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Renal Cancer
Published in Karl H. Pang, Nadir I. Osman, James W.F. Catto, Christopher R. Chapple, Basic Urological Sciences, 2021
Sabrina H. Rossi, Grant D. Stewart
Leiomyosarcoma is the most common, followed by liposarcoma.Leiomyosarcoma originates from smooth muscle cells in the renal capsule or perinephric tissue.Liposarcoma originates from fat.
Soft Tissue Sarcomas
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Thomas F. DeLaney, David C. Harmon, Karol Sikora, Francis J. Hornicek
Other chromosomal changes characteristic of specific sarcoma type include the reciprocal exchange t(11:22)(q24;ql2) seen in approximately 85%–90% of Ewing’s sarcoma and primitive peripheral neuro-ectodermal tumor (PNET). In this translocation, the EWS (Ewing sarcoma breakpoint region 1) gene from chromosome 22q12 is covalently linked to the erythroblast transformation-specific (ETS) family member, FLI-1, to form the EWS–FLI-1 fusion gene.15 The chimeric proteins that result from this translocation may alter transcription of a number of genes including upregulation of the transcription factor Gli1 that promotes the oncogenic potential of the Hedgehog pathway. A less common translocation t(21;22)(q22;q12) has also been identified and links EWS to a different ETS family member, ETS-related gene (ERG). Myxoid and round cell subtypes of liposarcomas display a reciprocal translocation t(12;16) (q13;p11). In this translocation, the CHOP (induced by DNA damage) gene is inserted adjacent to a novel gene called TLS. The fusion gene, called TLS–CHOP, shows sequence homology to the Ewing’s fusion gene. It fails to induce G1/S arrest, which is one of the functions of the non-oncogenic form of CHOP (GADD153). Identification of the fusion gene has been used as a diagnostic aid for these subtypes of liposarcoma.
Soft tissue sarcomas
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Christina Messiou, Dirk Strauss, Eleanor Moskovic
CT may demonstrate calcification (<10%) or even ossification. The hallmark of the well-differentiated liposarcoma variant is the presence of fat. On both CT and MRI, this tumour appears as a predominantly fatty mass with irregularly thickened linear or nodular septae, which are of decreased signal on T1-weighted images (T1WI) and increased signal intensity on T2-weighted images (T2WI) (Figure 23.2) (36). Areas of dedifferentiation appear more solid and heterogeneous. The myxoid, pleomorphic, and round cell liposarcomas do not generally contain substantial amounts of fat, and only 50% of masses demonstrate fat radiologically.
Retroperitoneal liposarcoma in older person – a rare case report
Published in The Aging Male, 2020
Navas Nadukkandiyil, Sameer Valappil, Marwan Ramadan, Essa Al Sulaiti, Hanadi Khamis Alhamad
A retroperitoneal tumor often develops in people in their 40s to 50s, and especially in men. It is a very rare tumor that accounts for less than 0.2% of all types of malignant tumors. Based on its histological features, liposarcoma was initially classified into four subtypes: the well-differentiated, myxoid, round cell and pleomorphic types. Later Evans introduced dedifferentiated liposarcoma in 1979, so now there are five subtypes. The feature of dedifferentiated liposarcoma is the histological coexistence of well to poorly differentiated liposarcoma and non-lipomatous differentiated areas [3]. The diagnosis is often difficult until the tumor reaches a great size to cause symptoms of pressure. As the mass grows, there are usually no initial subjective symptoms. Complete surgical removal is often difficult, and the tumor tends to recur, which make it hard to treat.
Pharmacotherapy for liposarcoma: current state of the art and emerging systemic treatments
Published in Expert Opinion on Pharmacotherapy, 2019
Lorena P. Suarez-Kelly, Giacomo G. Baldi, Alessandro Gronchi
Liposarcomas are soft tissue tumors that arise from adipose tissue and are one of the most common soft tissue sarcoma (STS) histotype found in adults, accounting for up to 25% of all cases [1,2]. Liposarcomas are subclassified into several subtypes: atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDL), dedifferentiated liposarcoma (DDL), myxoid/round cell liposarcoma (MRCL), and pleomorphic liposarcoma (PL) [1,3]. Classification into each specific subtype is done based on distinct biologic, histologic, cytogenic, and molecular features. Each liposarcoma subtype is distinct from one another with their unique clinical behaviors and prognosis. Therefore, it is crucial to understand the differences between each liposarcoma subtype when choosing the best treatment and management for this disease.
Investigational therapies in phase II clinical trials for the treatment of soft tissue sarcoma
Published in Expert Opinion on Investigational Drugs, 2019
Juan Martin-Liberal, Ezequiel Pérez, Xavier García Del Muro
Aberrations in tumor suppressor proteins are well-known alterations involved in carcinogenesis of multiples tumor types, including STS [47,48]. One of the mechanisms used by cancer cells to inactivate tumor suppression proteins is the overexpression of exportin 1 (XPO1). This molecule is responsible for exporting proteins from the cell nucleus to the cytoplasm [49]. Since tumor suppression proteins exert their activity in the nucleus, overexpression of XPO1 diminish the nuclear concentration of these proteins and therefore their activity. Subsequently, overexpression of XPO1 promotes carcinogenesis and it has been correlated with poor clinical outcomes in several malignancies, including sarcomas [50]. Selinexor is an oral inhibitor of XPO1 and it is the first of a novel class of compounds to show clinical activity in STS. It was assessed in a phase Ib study including 54 patients with pretreated advanced STS or bone sarcoma. Results confirmed its good safety profile and also showed early signs of activity: in spite that no PR was seen, SD for at least 4 months was reported in 17 patients (33%), of which 7 (47%) were de-differentiated liposarcoma [51].