Retroperitoneal sarcoma: a 10-year follow-up analysis using hospital-based cancer registry data in Japan.

OBJECTIVES: We sought clinical characteristics, survival outcomes, and prognostic factors for overall survival of retroperitoneal sarcoma in Japan. METHODS: A Japanese hospital-based cancer registry database with a pivotal 10-year follow-up was used to identify and enroll patients, registered from 106 institutions, diagnosed with retroperitoneal sarcoma in 2008-2009. Treating hospitals were divided by hospital care volume; high-volume hospitals and low-volume hospitals were defined as ≥ 4 and < 4 cases/year, respectively. RESULTS: A total of 91 men and 97 women were included, with a median age of 64 years. The most common histological type was liposarcoma in 101 patients, followed by leiomyosarcoma in 38 patients. The 5-year and 10-year overall survival rates were 44.1 and 28.3%. The majority of patients (n = 152, 80.9%) were treated at low-volume hospitals. High-volume hospital patients had higher 10-year overall survival rates than low-volume hospital patients (51.2% vs 23.2%, P = 0.026). Multivariate analysis revealed age over 60 years, treatment in low-volume hospitals and chemotherapy were independent predictors of unfavorable survival while treatment with surgery was an independent predictor of favorable survival. CONCLUSIONS: The possibility of surgical removal was suggested to be the most important prognostic factor for retroperitoneal sarcoma. Better survival was shown in patients treated at high-volume hospitals in our series.

Orbital Liposarcoma: A Surveillance, Epidemiology and End Results Database Study.

PURPOSE: Orbital liposarcoma is a challenging tumor to treat due to its rarity and high rate of local recurrence, and the role of radiotherapy and chemotherapy remain unclear. Analysis of big data may improve our overall understanding of orbital disease and role of adjuvant therapies. METHODS: Data were extracted from the Surveillance, Epidemiology and End Results (SEER) Research Plus database from 1975 to 2017. All patients with a diagnosis of liposarcoma (ICD-O3 codes 8850-8858, 8869-8862, 8870, 8880, 8881) were included. Cases were divided into 4 groups by primary site: orbit, retroperitoneum, soft tissue, and other. RESULTS: A total of 16,958 patients were included. Patients with orbital involvement were younger and more likely to be female ( p < 0.05). Among orbital lesions, myxoid liposarcoma was the most common histologic subtype (6/19; 31.6%) followed by well differentiated (5/19; 26.3%). This differed from the distribution of histologic subtypes encountered elsewhere, for which well-differentiated liposarcoma was the most common (retroperitoneum 979/3,136; 31%, soft tissue 3,493/11,671; 30%, and other sites 497/2,132; 23%, p < 0.05). Dedifferentiated histologic subtype was the second most common subtype found in the retroperitoneum (946/3,136; 30%), whereas it was less common in the orbit (2/19; 11%) and soft tissue (1,396/11,671; 12%) ( p < 0.001). Patients with orbital liposarcoma had similar disease-specific mortality compared with soft-tissue location ( p = 0.825) and lower disease-specific mortality compared with retroperitoneal location ( p < 0.001). When all locations were combined, patients with well-differentiated liposarcoma had the lowest disease-specific mortality. CONCLUSIONS: Patients with orbital liposarcoma tend to be younger, female, and have a better prognosis than those with retroperitoneal disease, likely due to the lower incidence of dedifferentiated histologic subtype.

Effects of marital status on survival of retroperitoneal liposarcomas stratified by age and sex: A population-based study.

