scholarly journals Correlation of Clinical, Histopathological and Histomorphometric Features of Canine Soft Tissue Sarcomas

2021 ◽  
Vol 14 (3) ◽  
pp. 151-158
Author(s):  
Ellen Cavalcanti ◽  
◽  
Leonardo Gorza ◽  
Bruna Sena ◽  
Brunno Sossai ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Mitotic Index ◽  
Medical Records ◽  
Soft Tissue Sarcomas ◽  
Nuclear Pleomorphism ◽  
Biological Behaviour ◽  
Undifferentiated Sarcoma ◽  
Histopathological Classification ◽  
Additional Tool ◽  
Degree Of Differentiation

Soft-tissue sarcomas (STS) represent a heterogeneous group of tumours with similar histological characteristics and biological behaviour. This study aimed to describe the correlation between clinical, histopathological and histomorphometric features of STS in dogs. Medical records were reviewed to identify all dogs in which an STS was diagnosed between 2006-2017. Thirty cases were included, and tumour samples and medical records were recovered. Most of the dogs were mixed breed (40%) and 80% of the STS were located in the subcutaneous connective tissue. Histopathological classification showed that undifferentiated sarcoma (17%) and peripheral nerve sheath tumour (30%) were the most common STS. Grade I STS were obtained in 50% of cases (15/30), and grade II or III tumours compromised 43% (13/30) and 7% (2/30) respectively. The mitotic index ranged from zero to 26 (5.8 ± 7.5). Increased nucleus:cytoplasm ratio was moderately associated with higher tumour grade (p = 0.05; rS = 0.361) and mitotic index (p = 0.05; rS = 0.355), while the number of microvessels was positively correlated with degree of differentiation (p = 0.05; rS = 0.362) and nuclear pleomorphism (p = 0.036; rS = 0.384). Histomorphometry proved to be useful in the evaluation of STS, representing an additional tool correlated with well-established prognostic factors (histopathological grade, degree of differentiation, nuclear pleomorphism).

scholarly journals DNA Methylation Profiling for Diagnosing Undifferentiated Sarcoma with Capicua Transcriptional Receptor (CIC) Alterations

2020 ◽  
Vol 21 (5) ◽  
pp. 1818 ◽  
Author(s):  
Evelina Miele ◽  
Rita De Vito ◽  
Andrea Ciolfi ◽  
Lucia Pedace ◽  
Ida Russo ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Soft Tissue ◽  
Abdominal Wall ◽  
Current Knowledge ◽  
Soft Tissue Sarcomas ◽  
Molecular Techniques ◽  
Round Cell ◽  
Undifferentiated Sarcoma ◽  
Dna Methylation Profiling ◽  
Methylation Profiling

Undifferentiated soft tissue sarcomas are a group of diagnostically challenging tumors in the pediatric population. Molecular techniques are instrumental for the categorization and differential diagnosis of these tumors. A subgroup of recently identified soft tissue sarcomas with undifferentiated round cell morphology was characterized by Capicua transcriptional receptor (CIC) rearrangements. Recently, an array-based DNA methylation analysis of undifferentiated tumors with small blue round cell histology was shown to provide a highly robust and reproducible approach for precisely classifying this diagnostically challenging group of tumors. We describe the case of an undifferentiated sarcoma of the abdominal wall in a 12-year-old girl. The patient presented with a voluminous mass of the abdominal wall, and multiple micro-nodules in the right lung. The tumor was unclassifiable with current immunohistochemical and molecular approaches. However, DNA methylation profiling allowed us to classify this neoplasia as small blue round cell tumor with CIC alterations. The patient was treated with neoadjuvant chemotherapy followed by complete surgical resection and adjuvant chemotherapy. After 22 months, the patient is disease-free and in good clinical condition. To put our experience in context, we conducted a literature review, analyzing current knowledge and state-of-the-art diagnosis, prognosis, and clinical management of CIC rearranged sarcomas. Our findings further support the use of DNA methylation profiling as an important tool to improve diagnosis of non-Ewing small round cell tumors.

