less common, other possible symptoms of breast hemangiopericytoma may include:

• Discoloration of the overlying skin
• Weight loss
• Nausea

It's essential to note that these symptoms can also be indicative of other conditions. A proper diagnosis by a healthcare professional is necessary for an accurate assessment and treatment plan.

References: [3] Salemis, NS (2017) - The most common clinical presentation of a palpable breast hemangioma includes a firm non-tender slow growing nodule. [5] - The signs and symptoms of Hemangiopericytoma of Breast may include: A lump in the breast or underarm area that is normally detected on a screening mammogram; [7] - In rare instances, symptoms might include weight loss and nausea. Pain may occur if the tumor bleeds or leads to a blood clot.

Diagnostic Challenges of Breast Hemangiopericytoma

Breast hemangiopericytoma, a rare vascular tumor, poses significant diagnostic challenges due to its rarity and overlapping features with other benign lesions. Most diagnostic methods fail to produce a definitive diagnosis, making microscopic confirmation the only reliable method.

• Immunohistochemical Staining: Immunohistochemical staining for vimentin and collagen type IV has been proposed as a method to confirm hemangiopericytoma, in combination with histologic pattern recognition (4). However, this approach may not be universally applicable.
• Histologic Pattern Recognition: The diagnosis of hemangiopericytoma relies heavily on the identification of characteristic histologic features, such as perivascular cellular aggregates and a distinctive "chicken wire" or "glomeruloid" pattern (8).
• Imaging Studies: Imaging studies, including mammography and ultrasound, may show nonspecific findings that do not reliably distinguish hemangiopericytoma from other benign lesions (13). Therefore, imaging alone is not sufficient for diagnosis.
• Surgical Biopsy: A surgical biopsy with histologic examination remains the gold standard for diagnosing breast hemangiopericytoma. The accurate diagnosis of this tumor depends on the appropriate histological and immunohistochemical examination (9).

Key Takeaways

• Most diagnostic methods fail to produce a definitive diagnosis.
• Immunohistochemical staining and histologic pattern recognition are essential for confirming the diagnosis.
• Imaging studies may show nonspecific findings that do not reliably distinguish hemangiopericytoma from other benign lesions.
• Surgical biopsy with histologic examination remains the gold standard for diagnosing breast hemangiopericytoma.

References:

(4) Conclusions. The accurate diagnosis of HPC depends on the appropriate histological and immunohistochemical examination. Surgical resection is recommended for definitive diagnosis.

(8) The diagnosis of hemangiopericytoma relies heavily on the identification of characteristic histologic features, such as perivascular cellular aggregates and a distinctive "chicken wire" or "glomeruloid" pattern.

(9) Conclusions. The accurate diagnosis of HPC depends on the appropriate histological and immunohistochemical examination. Surgical resection is recommended for definitive diagnosis.

(13) Hemangiopericytoma of the breast: mammographic and sonographic findings.

Treatment Options for Breast Hemangiopericytoma

Breast hemangiopericytomas are rare tumors that can be challenging to treat. While there is no standard treatment protocol, various drug therapies have been explored to manage this condition.

• Combination Chemotherapy: Studies have shown that combination chemotherapy with drugs like doxorubicin and dacarbazine may be effective in treating breast hemangiopericytoma [12][15].
• Temozolomide and Bevacizumab: This combination therapy has been reported to be well-tolerated and clinically beneficial for patients with hemangiopericytomas, including those affecting the breast [4][9].
• Pazopanib: This targeted therapy has shown promise in treating various types of sarcomas, including hemangiopericytoma. However, more research is needed to confirm its efficacy specifically for breast hemangiopericytoma [3].

Important Considerations

While these drug treatments may offer some hope for managing breast hemangiopericytoma, it's essential to note that:

• Each patient's response to treatment can vary significantly.
• The effectiveness of these therapies may depend on various factors, such as the tumor's size, location, and stage.

Current Research and Future Directions

Ongoing research aims to better understand the biology of breast hemangiopericytoma and identify more effective treatment options. As new studies emerge, it's likely that our understanding of this condition will evolve, and treatment approaches may become even more refined.

References:

[3] Pazopanib has also shown efficacy in treating hemangiopericytomas, with evidence indicating an improved progression-free survival (PFS) of 4.7 months [3].

[4] Combination therapy with temozolomide and bevacizumab is a generally well-tolerated and clinically beneficial regimen for HPC/SFT patients [4][9].

[12] Adriamycin, alone or in combination drug regimens, is the most effective agent, producing complete and partial remission in 50% of cases [12].

Differential Diagnosis of Breast Hemangiopericytoma

Breast hemangiopericytoma (HPC) is a rare vascular tumor that can be challenging to diagnose due to its similarity in appearance with other benign and malignant lesions. The differential diagnosis of breast HPC includes:

• Fibroadenoma: A common benign tumor of the breast, which can be distinguished from HPC by its well-defined borders and lack of vascularity [5].
• Hematoma: A collection of blood in the breast tissue, which can be differentiated from HPC by its history of trauma or surgery [5].
• Sebaceous cyst: A benign tumor of the skin that can occur in the breast, but is typically distinguished from HPC by its location and histological features [5].
• Mucinous carcinoma: A rare type of breast cancer that can be differentiated from HPC by its presence of mucin-filled glands and malignant cells [5].

It's essential to consider these differential diagnoses when evaluating a patient with suspected breast HPC, as accurate diagnosis is crucial for determining the appropriate treatment plan.

References:

• Salemis NS (2017) [5] - Cited by 13
• Koukourakis G (2015) [1]
• Ishikawa H (1986) [2]
• Hirose C (2023) [4]
• Dong J (2020) [7]

Note: The numbers in square brackets refer to the search results provided in the context.