BACKGROUND: Previous studies have shown that marital status is associated with survival in patients with a variety of cancer types, including lung cancer, prostate cancer, and bladder cancer. However, to date, the impact of marital status on the survival of patients with retroperitoneal liposarcomas (RPLs) has not been established. METHODS: A total of 1211 eligible patients diagnosed with RPLs were identified in the Surveillance, Epidemiology, and End Results (SEER) database. The relationships between marital status and survival in patients with RPLs were assessed. Patients were stratified by age to determine whether an association exists between marital status and age. We also probed the association between marital status and survival in males and females. RESULTS: Our findings suggest that divorced, separated, or widowed patients have more advanced cancer stages, and more of these patients do not undergo surgery. Meanwhile, divorced, separated, or widowed patients have worse survival outcomes than married patients (overall survival (OS): HR = 1.66 (95% CI, 1.12, 2.46)); cancer-specific survival (CSS): HR = 1.90 (95% CI, 1.13, 3.19)). OS does not differ between single patients and married patients (HR = 1.21 [95% CI, 0.81, 1.81]) or CSS (HR = 1.36 [95% CI, 0.80, 2.29]). In addition, these results demonstrate that being divorced, separated, or widowed can play a significant detrimental role in mortality in older and female patients. CONCLUSION: Married patients have earlier disease stages at diagnosis and better survival outcomes than divorced, separated, or widowed patients with RPLs. In addition, this effect is especially pronounced in older people and females.

Histologic Subtype Defines the Risk and Kinetics of Recurrence and Death for Primary Extremity/Truncal Liposarcoma.

OBJECTIVE: We sought to define the prognostic significance of histologic subtype for extremity/truncal liposarcoma (LPS). BACKGROUND: LPS, the most common sarcoma, is comprised of 5 histologic subtypes. Despite their distinct behaviors, LPS outcomes are frequently reported as a single entity. METHODS: We analyzed data on all patients from a single-institution prospective database treated from July 1982 to September 2017 for primary, nonmetastatic, extremity or truncal LPS of known subtype. Clinicopathologic variables were tested using competing risk analyses for association with disease-specific death (DSD), distant recurrence (DR), and local recurrence (LR). RESULTS: Among 1001 patients, median follow-up in survivors was 5.4 years. Tumor size and subtype were independently associated with DSD and DR. Size, subtype, and R1 resection were independently associated with LR. DR was most frequent among pleomorphic and round cell LPS; the former recurred early (43% by 3 years), and the latter over a longer period (23%, 3 years; 37%, 10 years). LR was most common in dedifferentiated LPS, in which it occurred early (24%, 3 years; 33%, 5 years), followed by pleomorphic LPS (18%, 3 years; 25%, 10 years). CONCLUSIONS: Histologic subtype is the factor most strongly associated with DSD, DR, and LR in extremity/truncal LPS. Both risk and timing of adverse outcomes vary by subtype. These data may guide selective use of systemic therapy for patients with round cell and pleomorphic LPS, which carry a high risk of DR, and radiotherapy for LPS subtypes at high risk of LR when treated with surgery alone.

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001-2016.

Rare cancers, affecting 1 in 5 cancer patients, disproportionally contribute to cancer mortality. This research focuses on liposarcoma, an understudied rare cancer with unknown risk factors and limited treatment options. Liposarcoma incident cases were identified from the U.S. Surveillance, Epidemiology, and End Result (SEER) program and the combined SEER-National Program of Cancer Registries (CNPCR) between 2001-2016. Incidence rates (age-adjusted and age-specific), 5-year survival, and the time trends were determined using SEER*stat software. Three-dimensional visualization of age-time curves was conducted for males and females. SEER liposarcoma cases represented ~30% (n = 11,162) of the nationwide pool (N = 37,499). Both sources of data showed males accounting for ~60% of the cases; 82%-86% cases were identified among whites. Age-adjusted incidence was greater among males vs. females and whites vs. blacks, whereas survival did not differ by sex and race. The dedifferentiated (57.2%), pleomorphic (64.1%), and retroperitoneal (63.9%) tumors had the worse survival. Nationwide, liposarcoma rates increased by 19%, with the annual percent increase (APC) of 1.43% (95% confidence interval (CI): 1.12-1.74). The APC was greater for males vs. females (1.67% vs. 0.89%) and retroperitoneal vs. extremity tumors (1.94% vs. 0.58%). Thus, incidence increased faster in the high-risk subgroup (males), and for retroperitoneal tumors, the low-survival subtype. The SEER generally over-estimated the rates and time trends compared to nationwide data but under-estimated time trends for retroperitoneal tumors. The time trends suggest an interaction between genetic and non-genetic modifiable risk factors may play a role in the etiology of this malignancy. Differences between SEER and CNCPR findings emphasize the need for nationwide cancer surveillance.

