Synovial Sarcoma Presenting Research Articles

Sarcomas of the Upper Extremity in Children

Bone and soft tissue sarcomas of the upper extremities in children are rare, but the practicing pediatric orthopaedist should be aware of their presentation, biology and treatment. This report presents a brief review of the presentation, diagnostic findings and treatment of osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, synovial sarcoma and infantile fibrosarcoma of the upper extremity in the pediatric population.

Variability in the presentation of synovial sarcoma in children: a plea for greater awareness

Variability in the presentation of synovial sarcoma in children: a plea for greater awareness

Synovial Sarcoma Presenting as Iliotibial Band Friction Syndrome

Iliotibial band friction syndrome is a common entity that is often quickly diagnosed in orthopedic clinics. However, synovial sarcoma is an elusive clinical entity that appears around many joints with variable presentations. This case report is an example of a patient with a classic presentation of iliotibial band friction syndrome that was diagnosed as a synovial sarcoma on further investigation.

Synovial Sarcoma- An unusual cause of Heel Pain

We report a case of 29-year-old female who presented with right heel pain that worsened over a period of two years. The onset of pain was followed by swelling at the medial aspect of right ankle. She was initially treated for plantar fasciitis with multiple steroid injections over the heel. Subsequent MRI revealed a well-defined heterogeneous lesion in continuity with the medial plantar nerve. Excision biopsy was performed and histopathological evaluation revealed monophasic synovial sarcoma. The patient subsequently underwent wide resection and free tissue transfer followed by radiotherapy and chemotherapy. This case highlights an unusual site and presentation of synovial sarcoma which led to delayed diagnosis and treatment.

Variability in the presentation of synovial sarcoma in children

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.

Paraspinal Synovial Sarcoma as an Unusual Postradiation Complication in Pediatric Abdominal Neuroblastoma

The development of a soft-tissue sarcoma is an infrequent but well-known long-term complication of radiotherapy. Malignant fibrous histiocytomas, extraskeletal osteosarcomas, fibrosarcomas, malignant peripheral nerve sheath tumors, and angiosarcomas are most frequently encountered. Radiation-associated synovial sarcomas are uncommon and exceedingly rare in pediatric patients. We report an unusual case of paraspinal synovial sarcoma presenting in an adolescent female 13 years after radiation therapy for her neuroblastoma.

Primary Synovial Sarcoma of the Prostate: Report of 2 Cases and Literature Review

Two cases of primary prostatic synovial sarcoma presenting as a prostatic mass are presented in patients aged 44 and 46 years. Histologically, both tumors were mainly composed of uniform spindle cells forming interlacing fascicles. Clusters of immature epithelioid cells were also observed among the spindle cells in case 1. Immunohistochemically, the tumor cells of both cases were strongly positive for vimentin, bcl-2, CD99, and E-cadherin, as well as focally positive for cytokeratin. However, they were negative for prostate-specific antigen, S-100 protein, CD34, CD117, muscle-specific actin, desmin, and calretinin. The presence of an SYT-SSX gene fusion resulting from t(X;18) was demonstrated from paraffin blocks by reverse transcriptase polymerase chain reaction in both cases. To the authors' knowledge, these represent the fifth and sixth reported cases of prostatic synovial sarcoma. Accurate diagnosis depends on morphologic and immunohistochemical examination and proper molecular analysis.

Synovial sarcoma presenting as a parotid mass: Case report and review of literature

Synovial sarcoma is an unusual neoplasm of mesenchymal derivation, which is uncommon in the head and neck sites. In the parotid gland, it is most likely to be misdiagnosed as a myoepithelial, primary mesenchymal, or metastatic neoplasm. We report a case of synovial sarcoma in a young woman who was seen with temporomandibular joint symptoms with a 10-year history. A review of the literature was undertaken to highlight the radiologic and histologic features that help in diagnosis as well as the suggested therapeutic protocols most likely to ensure better survival. Radiologic and histologic studies of the parotid mass led to a diagnosis of synovial sarcoma. A total parotidectomy was performed, and the individual remains tumor free at 36 months. Review of the literature suggests an aggressive long-term outcome for synovial sarcoma and advocates early diagnosis and radical surgical excision for a favorable prognosis.

Pulmonary Synovial Sarcoma Presenting as a Pneumothorax During Pregnancy

Synovial sarcoma is a clinically rare, but morphologically well-defined neoplasm, which accounts for approximately 10% of all malignant soft-tissue tumors. The diagnosis can be established with clinical and imaging evaluations together with immunohistochemical, electron microscopy, and molecular genetic studies. We describe a case of primary pulmonary synovial sarcoma presenting as a pneumothorax in a young woman at 34 weeks of gestation. Her persistent symptomatology ultimately led to a video-assisted thoracoscopy and thorascopic decortication. The diagnosis was established by pathology and immunohistochemistry of the cells, which were consistent with primary pulmonary synovial sarcoma. Malignancies, even those as uncommon as primary synovial sarcoma, should be considered in the differential diagnosis of pneumothorax during pregnancy.

