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A Combined Prosthetic and Surgical Approach
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Hitesh Koria, M. Stephen Dover, Steve Worrollo
In October 2012 the patient had a further decrease in her vision and developed diplopia. She had another MRI scan, which showed tumour growth above the eye, and a CT scan was carried out to determine bone involvement. A biopsy of the mass showed a myxoid liposarcoma. After further investigations and further discussion it was decided that the best treatment was an orbital exenteration with orbital implants followed by prosthetics.
Soft Tissues
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
DIFFERENTIAL DIAGNOSIS: ClinicalBecause the peak incidence of liposarcoma is in the seventh and eighth decade, and the middle 80% of cases occur between 25 to 79 years, a soft tissue tumor in adolescents and children is probably not a liposarcoma. Most soft tissue sarcomas, especially liposarcoma, fibrosarcoma, leiomyosarcoma, and synovial sarcoma possess a false capsule, and the differential diagnosis must be established by histological means. Traumatized lipomas may resemble liposarcoma on exploration, but these are usually subcutaneous lesions and not deeply situated tumors. MicroscopicTumor giant cells also occur in malignant fibrous histiocytoma (MFH) and pleomorphic rhabdomyosarcoma. The differential diagnostic features of the various soft tissue tumor giant cells are discussed under differential diagnosis, microscopic, of MFH.* Typical small signet ring lipoblasts are not present in MFH and pleomorphic rhabdomyosarcoma. In rhabdomyosarcoma cells are always observed with deeply eosinophilic (myofilament-rich) cytoplasm. These can be well appreciated in the frozen section. Generally, at the frozen section diagnosis the pathologist has no time to search for cross striations. The so-called myxoid liposarcoma must be differentiated occasionally from benign myxoma. The myxoid liposarcoma is composed of round closely packed lipoblasts and resembles embryonal fat tissue. The neoplasm is rich in capillaries (Figure 53). Benign myxoma does not possess vacuolated lipoblasts and in contrast to myxoid liposarcoma is poorly vascularized.201,208 Also inflammation of adipose tissue, panniculitis, must be considered in the differential diagnosis. The fat tissue may be infiltrated by large number of foamy histiocytes. In the frozen sections these faintly resemble immature fat cells. Numerous foam cells are large, and some form multinucleated giant cells. The absence of typical signet ring type lipoblasts, or bizarre lipoblastic giant cells with characteristic pyknotic nuclei aid in establishing the true nature of the lesion.
A multidisciplinary approach to soft-tissue sarcoma of the extremities
Published in Expert Review of Anticancer Therapy, 2020
Robert Nakayama, Tomoaki Mori, Yusuke Okita, Yutaka Shiraishi, Makoto Endo
Because of the high distinctiveness of RMS, other tumors are often referred to as non-RMS STS. The most common non-RMS STSs in AYA are synovial sarcoma and myxoid liposarcoma. More than half of patients with synovial sarcoma are in the AYA category. This malignancy can arise in any part of the body, but approximately 70% of lesions arise in the deep extremities [18]. Myxoid liposarcoma, the most common liposarcoma subtype in children and adolescents, accounts for 20–30% of liposarcomas, and this malignancy is also predisposed to arise in the deep extremities [19–22]. Most synovial sarcomas bear the SS18-SSX fusion gene, and more than 90% of myxoid liposarcomas bear the FUS-DDIT3 fusion gene [23,24]. Similarly, other STSs in the AYA group including clear cell sarcoma and alveolar soft part sarcoma tend to be characterized by tumor-specific genetic alternations [25]. The common extremity STSs in the elderly are undifferentiated pleomorphic sarcoma, leiomyosarcoma, and myxofibrosarcoma [26–29], and these cancers are usually characterized by nonspecific genetic alterations and complex unbalanced karyotypes [30].
Isolated intraosseous extra-gnathic orbital myxoma: a clinicopathologic case report
Published in Orbit, 2019
Fairooz P. Manjandavida, Shaifali Chahar, Brijal Dave
In our case, the tumor upon excision and gross examination was diagnosed as myxoid liposarcoma due to the presence of vascularity and bony invasion. Histopathology in combination with IHC helped in the definitive diagnosis. Angiomyxoma predominantly affects the pelvic-perineal region, and histopathologically it shows an abundance of small blood vessels. Also, angiomyxoma occurring in orbit is an extremely rare entity. Literature review reveals only four reported cases of angiomyxoma of the orbit.1,22 Myxoid liposarcoma must contain lipoblasts and complex arborizing or plexiform vascular patterns. Myxofibrosarcoma shows storiform cellular patterns and mixture of plump and spindle cells, infiltrative periphery and sends out long tentacles with frequent positive margins. Benign focal mucinosis contains fibroblastic-appearing cells embedded in a mucinous stroma. Myxolipoma is characterized by mature adipocytes and spindle cells in the myxoid stroma. IHC may be helpful in some cases. Myxomas are immunoreactive to Vimentin, CD34 (50%), SMA (10%), and factor XIIIa. It is negative for Desmin and S100. These markers differentiate myxomas from other varieties mimicking myxoma. In our case, IHC was positive for Vimentin and SMA and negative for S-100.
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
The use of radiation therapy in patients with liposarcomas has been evaluated and studies suggest MRCL to be a radiosensitive disease [29,56,57]. Chung et al. report the findings of their retrospective review of a prospectively maintained database of evaluating the use of perioperative radiotherapy on disease control in patients with myxoid liposarcoma compared to STS histologic subtypes [57]. In this study, patients with MRCL were found to have improved 5-year local recurrence free survival (98% vs. 90%, p = 0.008), 5-year metastasis-free survival (89% vs. 66%, p < 0.001), and 5-year OS (94% vs. 76%, p < 0.001) compared to other STS subtypes, respectively. Given the promising results of trabectedin and radiotherapy for the treatment of MRCL, a European phase I/II trial of trabectedin plus radiotherapy in patients with localized MRCL and metastatic STS (TRASTS trial, NCT02275286) is actively recruiting. Patients with localized MRCL are scheduled to receive three cycles of trabectedin in combination with radiotherapy (45 Gy) in 25 fractions (1.8 Gy/fraction). The preliminary results of the phase I portion of this study cohort were presented at the 2017 American Society of Clinical Oncology (ASCO) annual meeting and recently published [58]. Overall treatment was well tolerated and of the 13 patients evaluable for response a high proportion achieve a good pathological response on surgical specimen with 25% demonstrating complete response and 75% demonstrating ≤10% viable remaining tumor [58]. The phase II portion of the trial in the locally advanced MRCL cohort is ongoing and actively recruiting.