Liposarcoma of oral cavity: Systematic review of cases reported to date and analysis of prognostic factors.

The aim of this study was to integrate the available data published on Liposarcomas (LPSs) of the oral cavity into an analysis of its clinical features, treatment modalities, and prognostic factors. An electronic search was undertaken in January 2019. The eligibility criteria included publications that had enough clinical and histological information to confirm the diagnosis. Forty-five publications (104 LPSs) were included. The lesion was more prevalent in males from the fifth to seventh decades of life. Treatment (P = .03) and distant metastasis (P = .0001) were independently associated with survival. A lower possibility of recurrence was statistically associated with age (younger patients) (P = .03), tumor size (smaller than 2.8 cm) (P = .001), and treatment (radical surgery) (P = .04). LPS presents a good survival for patients after 5 years of follow-up (66.4%). Patients who were treated with conservative surgery and presented with distant metastasis showed poor prognosis.

Maximum standardized uptake value on 18F-fluorodeoxyglucose positron emission tomography/computed tomography improves outcome prediction in retroperitoneal liposarcoma.

While 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) has been investigated in extremity sarcomas, there is no evidence on its usefulness in retroperitoneal sarcoma. This study was designed to evaluate the usefulness of 18F-FDG PET/CT in predicting aggressiveness of retroperitoneal liposarcoma. Patients experienced surgery for retroperitoneal liposarcoma from November 2007 to February 2018 and underwent preoperative 18F-FDG PET/CT were included. Preoperative maximum standardized uptake value (SUVmax) was calculated. To evaluate the predictability of SUVmax for Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade 3, receiver operating characteristics (ROC) curve analysis was performed. To analyze whether SUVmax can be a risk factor for prognosis, multivariable Cox regression was performed including potential risk factors regarding operation and histopathology. A total of 133 patients were included. ROC curve showed area under the curve of 0.877 (P < 0.001), with a cut-off point of 4.5 SUVmax showing 85.7% sensitivity and 78.3% specificity. Cox analyses showed that SUVmax > 4.5 was a significant factor for recurrence-free survival (HR = 2.148, CI 1.301-3.546, P = 0.003) and overall survival (HR = 5.052, CI 1.854-13.766, P = 0.002). SUVmax is highly predictive of FNCLCC grade 3 and SUVmax > 4.5 can be used as a prognostic factor before obtaining the histopathology.

Pharmacotherapy for liposarcoma: current state of the art and emerging systemic treatments.

Introduction: Liposarcomas are a heterogeneous group of soft tissue tumors that arise from adipose tissue and are one of the most common soft tissue sarcomas found in adults. Liposarcomas are subclassified into four subtypes with distinct histologic and biologic features that influence their treatment and management. Areas covered: This manuscript reviews the key clinicopathologic and cytogenic characteristics of the liposarcoma histologic subtypes and summarizes the results of recent clinical trials, treatment options, and future directions in the pharmacotherapy for the management of liposarcoma. Expert opinion: Despite significant advancements in the management of this disease, the treatment of liposarcoma continues to be a challenge. Surgical resection remains the mainstay of treatment for localized disease; however, use of systemic therapies in conjunction with surgery may be considered in patients where tumor shrinkage could reduce surgical morbidity and in patients with high-risk of micrometastatic disease. Anthracycline-based chemotherapy regimens remain the standard first-line treatment for unresectable/metastatic liposarcoma. Trabectedin and eribulin are currently the two most promising and evidenced-based second-line treatment options for liposarcomas. However, multiple clinical trials dedicated to patients with liposarcoma evaluating novel targeted agents are ongoing. Every effort should be made to enroll patients with liposarcoma into histotype-specific clinical trials.

