Epithelioid sarcoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
The symptoms of epithelioid sarcoma usually develop in the second decade of life and start with symptoms such as a mass. Epithelioid sarcoma has a tendency to metastasize to the lymph nodes. The most common sites of epithelioid metastasis include the lungs, lymph nodes, and scalp. Common complications of epithelioid sarcoma include metastasis, nerve compression, and numbness. The prognosis of epithelioid sarcoma is good among female patients.
Natural History
- The symptoms of epithelioid sarcoma usually develop in the second decade of life and start with symptoms such as a mass. Epitheliod sarcoma has a tendency to metastasize to the lymph nodes. The most common sites of epithelioid metastasis include the lungs, lymph nodes, and scalp.
- Epithelioid sarcoma most commonly strikes young adults, yet no age group is immune. The disease has a tendency to develop local recurrences and metastasis thereafter to regional lymph nodes, lung, bone, brain, and other locations, including the scalp.[1] Generally speaking, epithelioid sarcoma has a high rate of relapse after initial treatment and tends to recur locally (at or near the original tumor site). Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases).[2] These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. The overall five-year survival rate for epithelioid sarcoma is anywhere from 25 to 78%. Importantly, the 10-year and 15-year survival rate drops off significantly.[3][4] Associated with a more positive outcome are younger age, female vs. male sex, distal vs. proximal location, smaller tumor size, and negative margins upon tumor resection.[5][4]
Complications
- Metastasis
- Contractures
- Nerve compression
- Muscle weakness
- Numbness
Prognosis
- The prognosis of epithelioid sarcoma is good among female patients.
- The five-year survival rate for patients with epithelioid sarcoma is approximately 50-70%.
- The ten-year survival rate for patients with epithelioid sarcoma is approximately 42-55%.
- Prognosis varies with:
- Age of diagnosis- Earlier age at diagnosis is associated with better outcome
- Gender
- Site- Proximal lesions have worse prognosis
- Microscopic pathology- Necrosis and vascular invasion have been associated with poor prognosis
- Tumor size- Tumors of more than 2 cm size have been associated with poor prognosis[6]
- Mitotic index
References
- ↑
- ↑ Levy, Antonin; Le Péchoux, Cécile; Terrier, Philippe; Bouaita, Ryan; Domont, Julien; Mir, Olivier; Coppola, Sarah; Honoré, Charles; Le Cesne, Axel; Bonvalot, Sylvie (2014). "Epithelioid Sarcoma: Need for a Multimodal Approach to Maximize the Chances of Curative Conservative Treatment". Annals of Surgical Oncology. 21 (1): 269–76. doi:10.1245/s10434-013-3247-4. PMID 24046109.
- ↑ Lev, Dina. "Epigenetic reprogramming of epitheliold sarcoma: a role for INI1-HDAC crosstalk".[dead link]
- ↑ 4.0 4.1 Casanova, Michela; Ferrari, Andrea; Collini, Paola; Bisogno, Gianni; Alaggio, Rita; Cecchetto, Giovanni; Gronchi, Alessandro; Meazza, Cristina; Garaventa, Alberto; Di Cataldo, Andrea; Carli, Modesto (2006). "Epithelioid sarcoma in children and adolescents". Cancer. 106 (3): 708–17. doi:10.1002/cncr.21630. PMID 16353216.
- ↑ Jawad, Muhammad Umar; Extein, Jason; Min, Elijah S.; Scully, Sean P. (2009). "Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database". Clinical Orthopaedics and Related Research. 467 (11): 2939–48. doi:10.1007/s11999-009-0749-2. PMC 2758965. PMID 19224301.
- ↑ Epithelioid sarcoma. Sarcomahelp (2016). http://sarcomahelp.org/epithelioid-sarcoma.html Accessed on February 8, 2016