scholarly journals Synovial Sarcoma of the Extremities: A Literature Review

2021 ◽  
Vol 11 (16) ◽  
pp. 7407
Author(s):  
Cosmin Ioan Faur ◽  
Daniel Laurentiu Pop ◽  
Ahmed Abu Awwad ◽  
Carmen Lacramioara Zamfir ◽  
Roxana Folescu ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Synovial Sarcoma ◽  
Soft Tissues ◽  
Spindle Cell ◽  
Joint Capsule ◽  
Chromosome X ◽  
Secondary Tumor ◽  
Cell Components ◽  
Synovial Sarcomas ◽  
Synovial Tissues

Synovial sarcoma (SS) is a rare and highly malignant tumor and a type of soft tissue sarcoma (STS), for which survival has not improved significantly in recent years. Synovial sarcomas occur mostly in adolescents and young adults (15–35 years old), usually affecting the deep soft tissues near the large joints of the extremities, with males being at a slightly higher risk. Despite its name, synovial sarcoma is neither related to the synovial tissues that are a part of the joints, i.e., the synovium, nor does it express synovial markers; however, the periarticular synovial sarcomas can spread as a secondary tumor to the joint capsule. SS was initially described as a biphasic neoplasm comprising of both epithelial and uniform spindle cell components. Synovial sarcoma is characterized by the presence of the pathognomonic t (X; 18) (p11.2; q11.2) translocation, involving a fusion of the SS18 (formerly SYT) gene on chromosome 18 to one of the synovial sarcoma X (SSX) genes on chromosome X (usually SSX1 or SSX2), which is seen in more than 90% of SSs and results in the formation of SS18-SSX fusion oncogenes.

scholarly journals Mediastinal Synovial Sarcoma: A Case Report and Literature Review

2003 ◽  
Vol 10 (7) ◽  
pp. 393-395 ◽  
Author(s):  
Linda SL Cheng ◽  
Gary MK Tse ◽  
Wilson WL Li ◽  
TW Lee ◽  
Anthony PC Yim
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Literature Review ◽  
Synovial Sarcoma ◽  
Spindle Cell ◽  
Diagnosis And Treatment ◽  
Soft Tissue Tumours ◽  
Unusual Location ◽  
Synovial Sarcomas ◽  
Proper Diagnosis

Synovial sarcomas are uncommon soft tissue tumours. Immunohistochemistry and cytogenetic techniques are essential for proper diagnosis and differentiation from other spindle cell neoplasms. A case of mediastinal synovial sarcoma is described, of which the unusual location, diagnosis and treatment form the basis of this report.

scholarly journals Primary Intraosseous Synovial Sarcoma with Molecular Confirmation: Expanding and Clarifying the Spectrum of This Rare Neoplasm

2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Kelsey E. McHugh ◽  
John D. Reith ◽  
Nathan W. Mesko ◽  
Scott E. Kilpatrick
Keyword(s):  
Soft Tissue ◽  
Synovial Sarcoma ◽  
Soft Tissues ◽  
Case Reports ◽  
Bone Lesion ◽  
Metastatic Potential ◽  
Metatarsal Bones ◽  
Synovial Sarcomas ◽  
Primary Bone ◽  
The Right

Synovial sarcoma is a well-known malignant tumor usually originating within deep soft tissues of the lower extremities of adolescents and young adults. Rare radiologically confirmed examples of primary bone synovial sarcoma have been documented, generally in isolated case reports. Herein, we report two cases of primary intraosseous synovial sarcoma, with molecular confirmation, involving the left humerus of a 45-year-old female and the right fourth metatarsal bone in a 36-year-old male. Additionally, we clarify the spectrum of primary intraosseous synovial sarcoma by separately analyzing reported cases with radiographic confirmation of bone origin and molecular support for the diagnosis. There are clinicopathologic differences between those tumors with documented molecular confirmation and those lacking such confirmation, specifically regarding their anatomic distribution (p<0.0001). Regarding the radiology of our two cases, the humeral lesion appeared almost entirely intramedullary without soft tissue extension; the midfoot lesion demonstrated a destructive, metatarsal-centered bone lesion, initially thought clinically to represent primary bone osteosarcoma. The diagnoses of monophasic synovial sarcoma were rendered via core needle biopsies, with molecular FISH confirmation of SYT gene rearrangement. Clinical follow-up data was only available for the female patient with the primary humeral lesion, who underwent surgical resection, with no local recurrence or distant metastasis at 7 months postsurgery. To our knowledge, these are the first reported examples of molecularly confirmed, primary intraosseous synovial sarcomas of the humerus and metatarsal bones. Primary intraosseous synovial sarcomas with molecular confirmation differ clinically from those lacking it; however, the demographic features and metastatic potential appear similar to primary soft tissue synovial sarcoma.

