BACKGROUND: We present the case of a patient with a rare synovial sarcoma (SS) of the tibial nerve. So far, only 4 cases of patients with SS originating from the tibial nerve have been described in the literature, and our patient is only the second patient whose limb was saved during treatment. Synovial sarcomas are malignant mesenchymal tumors, i.e., tumors arising from connective tissue. Synovial sarcomas account for 5-10% of all soft tissue sarcomas. However, the name synovial sarcoma is misleading, because the tumor does not originate from synovial cells, but rather from primitive mesenchymal cells. The name most likely originated from the localization around the large joints on the limbs, more often on the lower ones, in the area of the knee joints. We point out the aspects of correct and quick diagnosis and subsequent treatment, which has very important effect on the patient's prognosis. Primary less radical excision without prior biopsy verification leads to a higher risk of local recurrence, even if a proper reexcision was performed immediately after biopsy verification of the sarcoma. CASE PRESENTATION: A woman born in 1949 began to suffer at the end of 2020 with escalating pain under the left inner ankle with a projection to the sole and fingers. Her personal, family work and social history were insignificant. After the initial neurological examination, the patient was sent for an ultrasound examination of the ankle, which showed a lobular mass measuring 50 × 22 × 16 mm and according magnetic resonance imaging, the finding appeared to be a suspicious neurinoma of the tibial nerve. The tumor was surgically excised, without prior biopsy verification: a 50 × 20 mm tumor was dissected in the distal part of the tarsal canal, which grew through the structure of the tibial nerve and in some places into the surrounding area and appeared intraoperatively as a neurofibroma. But histologically the tumor was classified as monophasic synovial sarcoma. The patient was indicated for a wide reexcision of the skin with the subcutaneous tissue of size 91 × 20 × 15 mm. Now the patient is being treated with external radiotherapy to the tumor bed and she is able to walk. CONCLUSION: This report draws attention to a rare type of malignant nerve tumor, which both clinically and radiologically can mimic benign peripheral nerve sheath tumors. Synovial sarcoma should be considered in very painful resistances, typically located around the joints of the lower limbs, the growth of which can be slow. Because the size of the tumor is a negative prognostic factor, it is necessary to make a timely diagnosis using MR imaging and a biopsy with histological examination and to start treatment quickly. Surgical treatment should take place only after a biopsy with histological examination of the tumor so that it is sufficiently radical and does not have to undergo an additional reoperation, as happened in the case of our patient.
- MeSH
- časná diagnóza MeSH
- lidé MeSH
- nádory nervové pochvy * MeSH
- neurilemom * MeSH
- prognóza MeSH
- senioři MeSH
- synoviom * diagnostické zobrazování chirurgie MeSH
- Check Tag
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Synoviálni sarkom (SS) v oblasti hlavy a krku je u dětí velmi vzácné onemocnění a synoviální sarkom jazyka u kojence dosud nebyl popsán. V kazuistice popisujeme zkušenosti s léčbou bifázického SS jazyka u 8měsíční dívky, který je podle našich vědomostí první publikovanou kazuistikou v anglické literatuře.
Synovial sarcoma (SS) arising from head and neck region in children is very rare. No case of synovial sarcoma of tongue in an infant has been reported. We report a case of biphasic SS of tongue in 8-months-old female making it the first documented case of English language literature.
Synoviální sarkom je maligní mezenchymální vřetenobuněčný nádor s variabilní epiteliální diferenciací. Prezentujeme zde raritní případ mladé pacientky s primárním synoviálním sarkomem jater, jehož diagnóza byla ověřena molekulárně patologickou detekcí fuzního transkriptu SYT/SSX. Multidisciplinarita, ale i vysoké požadavky na odbornost předurčují pacienty se sarkomem k tomu, aby byli léčeni v centrech, která jim poskytnou potřebnou komplexní péči.
Synovial sarcoma is malignant mesenchymal fuso-cellular tumor with variable epithelial differentiation. We present the rare case of young female patient with primary synovial sarcoma of the liver. Molecular diagnosis was done by detection of SYT/SSX fusion transcript. Patients with sarcoma should be treated only in high specialized centres.
- MeSH
- biopsie MeSH
- fúzní onkogenní proteiny * MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mladý dospělý MeSH
- nádorové biomarkery MeSH
- nádory jater * diagnóza chirurgie patologie MeSH
- počítačová rentgenová tomografie MeSH
- synoviom * diagnóza chirurgie patologie MeSH
- Check Tag
- lidé MeSH
- mladý dospělý MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
- práce podpořená grantem MeSH
Aim: Synovial sarcoma is a clinically and morphologically distinct neoplasm of uncertain histogenesis predominantly affecting the extremities of adolescents and young adults. It is the fourth commonest soft tissue sarcoma in adults. It is regarded as an aggressive neoplasm with a reported 5-year survival rate ranging from 40-76%. The aim of our presentation is to verify the unusual presentation of long-standing pain at the site of a deep small tumour, without the development of swelling. Patients - methods and results: An 18-year-old young man suffered with localised pain in the medial lower part of the right thigh for 5 years. The pain appeared after prolonged exercise (cycling), and worsened with palpation. He had no pain at rest. There was no palpable mass in the thigh. A small spherical lesion was diagnosed as an obliterated venous varix one year prior to admission for an MRI. A small deep localised tumour was revealed by an ultrasonography and MRI shortly before surgery. Surgical excision was performed using preoperative ultrasonography. A solid 1 cm diameter demarcated tumour was removed from the deep inter-muscular septum. Surprisingly, the histological diagnosis of a monophasic synovial sarcoma was established. Thus the reoperation of the surgical margin excision was indicated. No malignant cells were found in the excised soft tissues. The patient is alive and well 1 year after the surgery. The controlled MRI of the thigh did not reveal any signs of a viable tumour. Discussion: The occurrence of long-standing pain at a tumour site prior to the development of swelling is uncommon. The pathophysiology of this pain is unclear. Awareness of this unusual presentation and appropriate investigation may enable the detection of synovial sarcoma at an early prognostically favourable stage.
- MeSH
- bolest diagnóza etiologie MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mladý dospělý MeSH
- stehno patologie MeSH
- synoviom chirurgie patologie MeSH
- Check Tag
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
