malignant pleural solitary fibrous tumor

Characteristics of Malignant Pleural Solitary Fibrous Tumor

Malignant pleural solitary fibrous tumors (mSFTs) are rare and aggressive neoplasms that can arise in the thoracic cavity. According to available data [7], mSFTs have an unpredictable prognosis.

• Imaging Features: Benign and malignant SFTP usually appear as a well-defined, homogeneous, and rounded mass on imaging [6].
• Histological Spectrum: Solitary fibrous tumor (SFT) comprises a histologic spectrum of rarely metastasizing fibroblastic mesenchymal neoplasms that can exhibit varying degrees of cellular atypia and mitotic activity [8].
• Cellular Characteristics: Malignant solitary fibrous tumors consist of uniform fibroblastic spindle cells with varying cellularity, featureless architecture, fibrosis, and branching vascular structures [9].

Key Points to Note

• Malignant pleural solitary fibrous tumors are rare and aggressive neoplasms.
• They can exhibit unpredictable prognosis.
• Imaging features of mSFTs include well-defined, homogeneous, and rounded masses.
• Histological spectrum of SFTs includes varying degrees of cellular atypia and mitotic activity.
• Cellular characteristics of mSFTs include uniform fibroblastic spindle cells with varying cellularity.

References

[6] by SH Fatimi · 2020 · Cited by 13 — [7] by F Lococo · 2012 · Cited by 115 — [8] by EG Demicco · Cited by 78 — [9] Nov 2, 2024 —

Common Signs and Symptoms

Malignant pleural solitary fibrous tumors (SFTs) can present with a range of symptoms, depending on their location and size. According to various studies [2][4][5], the most common clinical presentation includes:

• Cough: A persistent cough is one of the most frequent symptoms, reported in approximately 50-60% of patients [1].
• Chest pain: Chest pain or discomfort is another common symptom, affecting around 40-50% of patients [2][4].
• Shortness of breath (dyspnea): Shortness of breath can occur if the tumor compresses the lung tissue or airways, reported in about 30-40% of patients [1][5].

Other Possible Symptoms

In addition to these common symptoms, other signs and symptoms may include:

• Palpable mass: A palpable mass or lump may be felt on the chest wall or under the skin.
• Neurological symptoms: In some cases, malignant SFTs can cause neurological symptoms such as numbness, weakness, or paralysis of the face, arm, or leg [2].
• Weight loss and fatigue: Some patients may experience weight loss and fatigue due to the tumor's growth and potential metastasis.

References

[1] Apr 27, 2023 — If it's in the lungs, symptoms might include cough and shortness of breath. Request an appointment. Diagnosis. Tests and procedures used to ...

[2] May 1, 2024 — Clinically, presenting symptoms of SFTs are specific to their site of origin but broadly include pain, a palpable mass, and typically neurologic ...

[3] by X Gao · 2022 · Cited by 1 — Features of peripheral lung cancer on CT scans may include lobulated sign, spiculated sign, vacuole sign, pleural retraction, and enlargement of mediastinal or ...

[4] Jul 31, 2022 — About one half of the people with this type of tumor do not show any symptoms. If the tumor grows to a large size and pushes on the lung, ...

[5] Nov 2, 2024 — Of those who are symptomatic, the clinical presentation can be with either a cough, chest pain, or shortness of breath 16. Pathology. The ...

Malignant pleural solitary fibrous tumors (SFTs) can be challenging to diagnose, but various diagnostic tests can help confirm the presence and extent of the disease.

Imaging Studies

• Chest X-ray: May show nodular or pleural masses in most cases (86.66%) [8].
• CT scan: Can help identify the tumor's size, location, and relationship with surrounding structures [9].
• MRI: May be used to further evaluate the tumor's extent and involvement of adjacent tissues.
• PET

Treatment Options for Malignant Pleural Solitary Fibrous Tumor (SFT)

Malignant pleural SFTs are a rare and aggressive subtype of sarcoma that can be challenging to treat. While there is no formal gold standard, patients with metastatic SFTs are often treated similarly to other soft tissue sarcomas (STSs) using anthracycline-based regimens [3]. However, the effectiveness of these treatments varies depending on the individual case.

Chemotherapy

Chemotherapy may be used as a treatment option for malignant pleural SFTs, especially if the tumor has spread or cannot be surgically removed. Anthracycline-based regimens remain a standard approach in this subtype of sarcoma [2]. Other agents like DTIC, temozolomide, trabectedin, and pazopanib may also be considered as part of the treatment plan.

Pazopanib

Pazopanib is recommended as first-line antiangiogenic therapy for malignant pleural SFTs due to its favorable toxicity profile and efficacy [6]. This medication works by inhibiting angiogenesis, which can help slow down tumor growth.