Primary monophasic synovial sarcoma presenting as a pulmonary mass: a case report

IntroductionPrimary pulmonary synovial sarcoma is an extremely rare tumor with only few case reports in the literature.Case presentationA healthy 67-year-old woman was admitted for investigation of a pulmonary mass found on a routine X-ray. She had a history of breast cancer diagnosed and treated 13 years previously with left mastectomy followed by adjuvant endocrine therapy. No progression of the disease was reported. Thoracic computer tomography disclosed a soft-tissue mass in the lower lobe of the left lung arising in the vicinity of the pleura. No abnormal lymph nodes were noted. Further work-up for metastases was negative. Subsequently, the lower lobe of the left lung was removed and the diagnosis was a monophasic synovial sarcoma.ConclusionThe diagnosis of monophasic primary pulmonary synovial sarcoma requires clinical, imaging and immunohistochemical investigation to exclude alternative primary sources. The treatment of choice is excision (lobectomy or pneumonectomy), which in most of cases is helpful for diagnosis. The prognosis is usually poor.

Primary pleural synovial sarcoma presenting as a multiloculated cyst in an adolescent

Primary pleural synovial sarcoma (PPSS) is a rare pleural malignancy with a grave prognosis. Most cases present as a well-circumscribed mass with foci of haemorrhage and necrosis. We present an unusual case in a Nepalese boy that presented as a multiloculated cyst mimicking hydatid disease. The diagnosis was confirmed by histology and cytogenetic analysis.

A case of monophasic synovial sarcoma presenting as a vulvar mass

Background. Synovial sarcoma is the fourth most commonly occurring sarcoma, accounting for 8–10% of all sarcomas. They arise from unknown pleuripotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures. Synovial sarcoma is characterized by specific chromosomal translocation t (X; 18)(p11, q11). Case. We report the first case of monophasic synovial sarcoma arising in soft tissues of the vulva in a 33-year-old female. Complete excision of the mass was possible with tumor-free margins. Conclusion. Only four previous cases of biphasic synovial sarcoma arising in the vulva have been reported. The finding of the SYT-SSX2 translocation is generally associated with a better prognosis, besides tumor negative margins after excision offer the possibility of a better outlook for this patient.

Synovial Sarcoma Presenting as Posterior Tibial Tendon Dysfunction: A Report of Two Cases and Review of the Literature

Synovial Sarcoma Presenting as Posterior Tibial Tendon Dysfunction: A Report of Two Cases and Review of the Literature

Synovial sarcoma in older patients: clinicopathological analysis of 32 cases with emphasis on unusual histological features.

To analyse the clinicopathological features of synovial sarcoma presenting in patients over 60 years of age, an uncommon subset which have not been specifically studied. Thirty-two cases of primary synovial sarcoma in patients aged > or =60 years were retrieved from the authors' consultation files. These were analysed histologically and immunohistochemically and clinical follow-up was obtained in 26 cases (median duration 41 months). Mean age at diagnosis was 71.6 years (range 60-84) with 19 females and 13 males. Anatomical sites were lower limb (n = 13), upper limb (n = 5), lung/pleura (n = 5), trunk (n = 4), head/neck (n = 3), mediastinum (n = 1) and scrotum (n = 1). Histologically, 23 were monophasic and nine were biphasic; 14 were poorly differentiated, of which five showed focally marked pleomorphism. Unusual features in two cases each included organoid nodules, granular cell change, squamous metaplasia and papillary architecture. Ten patients developed local recurrence and 11 developed metastases, of whom seven died. Large tumour size, poorly differentiated morphology and high mitotic rate correlated with poor outcome. Less than 10% of synovial sarcomas occur in patients over 60, in which age group this diagnosis is often not considered. Despite inevitable bias in consultation material, it seems that these cases, when compared with younger age groups, more often show poorly differentiated histology and more often develop at unusual locations.

Synovial cell sarcoma presenting as spasmodic peroneal flatfoot

Commonest cause for spasmodic peroneal flatfoot is tarsal coalition with others being juvenile chronic arthritis, infections of the subtalar joint, trauma, osteoid osteoma and fibrosarcoma. We present a rare cause of synovial cell sarcoma of the sinus tarsi. This case report highlights the unusual clinical presentation, difficulties in its diagnosis and the resultant delay in diagnosis. We recommend MRI scan in addition to the usual imaging modalities and to consider this rare cause in the differential diagnosis of spasmodic peroneal flatfoot.

