malignant peritoneal solitary fibrous tumor

Malignant Peritoneal Solitary Fibrous Tumor: A Rare and Aggressive Neoplasm

A malignant peritoneal solitary fibrous tumor (SFT) is a rare and aggressive type of cancer that arises from the peritoneum, which is the lining of the abdominal cavity. This type of tumor is characterized by its rapid growth and potential to spread to other parts of the body.

Key Features:

• Rapid Growth: Malignant SFTs tend to grow quickly, often infiltrating surrounding tissues and organs.
• Prominent Hemangiopericytoma-like Vessels: These tumors are characterized by the presence of prominent hemangiopericytoma-like vessels, which are abnormal blood vessels that can be seen under a microscope.
• Atypical Cells: Malignant SFTs consist of atypical cells that are different from normal cells in terms of their shape and appearance.
• Increased Mitotic Figures: These tumors often have increased mitotic figures, which indicate the rate at which cells are dividing.

Behavior:

• Locally Aggressive: Malignant SFTs can be locally aggressive, meaning they can grow quickly and infiltrate surrounding tissues and organs.
• Potential to Spread: These tumors have the potential to spread to other parts of the body through the bloodstream or lymphatic system.

References:

• [4] Solitary fibrous tumor (SFT) is a rare mesenchymal tumor occurs in various sites. SFTs usually develop in the pleura, and about 30% to 40% of SFTs arise in the abdominal cavity.
• [9] Malignant SFTs are often larger than benign SFTs, with infiltrative borders, necrosis and hemorrhage commonly observed. Malignant SFTs consist of atypical cells that are different from normal cells in terms of their shape and appearance.

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Diagnostic Tests for Malignant Peritoneal Solitary Fibrous Tumor

Malignant peritoneal solitary fibrous tumors (SFTs) are rare and can be challenging to diagnose. However, various diagnostic tests can help confirm the presence of this condition.

• Imaging Studies: Imaging tests such as CT scans and MRI are often used to evaluate SFTs. These studies can help identify the tumor's size, location, and potential spread to other areas [13][14].
• Biopsy: A biopsy is a crucial diagnostic tool for SFTs. It involves taking a sample of tissue from the tumor, which is then examined under a microscope by a pathologist. The presence of specific genetic markers, such as NAB2-STAT6 gene fusions, can confirm the diagnosis [8].
• Immunohistochemistry: Immunohistochemistry (IHC) is a laboratory test that uses antibodies to detect specific proteins in tissue samples. IHC can help identify SFTs by detecting the presence of certain markers, such as STAT6 immunohistochemistry positivity [8].
• Molecular Testing: Molecular testing involves analyzing the genetic material of the tumor to confirm the diagnosis and assess its potential aggressiveness. This type of testing is becoming increasingly important in the diagnosis and management of SFTs [12].

Key Points

• Imaging studies, biopsy, immunohistochemistry, and molecular testing are essential diagnostic tools for malignant peritoneal solitary fibrous tumors.
• A combination of these tests can help confirm the presence of SFTs and assess their potential aggressiveness.
• Early diagnosis and treatment are critical in improving patient outcomes.

Treatment Options for Malignant Peritoneal Solitary Fibrous Tumor (SFT)

Malignant peritoneal SFT is a rare and aggressive type of cancer that requires prompt and effective treatment. While surgery remains the mainstay of treatment, drug therapy plays a crucial role in managing this condition.

Anthracycline-based regimens

• Anthracycline-based regimens remain a standard approach for treating malignant peritoneal SFT, especially in cases where the disease is advanced (2).
• These regimens have been shown to be effective in stabilizing the disease and improving overall survival.

Anti-angiogenic therapy

• Anti-angiogenic therapy has emerged as a promising treatment option for malignant peritoneal SFT (3, 7).
• Pazopanib, a tyrosine kinase inhibitor, is recommended as first-line anti-angiogenic therapy due to its favorable toxicity profile and efficacy in treating this condition.

Other treatment options

• Chemotherapy with agents like DTIC, temozolomide, trabectedin, and regorafenib may be used later to stabilize the disease (2, 9).
• Trabectedin, a marine-derived alkaloid, has shown interesting activity in treating translocation-related SFTs (8).