BCOR-CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma

2016 ◽  
Vol 69 (5) ◽  
pp. 792-801 ◽  
Author(s):  
Wan-Shan Li ◽  
I-Chuang Liao ◽  
Mei-Chin Wen ◽  
Howard Haw-Chang Lan ◽  
Shih-Chen Yu ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Synovial Sarcoma ◽  
Spindle Cell ◽  
Round Cell ◽  
Poorly Differentiated

Monophasic Synovial Sarcoma Arising in the Vulva: A Case Report and Review of the Literature

2008 ◽  
Vol 132 (4) ◽  
pp. 698-702
Author(s):  
Beverly E. White ◽  
Alan Kaplan ◽  
Dolores H. Lopez-Terrada ◽  
Jae Y. Ro ◽  
Robert S. Benjamin ◽  
...  
Keyword(s):  
Synovial Sarcoma ◽  
Soft Tissues ◽  
English Literature ◽  
Review Of The Literature ◽  
First Case ◽  
Additional Surgery ◽  
Synovial Sarcomas ◽  
The Right ◽  
Disease Free ◽  
Vulvar Mass

Abstract Synovial sarcomas most commonly arise in the soft tissue of the extremities. Less commonly, these tumors present in the head and neck, abdominal wall, and other sites. However, synovial sarcoma occurring in the vulvar area is extremely rare. Only 2 previous cases of biphasic synovial sarcoma of the vulva have been reported, but no case of vulvar monophasic synovial sarcoma has been described in the English literature. We report the third case of synovial sarcoma and apparently the first case of monophasic synovial sarcoma arising in soft tissues of the vulva. The patient was a 33-year-old woman who presented for evaluation of a painless vulvar mass. The tumor was located in the deep fibroadipose tissue of the right vulva (6.5 × 4.2 × 3.5 cm). The histology of the lesion was that of a monophasic synovial sarcoma with a hemangiopericytic vascular pattern. A subsequent molecular analysis revealed SYT-SSX2 gene fusion, which confirmed the diagnosis of synovial sarcoma. After an initial wide local excision, the patient developed a recurrence in the right groin and received chemotherapy and additional surgery. The patient is currently disease free, on adjuvant chemotherapy, and being followed up closely.

Internal Derangement of the Hip

2019 ◽  
pp. 232-246
Author(s):  
Imran M. Omar
Keyword(s):  
Bone Marrow ◽  
Soft Tissues ◽  
External Rotation ◽  
Joint Capsule ◽  
Internal Derangement ◽  
Loss Of Function ◽  
Contrast Resolution ◽  
Flexion Extension ◽  
Synovial Tissues ◽  
Supporting Structures

Chapter 107 discusses the hip and its component tissues, including the bones, labrum, synovial tissues, muscles, and tendons, and covers the MRI appearances of many of the most common pathologies that occur in and around the hip joint. The hip is a ball-and-socket joint consisting of the femoral head and the cup-shaped acetabulum. Because of its shape, the hip allows multi-axial movements, including flexion/extension, abduction/adduction, and internal/external rotation. A number of supporting structures, including the acetabular labrum and joint capsule, surrounding muscles and tendons, and bursae, help stabilize the hip and allow for a smooth range of motion. Injuries to any of these structures can result in hip pain and loss of function. MRI has become the test of choice to assess hip internal derangement because of its superior assessment of soft tissues and bone marrow and its contrast resolution, which improves conspicuity of pathologic conditions.

scholarly journals Monophasic Synovial Sarcoma of the Left Ventricle of the Heart: An Extremely Rare Case and Literature Review

2021 ◽  
Vol 9 (C) ◽  
pp. 109-113
Author(s):  
Anindhita Muthmaina ◽  
Ery Kus Dwianingsih ◽  
Shinta Andi Sarasati ◽  
Hendry Purnasidha Bagaswoto ◽  
Hasanah Mumpuni
Keyword(s):  
Left Ventricle ◽  
Spindle Cell ◽  
Cardiac Tumors ◽  
Cell Component ◽  
Exertional Dyspnea ◽  
Mesenchymal Marker ◽  
Cell Components ◽  
History Of ◽  
Synovial Sarcomas ◽  
Bcl2 Protein

BACKGROUND: Cardiac sarcomas account for <25% of all cardiac tumors. Of these, angiosarcomas are the most frequent. Synovial sarcomas (SS) are exceedingly rare. We present a case of primary left ventricle (LV) SS, a form of sarcoma particularly rare in the heart. CASE DESCRIPTION: A 19-year-old male was referred for further investigation of a LV tumor, presented with a 3-month history of exertional dyspnea and palpitations. He also experienced several syncopal episodes. The radiologic examination confirmed a mass in the LV, suspected for myxoma of the LV. Histopathologic examination revealed a malignant tumor with spindle cell components, suggesting leiomyosarcoma with differential diagnosis of monophasic SS. Immunohistochemistry demonstrated reactivity of the spindle cell component with the mesenchymal marker vimentin and BCL2 protein, while the smooth muscle marker, desmin, was negative, confirming the diagnosis of monophasic SS. CONCLUSIONS: Monophasic SS in the heart is diagnostically challenging since it shares the broad list of differential diagnoses of spindle cell tumors. Immunostaining is helpful to differentiate those entities to obtain a definitive diagnosis and proper treatment.