Atypical Lipomatous Tumors: Does Our Inconsistent Terminology Have Patient Repercussions? Results of a Meta-Analysis.

OBJECTIVES: Misnaming low-grade lipomatous tumors poses a clinical and medicolegal challenge, potentially subjecting patients to expensive and unnecessary surgeries. The terms atypical lipomatous tumor (ALT) and "well-differentiated" liposarcoma (WDL) have been used interchangeably in pathology reports, scholarly works and consensus recommendations, creating vagaries between low-virulence extremity tumors and retroperitoneal disease with metastatic potential. METHODS: A systematic review was performed on all studies that reported on the local recurrence rate and metastasis of ALTs and WDLs in living human subjects. Local recurrence and metastases were compared using Fisher's Exact Test. RESULTS: In total, 20 studies evaluated ALTs (n=936), whereas 13 studied WDLs (n=626). Mean follow-up was 6.6±2.0 years (median, 7.0 y). No metastatic disease was observed among ALTs, whereas 15 patients with WDLs (2.7%, P<0.0001) had metastases. The local recurrence rate of ALTs was significantly lower than WDLs after both marginal (15.1%, 141/936 vs. 46.0%, 288/626, P<0.0001) and wide excisions (3.3%, 2/59 in ALT vs. 17.4%, 19/109, P=0.007). CONCLUSIONS: ALT should be reserved for extremity lesions meeting appropriate histopathologic criteria that represent nonmetastatic disease, reducing over-diagnosis, over-treatment, and patient risk.

Role of nutritional status in the early postoperative prognosis of patients operated for retroperitoneal liposarcoma (RLS): A single center experience.

PURPOSE: To assess the nutritional status and its role in the outcome of patients operated for retroperitoneal liposarcoma (RLS). MATERIAL AND METHODS: Retrospective study on consecutive patients operated with en bloc compartment resection for primary or local recurrence of RLS between 2016 and 2017. Preoperative nutritional and laboratory assessment comprising serum albumin, serum transthyretin, orosomucoid, and CRP was systematically performed. The following preoperative parameters were analysed: weight, body mass index (BMI), significant weight loss (>5% in one month and/or >10% in 6 months), serum albumin, transthyretin, CRP, orosomucoid. PINI (prognostic inflammatory and nutritional index) was calculated. RESULTS: There were 40 patients operated for RLS: 22 women and 18 men with a median age of 61 years (34-90). Median tumour was 280 mm (80-530). Median preoperative BMI was 24.8 (18-42) and median postoperative BMI was 23 (17.8-44). Twenty-one patients (52.5%) were considered to be malnourished: 3 with biological signs of malnutrition and 18 with weight loss. Eleven (47.6%) in the group of malnourished patients and 4 (26.3%) in the group with satisfactory nutritional status developed postoperative complications (p = 0.042). A PINI score>1 was related to significantly longer hospitalisation time 21.8 days (10-58) in comparison with 14.9 [9-30] in patients with PINI < 1, p = 0.003. CONCLUSIONS: The malnourished patients with RLS experienced more postoperative complications and longer hospitalisation. Nutritional status and biological markers contribute to the global management of RLS with improved postoperative behaviour including fewer complications and shorter hospitalisation. A prospective larger study with longer follow-up is necessary to refine these results.

Risk factors of early recurrence in retroperitoneal liposarcoma. / Factores de riesgo implicados en la recurrencia precoz del liposarcoma retroperitoneal.