Aggressive, undifferentiated sarcoma with widespread metastasis in a six-month-old Neopolitan mastiff

1996 ◽  
Vol 32 (2) ◽  
pp. 97-101 ◽  
Author(s):  
NA Sanders ◽  
RL Kerlin ◽  
DM Dambach
Keyword(s):  
Soft Tissue ◽  
Lymph Nodes ◽  
Rapid Growth ◽  
Soft Tissue Sarcomas ◽  
Post Mortem ◽  
Cell Of Origin ◽  
Undifferentiated Sarcoma ◽  
Cervical Mass ◽  
Poor Differentiation

A six-month-old Neopolitan mastiff presented for a rapidly growing cervical mass. Undifferentiated sarcoma was diagnosed at post mortem based on histopathology and immunohistochemistry. Metastases to mediastinum, pleura, lungs, liver, kidneys, omentum, mesentery, and multiple lymph nodes were present. Soft-tissue sarcomas are reported infrequently in children and young dogs. The cell of origin often is difficult to determine due to poor differentiation and rapid growth of these neoplasms.

scholarly journals Endosialin and Associated Protein Expression in Soft Tissue Sarcomas: A Potential Target for Anti-Endosialin Therapeutic Strategies

Sarcoma ◽  
2016 ◽  
Vol 2016 ◽  
pp. 1-13 ◽  
Author(s):  
Daniel J. O’Shannessy ◽  
Hongyue Dai ◽  
Melissa Mitchell ◽  
Shane Huntsman ◽  
Stephen Brantley ◽  
...  
Keyword(s):  
Gene Expression ◽  
Soft Tissue ◽  
Protein Expression ◽  
Soft Tissue Sarcomas ◽  
Tissue Expression ◽  
Tissue Type ◽  
Expression Data ◽  
Interacting Protein ◽  
Undifferentiated Sarcoma ◽  
Tumor Types

Endosialin (CD248, TEM-1) is expressed in pericytes, tumor vasculature, tumor fibroblasts, and some tumor cells, including sarcomas, with limited normal tissue expression, and appears to play a key role in tumor-stromal interactions, including angiogenesis. Monoclonal antibodies targeting endosialin have entered clinical trials, including soft tissue sarcomas. We evaluated a cohort of 94 soft tissue sarcoma samples to assess the correlation between gene expression and protein expression by immunohistochemistry for endosialin and PDGFR-β, a reported interacting protein, across available diagnoses. Correlations between the expression of endosialin and 13 other genes of interest were also examined. Within cohorts of soft tissue diagnoses assembled by tissue type (liposarcoma, leiomyosarcoma, undifferentiated sarcoma, and other), endosialin expression was significantly correlated with a better outcome. Endosialin expression was highest in liposarcomas and lowest in leiomyosarcomas. A robust correlation between protein and gene expression data for both endosialin and PDGFR-βwas observed. Endosialin expression positively correlated with PDGFR-βand heparin sulphate proteoglycan 2 and negatively correlated with carbonic anhydrase IX. Endosialin likely interacts with a network of extracellular and hypoxia activated proteins in sarcomas and other tumor types. Since expression does vary across histologic groups, endosialin may represent a selective target in soft tissue sarcomas.

Clinical benefit of next generation sequencing in soft tissue and bone sarcoma: Rush University Medical Center’s experience.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e22552-e22552
Author(s):  
Mia C. Weiss ◽  
Alan Blank ◽  
Steven Gitelis ◽  
Mary J. Fidler ◽  
Marta Batus
Keyword(s):  
Overall Survival ◽  
Next Generation Sequencing ◽  
Soft Tissue ◽  
Clinical Decision Making ◽  
Soft Tissue Sarcomas ◽  
Bone Sarcoma ◽  
Next Generation ◽  
Undifferentiated Sarcoma ◽  
The Impact ◽  
Generation Sequencing

