Metastatic Alveolar Soft Part Sarcoma Research Articles

Atezolizumab as the First Systemic Therapy Approved for Alveolar Soft Part Sarcoma.

The objective was to review the pharmacology, efficacy, and safety of atezolizumab (Tecentriq) for the treatment of adult and pediatric patients aged 2 years and older with unresectable or metastatic alveolar soft part sarcoma (ASPS). A literature search was conducted using PubMed and MEDLINE databases, published abstracts, and ongoing studies from ClinicalTrials.gov between January 1, 1981, and May 31, 2023. Keywords included atezolizumab, Tecentriq, MPDL3280, immunotherapy, PD-L1, PD-1, pediatrics, sarcoma, and ASPS. All English-language studies involving atezolizumab for ASPS were included and discussed. Atezolizumab is an anti-programmed death-ligand 1 (PD-L1) monoclonal antibody designed to block the interaction between PD-L1 and the programmed cell death protein 1 (PD-1) receptor. Atezolizumab was granted approval by the FDA specifically for ASPS based on a phase II clinical trial in adult and pediatric patients (n = 49), which reported an overall response rate of 24% and a durable response rate at 6 and 12 months of 67% and 42%, respectively. Common grade 3/4 adverse reactions include musculoskeletal pain (8%), followed by hypertension (6%), weight gain (6%), headache (4%), and dizziness (4%). Advanced ASPS is a high-risk disease with limited treatment options. Atezolizumab appears to be a viable treatment option in ASPS demonstrating clinical efficacy and a manageable toxicity profile. With no other treatments that are FDA approved specifically for ASPS, and few demonstrating efficacy in the advanced setting, the approval of atezolizumab, including the first approval for pediatric patients, represents a landmark improvement to the therapeutic arsenal against this rare disease.

Atezolizumab (Tecentriq) for Alveolar Soft Part Sarcoma

Atezolizumab (Tecentriq – Genentech), an immune checkpoint inhibitor, has been approved by the FDA for treatment of unresectable or metastatic alveolar soft part sarcoma (ASPS) in patients 2>=years old. It was previously approved for treatment of non-small cell lung cancer, small cell lung cancer, hepatocellular cancer, and melanoma (see Table 1). Atezolizumab is the first drug to be approved in the US for treatment of ASPS. ASPS is a rare disorder that affects mostly adolescents and young adults; <1% of soft tissue sarcomas are ASPS.

Myositis ossificans circumscripta after surgery and radiotherapy and during sunitinib treatment: a case report

BackgroundMyositis ossificans circumscripta is a self-limiting, benign, ossifying lesion that can affect any type of soft tissue. It is most commonly found in muscles as a solitary lesion. A history of recent trauma has been reported in approximately 50% of cases. Clinically, MOC presents as a painful swelling, which rapidly increases in size. The pain and inflammatory symptoms spontaneously disappear after approximately 2–6 weeks, and the mass stabilizes or decreases. Radiologically, myositis ossificans circumscripta can be divided into two phases. The first is the acute phase, which is followed by the mature phase 2–6 weeks later. During the acute phase, the radiological aspect does not show any specific abnormality. In the mature phase, plain radiographs and computed tomography show blurred calcifications around a hypodense center. We describe here the first case of myositis ossificans circumscripta, with appropriate follow-up, occurring during sunitinib exposure.Case presentationWe report a case of myositis ossificans circumscripta in a 34-year-old man (ethnicity unknown) receiving sunitinib for metastatic alveolar soft part sarcoma of the left thigh after surgery and radiotherapy. Four months after the first dose of sunitinib, the patient experienced painful swelling in the surgical scar area. Magnetic resonance imaging showed diffuse and marked edema of the anterior compartment of the thigh, without nodular lesions circumscribing a central core, and without bone signal abnormality. The increased visibility of the intermuscular fascia and convergence of normal muscle fibers (black hole effect), without the displacement seen in tumors, were suggestive of myositis. Therefore, antiangiogenic treatment was discontinued, and the symptoms rapidly resolved within a few days. Three weeks after the discontinuation of sunitinib, the inflammatory findings completely disappeared. Two months after the diagnosis of myositis ossificans circumscripta, plain radiographs and computed tomography showed an extensive calcified mass measuring > 12 cm. The continuation of favorable clinical outcomes was confirmed.ConclusionsTo the best of our knowledge, this is the first case of myositis ossificans circumscripta with appropriate follow-up occurring during sunitinib exposure. Owing to multimodal treatment of sarcoma, we cannot rule out the radiotherapy and surgery causality.

