Sarcoma Family Research Articles

111 Primary Intracranial Ewing Sarcoma of the Mastoid Bone Mimicking an Inflammatory Process in a 5-Year-Old

Ewing sarcoma (ES) family of tumors (EFTs) of the head and neck are rare and account for only 1% to 4% of all EFTs. The head and neck is rarely involved as the primary site; an even rarer subset of EFTs is the ES involving the mastoid bone, for which there are only two reported cases based on our review of the world literature. A 5-year-old boy presented with fever and rapidly enlarging painful mass over the right mastoid region complicated by frontal headaches, right ear pain, and decreased hearing on the right side. The mass over the mastoid region measured 4.0 x 6.0 cm, and was hard, non-erythematous, and tender to palpation. MRI revealed lentiform epidural fluid collection with thick peripheral enhancement extending along the margins of mastoid into the right middle and posterior cranial fossa. Pathological evaluation showed small round blue cell proliferation embedded in fragments of trabecular bone. The tumor cells show patchy granular positivity for CD99. Periodic acid-Schiff (PAS) and PAS-D stains show glycogen granules in tumor cells. Tumor cells were negative for AE1/AE3, CD45, and desmin. Fluorescence in situ hybridization analysis confirmed that tumor cells are positive for EWSR1 gene rearrangement at chromosome 22q12. The EFT is a high-grade tumor, with local invasion and a strong tendency to metastasize. Identification of molecular characteristics of the ES/PNET family has contributed to clinical care by providing a greater degree of confidence for an often difficult diagnostic problem. Further understanding of the basic mechanisms of these tumor-specific molecules has the potential to lead to novel therapies. Given its rarity for affecting the skull, particularly the mastoid bone, our findings add to our global understanding of this uncommon subset of patients affected by ES.

Primary Vulvar Ewing Sarcoma in a 30-Year-Old Woman: A Case Report

Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered as Ewing sarcoma family of tumors (ESFT), characterized by chromosomal translocation t(11; 22) (q24; q12) leading to a chimeric transcript EWS-FLI1 in 85% of cases. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities in children, adolescents and young adults. It rarely occurs in the female genital tract. We report an extremely rare case of advanced vulvar Ewing sarcoma/PNET of the vulva confirmed by Fluorescence In Situ Hybridization (FISH) in a 30-year-old woman. The patient was treated by 6 cycles of chemotherapy followed by radiotherapy with favourable outcome.

Prognostic factors and survival in patients under 18 years of age with Ewing sarcoma family tumors: a 10-year experience

Objective: To describe the clinical and epidemiological characteristics, and to determine the prognostic factors, event-free survival (EFS) and overall survival (OS) of patients with Ewing Sarcoma family of tumors (ESFT). Materials and methods: retrospective study carried out in patients under 18 years of age with TFSE, seen at the Pediatric and Adolescent Oncology Unit of the Edgardo Rebagliati Hospital between 2006 and 2016. The descriptive analysis was performed by frequency distribution. Kaplan-Meier curves were used for the analysis of SLE and OS. Univariate and multivariate analysis was performed according to Cox regression model for demographic, clinical and surgical variables, and prognostic factors. The measure of strength of association was expressed as hazard ratio (HR) and 95% confidence interval (95% CI), and p<0.05 was considered for significant differences. Results: Twenty-nine cases of TFSE were presented. The median age was 9 years (range 2-17), 55% were male. The most frequent location was the pelvis (31%). Fifty-nine percent had metastases at diagnosis. The 3-year EFS in localized ESFT was 40.4% (±14.4 ES) and with metastases, 14.6% (±12.2, ES). The 3-year OS in localized TFSE was 53.9% (±17.8 ES) and in metastatic disease, 15.1% (±9.7, ES). Tumor size ≥5cm (HR 14.84, p=0.01) and the presence of metastases at debut (HR 3.23, p=0.01) were independent prognostic factors for worse OS. There was no significant difference in relation to prognosis according to sex, age, histologic type, surgical edge involvement or tumor location. Conclusions: ESFTs are highly aggressive. Prognostic factors contributing to lower EFS and OS are the presence of metastases at disease debut and tumor size ≥5cm.

Loss of MTAP expression is a negative prognostic marker in Ewing sarcoma family of tumors.