e22552 Background: The overall survival for metastatic sarcoma has remained at only 18-20%. In the era of next generation sequencing (NGS), much research is ongoing on identifying optimal treatments. The MULTISARC trial aims to determine if NGS can lead to improved overall survival by randomizing patients with metastatic STS to receive NGS (followed by possible NGS-guided therapy) or not. We present our center’s experience with NGS in sarcomas patients. Methods: Patients with soft tissue and bone sarcomas at Rush that had the Foundation Medicine assay sent on tumor samples between August 2017 and August 2018 were analyzed retrospectively. The impact of NGS on clinical decision making was determined based on patients being prescribed off-label FDA-approved therapy targeting identified mutation. Results: Thirty-four patients with bone/soft tissue sarcomas that had NGS sent on specimens were identified. Median age at diagnosis: 43 (18-78 years); 18 males, 16 females. Histologic subtypes: synovial sarcoma, myxofibrosarcoma, leiomyosarcoma, chondrosarcoma, sclerosing epitheloid fibrosarcoma, PEcoma, pleomorphic undifferentiated sarcoma, MPNST, liposarcoma- well and de-differentiated, angiosarcoma, osteosarcoma. 16/34 patients had targetable mutations with approved therapies in tumor types other than sarcoma. Four of these patients had therapy changed based on NGS results, 1 patient with metastatic chondrosarcoma (PTEN mutation, everolimus added), 1 patient with metastatic liposarcoma (CDK4 mutation, palbociclib added), 1 patient with metastatic osteosarcoma (CCD1/CDK4 and a PDGFRA mutation for which palbociclib followed by imatinib was added), and 1 patient with metastatic pleomorphic undifferentiated sarcoma (CDK4 mutation, palbociclib added). Targetable mutations for which clinical trials are available were identified in 25/34 (73%) of the cases. Conclusions: NGS was readily able to identify actionable mutations in close to 50% of patients with clinical trial opportunities in close to 75%. Four patients had therapy changed as a result of NGS testing. Although our study size is small, our data show potential for the use of genomic profiling to identify actionable targets, tailor therapy, and hopefully improve outcomes. [Table: see text]

Aberrant Immunohistochemical Expression in Nonrhabdomyosarcoma Soft Tissue Sarcomas of Infancy: Retrospective Review of Clinical Material

2002 ◽  
Vol 5 (6) ◽  
pp. 579-586 ◽  
Author(s):  
Neil James Sebire ◽  
Alan Drummond Ramsay ◽  
Gillian Levitt ◽  
Marian Malone ◽  
Rupert Anthony Risdon
Keyword(s):  
Soft Tissue ◽  
Smooth Muscle Actin ◽  
Diagnostic Category ◽  
Young Patients ◽  
Soft Tissue Sarcomas ◽  
Mitotic Rate ◽  
Soft Tissue Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma ◽  
Infantile Hemangiopericytoma

Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma.

scholarly journals The Minimalistic Malignancy- Low grade Fibromyxoid Sarcoma

2020 ◽  
Vol 1 (3) ◽  
pp. 7-14
Author(s):  
Anubha Bajaj
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Distant Metastasis ◽  
Soft Tissue Sarcomas ◽  
Benign Neoplasm ◽  
Low Grade ◽  
Biological Behaviour ◽  
Preclinical Stage ◽  
Tumour Incidence ◽  
Fibromyxoid Sarcoma

Low grade fibromyxoid sarcoma (LGFMS) is an exceptional, low grade, soft tissue sarcoma with indolent biological behaviour, extensive preclinical stage, enhanced localized tumour reoccurrence and delayed, distant metastasis. As the deceptively benign neoplasm was initially scripted by Evans in 1987, the tumefaction is nomenclated as “Evan’s tumour”. Incidence of sarcomas is nearly 1% of adult malignancies wherein low-grade fibromyxoid sarcoma represents roughly beneath <5% of soft tissue sarcomas 1.

scholarly journals Histologic and Immunohistochemical Analyses of Soft Tissue Sarcomas From brca2-Mutant/tp53-Mutant Zebrafish Are Consistent With Neural Crest (Schwann Cell) Origin

2016 ◽  
Vol 54 (2) ◽  
pp. 320-327 ◽  
Author(s):  
L. A. White ◽  
J. M. Sexton ◽  
H. R. Shive
Keyword(s):  
Soft Tissue ◽  
Neural Crest ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
Undifferentiated Sarcoma ◽  
Immunohistochemical Markers ◽  
Neural Tissues ◽  
Histologic Differentiation ◽  
Cancer Multiple ◽  
Immunohistochemical Analyses

