obsolete alveolar soft part sarcoma nonmetastatic

Based on the provided context, here are the signs and symptoms of alveolar soft part sarcoma (ASPS) in its non-metastatic stage:

Common Symptoms:

• A noticeable lump or swelling [3]
• May be painless, but can cause pain if it presses on nerves or muscles [5]
• Swelling or a lump in the arm or leg [5]

Typical Clinical Findings:

• A painless thigh or buttock mass is the most common presentation [11]
• The tumor may not cause notable symptoms until it has grown larger and begun pushing on nerves or muscles [10]

Other Possible Symptoms:

• Swelling or a lump in other areas, such as the arm, head, neck, chest, or abdomen [15]

It's essential to note that these symptoms can vary depending on where the tumor is located. In many cases, ASPS may not cause any additional signs and symptoms until it has metastasized to other sites [13].

References:

• [3] ASPS is a slow-growing, painless tumor and often found by accident.
• [5] Symptoms · Possibly pain in the affected area, if the cancer pushes on nerves or other areas of the body. · Swelling or a lump in the arm or leg.
• [10] Some ASPS tumors may cause a lump or swelling, but these changes are often painless.
• [11] The typical clinical findings are of a painless thigh or buttock mass, although ASPS can occur in the trunk, arm or elsewhere.
• [13] Alveolar soft tissue sarcoma generally presents as a soft, pain-less lump that is often not associated with any additional signs and symptoms.

Based on the provided context, it appears that diagnostic tests for alveolar soft part sarcoma (ASPS) are crucial in determining the presence and extent of the disease.

Imaging Studies

• Computed Tomography (CT): CT imaging is a useful tool in diagnosing ASPS, particularly in identifying the tumor's location and size. According to [2], ASPS is observed as an enhancing mass lesion with prominent feeding vessels on CT imaging.
• Magnetic Resonance Imaging (MRI): MRI is preferred over CT for confirming the diagnosis of soft-tissue sarcomas, including ASPS, due to its superior anatomical definition and multiplanar capabilities ([5]). However, contrast CT or preferably MRI of the brain should be considered in cases of ASPS and clear cell sarcoma due to a higher incidence of metastasis ([6]).

Biopsy

• Core-needle biopsy: This minimally invasive procedure involves removing a small sample of tissue from the tumor for histopathological examination. It is a useful diagnostic tool, especially when imaging studies are inconclusive.
• Incisional or excisional biopsy: These procedures involve surgically removing a larger portion of the tumor for further examination.

Other Diagnostic Modalities

• Plain radiography: This modality may be used as clinically indicated to assess the extent of disease and monitor response to treatment.
• Radionuclide scintigraphy with 99mTc: This imaging technique can help identify metastatic disease or assess the tumor's metabolic activity.

It is essential to note that a definitive diagnosis of ASPS often requires histopathological examination of a biopsy specimen. The choice of diagnostic modality depends on individual patient factors and clinical presentation.

References: [2] Fujiwara T (2023) - [Context 2] [5] Shakya S (2024) - [Context 5] [6] Hayes AJ (2024) - [Context 6]

Based on the available information, it appears that there are limited treatment options for non-metastatic alveolar soft part sarcoma (ASPS). However, I was able to find some relevant information in the search results.

According to [result 3], ifosfamide is one of the most commonly used drugs in soft tissue sarcoma, including ASPS. It has been reported to result in response rates of 20-25% in non-pretreated patients. However, it's essential to note that this information may not be specific to non-metastatic ASPS.

Another study mentioned in [result 6] also highlights the use of ifosfamide as a monotherapy for soft tissue sarcoma, including ASPS. The response rates achieved with ifosfamide are comparable to those reported in other studies.

Unfortunately, I couldn't find any specific information on the treatment of non-metastatic alveolar soft part sarcoma using modern or targeted therapies. However, it's worth noting that [result 9] mentions pazopanib as a targeted therapy with an FDA-approved indication for soft tissue sarcoma, but its effectiveness in non-metastatic ASPS is not specified.

In summary, while there are some treatment options available for soft tissue sarcoma, including ASPS, the specific treatment of non-metastatic alveolar soft part sarcoma appears to be limited. Ifosfamide may still be considered as a potential treatment option, but further research and clinical trials would be necessary to determine its efficacy in this context.

References:

• [result 3]
• [result 6]
• [result 9]

Differential Diagnosis of Alveolar Soft Part Sarcoma (ASPS) Nonmetastatic Disease

Alveolar soft part sarcoma (ASPS) is a rare and aggressive type of cancer that typically affects young adults. When ASPS presents as a nonmetastatic disease, the differential diagnosis can be challenging due to its rarity and similarity in presentation with other soft tissue tumors.

Possible Differential Diagnoses:

• Rhabdomyosarcoma: A malignant tumor of skeletal muscle origin, which is more common in children and young adults. It presents as a rapidly growing mass, often in the head and neck region or extremities.
• Fibrosarcoma: A rare type of cancer that arises from fibrous tissue, typically presenting as a slow-growing mass in the soft tissues.
• Malignant Fibrous Histiocytoma (MFH): A rare and aggressive tumor that arises from fibrohistiocytic cells, often presenting as a rapidly growing mass in the extremities or retroperitoneum.
• Synovial Sarcoma: A rare type of cancer that arises from synovial tissue, typically presenting as a slow-growing mass near joints.

Key Features to Consider:

• Age and sex of the patient: ASPS typically affects young adults, while rhabdomyosarcoma is more common in children.
• Location of the tumor: ASPS often presents in the orbit, oral cavity, or larynx, whereas rhabdomyosarcoma tends to occur in the head and neck region or extremities.
• Histological features: ASPS is characterized by its distinctive "organoid" nests of cells surrounded by endothelial-lined vascular spaces, which can be distinguished from other soft tissue tumors.

Prognostic Factors:

• Nonmetastatic disease at presentation results in a 77% survival rate at 2 years, 60% at 5 years, and 38% at 10 years [1].
• Surgical resection is the primary treatment for nonmetastatic ASPS, with a significant impact on patient outcomes.

References:

[1] Christopherson et al. (1952) - First description of Alveolar Soft Part Sarcoma. [11] Surveillance, Epidemiology and End Results database search (1973-2018) - Incidence and mortality rates for ASPS in the US population. [13] Clinical correlation and histological features to distinguish ASPS from other soft tissue tumors.

Note: The information provided is based on the search results within the context. If you need further clarification or would like me to expand on any of these points, please let me know!