Comparative Combinatorial Implications and Theranostics of Immunotherapy in the Impediment of Alveolar Soft Part Sarcoma.

Immune checkpoint inhibitors (ICIs), specifically programmed cell death receptor- 1/ligand 1 (PD-1/L1) inhibitors, have shown potential pharmacological efficacy in several cancers. Nonetheless, data pertinent to their therapeutic efficacy in alveolar soft-part sarcoma (ASPS) are limited. The retrospective aspects of ICIs (anti-PD1/PD-L1 blockers) to target ASPS are comparatively analyzed for clinical outcomes with other targeted immunotherapy modalities. We have conducted a systematic review without statistical analysis or comprehensive meta-analysis by collecting the articles published between 1952 and Sep 10th, 2020, by searching the following words: alveolar soft part sarcoma and immunotherapy including immune checkpoint, immune checkpoint inhibitors, and PD-1, PD-L1. We performed a pooled analysis of case reports, conferences, clinical trials, and other research reports pertinent to the efficacy of a PD-1 or PD-L1 antagonist in patients diagnosed with metastatic ASPS. The effective studies include 10 case reports, 2 conference reports, 5 clinical trials, and 2 additional research reports. A total of 110 patients were reported to be enrolled in the pooled analysis; among them, 87 (78.38%) received a PD-1/PD-L1 antagonist. For patients who received anti-PD-1/PD-L1as monotherapy, their clinical response rates (CRR) were 63.22% whereas those who received targeted therapy and immunotherapy had a CRR of 78.95% (15/19). In the patients treated with double immunotherapy, their CRR was 100% (4/4). Tumor mutational burden and mismatch repair status have significant implications for predicting the ASPS prognosis. Alveolar soft-part sarcoma patients with distant metastases can exhibit better clinical outcomes with immunotherapy, particularly toripalimab, atezolizumab, and axitinib combinatorial regimen with pembrolizumab. In addition, this review describes the therapeutic implications to guide personalized medicine depending on the expression patterns of PD-1/PD-L1 during the immunotherapy with ASPS.

Abstract CT168: Randomized phase 2 trial of sunitinib or cediranib in alveolar soft part sarcoma