The Ewing sarcoma family of tumors (ESFT) is a group of malignant small round cell neoplasms of bones and soft tissues closely histogenetically related. Methylthioadenosine phosphorylase (MTAP) deficiency has been recently associated with increased tumor aggressiveness and poor outcomes in different types of neoplasms. However, the expression of this biomarker and its biological role in ESFT remain largely unknown. Immunohistochemical expression of MTAP was accessed in 112 patients with ESFT in a tissue microarray platform and associated with clinicopathological parameters and overall survival(OS). Loss of MTAP expression was significantly associated with lower OS in both univariate and multivariate analyses. Loss of MTAP expression is an independent negative prognostic biomarker in ESFT.

Sarcoma family kinase activity is required for cortical spreading depression.

Objectives Sarcoma family kinase activity is associated with multiple diseases including ischemia and cancer; however, its role in the mechanism of migraine aura has been less well characterised. This study aims to investigate whether sarcoma family kinase is required for cortical spreading depression. Methods Cortical spreading depression was induced by topical application of K+ to the cerebral cortex and was monitored using electrophysiology in rats, and intrinsic optical signal in mouse brain slices. Drugs were perfused into the contralateral cerebral ventricle for pharmacological manipulations in rats. Western blot analysis was used for detecting the level of phosphorylated, and total, sarcoma family kinase in the ipsilateral cortex of rats. Key results The data demonstrate that a single cortical spreading depression in rats induced ipsilateral cortical sarcoma family kinase phosphorylation at the Y416 site. Deactivation of sarcoma family kinase by its inhibitor (3-(4-chlorophenyl) 1-(1,1-dimethylethyl)-1 H-pyrazolo[3,4- dpyrimidin-4-amine) suppressed the elevated enzyme activity and cortical susceptibility to cortical spreading depression. Interestingly, the inhibitory effect of the N-methyl-D-aspartate receptor antagonist NVP-AAM077 on cortical spreading depression was reversed by the sarcoma family kinase activator pYEEI (EPQY(PO3H2)EEEIPIYL), suggesting a link between this enzyme and N-methyl-D-aspartate receptors. Similarly, after deactivation of sarcoma family kinase, a reduction of sarcoma family kinase phosphorylation and cortical susceptibility to cortical spreading depression was observed with NVP-AAM077. Conclusions We conclude that activation of sarcoma family kinase is required for cortical spreading depression, and this process is regulated by recruiting N-methyl-D-aspartate receptors. This study provides novel insight for sarcoma family kinase function in the mechanism of migraine aura.

Primary orbital Ewing sarcoma family of tumors: a study of 12 cases.

PurposeThe purpose of this study is to discuss the clinical presentation, management, and outcomes of patients with primary orbital Ewing sarcoma family of tumors (ESFTs).Patients and methodsRetrospective study of 12 patients with biopsy-proven primary orbital ESFT.ResultsThe mean age at presentation of primary orbital ESFT was 12 years (median, 8 years; range, 5 months to 28 years). There were seven (58%) females and five (42%) males. The presenting complaints included proptosis (n=10; 83%) and swelling in the upper eyelid (n=2; 17%). The mean duration of symptoms was 9 weeks (median, 5 weeks; range, 2-24 weeks). Tumor epicenter was located in the superior orbit (n=6; 50%), lateral orbit (n=3; 25%), inferior orbit (n=2; 17%), and medial orbit (n=1; 8%). Computed tomography of the orbits revealed predominant bony lesion (n=10; 83%) or isolated soft tissue/extraosseous lesion (n=2, 17%). At presentation, extraorbital extension was noticed in 10 patients including intra cranial extension (n=7; 58%), extension into temporal fossa (n=4; 33%), nasal cavity (n=2; 17%), maxillary sinus (n=2; 17%), and ethmoid sinus (n=1, 8%). Systemic metastases at presentation was detected in five (42%) patients involving the bone marrow (n=4; 33%), kidney (n=1; 8%), and retroperitoneal lymphnode (n=1; 8%). Multi-modal treatment including a combination of neoadjuvant chemotherapy, excision biopsy/debulking, and/or radiotherapy was given. Over a mean follow-up period of 21 months (median, 7 months; range, 1-152 months), disease-related death occurred in 11 (92%) cases.ConclusionPrimary orbital ESFT is aggressive at presentation and is associated with poor prognosis.

