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Pleomorphic Liposarcoma

PLS


Presentation

  • A case presenting clinical evidence that PLS may arise from the epidural fat is reported. The clinical presentation, management, and outcome in a case of primary PLS of the thoracic spine, and a review of the literature, are presented.[ncbi.nlm.nih.gov]
Rigor
  • The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.[ncbi.nlm.nih.gov]
Back Pain
  • A 70-year-male presented with sudden onset lower extremity weakness, constipation, and back pain. Magnetic resonance imaging revealed an epidural lesion at T5 with noted mass effect compressing the spinal cord and extension to the T5-T6 foramen.[ncbi.nlm.nih.gov]
Diplopia
  • A 62-year-old female presented with pruritus, edema, proptosis and diplopia 5 months OS. Imaging revealed an intraorbital mass displacing the globe, with infiltration into the sinus.[ncbi.nlm.nih.gov]
Papule
  • We describe a unique case of an 85-year-old Caucasian gentleman with a 1 year history of a pedunculated, pink, non-tender papule on the dorsum of his left arm, measuring 1.0 cm in maximum dimension.[ncbi.nlm.nih.gov]
Breast Mass
  • A 70-year-old man admitted with left-sided painless progressively growing breast mass. Radiological investigation revealed liposarcoma of the pectoralis major muscle. The patient was treated by surgical removal.[ncbi.nlm.nih.gov]
  • masses of variable duration.[ijpmonline.org]

Treatment

  • PLS is a rare and high-grade malignancy with high recurrence, poor prognosis, and its treatment is still highly controversial.[ncbi.nlm.nih.gov]
  • They are distinct cancers in the ways they respond to treatment. How is it treated? Surgery is the most common treatment, but a combination of treatment is often used, Canter said, including chemotherapy, radiation and surgery.[cbc.ca]

Prognosis

  • It has a poor prognosis and unlike other variants of liposarcoma, lacks a molecular or genetic signature. Histologic studies of pleomorphic liposarcoma have defined this lesion as a high grade sarcoma, which contains a variable number of lipoblasts.[ncbi.nlm.nih.gov]
  • High-grade sarcoma Worse prognosis than dedifferentiated liposarcoma Better prognosis than high-grade myogenic sarcomas Deep-seated large neoplasms are associated with worse prognosis 20 mitoses per 10 high-power fields and necrosis are associated with[basicmedicalkey.com]

Etiology

  • (Etiology) The exact cause and mechanism of formation of Pleomorphic Liposarcoma is unknown It is thought to occur spontaneously due to certain genetic mutations (chromosomal abnormalities) Contrary to some beliefs, trauma does not contribute to the formation[dovemed.com]
  • Etiology Ninety percent of MRCLS lesions have a characteristic chromosomal translocation leading to the fusion of the DDIT3 and FUS genes on chromosome regions 12q13 and 16p11.[orpha.net]
  • Characterization of chromosomal copy number changes as well as gene expression analysis provides evidence for pathogenic mechanisms involved in tumor etiology or development.[cancerres.aacrjournals.org]

Epidemiology

  • […] liposarcoma (PLS) Definitions Pleomorphic high-grade sarcoma containing variable amounts of pleomorphic lipoblasts No areas of atypical lipomatous tumor are present No sarcomatous component of a different line of differentiation is present CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Definition / general Widespread high grade, pleomorphic tumor cells with some lipoblasts but no evidence of well differentiated liposarcoma Epidemiology 5% of all liposarcomas No gender preference Median age 54 - 70 years; rare in childhood Associated[pathologyoutlines.com]
  • Epidemiology of inflammatory bowel disease in the Songpa-Kangdong district, Seoul, Korea, 1986-2005: a KASID study. Inflamm Bowel Dis 2008;14:542–549. 7. Lee HJ, Yang SK, Kim KJ, et al.[synapse.koreamed.org]
  • One study, published in the American Journal of Epidemiology in 1989, that looked at 228 people with soft tissue cancers and 1,610 healthy people, found a greater risk of sarcomas among those who chewed tobacco.[livescience.com]
  • Summary Epidemiology The incidence is approximately 1/769, 000 per year and it accounts for 30% or less of all LS cases.[orpha.net]
Sex distribution
Age distribution

Prevention

  • Radiation therapy is used to prevent recurrence and is successful in 85 to 90 per-cent of cases, said the Sarcoma Initiative. Mayor Ford began the first of two chemotherapy treatments on Thursday.[humbernews.ca]
  • Is it possible to prevent a liposarcoma? Most liposarcomas develop in people with no known risk factors. Consequently, there is no way to prevent liposarcomas other than to avoid exposure to their risk factors listed above.[medicinenet.com]
  • However, neither surgery nor SBRT will prevent emergence of additional metastases elsewhere in the lung. Therefore, the role of chemotherapy needs to be further explored to address systemic metastasis.[en.wikipedia.org]
  • MDM2 binds TP53 and prevents it from exerting its effects on cell cycle progression and DNA repair.[ozradonc.wikidot.com]

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