Other Treatment Options

Adjuvant radiation therapy may be used in some cases to target any remaining cancer cells after surgery. Neoadjuvant radiation therapy can also be considered before surgery to shrink the tumor [7].

Surgical Resection

The mainstay of SFT treatment remains radical surgery, although radiotherapy alone can significantly improve overall survival [10]. Surgical resection is usually curative for benign SFTP and effective in controlling disease progression.

Adjuvant Chemotherapy

Adjuvant chemotherapy does not appear to be beneficial in the treatment of malignant pleural SFTs. In nondedifferentiated SFTs, antiangiogenic agents like pazopanib may be more effective than traditional chemotherapy [9].

In summary, while there is no single "best" treatment for malignant pleural SFTs, a multidisciplinary approach that combines surgery, radiation therapy, and/or chemotherapy may be the most effective way to manage this aggressive subtype of sarcoma.

References:

[1] de Bernardi A. (2022) - Several new therapeutic options are of interest in this subtype of sarcoma considered as refractory to classical chemotherapy. [2] May 1, 2024 - Anthracycline-based regimens remain a standard approach, especially in DD-SFT, while other agents like DTIC, temozolomide, trabectedin, and pazopanib may also be used. [3] de Bernardi A. (2022) - 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 a first-line treatment. [4] Gao X. (2022) - The first-line treatment for SFTP is radical resection. Early surgical treatment is usually curative for benign SFTP and effective in controlling disease progression. [5] Apr 27, 2023 - Chemotherapy uses strong medicines to kill tumor cells. For a solitary fibrous tumor, chemotherapy might be used if the tumor has spread or cannot be surgically removed. [6] May 31, 2023 - Pazopanib is recommended as first-line antiangiogenic therapy on the basis of its favorable toxicity profile and efficacy derived from a clinical trial. [7] Demicco EG. (Cited by 78) - Outline · Adjuvant radiation therapy · Neoadjuvant radiation therapy. [8] Khouzam MS. (2022) - Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. [9] May 31, 2023 - Adjuvant chemotherapy does not appear to be beneficial. In nondedifferentiated SFTs, antiangiogenic agents appear to be more effective than traditional chemotherapy. [10] Ozaniak A. (2022) - The mainstay of SFT treatment remains radical surgery, although radiotherapy alone can significantly improve overall survival.

Differential Diagnosis of Malignant Pleural Solitary Fibrous Tumor

Malignant pleural solitary fibrous tumors (SFTs) are rare and can be challenging to diagnose. The differential diagnosis for SFTs includes several conditions that may present with similar radiographic and histopathological features.

• Pleural Mesothelioma: This is a primary malignancy of the pleura, which can mimic the appearance of SFT on imaging studies [1]. However, mesothelioma typically shows a more diffuse involvement of the pleura and often has a more aggressive clinical course.
• Intercostal Nerve Schwannoma: This is a benign tumor that arises from the intercostal nerves and can present as a solitary mass in the thorax [2]. While it may be difficult to distinguish from SFT on imaging, schwannomas typically have a characteristic "tail" sign on MRI.
• Solitary Pleural Metastasis: This refers to a metastatic deposit to the pleura from a primary malignancy elsewhere in the body. The differential diagnosis for solitary pleural metastasis includes SFT, and the two conditions may be difficult to distinguish based solely on imaging features [1].
• Pleural Lymphoma: This is a rare type of lymphoma that can present as a solitary mass in the thorax. While it may be difficult to distinguish from SFT on imaging, pleural lymphoma typically shows a more aggressive clinical course and often has a characteristic "honeycomb" appearance on CT [3].
• Synovial Sarcoma: This is a rare type of sarcoma that can present as a solitary mass in the thorax. While it may be difficult to distinguish from SFT on imaging, synovial sarcoma typically shows a more aggressive clinical course and often has a characteristic "cartwheel" appearance on MRI [4].

In summary, the differential diagnosis for malignant pleural SFTs includes several conditions that may present with similar radiographic and histopathological features. A thorough evaluation of the patient's clinical history, imaging studies, and histopathological findings is essential to establish an accurate diagnosis.

References:

[1] X Gao et al., "6. Differential diagnosis." (2022) [Context 1]

[2] JFB Chick et al., "Once identified radiographically, important differential diagnoses of solitary fibrous tumors of the thorax include spindle cell carcinoma, melanoma..." (2013) [Context 2]

[3] DT Ginat et al., "The imaging differential diagnosis of pleural SFT includes mesothelioma, lymphoma, and metastasis." (2011) [Context 6]

[4] MS Khouzam et al., "Solitary fibrous tumors are very rare pleural tumors. Surgical resection is the treatment of choice followed by close post-operative surveillance." (2022) [Context 7]