A Retrospective Multicentric Study of Ewing Sarcoma Family of Tumors in Patients Older Than 50: Management and Outcome

Ewing’s sarcoma family of tumors (EFTs) is a group of rare and aggressive tumors. Data on EFTs in patients (pts) ≥ 50 years are limited and these pts are often not eligible for clinical trials. Some, but not all, studies have reported inferior outcome for older pts with EFTs. We conducted an IRB-approved retrospective analysis among centers of the French Sarcoma Group on pts diagnosed with EFTs at age ≥50 between 2000 and 2012. Clinical features, treatment modality and outcomes were analyzed. Seventy-seven pts were identified, including 36 females (46.8%) and the median age at diagnosis was 56 years (range: 50–86). The primary tumor was located in soft tissue in 59 pts (76.6%). Fifty-six pts (72.7%) had localized disease, among them 49 (87.5%) received chemotherapy in addition to local therapy. Their estimated 3-yr OS and event-free survival (EFS) rates were respectively 73.3% and 62.2%. Recurrence occurred in 43 pts. The estimated 3-yr OS rate was 37% in pts with metastatic disease at presentation. EFTs in pts ≥50 years are more likely to originate from soft tissue and their outcomes appear to be worse than that of younger pts treated with modern protocols.

Pediatric Tumors: Extraskeletal Ewing Sarcoma

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references. Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22)

Pediatric Tumors: Extraskeletal Ewing Sarcoma

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references. Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22)

1523P - Efficacy of second line treatment with etoposide and ifosfamide in adult patients with advanced Ewing Sarcoma family tumors

Background: Ewing Sarcoma family tumors (ES) are rare subtypes of sarcomas, and even less common in adult patients. For those not amenable to treatment with curative intent, sequential therapy with multi-agent combinations is the standard of care, usually followed by ifosfamide/etoposide (IE) at the time of progression, largely based on protocols that included pediatric patients. Nevertheless, less is known about the efficacy of this approach for adult patients with ES refractory to first-line therapy. Methods: We assembled a retrospective cohort of patients aged 18 or older diagnosed with metastatic/inoperable ES refractory to first-line combinations, treated with IE between 2010 and 2016. Patient´s characteristics, tumor variables, treatment outcomes and toxicity data were evaluated. Kaplan-Meier method was used to estimate overall survival and uni/multivariate analysis were carried out to identify factors associated with survival. Results: Among 18 adult patients, the mean age of diagnosis was 22 years, 73% were male and 84% had an ECOG of 0-1 at commencement of IE. Pelvis and thorax were the most common primary sites. The mean number of cycles of IE was 4. The disease control rate was 27%, with partial responses occurring in 16% of the patients (there were no complete responses). The median OS was 4,8 months (IC 95% 0,7-8,8). Toxicities ³ grade 3 occurred in 61% of the patient, including two treatment-related deaths. The main grade 4 toxicity was febrile neutropenia. Hospitalization were required in 55% of the cases. Conclusions: IE has limited efficacy and significant toxicity when used in the second-line setting for adult patients with advanced ES, and different approaches should be investigated for these patients. Legal entity responsible for the study: Instituto do Câncer do Estado de São Paulo Funding: None Disclosure: All authors have declared no conflicts of interest.

Immunohistochemical analysis of NKX2.2, ETV4, and BCOR in a large series of genetically confirmed Ewing sarcoma family of tumors

Ewing sarcoma is an aggressive neoplasm of pediatric and adolescent patients. Immunohistochemistry (IHC) can be used to support the morphologic diagnosis of Ewing sarcoma family of tumors (ESFT) in a convincing clinical/radiological context. Although neither NKX2.2 nor CD99 alone are entirely specific, when combined, the diagnostic specificity is high. The aim of the present study was to investigate the IHC expression of NKX2.2, ETV4 and BCOR in a large series of genetically confirmed ESFT. The results for CD99 and CAV-1 immunoreactivity, and the histological and fusion gene subtypes were retrieved from our previous study. NKX2.2 demonstrated moderate or strong nuclear positivity in 91.2% of the tumors. The staining intensity was heterogeneous. Many of the ESFT with negative NKX2.2 immunoreactivity were in bone. Strong/moderate ETV4 nuclear expression was detected in two small round cell tumors, both were negative for NKX2.2. No relationships could be found between expression of NKX2.2 and the histological subgroups or ESFT gene fusion subtypes. BCOR was negative in all ESFT. In conclusion, NKX2.2, ETV4 and BCOR IHC may be helpful in daily practice for distinguishing ESFT from CIC or BCOR-associated sarcomas, especially in hospitals without access to molecular assays. In addition, the combination of strong CD99 membranous positivity and nuclear NKX2.2 positivity seems to be very reliable for ESFT diagnosis in an appropriate clinicoradiological setting. So far no antibody is entirely specific for ESFT diagnosis, and the IHC or molecular results in round cell tumors of bone may be strongly influenced by decalcification processes.