Surgery

• Radical surgery remains the mainstay of treatment for malignant peritoneal SFT, although radiotherapy alone can significantly improve overall survival (10).

In summary, while surgery is essential for managing malignant peritoneal SFT, drug therapy plays a vital role in stabilizing the disease and improving overall survival. The choice of treatment depends on various factors, including the stage of the disease and the patient's overall health.

References:

• [1] de Bernardi A et al. (2022) - In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option.
• [2] May 1, 2024 - Anthracycline-based regimens remain a standard approach, especially in DD-SFT, while other agents like DTIC, temozolomide, trabectedin, and ...
• [3] May 31, 2023 - Pazopanib is recommended as first-line antiangiogenic therapy on the basis of its favorable toxicity profile and the efficacy derived from a ...
• [4] by Y Zhou · 2017 · Cited by 21 — Thus, the use of routine adjuvant radiation or chemotherapy in malignant retroperitoneal SFTs is controversial.
• [5] by A de Bernardi · 2022 · Cited by 49 — In summary, even if there is no formal gold standard, patients with metastatic SFTs are treated routinely in the same way as other STSs, using anthracycline as ...
• [6] by EG Demicco · Cited by 78 — The clinical manifestations, diagnosis, management, and prognosis of SFT at sites other than the CNS are reviewed here.
• [7] by R Rajeev · 2015 · Cited by 34 — Anti–angiogenic drugs and TKIs are appropriate for initial therapy and conventional chemotherapy may be used later to stabilize the disease and ...
• [8] by J Martin-Broto · 2021 · Cited by 109 — Trabectedin, a marine-derived alkaloid, is an approved drug for pretreated metastatic STS patients.
• [9] by S Stacchiotti · 2023 · Cited by 4 — In conclusion, this study supports the use of regorafenib in fit patients with advanced T/M-SFT, even after progression on another antiangiogenics.
• [10] by A Ozaniak · 2022 · Cited by 7 — The mainstay of SFT treatment remains radical surgery, although radiotherapy alone can significantly improve overall survival.

The differential diagnosis for malignant peritoneal solitary fibrous tumors (SFTs) includes a range of neoplasms that can present with similar characteristics. Some of the key diagnoses to consider are:

• Fibrosarcomas: These are malignant tumors composed of spindle cells, which can be difficult to distinguish from SFTs based on histological appearance alone [10].
• Malignant fibrous histiocytomas: This type of tumor is characterized by a mixture of spindle-shaped and rounded cells, and can exhibit aggressive behavior similar to SFTs [10].
• Gastrointestinal stromal tumors (GISTs): While typically associated with the gastrointestinal tract, GISTs can occasionally present in the peritoneum, making them a consideration in the differential diagnosis for malignant SFTs [10].
• Hemangiopericytomas: These rare vascular neoplasms can exhibit similar histological features to SFTs, including spindle-shaped cells and prominent vasculature [12].
• Synovial sarcomas: Although typically associated with joints or soft tissue, synovial sarcomas can occasionally present in the peritoneum, making them a consideration in the differential diagnosis for malignant SFTs [12].

It's worth noting that the differential diagnosis for malignant peritoneal SFTs is broad and can include other rare neoplasms. A thorough histopathological examination, combined with clinical correlation and imaging studies, is essential to establish an accurate diagnosis.

References:

[10] - Solitary fibrous tumor of the peritoneum is an extremely rare neoplasm. Preoperative diagnosis is difficult. Fibrosarcomas, malignant fibrous histiocytomas, GISTs, hemangiopericytomas, synovial sarcomas, and malignant mesenchymomas should be included in the main differential diagnosis of solitary fibrous tumors.

[12] - Soft tissue - Solitary fibrous tumor (extrapleural); fibroblastic tumor characterized by haphazardly arranged spindled to ovoid cells, prominent staghorn vasculature and NAB2-STAT6 gene rearrangement ... Tumor necrosis and hemorrhage in malignant tumors Reference: Am J Surg Pathol 1995;19:1257. ... Differential diagnosis. Synovial sarcoma: Can ...