Synovial sarcoma of kidney in a child: A rare presentation

We present a 4-year-old female who was suspected to have Wilms' tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms' tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction.
Source: Journal of Indian Association of Pediatric Surgeons - Category: Surgery Authors: Source Type: research

Related Links:

Abstract: Synovial sarcoma (SS) is a high-grade soft-tissue sarcoma occurring predominantly in older children and young adults. Only approximately 7% occur in the head and neck region, with SS representing less than 0.1% of all head and neck cancers. Orbital location is exceedingly rare with only 8 cases reported so far in the literature. It is noted for its propensity for late local recurrences and metastases. Histologically, SS is monophasic, biphasic, or poorly differentiated and harbors a specific chromosomal translocation t(X;18)(p11.2;q11.2) in>95% of cases. In this article, we describe a case of monophasic SS p...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Brief Report Source Type: research
Authors: Xing Z, Wei L, Jiang X, Conroy J, Glenn S, Bshara W, Yu T, Pao A, Tanaka S, Kawai A, Choi C, Wang J, Liu S, Morrison C, Yu YE Abstract Synovial sarcoma is the most common pediatric non-rhabdomyosarcoma soft tissue sarcoma and accounts for about 8-10% of all soft tissue sarcoma in childhood and adolescence. The presence of a chromosomal translocation-associated SS18-SSX-fusion gene is causally linked to development of primary synovial sarcoma. Metastases occur in approximately 50-70% of synovial sarcoma cases with yet unknown mechanisms, which led to about 70-80% mortality rate in five years. To explore the...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
We describe a case of a 42-ys-old female patient complaining a cervical swelling due to left intrathyroid nodule, measuring 35 mm in its greatest dimension. A Fine Needle Aspiration Cytology (FNAC) was performed and diagnosis of indeterminate neoplastic lesion, indefinite whether primary or metastatic, was formulated. After complete thyroidectomy, the histological picture of the nodule was characterized by a dual cellular population: several glandular structures composed by columnar cells with clear cytoplasm were embedded in a highly cellular stroma composed of spindle-shaped elements. Immunohistochemistry and molecular b...
Source: Pathologica - Category: Pathology Tags: Pathologica Source Type: research
We report a case of primary subcutaneous SS in the forearm of a young woman and discuss the histopathologic differential diagnosis with other similar neoplasms. This is the first reported case of primary cutaneous SS showing immunoreactivity for TLE1 in the nuclei of neoplastic cells, supporting the use of this marker for diagnosis of this rare cutaneous neoplasm.
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Extraordinary Case Report Source Type: research
ConclusionsPET –CT in high-grade bone and soft tissue sarcoma can add significant benefit to routine CT/MRI staging. Further prospective and multi-centre evaluation of PET–CT is warranted to determine the actual predictive value and cost-effectiveness of PET–CT in directing clinical management of clinically complex and heterogeneous high-grade sarcomas.
Source: Clinical Sarcoma Research - Category: Cancer & Oncology Source Type: research
Conclusion: FISH is a useful adjunct in the diagnostic assessment of different types of soft tissue sarcomas. It is easy to set up, is relatively inexpensive and has the ability to diagnose sarcomas with great accuracy, especially in cases which can not be accurately classified even after thorough histological and immunohistochemical evaluation. It may play a very important role in the accurate diagnosis and correct management of patients. PMID: 29580035 [PubMed - in process]
Source: Asian Pacific Journal of Cancer Prevention - Category: Cancer & Oncology Tags: Asian Pac J Cancer Prev Source Type: research
We report a case of intraarticular SS mimicking nodular synovitis and lateral meniscus rupture. Due to clinical and radiological presentation, arthroscopic synovectomy was performed, and histology confirmed nodular synovitis. After four years the lesion recurred and new arthroscopic biopsy was performed, revealing a monophasic SS with SYT/SSX1 translocation. Repeated histology of the first specimen confirmed appearance of a nodular synovitis microscopically, with no morphological criteria for a sarcoma, but molecular analysis showed positive SYT/SSX1 translocation. Wide extraarticular knee resection and reconstruction with...
Source: Orthopaedics and Traumatology: Surgery and Research - Category: Orthopaedics Source Type: research
Conclusions: There is good correlation between karyotyping and FISH. Complex FISH signals found in dedifferentiated liposarcomas may be related to an increased chromosome 12 copy number and ploidy. Karyotyping is an important baseline standard for the quality assurance of newly developed FISH probes. It also provides a global view of chromosomal changes and the opportunity to investigate the role of other genetic alterations and potential therapeutic targets.
Source: Applied Immunohistochemistry and Molecular Morphology - Category: Chemistry Tags: Research Article Source Type: research
aacute;pi Z Abstract Break-apart FISH probes are the most popular and reliable type of FISH probes used to confirm certain pathological diagnoses. The interpretation is usually easy, however, in some instances it is not so unequivocal. Our aim was to reveal and elucidate the problems occurring in the process of evaluation of the break-apart probe results. Altogether 301 soft tissue sarcomas with confirmed molecular tests using break-apart probes were assessed to reveal the frequency and type of unusual signal pattern. Among 89 synovial sarcoma (SS18) 11%, 12 alveolar rhabdomyosarcoma (FOXO1) 50%, 53 myxoid liposar...
Source: Pathology Oncology Research - Category: Pathology Authors: Tags: Pathol Oncol Res Source Type: research
Synovial sarcoma (SS) is a soft tissue sarcoma of the extremities developing in young adults that has rarely been reported in the orbit. SS is associated with a unique translocation, resulting in an SYT-SSX fusion gene. We analyze seven published periocular cases, together with the current one, to gain a better appreciation of the features of the tumor in this location and to compare the findings with those derived from non-ophthalmic studies. A 31-year-old woman developed an inferior orbital mass after experiencing periorbital and hemifacial pain for more than a decade.
Source: Survey of Ophthalmology - Category: Opthalmology Authors: Source Type: research
More News: Chemotherapy | Children | Neoadjuvant Chemotherapy Therapy | Neoadjuvant Therapy | Pediatrics | Sarcomas | Soft Tissue Sarcoma | Surgery | Synovial Sarcoma | Translocation | Urology & Nephrology