Initial FDG-PET/CT predicts survival in adults Ewing sarcoma family of tumors.

PurposeThe aim of this retrospective study was to determine, at baseline, the prognostic value of different FDG-PET/CT quantitative parameters in a homogenous Ewing Sarcoma Family of Tumors (ESFT) adult population, compared with clinically relevant prognostic factors.MethodsAdult patients from 3 oncological centers, all with proved ESFT, were retrospectively included. Quantitative FDG-PET/CT parameters (SUV (maximum, peak and mean), metabolic tumor volume (MTV) and total lesion glycolysis (TLG) of the primary lesion of each patient were recorded before treatment, as well as usual clinical prognostic factors (stage of disease, location, tumor size, gender and age). Then, their relation with progression free survival (PFS) and overall survival (OS) was evaluated.Results32 patients were included. Median age was 21 years (range, 15 to 61). Nineteen patients (59%) were initially metastatic. On multivariate analysis, high SUVmax remained independent predictor of worst OS (p=0.02) and PFS (p=0.019), metastatic disease of worst PFS (p=0.01) and high SUVpeak of worst OS (p=0.01). Optimal prognostic cut-off of SUVpeak was found at 12.5 in multivariate analyses for PFS and OS (p=0.0001).ConclusionsFDG-PET/CT, recommended at ESFT diagnosis for initial staging, can be a useful tool for predicting long-term adult patients outcome through semi-quantitative parameters.

Abstract 521: Dual-targeting of IGF-1R and ErbB3 pathways in Ewing’s Sarcoma cellular models with istiratumab (MM-141), a bispecific, tetravalent monoclonal antibody

Abstract Ewing’s sarcoma family tumors (ES) are aggressive tumors that often present as metastatic in bone and soft tissue and predominantly affect adolescents and younger adults. Current treatment for ES includes surgical resection followed by loco-regional radiotherapy and chemotherapy. Survival rates for patients with metastatic disease continue to offer a particularly difficult clinical challenge, with a five-year survival rate of 20-30% for these patients. ES is primarily a genetic disease caused by fusion between the 5’ segment of the Ewing sarcoma breakpoint region 1 gene (EWSR1) on chromosome 22 and the 3’ portion of Friend leukemia virus integration site 1 (FLI1) on chromosome 11. Fusions of EWSR1 and other ETS transcription factors result in dis-regulated transcription factors which promote malignant progression of ES tumors. Recent studies have shown that most ES cell lines and clinical samples express IGF-1R. Importantly, an activated IGF-1R pathway appears to be a prerequisite for malignant transformation by the EWS-FLI1 translocation, presumably via activation of the PI3K-AKT and MAPK pathways. These findings have led to the preclinical and clinical evaluation of multiple IGF-1R-targeted therapeutics with varying results. Clinical experience with anti-IGF1R targeting therapies has demonstrated striking anticancer activity in minor subsets of patients with ES. Importantly, the paucity of a clinically useful biomarker to select patients continues to hinder IGF-1R drug development in ES. Istiratumab is an investigational, bi-specific, monoclonal antibody that acts as a tetravalent inhibitor of PI3K/AKT/mTOR, a major pro-survival pathway tumor cells use as a resistance mechanism to anticancer therapies. Istiratumab is designed to interfere with this pathway by blocking ligand-induced signaling through the IGF-1R and ErbB3 receptors, based on findings that ErbB3 activation mediates resistance to the IGF-1R blockade. We will present data in multiple ES models demonstrating the importance of both IGF-1R and ErbB3 in this disease as a mechanism of growth and resistance. Furthermore, preclinical xenograft studies demonstrate that the combination of istiratumab with an irinotecan-based chemotherapy regimen offers significant benefit over chemotherapy alone. These studies suggest that clinical evaluation of istiratumab in ES is warranted. Citation Format: Isabel Yannatos, Adam Camblin, Zhenhua Li, Michael Curley, Gege Tan, Chrystal U. Louis, Vasileios Askoxylakis, Greg Finn, Birgit Schoeberl, Rachel Nering. Dual-targeting of IGF-1R and ErbB3 pathways in Ewing’s Sarcoma cellular models with istiratumab (MM-141), a bispecific, tetravalent monoclonal antibody [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2017; 2017 Apr 1-5; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2017;77(13 Suppl):Abstract nr 521. doi:10.1158/1538-7445.AM2017-521

