Myxoid liposarcoma originating in the retroperitoneum with metastasis to the calcaneus: a rare case report and review of literature.

Myxoid liposarcoma (MLPS) is known to have a variety of metastatic manifestations. We report a MLPS originating in the pelvis with metastasis to the calcaneus. The patient was a 72-year-old man who developed lumbar pain and right lower extremity pain 2 years ago. He visited a nearby clinic and underwent a radiographic examination. Computed tomography (CT) revealed a tumor in the right retroperitoneum. A CT-guided needle biopsy was performed, and pathological examination revealed myxoid liposarcoma. Wide surgical resection was not performed due to the patients' wishes, technical difficulties, and magnitude of the invasion, and the patient received heavy particle radiation therapy (HPRT) of 70.4 Gy. After HPRT, the tumor mass was slightly reduced. However, 11 months after HPRT, a recurrent lesion in the liver was observed. Although HPRT was performed again for the metastatic liver lesion (70.4 Gy), the tumor increased in size. Furthermore, 1 month later, the patient complained of pain in the left foot, and CT and magnetic resonance imaging revealed an osteolytic lesion in the calcaneus. A biopsy was performed, and pathological examination showed a metastatic lesion of myxoid-type liposarcoma. The patient wore a short lower limb orthosis and was able to walk but died 1 month later. Oncologists should note that MLPS can metastasize to the calcaneus.

Staging and Surveillance of Myxoid Liposarcoma: Follow-up Assessment and the Metastatic Pattern of 169 Patients Suggests Inadequacy of Current Practice Standards.

BACKGROUND: Unlike other sarcoma subtypes, myxoid liposarcoma (MLS) has a propensity for extra-pulmonary metastases. Computed tomography (CT) scan of the chest, abdomen, and pelvis has become an accepted practice for surveillance. However, recent literature suggests that this may be inadequate. This study aimed to assess the ability of current imaging methods to detect metastases adequately in this population. METHODS: The study identified 169 patients with MLS diagnosed between 2000 and 2016. The timing and location of metastases, the reasons leading to the MLS diagnosis, and the imaging methods were recorded. The locations of metastases were classified into the following categories: pulmonary, soft tissue, bone, retroperitoneal, intraperitoneal, solid organ, and lymph node. RESULTS: An initial diagnosis of metastasis was made at presentation with staging CT scan for 3 (10 %) of 31 patients, with a follow-up surveillance CT scan for 15 (48 %) of the patients or with subsequent imaging obtained in response to patient-reported symptoms for 13 (42 %) of the patients. The proportions of patients who had metastases in each location were as follows: soft tissue (84 %), pulmonary (68 %), intraabdominal (48 %), solid organ (48 %), bone (45 %), lymph node (32 %), and retroperitoneal (29 %). Although 14 patients had bone metastases, only 1 patient had a sclerotic/blastic presentation visualized on CT scan, and the diagnosis for the remaining 13 patients was determined by magnetic resonance imaging (MRI). CONCLUSION: Due to metastatic disease identified outside surveillance imaging for 58 % of the patients, the diversity of locations, and the significant failure of CT and bone scan to identify bone metastases, this study questioned the adequacy of CT scan for surveillance of MLS. Consideration should be given to the use of whole-body MRI for detection of metastasis in MLS.

Magnetic resonance imaging of trunk and extremity myxoid liposarcoma: diagnosis, staging, and response to treatment.

Myxoid liposarcoma (MLS) accounts for approximately 30% of all liposarcomas. The majority are intermediate-grade tumours, but the presence of >5% round cell component renders it a high-grade sarcoma with subsequent poorer outcome. MLS most commonly arises in the lower extremities, has a predilection for extra-pulmonary sites of metastatic disease, and is recognized to be radiosensitive. The purpose of the current article is to review the role of MRI in the management of MLS, including the characteristic features of the primary tumour, features which help to identify a round cell component and thus determine prognosis, the role of whole-body MRI for evaluation of extra-pulmonary metastatic disease, and the utility of MRI for assessing treatment response. The MRI differential diagnosis of MLS is also considered.

