obsolete alveolar soft part sarcoma metastatic

Alveolar soft part sarcoma (ASPS) is a rare and slowly growing malignant tumor that primarily affects young adults. It has a high potential for metastasis, with the lungs being the most common site for metastatic spread [3][5]. The tumor typically arises in the limbs or buttock as a painless mass, which can later metastasize to other parts of the body, including the brain and liver [1][4].

Historically, ASPS was considered an ultra-rare sarcoma with a poor prognosis due to its late metastatic spread. However, recent studies have shown that patients with metastatic ASPS may experience prolonged survival despite resistance to conventional chemotherapy [12]. The tumor's unique histogenesis and specific chromosomal translocation (der(17)t(X;17)(p11.2;q25)) contribute to its rarity and aggressive behavior [14].

In the past, the diagnosis of ASPS was often delayed due to its slow growth rate and lack of distinctive symptoms. However, with advancements in imaging techniques and molecular diagnostics, early detection and treatment have become more feasible.

It's worth noting that while ASPS is considered an ultra-rare sarcoma, it still accounts for a small percentage of all soft tissue sarcomas [10]. Further research is needed to understand the tumor's behavior and develop effective treatment strategies for patients with metastatic ASPS.

Alveolar soft part sarcoma (ASPS) is a rare and malignant tumor that can be challenging to diagnose, especially in its metastatic form. In the past, diagnostic tests for ASPS were limited, but with advancements in medical technology, several imaging modalities have been employed to aid in diagnosis.

• Computed Tomography (CT) scans: CT scans can help determine whether the sarcoma has spread and, if so, to which parts of the body [2]. However, they may not always provide a definitive diagnosis.
• Magnetic Resonance Imaging (MRI): MRI scans can show high signal intensity in T1-weighted and T2-weighted images, with internal and external multilobulated signal changes [3].
• Positron Emission Tomography (PET)/CT: PET/CT scans can help identify metastatic disease and assess the tumor's metabolic activity.
• Bone scan: A bone scan may be performed to evaluate for any potential skeletal metastases.

It is essential to note that while these diagnostic tests can aid in the diagnosis of ASPS, a definitive diagnosis often requires tissue biopsy and histopathological examination [9]. The diagnosis of ASPS is mainly based on pathological histological observations, including immunohistochemistry [6].

References: [2] - A computed tomography (CT) scan can determine whether the sarcoma has spread and, if so, to which parts of the body. [3] - Alveolar soft tissue sarcoma usually shows high signal in T1-weighted and T2-weighted MRI and features internal and external multilobulated signal change. [6] - The diagnosis requires comprehensive analysis of clinical history, histological morphology, and immunohistochemistry. [9] - Final diagnosis, however, is based on tissue biopsy, as for all other subtypes of soft-tissue sarcoma.

Treatment Options for Metastatic Alveolar Soft Part Sarcoma

Metastatic alveolar soft part sarcoma (ASPS) is a rare and aggressive cancer with limited treatment options. However, recent studies have explored various targeted therapies to improve patient outcomes.

• Axitinib and Pembrolizumab: A combination of axitinib, a tyrosine kinase inhibitor, and pembrolizumab, an immune checkpoint inhibitor, has shown promise in treating metastatic ASPS. This treatment approach was effective in an 80-year-old patient with a history of autoimmune disorders [3].
• Apatinib: Apatinib, a specific VEGFR-2 inhibitor, has been licensed by the China Food and Drug Administration for the treatment of advanced or metastatic ASPS. Clinical trials have demonstrated its efficacy in treating this rare cancer [15].
• Camrelizumab and Apatinib: The combination of camrelizumab, a programmed death-ligand 1 (PD-L1) inhibitor, and apatinib has been used to treat brain metastases in patients with ASPS. This treatment approach achieved partial remission in one patient [5].
• Nivolumab: Nivolumab, a PD-1 inhibitor, has been used as a salvage therapy for patients with metastatic ASPS who have failed previous treatments. One patient achieved stable disease for 7 months after receiving nivolumab [10].

Other Treatment Options

While these targeted therapies show promise, it's essential to note that the standard treatment for patients with advanced/metastatic soft-tissue sarcomas is systemic chemotherapy with doxorubicin [2]. However, ASPS often presents early with metastases and is typically insensitive to standard chemotherapy and radiotherapy.

References:

[1] Dorman K, Burkhard-Meier A, Di Gioia D, et al. Treatment of metastatic alveolar soft part sarcoma with axitinib and pembrolizumab in an 80-year-old patient with a history of autoimmune disorders [3].

[2] Surveillance, Epidemiology and End Results database search of the US population between 1973 and 2018.

[5] Alveolar soft-part sarcoma often affects young adults and is characterized by indolent behavior but early evidence of metastatic spread.

[10] Background: Evidence suggests that advanced or metastatic alveolar soft part sarcoma (ASPS) with high metastatic potential is chemo-resistant. However, the benefits of tyrosine kinase inhibitors have been demonstrated for the treatment of ASPS.

[15] Apatinib, a specific VEGFR-2 inhibitor, was licensed by the China Food and Drug Administration for the treatment of advanced or metastatic ASPS.

Based on the provided context, it appears that differential diagnosis for alveolar soft part sarcoma (ASPS) metastasis is a complex and challenging task.

Possible Differential Diagnoses:

• Metastatic renal cell carcinoma: This type of cancer often presents in older patients (usually > 40 years) with distant metastasis at diagnosis [10].
• Epithelioid sarcoma or solitary fibrous tumor: Dasatinib has been shown to have no significant antisarcoma activity in patients with these types of tumors [7].
• Metastatic carcinoma, melanoma, or lymphoma: These malignant lesions can also present as soft tissue masses and may be considered in the differential diagnosis for ASPS metastasis [1].

Key Features of Alveolar Soft Part Sarcoma (ASPS) Metastasis:

• Young peak age incidence
• Frequent metastasis to the brain
• Late metastases and an extended clinical course
• Survival rates of 77% at 2 years [13]

Differential Diagnosis Considerations:

• The differential diagnosis for ASPS metastasis is fairly broad and may include various types of soft tissue sarcomas, as well as other malignant lesions.
• A thorough clinicopathological analysis, including molecular genetic studies and diagnostic histology, is essential for accurate diagnosis.

It's worth noting that the provided context suggests that alveolar soft part sarcoma (ASPS) metastasis is a rare and complex entity, with limited information available on its differential diagnosis. Further research and investigation may be necessary to determine the most likely causes of ASPS metastasis in individual cases.

References:

[1] by GD Demetri · 2005 · Cited by 55 [7] by SM Schuetze · 2017 · Cited by 124 [10] [13]