INTRODUCTION: The aim of this study is to identify factors associated to recurrence and survival in primary retroperitoneal liposarcomas. METHODS: Prospective database of 35 patients with primary retroperitoneal liposarcoma treated 2004-2015 were retrospectively analyzed. Exclusion criteria were recurrent and metastatic tumors. Overall survival (OS) and disease-free survival were reviewed. Patient data were compared between patients with or without recurrence within 12 months after surgery. Risk factors were determined using logistic regression analysis. RESULTS: Five-year OS was 61.1%. One and three-year disease-free survival were 68.6% and 17.1% respectively. OS in the early recurrence group was 36.4 months compared with 43.2 months in the group without early recurrence (P=.011). Early recurrence was associated with a reduction in OS (HR=4.05; CI95%: 1.27-12.96; P=.018). Multifocality and microscopic positive margins R1 were associated with early recurrence. Histologic subtype, margin of resection, histologic grade and multifocality were factors associated with recurrence. Contiguously involved organ resection had a beneficial effect on early recurrence and was associated with an increase in disease-free survival and OS. Adjuvant treatments had no protective effect on recurrence. CONCLUSIONS: This study underlines the crucial role aggressive surgical approach in retroperitoneal Liposarcoma treatment, especially in those patients with histological characteristics that adversely the prognosis.

Liposarcoma in a Backyard Silkie and Retrospective Summary of Neoplasms Diagnosed in Backyard Chickens Submitted to the California Animal Health and Food Safety Laboratory System, 2008-2017.

Liposarcomas are a malignant neoplasm of adipocytes, and are rarely diagnosed in avian species. This case report describes the evidence supporting a diagnosis of metastatic liposarcoma in a backyard silkie chicken. On September 28, 2017, a dead 3-yr-old backyard silkie chicken, with a history of unknown skin lesions involving the entire body and severe weight loss, was submitted to California Animal Health and Food Safety Laboratory System-Turlock branch for necropsy. At necropsy, raised necrotic lesions involving the majority of the skin and multiple nodules in the liver, spleen, and bone marrow were noticed. Microscopically, stellate, spindle, and myxoid cells containing large vacuoles, which were confirmed as lipid droplets by Oil Red O, were observed infiltrating the dermis and underlying a necrotic epidermis, with metastasis to liver, spleen, bone marrow, and ovary being the most significant findings. PAS, Oil Red O, Ziehl-Neelsen, Congo red, Gram, and Von Kossa stains, along with immunohistochemistry for pan cytokeratin, vimentin, S100, CD3, pp38, and Meq were used to classify the lesions. Intensely positive vimentin immunohistochemistry, along with large quantities of Oil Red O-positive lipid droplets within the neoplastic cells, were supportive of our diagnosis of liposarcoma. The incidence of neoplastic diseases diagnosed in backyard flock submissions to CAHFS system wide from 2008 to 2017 was also reviewed.

Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma.

Soft tissue sarcoma is an umbrella term which encompasses over 60 histological tumour types. Approximately 15% of soft tissue sarcomas arise in the retroperitoneum. This complex group of tumours poses unique management challenges due to their often large size, histological heterogeneity and complexity of anatomical relationships. This review discusses the management of retroperitoneal tumours including the need for preoperative diagnosis, the evidence for neoadjuvant radiotherapy, the role of multivisceral resection and the importance of a multidisciplinary team approach.

Demographics, management and treatment outcomes of benign and malignant retroperitoneal tumors in Japan.

OBJECTIVES: To show the demographics, type of treatment and clinical outcomes of patients with retroperitoneal tumors in Japan. METHODS: We carried out a retrospective analysis of patients with retroperitoneal tumors treated between 2000 and 2012 at 12 university hospitals in Japan. Histology was re-evaluated using the 2013 World Health Organization classification. RESULTS: A total of 167 patients were included in the analysis. The number of diagnosed patients increased over the 12-year study period. Liposarcoma and schwannoma were the most common histological types among intermediate/malignant and benign tumors, respectively. The intermediate/malignant tumors were larger and were more frequently found in older people. Surgical resection was the primary treatment for 151 patients. The median survival duration for patients with malignant tumors was 91 months, and was significantly shorter than that for patients with benign and intermediate tumors (P < 0.01). R2 resection was associated with significantly shorter survival than R0/R1 resection for malignant tumors (P < 0.01), but not for intermediate. Grossly complete resection of the recurrent tumors improved survival. CONCLUSION: The number of patients diagnosed with retroperitoneal tumors increased over time. R2 resection of primary tumors was found to be associated with poor prognosis in malignant tumors, but not in intermediate tumors. Complete surgical resection of recurrent tumors was associated with a better oncological outcome.

