A 7-year-old female spayed Bernese Mountain dog was presented for evaluation of hematuria. Incidentally, a right stifle sarcoma was diagnosed via cytology, which raised concern for histiocytic sarcoma (given the patient\'s signalment) versus another joint-associated sarcoma. Histopathology and immunohistochemistry revealed a CD18-negative, non-histiocytic origin cell population. Findings were consistent with a joint-associated grade II soft tissue sarcoma (STS). The patient\'s hematuria was progressive over 5 months, and urinary bladder transitional cell carcinoma (TCC) was diagnosed via cystoscopy and histopathology. An enlarged right medial iliac lymph node was identified on routine restaging via abdominal ultrasound 3 months later. Cytology of the lymph node revealed a markedly pleomorphic cell population, again raising concern for histiocytic sarcoma (HS). Other differentials included an anaplastic metastatic population from the joint-associated STS or the TCC. Immunocytochemistry revealed a cytokeratin-positive, CD18-, CD204-, and vimentin-negative cell population, consistent with a carcinoma. DNA was extracted from cytology slides to sequence cells for BRAF mutation status. Sequencing revealed a homozygous V596E (transcript ENSCAFT00845055173.1) BRAF mutation, consistent with the known biology of TCC. In neither case was HS truly present in this patient, but immunocytochemistry provided information that helped to optimize the patient\'s chemotherapy recommendations.

Primary mediastinal sarcoma is a rare tumour that usually presents with nonspecific symptoms such as hoarseness, dyspnoea, and chest pain. Superior vena cava (SVC) syndrome is an extremely uncommon complication that is caused by the compression, invasion, and thrombosis of the SVC or brachiocephalic veins. SVC syndrome can present as asymptomatic cases or as rare life-threatening emergencies with upper airway obstruction and increased intracranial pressure. This report describes the case of a 58-year-old female who presented with swelling of the face, neck, and upper limbs associated with dyspnoea on exertion. The radiological investigations revealed a large well-defined central necrotic peripherally enhancing lesion in the superior mediastinum extending anteriorly with the compression of brachiocephalic veins. A histopathological examination detected spindle cells arranged in fascicles with nuclear atypia with immunohistochemistry positive for creatine kinase (CK), smooth muscle actin (SMA), desmin and CD99. These findings established the diagnosis of a mediastinal monophasic synovial sarcoma with SVC obstruction. The patient was initiated on palliative radiotherapy for the management of the SVC, followed by systemic biological treatment with the tyrosine kinase inhibitor pazopanib, and was clinically improved. It is essential to promptly diagnose and treat this condition, especially when SVC syndrome manifests.

UNASSIGNED: Synovial cell sarcoma (SS) is an extremely rare mesenchymal malignancy, representing nearly 10% of all soft-tissue sarcomas. These high-grade soft tissue sarcomas commonly arise in the para-articular regions of lower extremities. However, 15% of Synovial sarcomas has been described at Unusual locations, including head, neck, and trunk. Herein, we describe the twelfth case of primary synovial cell sarcoma of thyroid with a literature review.
METHODS: A 43-year-old woman presented with complaint of a progressive neck mass for the last five-months. She developed with dysphagia and dyspnea nearly 2 months prior, without signs of hoarseness, and weight loss. Ultrasonography in which revealed a heterogeneous, hypervascularized thyroid mass. After total thyroidectomy immunohistochemistry was in favor of primary synovial cell sarcoma of thyroid. The diagnosis was confirmed via Molecular genetic analysis of the SYT-SSX fusion gene transcript using the RT- polymerase chain reaction method. Clinical Discussion: Primary thyroid SVS is an extremely rare malignancy with poor biological behavior. SVS has been known for its tendency to local and distal re-occurrence after a few years of treatment. SS can be classified into two subtypes of monophasic or biphasic based on the presence of mesenchymal and/or epithelial components. Accordingly, the most accurate diagnostic tool for SS is considered to be molecular genetic analysis for SYT/SSX fusion transcript.
CONCLUSIONS: Herein, we reported an extremely rare case of SVS of thyroid gland. These high-grade soft tissue sarcomas mainly present with an asymptomatic rapid growing neck mass. Unspecific clinical presentations and extreme rarity of this disorder, make the diagnosis of thyroid SVS very challenging. Due to paucity of data, there is not enough evidence to establish a reliable mortality rate. However, the prognosis of thyroid SVS seems unfavorable.