Diagnosing malignant bone tumours

The Journal of Bone and Joint Surgery. British volumeVol. 72-B, No. 5 ArticlesFree AccessDiagnosing malignant bone tumoursRJ Grimer, RS SneathRJ GrimerSearch for more papers by this author, RS SneathSearch for more papers by this authorPublished Online:1 Sep 1990https://doi.org/10.1302/0301-620X.72B5.2211748AboutSectionsPDF/EPUB ToolsAdd to FavouritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InRedditEmail FiguresReferencesRelatedDetailsCited byA virtual multi-disciplinary meeting is a cost-effective method of triaging referrals to a regional musculoskeletal oncology service9 May 2021 | Scottish Medical Journal, Vol. 66, No. 3Use of Healthcare Services Two Years before Diagnosis in Danish Sarcoma Patients, 2000–2013Sarcoma, Vol. 2019Has the Scottish Managed Clinical Network for Sarcoma influenced the survival outcomes for primary malignant bone tumours?Journal of Orthopaedics, Vol. 16, No. 3Longer duration of symptoms at the time of presentation is not associated with worse survival in primary bone sarcomaJ. M. Lawrenz, J. F. Styron, M. Parry, R. J. Grimer, N. W. Mesko27 April 2018 | The Bone & Joint Journal, Vol. 100-B, No. 5Identification of adult knee primary bone tumour symptom presentation: A qualitative studyManual Therapy, Vol. 26UK guidelines for the management of bone sarcomas4 May 2016 | Clinical Sarcoma Research, Vol. 6, No. 1Biopsy and the diagnostic evaluation of musculoskeletal tumours: critical but often missed in the 21st century2 August 2015 | ANZ Journal of Surgery, Vol. 86, No. 3Singapore Cancer Network (SCAN) Guidelines for the Initial Evaluation, Diagnosis, and Management of Extremity Soft Tissue Sarcoma and Osteosarcoma15 October 2015 | Annals of the Academy of Medicine, Singapore, Vol. 44, No. 10Alarm symptoms of soft-tissue and bone sarcoma in patients referred to a specialist center1 September 2014 | Acta Orthopaedica, Vol. 85, No. 6Limb Salvage in Children22 April 2014The final diagnosis in patients with a suspected primary malignancy of boneA. M. Malhas, R. J. Grimer, A. Abudu, S. R. Carter, R. M. Tillman, L. Jeys1 July 2011 | The Journal of Bone and Joint Surgery. British volume, Vol. 93-B, No. 7Earlier diagnosis of bone and soft-tissue tumoursR. J. Grimer, T. W. R. Briggs1 November 2010 | The Journal of Bone and Joint Surgery. British volume, Vol. 92-B, No. 11Principles of Detection and DiagnosisVariability in the presentation of synovial sarcoma in childrenThe Journal of Bone and Joint Surgery. British volume, Vol. 90-B, No. 8Ewing's sarcoma of the foot masquerading as osteomyelitisFoot and Ankle Surgery, Vol. 10, No. 1KalkaneusfrakturTumours and Tumour-like LesionsThe masqueradersJournal of Bodywork and Movement Therapies, Vol. 6, No. 3Tumoren im Bereiche der SchulterMusculoskeletal TumoursSystematik der Störungen am BewegungsapparatPaediatric surgical oncologyEuropean Journal of Surgical Oncology (EJSO), Vol. 21, No. 2Metastatic spinal cord compression ? options for surgical treatmentActa Neurochirurgica, Vol. 123, No. 3-4Computed tomographic appearances of the pelvis following hindquarter amputationThe British Journal of Radiology, Vol. 65, No. 780Ewing’s Sarcoma: Experience with 12 Cases15 July 2016 | Journal of Chemotherapy, Vol. 4, No. 1Bone TumorsTumours and Tumour-Like Lesions Vol. 72-B, No. 5 Metrics History Published online 1 September 1990 Published in print 1 September 1990 InformationCopyright © 1990, The British Editorial Society of Bone and Joint Surgery: All rights reservedPDF download

The Early Clinical Presentation of Synovial Sarcoma

While synovial sarcoma most commonly presents as a painless mass, occasionally the cancer emerges in a misleading manner resulting in an unfavorable delay or error in diagnosis. A review of the litrature reveals 4 such occult patterns: pretumor phase characterized only by pain or tenderness; the acute inflammatory lesion presenting as a "hot" arthritis or bursitis; the chronic contracture; the post traumatic tumor. These conditions, especially when otherwise unaccounted for, are indications for biopsy. Four avoidable pitfalls in biopsy management also emerged from the review.