The zebrafish ( Danio rerio) provides a powerful model for analyzing genetic contributors to cancer. Multiple zebrafish lines with cancer-associated genetic mutations develop soft tissue sarcomas that are histologically consistent with malignant peripheral nerve sheath tumor (MPNST). The goal of this study was to determine the phenotype of soft tissue sarcomas in a brca2-mutant/ tp53-mutant zebrafish line using immunohistochemical markers that are commonly expressed in mammalian MPNST. We classified 70 soft tissue sarcomas from a brca2-mutant/ tp53-mutant zebrafish cohort as MPNST, undifferentiated sarcoma, or other tumor based on histologic features. The expression of S100, CD57, and glial fibrillary acidic protein (GFAP) was analyzed in nonneoplastic neural tissues and tumor specimens by immunohistochemistry. Each marker was expressed in nonneoplastic neural tissues. In MPNST, S100 and CD57 were widely expressed in neoplastic cells, with greater consistency observed for CD57 expression. In undifferentiated sarcomas, results were variable and correlated to anatomic location. Coelomic undifferentiated sarcomas often exhibited widespread CD57 expression but limited S100 expression. In comparison, ocular undifferentiated sarcomas exhibited limited expression of both CD57 and S100. Overall, CD57 and S100 expression was significantly higher in MPNST than in undifferentiated sarcomas. GFAP was not expressed in any of the tumors. This study identified commercially available antibodies that are useful for analyzing S100, CD57, and GFAP expression in zebrafish. This study further shows a correlation between degree of histologic differentiation and expression of these markers in soft tissue sarcomas from brca2-mutant/ tp53-mutant zebrafish and suggests that these cancers are derived from the neural crest with differentiation toward myelinating Schwann cells.

Discordance of histo-pathological diagnosis of patients with soft tissue sarcoma referred to tertiary care center.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11064-11064 ◽  
Author(s):  
Sameer Rastogi ◽  
Aditi Aggarwal ◽  
Kamal Raj Soti ◽  
Ilavarasi Vanidassane ◽  
Mehar C Sharma ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Treatment Plan ◽  
Care Center ◽  
Tertiary Care ◽  
Soft Tissue Sarcomas ◽  
Pathological Diagnosis ◽  
Histopathological Diagnosis ◽  
Undifferentiated Sarcoma ◽  
Major Discrepancy ◽  
Set Up

11064 Background: Reaching to the correct histo-pathological diagnosis of soft tissue sarcomas (STS) is a great challenge and is cornerstone for treatment planning. Need of expertise for diagnosis is limited by lack of expert pathologists and dedicated sarcoma oncologists in India. Through this study we highlight the pattern of pathological diagnosis and accuracy outside specialist centre. Methods: We did retrospective analysis of all patients referred to us with diagnosis of STS in the last 12 months (January 2016 to 2017). According to protocol, all patients had pathology review from our institute. If blocks were available then they were reviewed and if necessary, fresh biopsy was performed. Besides, pathological diagnosis was reviewed in joint clinic, giving clinico-radiological inputs to sarcoma pathologists. For patients diagnosed outside and had discordant report, we divided them into major discrepancy (including change of diagnosis of sarcoma to benign or other histological entity that could potentially change the treatment plan) or minor discrepancy (like mild change in grade or histopathological diagnosis not affecting the treatment plan). Results: There were 149 patients registered with median age of 36 years (14-77 years) and 93 patients (62.4%) were males. 85(57%) patients had localized disease. Most common subtypes were synovial sarcoma 16%, liposarcoma 9%, soft tissue ewings sarcoma 9%, MPNST 9%, leiomyosarcoma 8%, pleomorphic undifferentiated sarcoma 8% etc. Of 149 patients, 42 had not been worked up outside and thus comparison was not possible while 4 patients couldn’t retrieve blocks and repeat biopsy could not be performed. Of 97 patients (biopsy = 84, FNAC = 13) who had diagnosis from outside, 37% had major discrepancy and 24% had minor discrepancy compared with our biopsy review. Major discrepancy was more in non extremity than extremity STS (p = 0.003). Conclusions: Pathological diagnosis of more than half of patients referred from outside was discordant with respect to diagnosis of our centre with major implications on 37%. We believe this is due to lack of sarcoma pathology experts and virtually non-existent multidisciplinary clinics in set up outside tertiary care centres.

