Intraneural synovial sarcoma of the tibial nerve

Hashimoto, Kazuhiko; Nishimura, Shunji; Fujii, Keiji; Kakinoki, Ryosuke; Akagi, Masao

doi:10.1177/2036361318776495

Cited by 7 publications

(3 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[33][34][35] However, most of the MPNSTs do not express KRT7 or KRT19 and are typically negative for SS18-SSX. 36,37 Similarly, SFT and primary renal synovial sarcomas share TLE1, CD34, BCL2, and CD99 expression in their immunoprofile. 38 While SFT exhibits STAT6 positivity, primary renal synovial sarcoma has a SS18-SSX positive staining pattern.…”

Section: Discussionmentioning

confidence: 99%

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients

Challa

Mohanty²,

Jha³

et al. 2023

Int J Surg Pathol

View full text Add to dashboard Cite

Primary renal synovial sarcoma is a rare aggressive mesenchymal neoplasm of the kidney that accounts for less than 1% of renal sarcomas. Herein, we describe the clinicopathologic and molecular findings of 14 renal synovial sarcoma patients in one of the largest case series to date and to our knowledge, the only renal synovial sarcoma series to use novel SS18-SSX IHC. Clinicopathologic, IHC, molecular, management, and follow-up data were reviewed and analyzed. Macroscopically, the tumors had either homogeneous, tan-white, and solid ( n = 10), variegated and solid ( n = 3), or variegated and solid-cystic ( n = 1) cut surfaces. Spindle cell ( n = 10), round cell ( n = 3), and round to epithelioid morphologies ( n = 1) were observed. SS18-SSX IHC was positive in all 14 tumors (diffuse, n = 10; multifocal, n = 2; focal, n = 2). All the tumors harbored SS18::SSX1/2 gene rearrangement. Metastases to the liver, brain, and lung ( n = 1); liver and bone ( n = 1); liver and diaphragm ( n = 1) were identified. Adjuvant chemotherapy was administered in 11/12 patients. Follow-up was available for 10 patients (time period range: 5 to 24 months). Four patients died of disease, and six patients are alive with no recurrence or metastasis. As SS18-SSX IHC showed an excellent concordance with the FISH results, this may reliably be used in the IHC panel of spindle/round cell sarcomas of the kidney and as a molecular surrogate for renal synovial sarcoma, particularly in a resource-limited setting. Also, the tumors with focal SS18-SSX expression had lower break apart signals in the FISH assay (19% and 23% in two tumors with focal SS18-SSX IHC positivity).

show abstract

Section: Discussionmentioning

confidence: 99%

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients

Challa

Mohanty²,

Jha³

et al. 2023

Int J Surg Pathol

View full text Add to dashboard Cite

show abstract

“…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 [1][2][3][4].…”

Section: Backroundmentioning

confidence: 90%

Synovial sarcoma of the tibial nerve - case report of a rare tumor in a rare location requiring early diagnosis

et al. 2023

View full text Add to dashboard Cite

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.

show abstract

“…Synovial sarcoma arising within a peripheral nerve stands as an exceedingly rare occurrence, with only 39 published cases to date. Most of these cases are reported individually, although the most extensive series encompassed ten patients [64][65][66]. These tumors can pose diagnostic challenges in terms of diagnosis, as their imaging characteristics can closely resemble those of BPNSTs, potentially leading to unplanned excision [65,67].…”

Section: Uncommon Primary Sites Of Synovial Sarcoma In the Extremitiesmentioning

confidence: 99%

Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

Cho,

Lee,

Kim

et al. 2023

Cancers

View full text Add to dashboard Cite

Synovial sarcomas are rare and highly aggressive soft-tissue sarcomas, primarily affecting adolescents and young adults aged 15–40 years. These tumors typically arise in the deep soft tissues, often near the large joints of the extremities. While the radiological features of these tumors are not definitely indicative, the presence of calcification in a soft-tissue mass (occurring in 30% of cases), adjacent to a joint, strongly suggests the diagnosis. Cross-sectional imaging characteristics play a crucial role in diagnosing synovial sarcomas. They often reveal significant characteristics such as multilobulation and pronounced heterogeneity (forming the “triple sign”), in addition to features like hemorrhage and fluid–fluid levels with septa (resulting in the “bowl of grapes” appearance). Nevertheless, the existence of non-aggressive features, such as gradual growth (with an average time to diagnosis of 2–4 years) and small size (initially measuring < 5 cm) with well-defined margins, can lead to an initial misclassification as a benign lesion. Larger size, older age, and higher tumor grade have been established as adverse predictive indicators for both local disease recurrence and the occurrence of metastasis. Recently, the prognostic importance of CT and MRI characteristics for synovial sarcomas was elucidated. These include factors like the absence of calcification, the presence of cystic components, hemorrhage, the bowl of grape sign, the triple sign, and intercompartmental extension. Wide surgical excision remains the established approach for definitive treatment. Gaining insight into and identifying the diverse range of presentations of synovial sarcomas, which correlate with the prognosis, might be helpful in achieving the optimal patient management.

show abstract

Intraneural synovial sarcoma of the tibial nerve

Cited by 7 publications

References 21 publications

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients

Synovial sarcoma of the tibial nerve - case report of a rare tumor in a rare location requiring early diagnosis

Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

Contact Info

Product

Resources

About