Soft-­Tissue Tumors: (Rhabdomyosarcoma and Other Soft-­Tissue Sarcomas)

2013 ◽  
pp. 241-272 ◽  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors

Epithelioid Hyalinizing Sarcoma With MGA-NUTM1 Fusion

2020 ◽  
Vol 154 (6) ◽  
pp. 859-866
Author(s):  
Caroline I M Underwood ◽  
Diana M Cardona ◽  
Rex C Bentley ◽  
Guomiao Shen ◽  
Xiaojun Feng ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Immunohistochemical Study ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Molecular Testing ◽  
Molecular Alterations ◽  
Poorly Differentiated ◽  
Next Generation Sequencing Ngs ◽  
Generation Sequencing

Abstract Objectives Soft tissue sarcomas are a group of tumors derived from the mesenchymal origin. Historically, they have been classified according to morphologic and immunohistochemical characteristics. The advent of multiplexed next-generation sequencing (NGS), specifically RNA sequencing, has modified the classification of such tumors and others by determining categorization based on molecular alterations. The NUTM1 rearrangement, previously thought to be present only in carcinomas, has recently been reported in poorly differentiated high-grade sarcomas of the soft tissue. We present the first reported case of an epithelioid hyalinizing sarcoma harboring the MGA-NUTM1 fusion in an acral site. Methods Histopathologic, immunohistochemical, and molecular testing were performed on resection tissue. Results Histologically, the tumor showed an epithelioid morphology with prominent background hyalinization. Immunohistochemically, the tumor expressed CD99 and nuclear NUT-1. By NGS the tumor harbors MGA-NUTM1 fusion. Conclusions Our findings support more extensive use of NGS for accurate sarcoma classification and identification of potential therapeutic targets. Furthermore, they corroborate the fact that NUTM1-rearranged soft tissue tumors represent a spectrum of heterogeneous morphologic entities. This case also highlights the utility of NUT-1 immunohistochemical study as a possible screening tool for NUTM1-fused sarcomas.

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.

Surgical Management and Minimally Invasive Approaches for the Treatment of Metastatic Sarcoma

2013 ◽  
pp. 457-464
Author(s):  
Peter Hohenberger ◽  
Bernd Kasper ◽  
Kamran Ahrar
Keyword(s):  
Soft Tissue ◽  
Minimally Invasive ◽  
Early Stage ◽  
Tumor Biology ◽  
Local Treatment ◽  
Soft Tissue Sarcomas ◽  
Metastatic Tumor ◽  
Soft Tissue Tumors ◽  
Tumor Spread ◽  
Systemic Treatments

Soft tissue sarcomas describe a very heterogeneous group of soft tissue tumors mainly arising in the lower extremities. If diagnosed at an early stage and a complete resection of the primary tumor is achieved, the patients' prognosis is excellent. However, metastatic tumor spread is common with only limited treatment possibilities. Despite an improved insight into tumor biology of sarcomas, no notable improvement has been gained in the last 20 years regarding prognosis of patients. Metastatic lung disease has long been the preserve of systemic treatments, local treatments being considered in a purely palliative intention. Several studies have objectified benefit to the local treatment of metastases, especially in an oligometastatic state. The development of techniques for stereotactic radiotherapy on the one hand and the refusal or contraindication for surgery on the other hand inaugurated studies in this direction. Besides surgery and radiotherapy, other local modalities have been investigated in the last few years such as thermal therapy (radiofrequency and laser ablation) or combined modalities (isolated limb perfusion and deep-wave hyperthermia plus chemotherapy) to help patients with metastatic soft tissue sarcoma. Minimally invasive, image-guided therapies such as thermal ablation should be considered particularly in patients who are not suitable surgical candidates or may have exhausted all other viable surgical options. Some of these techniques will be reviewed in this article, and their value for the patients will be evaluated in the light of indication from tumor biology and technical feasibility. These highly selected and specific procedures should only be performed after decision making in an interdisciplinary sarcoma-board.

Imaging of the Knee and Surrounding Structures Following Tumor Surgery

2018 ◽  
Vol 22 (04) ◽  
pp. 481-505
Author(s):  
Naomi Winn ◽  
Radhesh Lalam
Keyword(s):  
Soft Tissue ◽  
Treatment Options ◽  
Management Strategies ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Artifact Reduction ◽  
Soft Tissue Lesion ◽  
Metallic Implants ◽  
Primary Bone ◽  
Tumor Management

AbstractLimb salvage is a key goal of tumor management around the knee, with surgical, medical, and radiologic treatment options. Primary bone and soft tissue sarcomas are optimally treated in specialist tertiary centers; however, metastatic disease is encountered in all aspects of radiologic practice, with overlap in the management strategies. Both specialist and generalist radiologists therefore need to be familiar with the expected normal appearances following these therapies and be able to recognize potential complications. This review article describes the techniques available for imaging the knee following treatment of bone and soft tissue tumors, with particular reference to artifact reduction. The therapeutic options for managing bone and soft tissue lesion are discussed, with emphasis on imaging appearances. Surgical, medical, and radiologic treatments are described. Complications and their imaging appearances are reviewed including local recurrence of tumor, infection, complications related to metallic implants, postradiation changes, and amputation. Normal imaging appearances and complications following radiologic treatment (namely radiofrequency ablation) of bone and soft tissue tumors are presented.

