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Soft tissue sarcomas
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Christina Messiou, Dirk Strauss, Eleanor Moskovic
MRI may not reveal involvement of the underlying bone, which occurs in 20% of cases, with features including periosteal reaction, bone remodelling, and direct invasion. MRI findings are generally those of a non-specific heterogeneous mass; however, many display smooth well-defined margins with cystic contents, leading to an erroneous diagnosis of a benign ganglion or myxoma (29,44). Changes compatible with haemorrhage are seen in 40% of cases, and fluid-fluid levels are seen in around 20% of lesions. The so-called ‘triple-signal’ with areas hyperintense, isointense, and hypointense to fat on T2WI has been noted in 35% of cases, representing a mixture of cystic and solid areas with haemorrhage and fibrous tissue (Figure 23.8) (45). As with other STSs, the lung is the main site of metastasis in synovial sarcoma, which occurs in over 50% of patients, with as many as 25% present with metastatic disease. Of note, synovial sarcoma is one of the few STSs which commonly spread to regional lymph nodes, occurring in up to 20% of cases; this should be borne in mind when imaging the tumour so that the regional lymph nodes are included in the area scanned (11,43,44).
Pleuropulmonary Blastoma
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Raúl Barrera-Rodríguez, Carlos Pérez-Malagón
Synovial sarcoma is the main differential diagnosis for PPB in young adults. The most common sites of origin are the thigh, knee, ankle, foot, and upper extremities, but in unusual cases, synovial sarcoma may arise within the chest wall, mediastinum, heart, lung, or pleura. Rarely are PPBs seen in teenagers and typically are more heterogeneous than synovial sarcomas, but the spindle cell components of PPB and synovial sarcoma can be remarkably similar. As synovial sarcoma is linked to a specific chromosomal translocation t(X;18)(p11;q11), which fuses the SYT gene from chromosome 18 to a homologous gene at Xp11 (SSX1, SSX2, or SSX4), immunohistochemical demonstration of airway epithelial cell markers or identification of fusion protein SYT–SSX is helpful for making a diagnosis of synovial sarcoma.
Benign tumors
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Synovialoma, also called benign xanthomatous giant cell tumor or villonodular pigmented synovitis, derives from the synovial membrane of the joints or tendon sheath. It is frequent in the hand and more common in women. When located on the dorsal aspect of the distal digit it appears as a solitary or lobulated, firm to hard, skin-colored tumor, to which the skin is firmly attached. When the tumor is located in the digital pulp it is easily movable and when extirpated it appears as a corymbiform yellow-brownish lesion. It is not tender except when pressed too hard and does not usually interfere with nail growth. Localization in the lateral nail fold may cause a paronychia-like lesion.257 When under the nail, it causes nail deformity.258 Treatment is by meticulous extirpation. Radiographs do not show calcifications in contrast to its very rare malignant counterpart synovial sarcoma. Clinical differential diagnosis comprises MPC, epidermal cyst, neuroma, rheumatoid nodules, Heberden nodes, FK, epidermal cyst, tendon sheath fibroma, multicentric reticulohistiocytosis, tendinous xanthoma, epithelioid sarcoma, and other sarcomas as well as metastases, but in rare cases also granuloma anulare and erythema elevatum diutinum.259
Monophasic synovial sarcoma mimicking schwannoma: a case report of a rare peripheral nerve tumor and literature review
Published in Neurological Research, 2023
Crescenzo Capone, Alessandra Turrini, Giulio Rossi, Vanni Veronesi, Carlo Sacco, Guido Staffa
The surgical resection was fixed in 10% buffered formalin and paraffin-embedded for 18 h. Serial hematoxylin-eosin-stained sections were performed for conventional light microscope examination. The lesion consists of a spindle-shaped cell proliferation made up of uniform, relatively monomorphic spindle cells with a high nuclear-cytoplasmic ratio, and finely stippled chromatin. The neoplastic cells were closely packed, growing in short, interlacing and intersecting fascicles around a branching, ‘hemangiopericytoma-like’ vascular network (Figure 2). The mitotic rate was low (about 3 mitoses/10 HPFs) and necrosis absent. Immunohistochemical analysis showed diffuse expression of CD56 and bcl2, moderate staining for EMA and focal reactivity with pan-cytokeratins, whereas S100 protein, smooth muscle actin, desmin, and CD34 were entirely negative (Figure 3). A diagnosis of monophasic synovial sarcoma was made.
Risk factors for the development of local recurrence in extremity soft-tissue sarcoma
Published in Expert Review of Anticancer Therapy, 2022
Fabio Tirotta, Raza Sayyed, Robin L Jones, Andrew J Hayes
There are many histopathological subtypes of STS. The most recent WHO classification identifies over 80 histopathological variants of indeterminant or malignant STS [2]. The historical attempt to categorize histopathological subtypes of STS by cellular differentiation on the basis of histological appearances, allowing for a localization of certain subtypes of STS to certain structures, is a gross oversimplification of the biological factors governing STS. While on occasion certain STS subtypes do reproducibly localize to similar mesenchymal structures (for instance, leiomyosarcomas are often found to arise from major blood vessels and as such have presumably arisen from vascular smooth muscle), for the most part this is not the case. Synovial sarcoma has no relation to synovium and as such their title is an historical misnomer. Meanwhile, many variants of liposarcoma (except for retroperitoneal liposarcoma arising from the perinephric fat) have no anatomical predilection to fat, and are found in a wide variety of non-fatty location. STS are heterogeneous, exhibiting great anatomical and pathological variability, and in return, the variations affect LR. This means decisions regarding treatment strategies to minimize the risk of LR need to be bespoke rather than protocol driven.
Synovial sarcoma mimicking a thoracic dumbell schwannoma- a case report*
Published in British Journal of Neurosurgery, 2020
Susanth Subramanian, Gandham Edmond Jonathan, Bimal Patel, Krishna Prabhu
Synovial sarcoma is a rare mesenchymal malignant neoplasm that accounts for less than 10% of soft tissue sarcomas. About 95% of the sarcomas occur in the extremities in young adults.4 Synovial sarcomas can arise from any anatomic site. Most common site of primary involvement is the para-articular region of the extremities. However, the reports of synovial sarcoma arising from the heart, lungs, small intestine, spine, and peripheral nerves also have been reported in the literature.2,5 Primary synovial sarcoma of the spine is a rare tumour, arising from the paravertebral regions, paraspinal muscles or epidural spaces. The first case of pediatric intradural synovial sarcoma in literature was reported by Green et al. in 20052. However, the primary thoracic intradural spinal synovial sarcomas in the adult are rare to date. The spine is a rare site for metastatic synovial sarcoma.6