Unusual cardiac tamponade: Giant primary cardiac myxoid liposarcoma.

Primary cardiac myxoid liposarcoma is an extremely rare disease. We reported a 61-year-old man with a 1-month history of aggressive dyspnea and anorexia due to a giant primary cardiac myxoid liposarcoma. Radical resection, whenever feasible, is considered to be an appropriate surgical strategy for this disease. Heart transplantation may be an alternative therapy for such isolated and unresectable tumor.

[Neoadjuvant Use of Isolated Limb Perfusion in Large Myxoid Liposarcoma of the Thigh: a Case Report]. / Neoadjuvantní uzití izolované perfuze koncetiny u objemného myxoidního liposarkomu stehna: kazuistika.

Large unresectable STS presents a therapeutic challenge. Several options are being explored to avoid amputation without compromising the oncological outcome. Neoadjuvant chemotherapy delivers inconsistent and rather unsatisfactory results, preoperative radiotherapy compromises healing, hence it can impede adjuvant systemic treatment. We present a case report of neoadjuvant use of isolated limb perfusion with TNF-alfa and Alkeran (Melphalan) in a patient with initially unresectable large myxoid liposarcoma of the thigh. We achieved 55% reduction in size of the tumor that allowed for wide resection with a safe margin. Pathology confirmed 99% tumor necrosis. The patient has a full function of his extremity and is disease-free at one year follow-up. ILP should be considered as a treatment option which, in selected cases, can contribute to limb sparing surgery. Key words: sarcoma, soft tissue, regional perfusion, chemotherapy, surgery, orthopedic, limb salvage.

Report of two primary renal tumors with myxoid features. Differential diagnosis between benign and malignant entities.

Renal mesenchymal neoplasms are rare entities which can have a benign or a malignant behavior. Herein we describe two renal mesenchymal tumors with myxoid stroma, investigating the wide spectrum of differential diagnosis. With our first case we considered some benign entities such as myxoma, myxoid leiomyoma, and mixed epithelial and stromal tumor; with our second case we considered some sarcomas with myxoid features such as myxofibrosarcoma, low-grade fibromyxoid sarcoma, dedifferentiated liposarcoma, and myxoid liposarcoma. During the diagnostic process, it is important to integrate histopathological, immunohistochemical, and molecular data in order to avoid misdiagnosis. We concluded our second case report was a myxofibrosarcoma grade 1. To the best of our knowledge, we described the fourth primary renal myxofibrosarcoma reported in literature.

Primary low grade myxoid liposarcoma of the ovary: A case report and review of literature.

A 20-year-old female patient presented with the complaint of pelvic pain. Radiological studies showed a cystic mass in the left ovary. Histological examination revealed a myxoid tumor with a chicken wire-like, thin vascularization. The diagnosis of the lesion, morphologically resembling myxoid liposarcoma was supported by demonstration of rearrangement of the DDIT3 gene by fluorescence in situ hybridization.

A case of laparoscopic resection of myxoid liposarcoma of the greater omentum.

Primary omental liposarcoma is a rare clinical entity with less than 20 cases being reported in the literature. Laparotomy has been the traditional approach for resection, with no reports of laparoscopic resection. A 39-year-old lady presented at the Sarawak General Hospital, Kuching, Malaysia with a history of a progressive, painless left upper quadrant abdominal swelling for a year. CT scan showed a well-defined heterogeneously enhancing cystic mass measuring 7.5x7.5x8.1cm with a poor plane with the adjacent greater curvature of stomach and transverse colon. Upper and lower endoscopy was normal. The tumour was completely dissected from the adjacent transverse colon and removed laparoscopically. Histopathological examination of the resected specimen revealed a myxoid liposarcoma. She had an uneventful recovery and was discharged well on the third postoperative day. She subsequently underwent adjuvant chemotherapy and was well at 1-year follow-up with PET CT showing no evidence of recurrence or metastases.