Abstract Background: Alveolar soft part sarcoma (ASPS) is a rare, highly vascular tumor with few effective treatment options. Cediranib (C) and sunitinib (S) are potent oral inhibitors of all 3 VEGF receptors. Cediranib showed substantial single-agent activity (objective response rate [ORR] = 35%) in our previous trial in patients (pts) with metastatic ASPS (NCT00942877). Here we report a phase 2 randomized multicenter trial of single agent C or S in pts with ASPS (NCT01391962). Methods: We conducted a multicenter phase 2 trial with an optimal 2-stage design targeting an ORR of 40%. Enrolled pts were >16 years with metastatic ASPS, previously not treated (N) and unresectable, or previously treated (T), who have progressed per RECIST 1 within the 6-month period preceding enrollment. Pts were randomized to receive C (30 mg) or S (37.5 mg) orally, once a day, in 28-day cycles and could crossover to the other treatment arm at disease progression. ORR (primary endpoint), median progression-free survival (mPFS), and PFS rate at 24 weeks for the 2 arms (C and S) were evaluated; T and N cohorts were assessed separately in each arm. Arm accrual closed if ≤ 1 of the first 10 enrolled pts responded to the first treatment. Results: Thirty-four pts (47% white, 29.4% black, 17.6% Asian, 5.8% Pacific Islander) were enrolled; 29 pts were evaluable for response. One pt on each of the initial treatment arms had a confirmed partial response (PR), rates of 6.7% (1/154) and 7.1% (1/14) for C and S tx or tx-naive, respectively. Among pts who crossed over, there was 1 PR in a pt receiving C after initially responding (PR) on S (1/9; ORR 11.1%). Twenty-four pts had a best response of stable disease (86.7% and 78.6%) for C and S, respectively. The mPFS was 7.6 months (mo) (95% CI: 3.7-9.9 mo) and 5.5 mo (95% CI: 1.8-14.5 mo) for C and S, respectively administered as first therapy (p=0.92). PFS rate at 24 weeks was 62.5% (95% CI: 29.5-76.2%) and 50% (95% CI: 25.9-70.1%) for pts receiving C and S respectively, as initial therapy. There was no difference in mPFS between T or N pts in the C (6.7 mo [95% CI: 1.4-9.9 mo] vs 8.3 mo [95% CI: 2.7 - 16.6 mo]; P=0.35) arm, but some evidence of a potential difference in the S (4.8 mo [95% CI: 0.9-7.9 mo] vs 14.7 mo 95% CI: 1.8 - 21.6 mo]; P=0.058) arm. Overall, 43.7% (C) and 77.8% (S) of pts experienced grade ≥3 adverse events (AEs) at least possibly related to the study drug. Common grade ≥3 AEs included: diarrhea (C), neutropenia (S), hypertension (C and S). AEs were in line with the known safety profiles of each agent. As of August 2021, 1 pt (unevaluable for ORR) remains on study. Conclusions: The study did not meet its endpoints for ORR. There were no differences in mPFS for the 2 treatment arms. The selection of pts with more aggressive disease, who had progressed in the 6 months prior to enrollment, may account for the low patient response rates compared to our previous study of cediranib in ASPS. Funded by NCI Contract No. HHSN261200800001E. The study was a collaboration between NCI and Pfizer. Citation Format: Naoko Takebe, James Nguyen, Shivaani Kummar, Albiruni Abdul Razak, Sant P. Chawla, Suzanne George, Shreyaskumar R. Patel, Mary Louise Keohan, Sujana Movva, Geraldine O’Sullivan, Khanh Do, Larry Anderson, Lamin Juwara, Brooke Augustine, Seth Steinberg, Laura Kuhlmann, S. Percy Ivy, James H. Doroshow, Alice P. Chen. Randomized phase 2 trial of sunitinib or cediranib in alveolar soft part sarcoma [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr CT168.

Alveolar soft tissue sarcoma: a report of 50 cases at a single institution

Background Alveolar soft-part sarcoma (ASPS) is a rare soft tissue sarcoma subtype, occurring mainly in young people, with poor prognosis. Materials and methods We conducted a retrospective analysis of localized or metastatic ASPS patients admitted to the First Affiliated Hospital of Zhengzhou University (China) from 2012 to 2020, focusing on treatment and prognosis. Results The median age at diagnosis was 24 years (range: 1.4–78 years). Women (n = 29, 58%), especially those aged <30 years, dominated this series. The most common metastasis site was lung. Thirty-one (62%) patients developed lung metastasis (localized: n = 9 [18%]; metastatic: n = 22 [44%]). Only a tumor maximum diameter ≥ 5 cm was associated with a high lung metastasis rate (p = 0.039). The mean follow-up time was 37.5 months (1–108 months), and the 5-year overall survival (OS) rate was 84.7%. Univariate analysis indicated that distant metastasis observed at the initial visit and incomplete resection of the primary tumor were associated with poor OS. For localized cases, neither surgery plus radiotherapy (p = 0.486) nor surgery plus chemotherapy (p = 0.536) improved progression-free survival compared to surgery alone. Among the metastatic cases, the disease control rate (PR + SD) was higher for targeted therapy (60%) and combined immunosuppressive therapy (100%) than for conventional cytotoxic chemotherapy (26%). Conclusions Postoperative adjuvant radiotherapy and chemotherapy do not provide good local control for patients with localized disease. Although there is no standard treatment strategy for patients with advanced or metastatic disease, they can benefit from targeted therapy and/or immunosuppressive therapy.