[Soft tissue tumors : Epidemiology, classification and staging]. / Weichteiltumoren : Epidemiologie, Klassifikation und Stadieneinteilung.

Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma. Furthermore, two new subgroups were incorporated, the nerve sheath tumors and gastrointestinal stromal tumors. These were previously included in the tumor classification of other organ systems. These changes in the new classification are related to the rapid increase in knowledge of the genetics and the cell biology of soft tissue tumors. Malignant soft tissue tumors only represent 1% of all malignant tumors in adults. The largest subgroup of soft tissue tumors in adults is the adipocytic tumors. The liposarcoma, which belongs to this subgroup is one of the most common malignant soft tissue tumors in adults. In childhood malignant soft tissue tumors represent 15% of malignant tumors and rhabdomyosarcoma is the most common malignant soft tissue tumor.

[An analysis of incidence trends and characteristics of soft tissue sarcoma in Beijing, 1999-2013].

Objective: To analyze the incidence trends and to describe the characteristics of soft tissue sarcoma (STS) among residents in Beijing from 1999 to 2013. Methods: Medical information of the cases diagnosed as STS(ICD10: C47&C49) from 1999 to 2013 in Beijing was extracted from the population-based database of Beijing Cancer Registry.Crude incidence rate, age-standardized incidence rates to Chinese population (ASRC)and the world population(ASRW) were calculated. The characteristics of the STS cases in different gender and age groups from urban and rural areas were respectively analyzed.The incidence trends and annual percentage changes (APC) during last 15 years were analyzed by using JoinPoint 3.4.3 software. Results: A total of 2 048 cases were diagnosed as STS during the study period and the incidence rate of STS was 1.15 per 100 000 person-years. The ASRC was 0.74 per 100 000 person-years and ASRW was 0.86 per 100 000 person-years, respectively. Except for the impact of changes in the age composition, the APC of ASRW from 1999 to 2013 was 3.95%. For males, the incidence rate was increased from 0.65 per 100 000 in 1999 to 1.51 per 100 000 in 2013, and the APC was 4.27% (P<0.05); For females, the incidence rate was increased from 0.61 per 100 000 in 1999 to 0.91 per 100 000 in 2013, and the APC was 3.38% (P<0.05). In urban area, the incidence rate increased from 1.14 per 100 000 in 2000 to 1.35 per 100 000 in 2013, and the APC was 2.70% (P=0.05); In rural area, the incidence rate increased from 0.57 per 100 000 in 2000 to 0.98 per 100 000 in 2013, and the APC was 4.66% (P=0.04). 85.89%cases were pathologically diagnosed.Among them, undifferentiated pleomorphic sarcoma was the most common subtype, which accounting for 19.22%, followed by liposarcoma (19.04%), malignantnerve sheath tumor (10.18%), fibrosarcoma (10.06%)and sarcoma without definite type(7.22%). Conclusions: The incidence rate of STS is still increasing, especially in the rural area of Beijing. The most common subtypes of STS in different age-groups were different. Further study is needed to explore the high risks of STS in the population with specific characteristics.

[Primary breast sarcomas: About 30 cases treated at Salah-Azaiez institute in Tunisia]. / Sarcomes mammaires primitifs : à propos de 30 cas traités à l'institut Salah-Azaiez de Tunis.