Extraskeletal and Skeletal Ewing Sarcoma Family of Tumours in Adults: A Clinico Radiological Review of 50 Cases

This review describes the imaging characteristics of Ewing sarcoma family of tumours ESFT exclusively in an adult population The magnetic resonance imaging MRI and computed tomography CT appearances in adult patients with ESFT is described The imaging characteristics in this largest case series of adult ESFT reported so far were similar to those reported in the paediatric ESFT Our incidence of extra skeletal ESFT of is the highest reported so far in the adult population and is higher than the reported incidence of in the paediatric and adolescent population MRI was found to be superior to CT scan in identifying tumour involvement of the bone marrow and in delineating the soft tissue involvement due to its multi planar imaging capability

Pretreatment neutrophilto lynphocyte ratio in ewing sarcoma family of tumors: An analysis of 570 patients.

e22504 Background: Ewing sarcoma family tumor (ESFT) is a systemic disease and inflammatory in nature. Inflammatory biomarkers have been identified as prognostic marker in various malignancies including sarcoma. Here we have evaluated the prognostic implication of pretreatment neutrophil to lymphocyte ratio (NLR) in ESFT. Methods: We have calculated baseline NLR from peripheral blood parameters in 570 patients of ESFT treated with uniform chemotherapy protocol in our institution from June’2003 to Feb’2015. Treatment protocol consists of neoadjuvant chemotherapy followed by surgery and/or radiotherapy as local treatment modality and adjuvant chemotherapy. NLR was dichotomized as high and low with high NLR defined as value over the median. Data was censored on 30th Jan’2016. Results: Median age was 15 years (range: 0.1-55) with male: female ratio of 40:17 and 41% (n = 236) patients had metastasis at presentation. Most common sites of tumor were long bones in 209 (37%), thorax in 108 (19%) and pelvis in 90 (16%) patients. Hundred-one (18%) had a soft tissue primary. Twenty-five percent (n = 144) patients had systemic symptoms at presentation. Median symptom duration was 4 months (range: 0.5-36) with median tumor size 8.8 cm (range: 1.6-26). Median NLR was 1.55 (range: 0.08-12.87). High NLR was associated with older age (p = 0.002), female sex (p = 0.02), low serum albumin (p = 0.003) and high lactate dehydrogenase level (p = 0.03). After median follow-up of 32 months (range: 0.8-152), 5-year event-free survival (EFS) was 25.3% & 35.8% and overall survival (OS) was 38% & 48.7%, respectively in whole cohort and in those with localized disease. In the whole cohort, high NLR ( > 1.55) emerged as independent prognostic factor predicting inferior EFS (Hazard ratio-1.36, p = 0.03) along with metastasis at presentation (p < 0.001) and tumor diameter > 8 cm (p = 0.005), in multivariate analysis. NLR couldn’t predict OS. Conclusions: High pretreatment NLR emerged as independent prognostic factor predicting inferior EFS in patients with ESFT. It is an easily available and cost effective biomarker. Prognostic nature of NLR should be evaluated in a prospective study and if proven, should be included in prognostic model to tailor therapy.

A Comparison of Pediatric vs. Adult Patients with the Ewing Sarcoma Family of Tumors.