scholarly journals Underlying Ossification Phenotype in a Murine Model of Metastatic Synovial Sarcoma

2020 ◽  
Vol 21 (7) ◽  
pp. 2636
Author(s):  
Matthew Kirkham ◽  
Austen Kalivas ◽  
Kaniz Fatema ◽  
Sarah Luelling ◽  
Brooke H. Dubansky ◽  
...  
Keyword(s):  
Young Adults ◽  
Mouse Model ◽  
Synovial Sarcoma ◽  
Soft Tissues ◽  
Bone Development ◽  
Survival Rates ◽  
Primary Tumors ◽  
Synovial Sarcomas ◽  
Worse Prognosis ◽  
Genetic Mouse Models

Synovial sarcoma, an uncommon cancer, typically affects young adults. Survival rates range from 36% to 76%, decreasing significantly when metastases are present. Synovial sarcomas form in soft tissues, often near bones, with about 10% demonstrating ossification in the tumor. The literature is inconclusive on whether the presence of ossification portends a worse prognosis. To this end, we analyzed our genetic mouse models of synovial sarcoma to determine the extent of ossification in the tumors and its relationship with morbidity. We noted higher ossification within our metastatic mouse model of synovial sarcoma. Not only did we observe ossification within the tumors at a frequency of 7%, but an even higher frequency, 72%, of bone reactivity was detected by radiography. An enrichment of bone development genes was associated with primary tumors, even in the absence of an ossification phenotype. In spite of the ossification being intricately linked with the metastatic model, the presence of ossification was not associated with a faster or worse morbidity in the mice. Our conclusion is that both metastasis and ossification are dependent on time, but that they are independent of one another.

Ultrastructural Features of a Poorly Differentiated Neoplasm Resembling Synovial Sarcoma

1977 ◽  
Vol 35 ◽  
pp. 526-527
Author(s):  
P. K. Simons
Keyword(s):  
Fine Structure ◽  
Light Microscopy ◽  
Synovial Sarcoma ◽  
Spindle Cell ◽  
White Male ◽  
Biphasic Pattern ◽  
Malignant Mesenchymal Tumor ◽  
Synovial Sarcomas ◽  
Poorly Differentiated ◽  
Histologic Differentiation

Synovial sarcoma is a malignant mesenchymal tumor most commonly occurring in the extremities, but is occasionally seen in the trunk, head, and neck regions. The well developed lesion exhibits a distinct biphasic histologic morphology consisting of a spindle cell fibrosarcomatous stroma and epithelial-lined pseudoacinar structures with an associated mucopolysaccharide secretion. The presence of this biphasic pattern is dependent upon the degree of histologic differentiation. Few studies of the fine structure of synovial sarcomas are available and none of the literature has been concerned with these neoplasms occurring in the less common areas. The ultrastructural features of a tumor classified as a poorly differentiated synovial sarcoma are presented.A mass of irregularly shaped, cerebreform tissue was surgically removed from the left side of the neck of a 61 year old white male. The majority of the tissue was fixed in neutral buffered formalin and prepared for light microscopy.

Monophasic spindle cell myxoid synovial sarcoma of the hand: promising results with marginal resection

2021 ◽  
Vol 14 (1) ◽  
pp. e238366
Author(s):  
Sandeep Vijayan ◽  
Afsal P Mohammed ◽  
Mahesh Suresh Kulkarni ◽  
Sharath Kumar Rao
Keyword(s):  
Synovial Sarcoma ◽  
Elderly Woman ◽  
Spindle Cell ◽  
Normal Function ◽  
Malignant Neoplasms ◽  
High Incidence ◽  
Male Preponderance ◽  
Synovial Sarcomas ◽  
Prompt Diagnosis

Synovial sarcomas are deep-seated, genetically distinct, malignant neoplasms seen in young adults, with a male preponderance. They have unusual clinical and pathological presentation and mimic many other sarcomas and carcinomas, making the diagnosis quite challenging. Although four variants are identified, occurrence in the hand is extremely rare and leads to significant morbidity. There is a high incidence of local recurrence and distant metastasis within the first 2 years. We report the case of an elderly woman with monophasic spindle cell synovial sarcoma who presented with painless swelling in her palm and underwent local excision of the neoplasm. At 2-year follow-up, she remains totally asymptomatic with normal function of the hand. We also attempt to give an overview about monophasic spindle cell synovial sarcoma with the differentials, which would help surgeons in prompt diagnosis and appropriate management.

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