Efficacy of targeted therapy in alveolar soft part sarcoma: A systematic review and meta-analysis.

e23543 Background: Alveolar soft part sarcoma (ASPS) is a rare subtype of soft tissue sarcoma, characterized by a specific unbalanced translocation leading to the fusion of the TFE3 gene on chromosome-X to the ASPSCR1 gene on chromosome-17. Despite its indolent course, ASPS presents a challenge in treatment due to its resistance to conventional anthracycline-based chemotherapy and lack of large scale trial data for this rare sarcoma. This review aimed to assess the efficacy of tyrosine kinase inhibitors (TKI) and immune checkpoint inhibitors (ICI) in metastatic alveolar soft part sarcoma. Methods: A systematic search was performed on Embase and Medline databases for studies that assessed best response of patients with unresectable or metastatic ASPS to TKI and ICI therapy, according to the Response Evaluation Criteria in Solid Tumors (RECIST) edition 1.0 or 1.1. This study followed the Preferred Reporting Items for Systematic Reviews (PRISMA) protocol. Four independent reviewers screened abstracts and extracted the data; any discrepancy was resolved by discussion among reviewers. Pooled objective response rate (ORR) and disease control rate (DCR) were obtained using the Freeman-Tukey double-arcsine transformation using random effects model on STATA software (version. 16.1, StataCorp). Results: 27 articles and abstracts published between 2011 and 2020 were included in the review, resulting in 2 randomized clinical trials (104 participants), 14 single arm prospective trials (214 participants), and 11 retrospective studies (120 patients). Among clinical trials, the pooled ORR and DCR were 18% (95% confidence interval [CI] 8 - 30%; I2 = 72.25%; p &lt; 0.01) and 87% (95% CI 97 - 93%; I2 = 43.2%; p = 0.03) respectively. Conclusions: The response rate to targeted therapy in metastatic ASPS is not only clinically meaningful, but also comparable to that of first-line chemotherapy. The majority of patients receiving targeted therapy achieved disease control. Patients who had refractory or progressive disease to one targeted agent demonstrated response to other agents. More randomized trials are warranted to expand treatment options and compare to standard of care regimens.

Metastatic alveolar soft part sarcoma masquerading as primary CNS tumor

Alveolar soft part sarcoma (ASPS) is a very rare malignant, highly aggressive and profusely vascular soft tissue sarcoma of controversial histological origin accounting for less than 1% of the soft tissue sarcoma cases worldwide. Though it has an indolent clinical course, however, because of its propensity to recur and frequently metastasize, it usually has a fatal outcome. The knowledge of its microscopic features and TFE3 staining facilitate earlier diagnosis. Surgical resection helps in increasing the disease-free period, but the role of adjuvant chemotherapy and radiotherapy is disputed. After resection, it should be frequently monitored because of its malignant potential and dismal prognosis. We present a case report of twenty-year-old male patient whose entire clinical presentation was that of a primary brain malignancy with no symptoms anywhere else in the body but was diagnosed to be a case of alveolar soft part sarcoma of para lumbar region with brain and lung metastasis. To the best of our knowledge, it is an extremely uncommon presentation with only fourteen cases have been reported so far. Keywords: Alveolar soft part sarcoma, brain metastasis, TFE3 nuclear positivity.

Target therapy for metastatic alveolar soft part sarcoma: a retrospective study with 47 cases.

BackgroundAlveolar soft part sarcoma (ASPS) is a translocation-associated soft-tissue tumor resistant to conventional cytotoxic agents. This report aims to compare the efficacy of anlotinib versus pazopanib as targeted monotherapy in metastatic ASPS and to determine the impact of drug dosage reduction on disease control.MethodsSixteen and 31 patients with metastatic ASPS were respectively treated with anlotinib and pazopanib monotherapy at a single institution. Objective response rate (ORR), progression-free survival (PFS), and overall survival (OS) were retrieved and compared between both therapeutic arms. Adverse events (AEs) within each group were recorded. Kaplan-Meier survivorship curves computed the impact of drug dosage reduction on PFS.ResultsThe anlotinib group showed an ORR of 31.2%, compared to 35.5% in the pazopanib arm (P=0.772). Median PFS was 23.6 months [95% confidence interval (CI), 16.2–31.0 months] in patients treated with anlotinib, but dropped to 13.7 months (95% CI, 10.8–16.7 months) in those managed with pazopanib (P=0.023). One (6.3%) patient on anlotinib and 11 (35.5%) on pazopanib developed AEs requiring drug dosage reduction (P=0.029), which significantly reduced patients’ PFS in the latter setting (10.5 vs. 15.8 months, P=0.012). In patients without dosage reduction, anlotinib showed a bordering advantage than pazopanib on median PFS (24.5 vs. 15.8 months, P=0.112).ConclusionsCompared to pazopanib, anlotinib yielded longer PFS and lower incidence of AEs in ASPS patients. Drug dosage reduction was more frequently encountered with the former agent and affected the disease control.