This study sought to identify differences in clinical characteristics, outcomes, and treatments between adult and pediatric patients with the Ewing sarcoma family of tumors (ESFT). By using the Surveillance, Epidemiology, and End Results database from 1983 to 2013, 1,870 patients were analyzed (n = 976 pediatric, n = 894 adult). Between the two groups, demographic, tumor, and treatment characteristics were collated and compared. The chi-square test determined differences in proportions of the variables between groups. Survival analysis was performed using the Kaplan-Meier method; distributions were compared using the log-rank test. Univariate and multivariate analyses were performed to examine variables correlating with overall survival (OS), the primary endpoint. Adult patients had a poorer prognosis and were more likely to present with primitive neuroectodermal tumor (PNET) histology, along with distant metastasis and soft tissue primary site. In patients undergoing surgery, radiation therapy (RT) was not associated with higher OS in either children or adults. If no surgery was performed, receipt of RT was associated with higher OS in adults but not children. Adulthood negatively correlated with OS on multivariate analysis when adjusting for potential confounding factors. Other salient factors associated with OS were male gender, metastatic disease, non-extremity bone location, treatment era, and PNET histology. However, when examining the most recent subset (patients treated from 2004 to 2013), RT was associated with improved OS in both pediatrics and adults, which was an independent predictor on multivariate analysis. Adult patients with ESFT have inferior survival compared to pediatric patients, likely related to earlier clinical detection in the latter.

Cytogenetically confirmed primary Ewing’s sarcoma of the pancreas

Ewing’s sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. It is part of the Ewing’s sarcoma family of tumours (ESFTs) that also...

BCOR-CCNB3 Undifferentiated Sarcoma-Does Immunohistochemistry Help in the Identification?

Recent methodology has enabled the identification of some new genetic subgroups within the melting pot of lesions presently classified by the 2013 WHO classification as "undifferentiated/unclassified sarcomas". One of these subgroups is characterized by a paracentric inversion of the X chromosome with consequent formation of a BCOR-CCNB3 fusion. Clinical and pathological features of these tumors overlap with the Ewing sarcoma family as well as other soft tissue sarcomas, thus making them difficult to diagnose. To investigate the morphological and immunohistochemical characteristics of BCOR-CCNB3 positive sarcoma, we reviewed two sarcoma series, comprising 632 and 121 cases. The 11 tumors harboring the BCOR-CCNB3 fusion, identified by CCNB3 immunohistochemistry and/or RT-PCR, were reevaluated for morphological characteristics and further immunohistochemical investigations for CCNB3, SATB2, and Pax8 were performed. Tumors harboring a BCOR-CCNB3 fusion (11/753) occured exclusively in males, with a mean age at diagnosis of 12.9 years, and were mainly axially located. In this group of either spindled or round cell tumors, vesicular nuclei with finely dispersed chromatin, inconspicuous nucleoli and an arciform vascular pattern were pathognomonic. More than 50% of cases stained positive for SATB2 and Pax8, raising the hypothesis of a potential use of these markers in the identification of BCOR-CCNB3 positive undifferentiated/unclassified sarcomas. CCNB3 was confirmed as a useful ancillary immunohistochemical marker.

Rare adrenal gland incidentaloma: an unusual Ewing\u2019s sarcoma family of tumor presentation and literature review

BackgroundMembers of the Ewing’s sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland.Case presentationWe report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery.ConclusionsHistopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients.

Primary Ewing Sarcoma of Vulva: A Case Report and a Review of Literature

IntroductionEwing sarcoma family of tumour represents a group of bone and soft-tissue tumours with variable neuroectodermal differentiation. Extraosseous Ewing sarcoma (EES) commonly arises in soft tissues of trunk or extremities with a few reported rare sites include larynx, nasal cavity, neck, lung, retroperitoneum, mediastinum and genital tract. Till now 28 cases of extraosseous Ewing sarcoma of vulva and vagina have been reported in the literature.Case ReportWe report 29th case of Ewing sarcoma vulva in sixth youngest, 16-year-old patient with third largest size tumour. Her all routine blood reports were found to be normal, and MRI showed highly vascular vulval mass with terminal urethral involvement. Tru-cut biopsy revealed uniform sheets of small round cells with geographical necrosis and perivascular clusters of tumour cells. Immunohistochemical staining demonstrated that the cells were focally positive for CK and vimentin and showed membranous positivity for CD99 and nuclear positivity for FLI1, whereas all other IHC markers to rule out differentials of small round cell tumour were negative. Based on HPE and IHC, which is polyimmunophenotypic, has confirmed the diagnosis of an ES/PNET. After ruling out for the metastatic lesions, 14 cycles of chemotherapy with compressed VDC/IE were planned, but after receiving her 1st cycle itself, she expired on 10th day of post-chemotherapy.ConclusionEES is a rare fast growing aggressive tumour requiring ancillary techniques for exact diagnosis and multimodality treatment for better survival.