scholarly journals Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors

2009 ◽  
Vol 19 (6) ◽  
pp. 1499-1511 ◽  
Author(s):  
M. van Vliet ◽  
M. Kliffen ◽  
G. P. Krestin ◽  
C. F. van Dijke
Keyword(s):  
Soft Tissue ◽  
Mr Imaging ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Imaging Features

scholarly journals Undifferentiated pleomorphic sarcoma: Diagnosis of exclusion

2017 ◽  
Vol 03 (02) ◽  
pp. 133-135
Author(s):  
Pragati S. Upasham ◽  
Sharayu P. Dighavkar ◽  
Prakash M. Roplekar
Keyword(s):  
Soft Tissue ◽  
Lower Extremity ◽  
Fibrous Histiocytoma ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
High Grade ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Malignant Soft Tissue Tumors ◽  
Pleomorphic Sarcoma ◽  
Malignant Soft Tissue

AbstractMalignant soft-tissue tumors which were designated as malignant fibrous histiocytoma are regrouped by the WHO (in 2002) under the new entity termed as “undifferentiated pleomorphic sarcoma.”1 It accounts for less than 5% of all adult soft-tissue sarcomas. Here, we report the lesion in a 70-year-old man who presented with high-grade undifferentiated pleomorphic sarcoma in the lower extremity.

Synovial Sarcoma in the Foot of a 5-Year-Old Child

2016 ◽  
Vol 106 (4) ◽  
pp. 283-288 ◽  
Author(s):  
Gary M. Lepow ◽  
Daniel L. Grimmer ◽  
Onya V. Lemar ◽  
Evan A. Bridges
Keyword(s):  
Soft Tissue ◽  
Synovial Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Excisional Biopsy ◽  
Soft Tissue Tumors ◽  
Tibialis Posterior ◽  
Unexpected Finding ◽  
Tibialis Posterior Tendon ◽  
History Of ◽  
Soft Tissue Masses

The purpose of this case report is to present a rare finding of synovial sarcoma in a 5-year-old child. Most soft-tissue masses of the foot are too often presumed to be small and benign; therefore, compared with soft-tissue sarcomas, they are difficult to clinically differentiate and treat. A 5-year-old girl presented with a painful lesion that was diagnosed as synovial sarcoma after an excisional biopsy was performed. This was an unexpected finding of synovial sarcoma involving the tibialis posterior tendon of her right foot. The patient presented with an 8-month history of tenderness and an antalgic gait. We would like to encourage that all soft-tissue tumors of the foot be preoperatively evaluated with the aid of diagnostic imaging so that a well-planned biopsy assessment can be performed, with adequate margins excised.

scholarly journals Bovine Papillomavirus DNA and S100 Profiles in Sarcoids and Other Cutaneous Spindle Cell Tumors in Horses

2016 ◽  
Vol 54 (1) ◽  
pp. 44-52 ◽  
Author(s):  
E. D. Epperson ◽  
W. L. Castleman
Keyword(s):  
Soft Tissue ◽  
Spindle Cell ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Bovine Papillomavirus ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Spindle Cell Tumors ◽  
Tumor Types

Histopathologic differentiation between deep dermal or subcuticular equine sarcoids (ie, nodular sarcoids) and other spindle cell tumors in the dermis and subcutis such as peripheral nerve sheath tumors (PNSTs) can be challenging based on morphologic criteria alone. It has been proposed that polymerase chain reaction (PCR) for bovine papillomavirus (BPV) DNA and S100 immunohistochemistry be used as diagnostic tests to separate equine sarcoids from PNSTs. We reviewed 197 skin-associated spindle cell tumors (ie, soft tissue sarcomas), including PNSTs and sarcoids, received at the University of Florida between 1995 and 2013 and performed BPV PCR and S100 immunohistochemistry on archived paraffin-embedded tissues. We found that BPV DNA was demonstrable in 70% of the sarcoids, 59% of the PNSTs, 37% of the fibrosarcomas, and 22% of other tumors (myxosarcomas, fibromas, and other sarcomas) diagnosed on histomorphologic characteristics. Positive S100 staining was only seen in 12 tumors in the study (5 fibrosarcomas, 3 sarcoids, 2 PNSTs, and 2 other sarcomas). The results demonstrate that BPV is associated with many skin-associated spindle cell soft tissue tumors in horses in addition to sarcoids. S100 was rarely expressed in equine soft tissue sarcomas in the skin but was expressed in many tumor types, including PNSTs and sarcoids. Because 41% of the PNSTs classified by histomorphology in this study were BPV negative and 94% were S100 negative, it is reasonable to classify these as soft tissue sarcomas with nerve sheath tumor histomorphology rather than as either PNSTs or sarcoids.

scholarly journals Bone and Soft Tissue Tumors: New Treatment Approaches

Cancers ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1832
Author(s):  
Shinji Miwa ◽  
Norio Yamamoto ◽  
Hiroyuki Tsuchiya
Keyword(s):  
Soft Tissue ◽  
Surgical Resection ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Treatment Approaches ◽  
New Treatment

Bone and soft tissue sarcomas require intensive treatments, including chemotherapy, surgical resection with safe margin, and radiotherapy [...]

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