ICREATE: imaging features of primary and metastatic alveolar soft part sarcoma from the EORTC CREATE study

BackgroundAlveolar Soft Part Sarcoma (ASPS) is a rare, slow-growing, but highly vascular soft tissue sarcoma, characterised by a high rate of metastases at presentation. Although imaging features of the primary are well described, less detail is available on the imaging pattern of metastatic ASPS. The EORTC 90101 (CREATE) study assessed the efficacy of Crizotinib in patients with metastatic ASPS and presents a unique opportunity to describe the imaging phenotype of primary and metastatic ASPS, based on prospectively collected imaging.MethodsA retrospective review of the staging CT scans of 32 patients with ASPS from the CREATE study was undertaken and the imaging features of primary and metastatic disease were assessed.ResultsImaging of the primary tumour was available in 7/32 cases (28%). All primary tumours demonstrated marked vascularity with prominent feeding vessels (7/7, 100%). The most frequent sites of metastases included lung (30/32, 94%), nodal (7/32, 22%), bone (5/32, 16%) and muscle/subcutaneous (5/32, 16%). Features of hypervascularity were identified at all sites, more appreciable in the lungs, with feeding vessels frequently demonstrated in pulmonary metastases (21/32, 66%).ConclusionAnalysis of imaging from the CREATE cohort of patients with metastatic ASPS demonstrates that metastases from ASPS are predominantly hypervascular and demonstrate feeding vessels comparable to primary ASPS, suggesting potential sensitivity of this rare sarcoma for antivascular/antiangiogenic treatment approaches.

Metastatic Alveolar Soft Part Sarcoma to the Breast: A Case Report

Abstract Introduction/Objective Metastases to the breast are rare (less than 3% of all breast malignancies). In adults, malignant melanoma is the most common type of metastasis followed by carcinomas from various primary sites. Here, we describe a case of metastatic alveolar soft part sarcoma (ASPS) to the breast. Only a handful similar presentations are reported in the literature. Methods Here we describe the case of a 43-year-old female patient with a recent diagnosis of left base of tongue alveolar soft part sarcoma (in an outside hospital), presenting with a palpable mass in her left breast. Sonographic study of the left breast demonstrated a 1.4 cm oval hypoechoic mass with partially microlobulated margins at 1 o’clock. Results Histopathologic examination of the ultrasound guided core biopsy showed a “pink” neoplasm, composed of plump epithelioid tumor cells with an eosinophilic and granular cytoplasm, round to oval nuclei and conspicuous nucleoli. They are arranged in organoid nests separated by sinusoidal spaces. An immunohistochemical study shows the tumor cells to be negative for keratin AE1/AE3, keratin 7, keratin 5/6, GATA-3, Pax-8, estrogen receptor, HER2, p63, S100 and CD117. FISH test for TFE3 (Xp 11.23) is positive. PAS with diastase staining highlights the intracytoplasmic granules. Theses morphologic, clinical and molecular findings confirm the diagnosis of metastatic ASPS. Conclusion The differential diagnosis of “pink” tumors of the breast include primary tumors like secretory carcinoma and apocrine carcinoma and metastatic lesions of renal, thyroid, skin (melanoma), oral cavity and soft tissue. Although extremely rare, in the right clinical context, metastatic ASPS to the breast can occur and should be included in the differential diagnosis.

