Teratoma, rhabdomyosarcoma and other tumours
Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven in Succeeding in Paediatric Surgery Examinations, 2017
From the statements above, choose the most appropriate treatment for the following cancer patients. The same statement can be used more than once to answer any of the questions. An 11-year-old girl with phaeochromocytoma.A 15-year-old with synovial sarcoma.A 12-year-old with stage II yolk sac testicular tumour postorchidectomy.A 15-year-old with stage III Hodgkin’s lymphoma.A 5-year-old with stage III neuroblastoma.A 13-year-old with malignant melanoma.A 15-year-old with stage IVB osteogenic sarcoma of the distal femur.An 8-year-old with alveolar sarcoma.A 6-year-old with stage IV neuroblastoma.A 10-year-old postorchidectomy boy with stage IV testicular yolk sac tumour.
Pleuropulmonary Blastoma
Dongyou Liu in Handbook of Tumor Syndromes, 2020
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.
Soft tissue sarcomas
Anju Sahdev, Sarah J. Vinnicombe in Husband & Reznek's Imaging in Oncology, 2020
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).
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
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.
Investigational therapies in phase II clinical trials for the treatment of soft tissue sarcoma
Published in Expert Opinion on Investigational Drugs, 2019
Juan Martin-Liberal, Ezequiel Pérez, Xavier García Del Muro
Another type of adaptive cell therapy is T-cell receptor (TCR) treatment. Similarly to CAR T-cell therapy, TCR therapy involves T-cells engineering to make them express TCR α and β chains that confers antigen-specificity to the T-cells. The main difference with CAR T-cell therapy is that TCR treatment is dependent on the presentation of the tumor antigen by the molecules of the human leukocyte antigen (HLA) system [71]. Since synovial sarcoma expresses the cancer testis antigen NY-ESO-1 in 70–80% of cases, TCR therapy against NY-ESO-1 has been assessed in this STS subtype [79]. A pilot trial was conducted in advanced synovial sarcoma and melanoma patients with HLA-*0201 allele using a TCR treatment specific for NY-ESO-1. In total, 11 out of the 18 patients (61%) recruited with synovial sarcomas achieved PR and OS rates at 3 and 5 years were 38% and 14%, respectively [80]. These results indicate that this treatment strategy is active in synovial sarcoma. Indeed, a TCR therapy targeting NY-ESO-1 has recently gained breakthrough therapy designation by the FDA for advanced synovial sarcoma patients with HLA-A * 201, HLA-A * 205 or HLA-A * 206 alleles [71]. Further investigation is ongoing.
Related Knowledge Centers
- Joint Capsule
- Synovial Membrane
- Tendon Sheath
- Joint
- Heart
- Soft Tissue
- Cancer
- Brain
- Soft-Tissue Sarcoma
- Prostate