Solitary metastasis of myxoid liposarcoma from the thigh to intraperitoneum: a case report.

BACKGROUND: The purpose of case report was to present a rare case of a solitary metastasis of myxoid liposarcoma and discuss the clinical and pathological information for patients treated for metastatic myxoid liposarcoma. CASE PRESENTATION: We report our experience with a case of solitary metastasis of myxoid liposarcoma from the thigh to intraperitoneum. The patient was a 60-year-old man who was referred for abdominal discomfort and fatigue. Enhanced computed tomography showed a 25-cm intra-abdominal tumor. He had undergone a wide local excision for a right thigh myxoid liposarcoma 6 years earlier. At laparotomy, a huge multi-lobular cystic mass was identified at the small bowel mesentery. Wide local excision was performed, and the mass was diagnosed as metastatic myxoid liposarcoma. He was discharged without postoperative complications. CONCLUSIONS: We experienced a single intraperitoneal metastasis in a patient with myxoid liposarcoma after radical surgery of the primary site.

Isolated intraosseous extra-gnathic orbital myxoma: a clinicopathologic case report.

A 19-year-old female student presented with painless, progressive proptosis in the right eye for the past six months causing significant cosmetic blemish and double vision. Clinical evaluation revealed normal visual acuity, normal pupillary reflex, severe proptosis, and dystopia. Orbital imaging revealed a heterogeneous mass in superior orbit displacing the globe inferiorly and causing extensive bony erosion of frontal bone superiorly and invading the frontal sinus. Excision biopsy was performed with en-bloc removal of the tumor. Surgical delivery of a slimy mass with infiltrated bony tissue led to a presumptive diagnosis of myxoid liposarcoma. Careful histopathologic analysis coupled with immunohistochemical studies were performed which established the rare diagnosis of low-grade myxoid spindle cell neoplasm with features suggestive of intraosseous myxoma. On follow-up at 15 months, our patient revealed no signs of recurrence. Isolated intraosseous orbital myxoma is a rare entity; only two such cases have been reported in the literature.

Diagnosis and Treatment of Myxoid Liposarcomas: Histology Matters.

OPINION STATEMENT: Sarcomas are a heterogeneous group of rare malignancies that arise from mesenchymal cells and can occur anywhere in the body. Herein, the focus will be on one subtype of sarcoma that arises from adipocytic tissue, liposarcoma. Specifically, the review will focus on one type of liposarcoma, myxoid liposarcoma. Given the rarity of this tumor, it is imperative that these patients are treated at a sarcoma center, where a multidisciplinary approach incorporates all the modalities available including clinical trials. As the understanding of the biology of myxoid liposarcomas progresses, more targeted therapies are being developed that will lead to better tolerated treatments and improved survival for patients. In this review, we will be discussing the pathophysiology, clinical presentation, diagnostic workup, and available treatment options including surgery, radiation, chemotherapy, and clinical trials.

Quantification of fat content in lipid-rich myxoid liposarcomas with MRI: a single-center experience with survival analysis.

OBJECTIVE: To determine the fat content of myxoid liposarcomas (MLS) on MRI and to identify any association between lipid content and survival. MATERIALS AND METHODS: The fat percentage of MLS diagnosed between January 2006 and December 2016 at a single institution was assessed by two radiologists on preoperative MR images. A Cox proportional hazard model was used to determine any association between tumor fat percentage and survival time. Tumor fat percentage was the single predictor in the model. A significance level of 0.05 was used. The Kaplan-Meier estimator was also used to provide a nonparametric estimate of the survivor function within the entire sample and within two patient subgroups consists of lipid-rich and lipid-poor tumors. Lipid-rich tumors were defined as any tumors showing more than 20% of fat on MRI. A 20% cutoff was determined arbitrarily. RESULTS: Of the 43 cases identified through retrospective review, 8 tumors demonstrated ≥10% fat on MRI, and 4 tumors demonstrated ≥20% fat (highest fat percentage, 38%). There was no significant survival difference between patients with high tumor fat, which was defined as ≥20% fat, compared with those with little to no tumor fat. CONCLUSION: Myxoid liposarcomas may demonstrate a higher fat content on MRI than has previously been reported in the literature. Increased tumor fat percentage in lipid-rich tumors was not found to be associated with increased risk of death. Radiologists must be aware of the existence of MLS lesions with higher fat content.

