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]
Gastrointestinal
- Abdominal Pain
The patients, including this case, ranged in age from 45 to 78 years (mean and median 59 y) and frequently presented with abnormal uterine bleeding and/or abdominal pain. [ncbi.nlm.nih.gov]
However, symptoms and signs may include the following, depending on the location of the tumor : Painful swelling and/or a lump developing under the skin Numbness Fatigue Abdominal pain Chest pain Urinary difficulties Swallowing difficulties Nausea Vomiting [medicinenet.com]
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]
In a phase III trial, treatment emergent adverse events occurred in 224 (99%) of 226 patients who received eribulin. [journals.lww.com]
Long-term neoplasia risk after azathioprine treatment in inflammatory bowel disease. Lancet 1994;343:1249–1252. 11. [synapse.koreamed.org]
Prognosis
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 [basicmedicalkey.com]
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]
Etiology
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]
(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]
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
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]
[…] 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]
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]
Prevention
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]
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]
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]
Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.medscape.com]