&lt;p&gt;The efficacy and safety of apatinib in metastatic alveolar soft part sarcoma: a case series of six patients in one institution [Corrigendum]&lt;/p&gt;

&lt;p&gt;The efficacy and safety of apatinib in metastatic alveolar soft part sarcoma: a case series of six patients in one institution [Corrigendum]&lt;/p&gt;

&lt;p&gt;The efficacy and safety of apatinib in metastatic alveolar soft part sarcoma: a case series of six patients in one institution&lt;/p&gt;

Background: Evidence suggests that advanced or metastatic alveolar soft part sarcoma (ASPS) with high metastatic potential is chemo-resistant. However, the benefits of tyrosine kinase inhibitors have been demonstrated for the treatment of ASPS.Purpose: This study aimed to investigate the efficacy and safety of apatinib, aspecific VEGFR-2 inhibitor, in ASPS patients. This retrospective analysis involved six patients with metastatic ASPS not amenable to curative treatment.Patients and methods: Apatinib was administered at a dose of 500mg per day. Tumor responses were assessed according to the Response Evaluation Criteria in Solid Tumors (RECIST 1.1) guidelines. Survival analysis was performed using the Kaplan–Meier test, and a safety profile was recorded.Results: The mean age of patients was 26.5 (range, 17–32) years. The median progression-free survival (PFS) was 18.53 months (95% CI, 12.23-NE). However, median overall survival (OS) has not been reached. Twenty-four month PFS and OS rates were 50.0% and 100.0%, respectively. One patient achieved a complete response, and the remaining patients achieved partial responses, with an objective response rate of 100%. Median follow-up was 20.6 (range, 12.43–34.13) months. The most common adverse events included gastrointestinal discomfort (4/6[66.7%]), hair hypopigmentation (4/6[66.7%]) and hand-foot skin reaction (3/6[50.0%]).Conclusion: Apatinib shows beneficial activity in metastatic ASPS patients, and further studies are warranted with more cases and longer follow-up periods to fully characterize clinical efficacy and safety of apatinib in ASPS.

Alveolar soft-part sarcoma: Primary metastatic disease and metastatic relapse occurring during long-term follow-up: Treatment results of four Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry.

Patients with metastatic alveolar soft-part sarcoma (ASPS) are known to have a very poor prognosis. Little is known about best treatment of primary metastatic disease (MD) or relapsed metastatic disease (rMD). Patients with localized disease (LD), primary MD, and metastatic recurrence after complete remission (CR) treated within the CWS-86, -91, -96, -2002P trials and the recent registry SoTiSaR (1985-2016) were analyzed. Fifteen of 61 patients had primary metastases at initial diagnosis at the age of 14.6years (range, 7.8-19.7). Nine of 46 patients with initial LD suffered of rMD at a median age of 9.9years (range, 3.5-30), 3.75 years (0.75-21) after CR of primary disease. Complete resection (microscopically or macroscopically) was possible in 2 of 15 patients with MD and in 5 of 9 with rMD. RT was administered in 4 of 15 MD and 1 of 9 rMD. Chemotherapy was administered to 11 of 15MD and 3 of 9rMD, targeted therapy to 3 of 15MD and 1 of 9rMD. Median time to progression of patients treated with targeted therapy (n=4), CHT (n=14), and resection only (n=6) was 56, 17, and 23months, respectively. The 5-year event-free survival and overall survival (OS) rates were 19.8% and 61%, respectively, for patients with MD compared with 79% and 98% for patients with LD. The 5-year progression-free survival and OS were 67% and 100% for patients with rMD. Complete tumor resection correlates with long-term survival in patients with primary and relapsed MD.

Activity and safety of crizotinib in patients with advanced, metastatic alveolar soft part sarcoma (ASPS) with rearrangement of TFE3: European Organization for Research and Treatment of Cancer (EORTC) phase 2 trial 90101 CREATE.

11540Background: ASPS is an orphan disease associated with rearrangement of transcription factor E3 (TFE3), leading to abnormal MET expression. We assessed crizotinib in pts with ASPS (NCT01524926)...

Cediranib phase II study in children with metastatic alveolar soft part sarcoma (ASPS).

10540Background: ASPS, a rare, highly vascular sarcoma with a clinically indolent course, frequently presents with metastases at diagnosis. Standard sarcoma chemotherapy is ineffective. Vascular en...

Brain metastatic alveolar soft-part sarcoma: Clinicopathological profiles, management and outcomes.