Early detection of metastases using whole-body MRI for initial staging and routine follow-up of myxoid liposarcoma.

OBJECTIVE: To define the role of whole-body MRI (WBMRI) for initial staging and routine follow-up of myxoid liposarcoma (MLS). MATERIALS AND METHODS: A retrospective review of all the patients with MLS who underwent WBMRI for initial staging and routine follow-up at our institution between October 1, 2006, and September 30, 2016 was performed. Patient demographics, clinical presentation, imaging findings, tumor histology, and occurrence and location of metastatic disease were recorded. Thirty-three patients who underwent a total of 150 WBMRI examinations were included in the study. RESULTS: Nine patients (27%) were diagnosed with metastases between 0 and 60 months (median 10; interquartile range, 7-13) from the diagnosis of the primary tumor. The initial site of metastatic disease was extrapulmonary in all patients. Only two patients developed pulmonary metastases, which were diagnosed by CT chest 9 and 29 months after the diagnosis of extrapulmonary metastases. The first metastasis was diagnosed by WBMRI in seven patients (78%), by thoracic CT in one patient, and by abdominal CT in one patient. Eight of nine patients (89%) were asymptomatic at the time of diagnosis of the metastases. In seven patients (78%), WBMRI demonstrated metastases included within the field of view of, but occult on a contemporaneous CT scan. CONCLUSION: Our 10-year institutional experience demonstrates that WBMRI facilitates early detection of extrapulmonary MLS metastases before the onset of clinical symptoms and pulmonary metastases. WBMRI also depicts extrapulmonary metastases that are occult on CT scans. The current surveillance strategies are insufficient for screening for extrapulmonary MLS metastases.

18F-FDG PET/CT and MRI features of myxoid liposarcomas and intramuscular myxomas.

OBJECTIVE: To examine the imaging characteristics of intramuscular myxomas (IM) and myxoid liposarcomas (MLS) on 18F-FDG PET/CT and MRI. MATERIALS AND METHODS: With IRB approval, our institutional imaging database was searched for pathologically proven IM and MLS evaluated by 18F-FDG PET/CT and MRI. PET/CT and MRI imaging characteristics were recorded and correlated with pathologic diagnosis. RESULTS: We found eight patients (2 M, 6 F) with IM (mean age 65.6 ± 10.4 years) and 16 patients (7 F, 9 M) with MLS (mean age 42.8 ± 16.3 years). MRI was available in 7/8 IM and 15/16 MLS patients. There was no significant difference between the two groups in SUVmax (IM 2.7 ± 0.8, MLS 3.0 ± 1.0; p = 0.35), SUVmean (1.7 ± 0.4, 1.5 ± 0.5; p = 0.40), total lesion glycolysis (101.8 ± 127.3, 2420.2 ± 4003.3 cm3*g/ml; p = 0.12), metabolic tumor volume (62.3 ± 71.1, 1742.9 ± 3308.0 cm3; p = 0.17) or CT attenuation (p = 0.70). MLS occurred in younger patients (p = 0.0015), were larger (16.4 ± 8.2 vs. 5.6 ± 2.5 cm; p = 0.0015), more often T1 hyperintense (p = 0.03), with nodular enhancement (p = 0.006), and macroscopic fat on CT (p = 0.0013) and MRI (p = < 0.001) compared to myxomas. CONCLUSIONS: IM and MLS most commonly demonstrate low-grade FDG activity and overlapping metabolic measures on PET/CT. MRI is useful in differentiation, but MLS can present without macroscopic fat on MRI, underscoring the importance of radiologic-pathologic correlation for accurate diagnosis.

