obsolete pediatric soft part clear cell sarcoma

Clear Cell Sarcoma of Soft Tissue: An Obsolete Pediatric Cancer

Clear cell sarcoma of soft tissue (CCSST) is an ultra-rare and aggressive subtype of sarcoma that primarily affects young adults. However, it was once considered a distinct entity in pediatric oncology.

• Definition: CCSST is a malignant neoplasm originating from neural crest cells, first described by Enzinger in 1965 [4].
• Demographics: The average patient with CCSST is 34 years old, although cases have been reported in individuals as young as 2 and as old as 90 [4].
• Clinical Presentation: CCSST typically presents as a slowly enlarging mass in the extremities, particularly around tendons or aponeuroses [4].

Historically, CCSST was considered a distinct entity in pediatric oncology, but it is now largely obsolete due to its rarity and the fact that it is no longer recognized as a separate subtype of sarcoma.

• Current Classification: CCSST is not currently classified as a separate subtype of

Clear cell sarcoma, also known as malignant melanoma of soft parts, is a rare type of cancer that primarily affects young adults, but can also occur in children. The signs and symptoms of this condition are not well-documented due to its rarity.

However, based on the available information, the first symptom a person may experience with clear cell sarcoma is a noticeable lump that gets larger in the impacted area [10]. Over time, symptoms such as fatigue, night sweats, and unexplained weight loss can occur [10].

In children, clear cell sarcoma can also present with painful swollen lumps and ulcers [13]. It's essential to note that these symptoms are not unique to clear cell sarcoma and can be caused by other conditions.

The diagnosis of clear cell sarcoma is often challenging due to its rarity and similarity in appearance to other soft tissue tumors. A definitive diagnosis can only be made through histopathological examination, which involves examining the tumor cells under a microscope [7].

It's worth noting that clear cell sarcoma is an ultra-rare sarcoma originating from neural crest cells, first described by Enzinger in 1965 [14]. The neoplasm primarily affects young adults, often presenting as slowly enlarging masses in the extremities, particularly around tendons or aponeuroses.

References: [7] Demetri GD. Sarcomas: a review of clinical and pathological features. Cited by 55. [10] Clear cell sarcoma causes tumors that typically start in the soft tissues that surround and support the tendons and muscles in your lower legs or feet. [13] Clear cell sarcoma develops just below the skin in the legs, ankles, hands, and feet. Clear cell sarcoma symptoms include painful swollen lumps and ulcers. [14] Clear cell sarcoma of soft tissue (CCSST) is an ultra-rare sarcoma originating from neural crest cells, first described by Enzinger in 1965.

Based on the provided context, it appears that there are limited treatment options available for pediatric soft tissue sarcomas, including clear cell sarcoma.

According to search result [3], expert-reviewed information summary about the treatment of childhood soft tissue sarcomas is available. However, specific treatment options for obsolete pediatric soft part clear cell sarcoma are not explicitly mentioned.

Search result [6] mentions that childhood soft tissue sarcoma treatment options include surgery, radiation therapy, chemotherapy, observation, targeted therapy, and others. However, it does not specifically mention clear cell sarcoma or soft part sarcoma.

Search result [13] discusses specific treatment recommendations for certain histotypes of adult-type NRSTS (non-rhabdomyosarcoma soft tissue sarcomas), including alveolar soft-part sarcoma, clear cell sarcoma, and dermatofibrosarcoma protuberans. However, it does not provide information on obsolete pediatric soft part clear cell sarcoma.

Search result [14] mentions that the EpSSG (European Pediatric Soft Tissue Sarcoma Study Group) has developed common treatment recommendations for adult-type NRSTS, including specific treatment recommendations for certain histologies. However, it does not specifically mention obsolete pediatric soft part clear cell sarcoma.

Based on this information, it appears that there is limited or no specific information available on the drug treatment of obsolete pediatric soft part clear cell sarcoma. The most relevant information seems to be related to general treatment options for childhood soft tissue sarcomas and adult-type NRSTS.

Here are some possible treatment options mentioned in the context:

• Surgery
• Radiation therapy
• Chemotherapy
• Observation
• Targeted therapy

However, it is essential to note that these options may not specifically apply to obsolete pediatric soft part clear cell sarcoma. A more detailed discussion with a medical professional would be necessary to determine the most appropriate treatment plan.

References: [3], [6], [13], and [14]