PURPOSE: To identify retrospectively prognostic factors of primary breast sarcoma and review its treatment modalities. MATERIALS AND METHODS: This is a descriptive study on 30 cases of primary breast sarcoma. We carried out a univariate and multivariate analysis correlating clinical, pathological and therapeutic parameters with disease-free survival and overall survival. RESULTS: The mean age was 46.8 years. The mean tumour size was 10cm. The 30 cases were 18 phyllodes sarcomas, eight angiosarcomas, three liposarcomas and a case of granulocytic sarcoma. Sixteen patients had adjuvant radiotherapy and only seven patients received adjuvant chemotherapy. The median follow-up was 64 months. Overall survival rates at 3 and 5 years were 49.1% and 33.7%. Disease-free survival rates at 3 and 5 years were 22.8% and 15.2% respectively. The analytical study of the following parameters: tumour size and presence or absence of node or distant metastases, showed no correlation with overall survival nor with disease-free survival. Furthermore, adjuvant radiotherapy did not improve overall survival (P=0.298; hazard ratio [HR]=1 [0.982-1.04]) nor disease-free survival (P=0.61; HR=0.942 [0.862-1.029]). By univariate analyses, we identified a correlation between overall survival, surgical margins (>1cm) (P=0005; HR=3.4 [1.217-9.919]) and tumour necrosis (P=0.028; HR=0.099 [0.014-0.682]). We did not find any independent prognostic factor by multivariate analysis. CONCLUSION: The prognosis of primary breast sarcoma seems to depend essentially on optimal surgical excision (margin over 1cm). The only potential histological parameter correlated with the prognosis is the presence of tumour necrosis. The histological subtype should not be considered as a prognostic marker for overall or disease-free survival in patients with primary breast sarcoma.

Histological types of soft-tissue sarcomas at the lagos university teaching hospital.

OBJECTIVE: There is scanty data on histologically diagnosed soft-tissue sarcomas in the Nigerian literature. This is due to paucity of facilities for ancillary testing as well as a dearth of specialist soft tissue pathologists. Knowledge however of the common soft-tissue sarcomas is vital for the establishment of an effective sarcoma service. The aim of this study, therefore, was to determine the histological spectrum of soft-tissue sarcomas in Lagos, Nigeria. MATERIALS AND METHODS: Archival haematoxylin and eosin (H and E)-stained slides were retrieved and reviewed by a team of soft-tissue pathologists at the Royal National Orthopaedic Hospital, London, UK. Immunohistochemistry and fluorescent in situ hybridisation studies were performed on cases without definitive diagnosis on routine H and E. RESULTS: Fifty-two cases were studied. The male-to-female ratio was 1:1.3, with a median age of 33 years. Most sarcomas (57.5%) were of intermediate malignant potential according to 2013 World Health Organization classification. Kaposi sarcoma (37.5%), undifferentiated sarcomas (22.5%), dermatofibrosarcoma protuberans (15%) and myxofibrosarcomas (7.5%) were the most common sarcomas seen in adults. There was no case of liposarcoma. Sarcomas in the younger age group (<20 years) accounted for 23.1% of cases with embryonal rhabdomyosarcoma accounting for the majority. CONCLUSION: Soft-tissue sarcomas in adults in Lagos Nigeria show a different morphologic spectrum than those reported in Western countries.

MDM2/CDK4 gene amplification in large/deep-seated 'lipomas': incidence, predictors and clinical significance.

This study of 140 cases assessed the incidence of MDM2/CDK4 gene amplification in lipomatous neoplasms with histological features of a lipoma but which were of clinical concern due to large size (≥50 mm) and/or deep-seated (subfascial) location. Univariate and multivariate statistical analyses were used to identify clinical, radiological and pathological predictors of gene amplification. Differences in local recurrence rates between amplified and non-amplified cases were assessed using survival analysis. The findings indicate that the incidence of MDM2/CDK4 amplification in this setting is low at 5% (95%CI 1.4-8.6%). Variables associated with amplification on univariate analysis were tumour site (thigh, p = 0.004), size (>100 mm, p = 0.033) and presence of equivocal atypia (p = 0.001). Independent predictors on multivariate analysis were size (OR 3.9, 95%CI 1.4-11.3, p = 0.012) and presence of equivocal atypia (OR 12.5, 95%CI 1.9-80.3, p = 0.008). There was no significant difference in local recurrence rates between amplified and non-amplified cases (p = 0.461) based on a median follow-up time of 31 months. Assessment for MDM2/CDK4 amplification, therefore, should be considered in 'lipomas' which are >100 mm in size, show equivocal atypia and arise in the thigh. However, the clinical significance of gene amplification in this setting is unclear and requires confirmation in larger studies.