MRI characteristics associated with high-grade myxoid liposarcoma.

AIM: To identify magnetic resonance imaging (MRI) features differentiating high-grade (>5% round-cell component) from low-grade myxoid liposarcomas (LPS) (≤5% round-cell component). MATERIALS AND METHODS: Informed consent was waived. Patients with myxoid LPS and MRI before biopsy, neoadjuvant therapy, and surgery were included retrospectively. High-grade components were recorded from histological specimens by a pathologist (24 years of experience). Images were evaluated by a senior radiologist (>12 years of experience) for tumour size, location, tissue layer, and MRI features (signal intensity, heterogeneity, margin, and perilesional characteristics). Descriptive statistics, Fisher's exact test to identify associations with a round-cell component, and multivariate logistic regression to identify independent predictors of high-grade tumours were used. RESULTS: Thirty-one patients (16 women [mean 51.1 years; range 19-79 years] and 15 men [mean 45.5 years; range 18-95 years]) with myxoid LPS (23 low-grade, eight high-grade) were included. All high-grade lesions had lipid signal, a peritumoural capsule and peritumoural contrast enhancement, and more commonly exhibited heterogeneous signal; however, the average size of ≥10 cm was the strongest independent indicator of high-grade status (odds ratio [OR], 14.6; 95% confidence interval [CI]: 1.6, 131). CONCLUSION: Size ≥10 cm is most strongly associated with high-grade myxoid LPS (round-cell component >5%). Other features possibly differentiating high-grade from low-grade status include lesion margin, lipid signal, and perilesional characteristics.

Paratesticular dedifferentiated liposarcoma with prominent myxoid stroma: report of a case and review of the literature.

Paratesticular sarcoma is rare, but liposarcoma is its most common type. Paratesticular liposarcoma sometimes presents as dedifferentiated liposarcoma. Both high-grade and low-grade dedifferentiation have been reported. Herein, we presented a unique case of a 64-year-old man with low-grade dedifferentiated liposarcoma with prominent myxoid stroma. Well-differentiated liposarcoma components extended along the spermatic cord. The constituent cells of the dedifferentiated component were peculiar in that, they were relatively uniform cells with atypia and did not have pleomorphism to such an extent that it mimicked myxofibrosarcoma. This myxoid component was confidently differentiated from myxoid liposarcoma with the help of immunohistochemical analysis using CDK4 and MDM2. These two markers were also expressed in the well-differentiated component. It could therefore be confirmed that this sarcoma is dedifferentiated liposarcoma but is not mixed-type liposarcoma comprising well-differentiated liposarcoma and myxoid liposarcoma.

[Anterior Mediastinal Myxoid Liposarcoma Detected by Chest Computed Tomogram in the Patient with Pneumothorax].

A 57-year-old woman visited our hospital with left chest pain. Chest computed tomography (CT) scanning showed left pneumothorax with apical bullae and a nodular shadow in the left anterior mediastinum accidentally. However, a week later, we could not detect a mediastinal shadow on chest CT image after healing of left pneumothorax. Video assisted thoracoscopic surgery was scheduled in order to remove bullae and evaluate an anterior mediastinal lesion. The mediastinal lesion was tumorous and resected with around pericardial fat tissue. Pathological diagnosis was a myxoid liposarcoma of 15×10mm in size without infiltration into the surrounding tissue. The postoperative course was uneventful without recurrence 6 months later.

Phase II study of amrubicin (SM-5887), a synthetic 9-aminoanthracycline, as first line treatment in patients with metastatic or unresectable soft tissue sarcoma: durable response in myxoid liposarcoma with TLS-CHOP translocation.