scholarly journals Patient and Tumour Characteristics of Adult Head and Neck Soft Tissue Sarcomas: A Systematic Review and Meta-Analysis

Sarcoma ◽  
2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Sakshi Andersen ◽  
Henriette Mann ◽  
Anders Krarup-Hansen ◽  
Christel Bræmer Lajer ◽  
Christian Grønhøj
Keyword(s):  
Systematic Review ◽  
Soft Tissue ◽  
Head And Neck ◽  
Treatment Guidelines ◽  
Meta Analysis ◽  
Soft Tissue Sarcomas ◽  
Disease Free Survival ◽  
Biological Behaviour ◽  
Grade 3 ◽  
Tumour Characteristics

Background. Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due to the rarity and varied biological behaviour among various subtypes. This systematic review examines the characteristics of tumours and patients with HNSTS. Materials and Methods. A systematic literature review and meta-analysis were performed using the electronic databases PubMed and Embase. Eight eligible studies were identified, and 13 variables were extracted from each study including 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) rate. Results. We identified eight studies (n = 1,120 patients; 739 males (66%)) from six different countries). In total, 24 histological subtypes were found, and 20% of the sarcomas (n = 215) could not be subclassified. 607 sarcomas (57%) were <5 cm in diameter, and 945 sarcomas (84%) were grade 3. 1,059 patients (90%) underwent surgery. Estimated 5-year OS was 74% (95% CI; 0.63–0.84%) and 5-year DFS was 56% (95% CI; 38–74%). Conclusion. HNSTS holds a relative poor prognosis possibly explained by the heterogeneity of the disease. Treatment of HNSTS has shown to be highly diverse, underlining the importance of uniformed treatment guidelines in order to achieve improved survival outcomes.

Clinical features and results of therapy for children with paraspinal soft tissue sarcoma: a report of the Intergroup Rhabdomyosarcoma Study.

1991 ◽  
Vol 9 (5) ◽  
pp. 796-801 ◽  
Author(s):  
J A Ortega ◽  
M Wharam ◽  
E A Gehan ◽  
A H Ragab ◽  
W Crist ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Complete Response ◽  
Tumor Control ◽  
Clinical Group ◽  
Undifferentiated Sarcoma ◽  
Group I ◽  
Field Radiation ◽  
Extended Field ◽  
Extraosseous Ewing’S Sarcoma

Soft tissue sarcomas of the paraspinal region comprised 3.3% (56 of 1,688) of the patients entered and eligible on Intergroup Rhabdomyosarcoma Studies I (IRS-I) and II (IRS-II) (1972 to 1984). These lesions tended to be greater than 5 cm in diameter at diagnosis, invaded the spinal extradural space, and were of the extraosseous Ewing's sarcoma or undifferentiated sarcoma subtype in 55% (30 of 56) of the cases. Patients with tumors in clinical groups II, III, and IV were treated with radiotherapy (XRT) and vincristine-dactinomycin (VA) or VA plus cyclophosphamide (VAC) +/- doxorubicin. Clinical group I patients treated on IRS-II did not receive XRT, while those on IRS-I were randomized to receive VAC +/- XRT. Forty-four of the paraspinal patients (79%) achieved a complete response (CR) compared with 77% (1,260 of 1,632) for patients with disease in other sites. Twenty-seven patients (55%) subsequently relapsed (five local, three regional, four local and distant, and 14 distant). The proportion of patients surviving 5 years by clinical group (stage) from I to IV were 50%, 50%, 62%, and 27%, respectively. Paraspinal patients had somewhat poorer survival than patients with disease in other sites, both in IRS-I and IRS-II; the percentage of paraspinal patients surviving 5 years was 50% and 52% for IRS-I and IRS-II, respectively, whereas these percentages were 55% and 63% for patients with disease in other sites. Histology did not influence the CR rate, but unexpectedly, patients who had embryonal rhabdomyosarcoma (RMS) had the poorest overall survival rate. We concluded that patients with paraspinal lesions may require extended-field radiation therapy to reduce the high local failure rate and more intensive chemotherapy to achieve better local and systemic tumor control.

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