Alveolar soft-part sarcoma (ASPS) is a rare sarcoma that presents in the buttocks or thigh of young adults and often metastasizes to the brain. The present study examined the clinical features and morphology of brain metastatic ASPS. The case records of eight patients with brain metastatic ASPS admitted between November 2008 and March 2015 were reviewed. The relevant clinical data (including patient age and sex, neuroimaging studies, histopathological and immunohistochemical features, surgical records and follow-up reports) were collected through a review of patient records. The sex distribution was 3:1 male to female and the age ranged between 15 and 33 years at the time of surgery. In total, five patients with brain metastases had concurrent pulmonary metastases. The lesions were hypointense on T1-weighted images in every patient, hyperintense on T2-weighted images in six patients and contrast enhancement was present in all patients. The most notable immunohistochemical feature was strong immunohistochemical staining for TFE3 in each patient. Gross total resection was performed in all eight patients, with two patients undergoing adjuvant radiotherapy and one undergoing adjuvant chemotherapy. Four recurrent cases were observed during the follow-up. TFE3 staining and knowledge of its microscopic characteristics would facilitate earlier diagnosis: Early diagnosis with a multidisciplinary, multimodal approach to treatment is required to achieve extended disease-free survival in patients with brain metastatic ASPS.

Long-term results of therapy with sunitinib in metastatic alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is a rare, highly vascularized soft tissue sarcoma characterized by a high frequency of metastatic disease and resistance to classical chemotherapy. The purpose of our analysis was to assess long-term sunitinib activity in the treatment of metastatic ASPS. Between 2009 and 2015, 15 patients were diagnosed with metastatic ASPS and received therapy with sunitinib at initial continuous daily dosing of 37.5 mg. Median age was 32 years. The primary tumor sites were lower extremities (8), trunk-retroperitoneum/pelvis (2), upper extremity (3) and other (2). All patients had unresectable disease (primary or relapse in the form of metastases to the lungs ± bones). Five patients received systemic therapy before initiating sunitinib. Median follow-up from start of sunitinib was 38 months (range 5-69 months). At the time of analysis 4 patients continue therapy and 9 are still alive. Six patients had RECIST partial remission as best response, 8 had stable disease, and 1 had disease progression. The median progression-free survival was 19 months, with 86% of patients being free of progression at 6 months. Median overall survival was 56 months; the 5-year overall survival rate was 49%. Five patients were treated with sunitinib longer than 2 years. All patients experienced some side effects: 8 patients (53%) had CTCAE grade 3/4 toxicity, 7 patients required dose reduction. The most common toxicities were neutropenia, thrombocytopenia, hypothyroidism, arterial hypertension, and hand-foot syndrome. Our analysis confirms the long-term efficacy of sunitinib in patients with advanced ASPS.

Alveolar soft part sarcoma with brain metastases

Metastatic tumors are the most common mass lesions in the brain. This case reports a rare form of sarcoma with metastasis to the brain. The appropriate management of a patient with metastatic alveolar soft part sarcoma to the brain is discussed. Author describes a 32-year-old gentleman diagnosed with primary tumor at gluteus and distant metastases at lower lobe of right lung and the brain. Histopathology proves diagnosis as alveolar soft part sarcoma. Craniotomy with excision of brain lesion was done. Repeated magnetic resonance imaging of the brain after 2 months showed rapidly growing new lesions. The next step of management was made by the oncology team as recurrence rate was high and due to multi-systemic involvement. Patient was planned for palliative chemotherapy and to be reassessed later. This case report discusses the appropriate approach to any form of brain metastases and the role of early follow-up especially after surgery for better outcome and choice of post operative management such as radiotherapy or chemotherapy or both for malignant tumors. Based on this report, it was concluded that every brain tumor patient should be frequently monitored even in the outpatient setting as most of them are metastatic and rapidly spreading. The patient should be considered for radiotherapy or chemotherapy or both after surgery if the histopathology result is suggestive of malignancy.

1408P - A phase II trial of pazopanib in patients with metastatic alveolar soft part sarcoma

1408P - A phase II trial of pazopanib in patients with metastatic alveolar soft part sarcoma