PURPOSE: Amrubicin is a third generation synthetic 9-aminoanthracycline that specifically inhibits topoisomerase II. Amrubicin preferentially concentrates in tumor cells leading to tumor cell death without causing cardiac toxicity. This phase II multicenter study was done to evaluate the efficacy and tolerability of amrubicin in advanced soft tissue sarcoma (STS). PATIENTS AND METHODS: 24 eligible patients with chemotherapy-naive metastatic or unresectable STS were treated with amrubicin 40 mg/m(2) intravenously daily for three consecutive days in 21 days cycles with growth factor support. Patients continued to receive treatment, as long as it was tolerated, in the absence of significant disease progression. The disease was followed on imaging scans every 6 weeks. The primary endpoint of the study was the best overall response rate. RESULTS: The best overall response rate was 13% in 23 evaluable patients. Median progression-free survival was 5.8 months, and median overall survival was 26 months. Grade 3 to 4 toxicities of febrile neutropenia and anemia occurred in 21% of treated patients. One patient with metastatic myxoid liposarcoma with TLS-CHOP translocation had a durable response and received 40 cycles of amrubicin. There was no significant cardiac toxicity. CONCLUSIONS: Amrubicin has efficacy comparable to doxorubicin in adult STS, is well tolerated and has no significant cardiac toxicity up to a cumulative dose of 4800 mg /m(2). Topoisomerase II inhibition with amrubicin warrants further study as a potential 'targeted therapy' for TLS-CHOP-translocated myxoid liposarcoma. Results from this trial favor the use of amrubicin for the treatment of STS.

Creating an Intraoperative MRI Suite for the Musculoskeletal Tumor Center.

BACKGROUND: Altered anatomy in a previously irradiated surgical bed can make accurate localization of anatomic landmarks and local recurrence nearly impossible. The use of intraoperative MRI (iMRI) has been described in neurosurgical settings, but to our knowledge, no such description has been made regarding its utility for local recurrence localization in sarcoma surgery. CASE DESCRIPTION: A 58-year-old female presented after previously undergoing two previous resection and reresection procedures of a myxoid liposarcoma located adjacent to her proximal femoral vasculature. After postoperative radiation therapy, she was referred to our institution where she underwent two additional reexcisions of local recurrences during a 3-year span, eventually undergoing a regional rotational muscle flap for coverage. Two years after her third reexcision procedure, she presented with two additional, nonpalpable surgical-bed local recurrences. After converting an MRI bed and scanner to allow for proximal thigh imaging in an iMRI surgical suite, the patient underwent a successful resection that achieved negative margins. To date, she remains without evidence of disease at 37 months. LITERATURE REVIEW: Real-time iMRI in neurosurgical studies has shown a high rate of residual disease leading to immediate subsequent reexcision, thus lending to improved rates of negative margin resection. To our knowledge, this is the first example using iMRI technology to remove a recurrent soft tissue sarcoma that otherwise was clinically nonlocalizable. CLINICAL RELEVANCE: The use of an iMRI surgical suite can aid with identification of soft tissue nodules in conditions such as an altered tumor bed from prior resection and radiotherapy, which otherwise make recurrences difficult to localize. A team approach between administration, surgeons, and engineers is required to design and pragmatically implement the use of an MRI-compatible table extension to enhance existing iMRI surgical suite technology for extremity sarcoma resection procedures.

Myxoid Liposarcoma Originating In The Anterior Mediastinum.

Myxoid liposarcomas of the head and neck are mostly metastatic in nature, with the primary lesion usually in lower extremities or retroperitoneum. Primary mediastinal liposarcomas are even rarer. Although there have been previous cases reporting their incidence, there is no evidence of any case comprising of both the neck and the mediastinal region yet. We here present a case of a 43 year old male with a primary liposarcoma of the myxoid subtype originating in the anterior mediastinum. This is the first time such a case has presented in literature. The patient presented with a right sided neck swelling associated with pain and shortness of breath upon lying down. CT neck revealed an enhancing mass extending from right cervical region to axilla and encasing the subclavian artery and involving a portion of the right lung. The patient underwent wide local excision of the mass along with chemoradiotherapy to prevent recurrence.