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Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct investigation and management, and treatment is largely based on what is known for synovial sarcoma of the upper and lower limbs. This PROSPERO-registered study aims to review the diagnostic methods, treatment regimens, and survival outcomes for patients with synovial sarcoma of the larynx. A systematic search of databases Medline, Embase, SCOPUS, and Web of Science was undertaken in December 2017. The literature search identified 1031 potentially relevant studies, and after the deletion of duplicates and excluded papers, 98 full-text articles were screened. A total of 39 cases were reviewed from 32 studies in the data extraction. The average age at the time of laryngeal synovial sarcoma diagnosis was 32 years (range, 11-79 years). In all cases (n = 39), patients underwent wide surgical excision, with 20 patients requiring a partial or total laryngectomy. A total of 18 patients received adjuvant and 3 received neoadjuvant radiotherapy. Chemotherapy was used in 10 cases, with ifosfamide the most frequently used agent. There was considerable variability in the order and combinations of the abovementioned treatments. No clinicopathologic factors or treatment regimens were associated with improved overall survival or lower rate of recurrence. There is a paucity of literature and heterogeneity in clinical approaches to this highly aggressive sarcoma. Reporting of cases must be standardized and formal guidelines must be established to guide clinical management.

BACKGROUND: Synovial cell sarcomas are usually seen in a juxta-articular location. However, they occur rarely in the head and neck region.
METHODS: We report a rare case of brachial plexus synovial sarcoma in a 24-year old South Asian man treated successfully with surgical excision followed by radiotherapy.
CONCLUSIONS: Synovial sarcoma arising from the brachial plexus is rare. The treatment is multimodal with complete excision (often challenging owing to the proximity of the neurovascular structures) and adjuvant therapy.

Soft tissue sarcomas (STSs) account for less than 1% of adult solid tumors and about 7% of pediatric malignancies, causing 2% of cancer-related deaths. With the advent of PET-computed tomography (CT), the value of (18) fluorine-2-fluoro-2-deoxy-d-glucose (FDG) PET imaging to improve the management of STSs has been explored. FDG PET imaging has been found useful in restaging and treatment response assessment. This article reviews current knowledge and application of FDG PET-CT in initial diagnosis, staging, restaging, treatment response monitoring, and prognosis, with a brief overview of the most common histologic subtypes of STS.

Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group encompassing more than 50 different histological diagnoses arising from primitive mesenchymal tissue. Together, they comprise about half the soft tissue sarcomas diagnosed in children and young adults. Despite each histologies relative rarity, their management schema is similar among the different NRSTS histologies. Surgical management is an important component of the multimodal treatment strategy of all these tumors. Resection with negative margins, while maintaining function, plays an important role as a primary treatment of these patients as well as diminishing the risks of local and distant recurrence.

A synovial sarcoma (also known as malignant synovioma) is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare, only very limited cases are reported. Here, we report a case of primary synovial cell sarcoma of the left side of the heart in a 26-year-old female. She presented with dyspnea and weakness only. She was operated for left-sided mass covering mitral valve; further histo-pathology revealed the mass as synovial cell sarcoma. In Indian scenario, this type of rare case needs documentation.

A 2.5-year-old, male, neutered cat presented with a 5-month history of progressive right hind limb lameness and an enlarged right popliteal lymph node. Radiographs revealed significant bony lysis of the tarsus and distal tibia, and fine-needle aspirate of the bone lesion and lymph node revealed a neoplastic population of cells with uncertain origin. Amputation was elected, and the mass was submitted for histology and cellular culture for better characterization. Histologic examination revealed a mixture of spindle-shaped cells and larger, round to polygonal cells. All cells were immunoreactive for vimentin, and only the larger polygonal cells were also positive for cytokeratin. All cells were negative for desmin, smooth muscle actin, cluster of differentiation (CD)3, CD18, CD79a, macrophage antibody (MAC)387, and glial fibrillary acidic protein. Cultured neoplastic cells failed to express CD18, and were not able to secrete the pro-inflammatory cytokines tumor necrosis factor-α, interleukin-1 (IL-1)β, and IL-6 when stimulated by lipopolysaccharide, disproving that the cells originated from the macrophage or monocyte line. Ultrastructurally, neoplastic cells were characterized by abundant rough endoplasmic reticulum, interdigitating cellular processes, and membrane condensations. Based on location and cytologic, histologic, ultrastructural, and functional studies, this neoplasm was considered a synovial cell sarcoma.