1/33. Intraneural synovial sarcoma of the median nerve.Intraneural synovial sarcomas are extremely rare in the main nerve trunks of the upper limb. We report on a 16-year-old youth who presented with a painless mass on the flexor aspect of the wrist with the clinical appearance of a ganglion. At operation there was a tumour of the median nerve that was shown on histology to be an intraneural synovial sarcoma.- - - - - - - - - - ranking = 1keywords = median nerve, median, nerve (Clic here for more details about this article) |
2/33. Successful restoration of the trapezius muscle using pedicle latissimus dorsi. A case report.Reconstruction of the trapezius muscle using a pedicle latissimus dorsi flap was performed in a 27-year-old man with a large synovial sarcoma in his shoulder girdle. Size and location of the tumor required combined resection of surrounding muscles, including the trapezius, levator scapulae, and rhomboid major and minor. Thus, an extensive defect of the suspending muscles of the scapula was created after accomplishing an adequate resection of the tumor. The flap was performed to restore the trapezius functionally because there were no adjacent muscles available. The transferred muscle compensated for loss of the trapezius, thereby recovering excellent shoulder function. Although an opportunity of its application is thought to occur infrequently, the pedicle latissimus dorsi can activate scapular motion successfully in the absence of the levator scapulae. The technique may be extended to salvage failed conventional reconstruction after spinal accessory nerve palsy.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
3/33. Malignant peripheral nerve sheath tumor with a t(X;18).We describe an ankle tumor arising in a 16-year-old girl. The tumor demonstrated histology typical of a malignant peripheral nerve sheath tumor (MPNST), but exhibited a variant form of the (X;18) translocation associated with synovial sarcoma. Immunohistochemical stains were positive for vimentin, CD57, collagen type iv, and Bcl-2. Routine and molecular cytogenetic studies showed an unbalanced 3-way chromosomal translocation that involved chromosomes X, 18, and 1. Electron microscopic findings were noncontributory. This unusual tumor raises the following questions and possibilities: (1) As the t(X;18) suggests, could this tumor be a monophasic synovial sarcoma with the histologic features of an MPNST? (2) Or, as the histology suggests, is this tumor an MPNST that has a t(X;18)? (3) Finally, could MPNST histology, a t(X;18), and no defining immunohistochemical or electron microscopic features represent an as yet unrecognized part of a spectrum that spans from synovial sarcoma to MPNST or other spindle cell tumors?- - - - - - - - - - ranking = 0.0099323956423489keywords = nerve (Clic here for more details about this article) |
4/33. Synovial sarcomas of three children in the first decade: clinicopathological and molecular findings.Synovial sarcoma in children below the age of 10 years is rare. We report on three cases of synovial sarcoma which were diagnosed in three children aged 3, 8 and 8 years, respectively. These tumors were located in the hip of the 8-year-old, the foot of the 3-year-old, and the elbow of the other 8-year-old. Histologically, one tumor was a biphasic synovial sarcoma, and the other two, which had been initially diagnosed as infantile fibrosarcoma, were of the monophasic fibrous type. In the three cases, a reverse transcription-polymerase chain reaction (RT-PCR) using ribonucleic acid extracted from formalin-fixed, paraffin-embedded tissues detected SYT-SSX1 fusion gene transcripts resulting from translocation t(X;18)(p11.2;q11.2), which is specific for synovial sarcoma. ETV6-NTRK3 fusion gene transcripts that result from t(12;15)(p13;q25), which is characteristic of congenital/infantile fibrosarcoma, were not demonstrated. In conclusion, other pediatric soft tissue sarcomas, such as congenital/infantile fibrosarcoma, spindle cell rhabdomyosarcoma, leiomyosarcoma and malignant peripheral nerve sheath tumor, should be distinguished from synovial sarcoma in children, especially the monophasic fibrous type. RT-PCR analysis is a useful approach to the final diagnosis of synovial sarcoma arising at such an early age.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
5/33. Intraneural monophasic synovial sarcoma: a case report.STUDY DESIGN: A case report. OBJECTIVES: To illustrate a rare case of synovial sarcoma arising within a peripheral nerve. SUMMARY OF BACKGROUND DATA: A synovial sarcoma arising within a peripheral nerve is very unusual. Only five cases of primary synovial sarcoma within a peripheral nerve have been reported. This is the first case with involvement of the nerve root. The authors diagnosed the tumor arising within the S1 nerve root as synovial sarcoma using cytogenetic analysis that detected the chimeric SYT/SSX gene. methods: In addition to the immunohistochemical study, a reverse transcription-polymerase chain reaction (RT-PCR) assay was conducted for the SYT-SS10 fusion gene using archival formalin-fixed paraffin-embedded tumor specimens. RESULTS: Computed tomography scan, magnetic resonance imaging performed before surgery, and the intraoperative findings showed that the tumor was embedded within the S1 nerve root. Although the histologic findings were suggestive of a malignant peripheral nerve sheath tumor, the results of the cytologic study confirmed its diagnosis of synovial sarcoma. CONCLUSION: Primary intraneural synovial sarcoma, although rare, must be distinguished from malignant peripheral nerve sheath tumor. The molecular assay of the detection of the SYT/SSX fusion gene is useful to make a definite diagnosis of monophasic synovial sarcoma.- - - - - - - - - - ranking = 0.015891833027758keywords = nerve (Clic here for more details about this article) |
6/33. Uncommon hand tumours.This paper presented clinical examples of some rare or uncommon presentations of tumours of the hand, including glomus tumour, intraosseous dermoid, fibrolipoma of the radial nerve, blastomycotic cyst and synovial sarcoma. Each lesion is illustrated by review of case histories, radiological and pathological features.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
7/33. Intrarenal solitary fibrous tumor of the kidney report of a case with emphasis on the differential diagnosis in the wide spectrum of monomorphous spindle cell tumors of the kidney.Solitary fibrous tumor (SFT) is a neoplasm that can occur in the urogenital tract, and is also reported occurring in the spermatic cord, seminal vesicles, urinary bladder, prostate, and kidney. Furthermore, it is most important to consider its existence in the kidney, because it is usually diagnosed as renal cell carcinoma pre-operatively. To our knowledge, only 10 cases of SFT have been reported in the kidney to date. We report the clinico-pathological features of an intrarenal SFT occurring in a 31-year-old woman. The tumor, measuring 8.6 cm in its greatest diameter, completely replaced the cortex and the medulla of the middle region of the right kidney, compressing the pelvis. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumor was composed of a proliferation of bland-looking vimentin , CD34 , bcl2 and CD99 spindle cells exhibiting a haphazard to storiform growth pattern, pushing borders, and a low mitotic rate (2 mitoses x 10 HPF). We placed emphasis on the differential diagnostic problems, i.e., its differentiation from other primary monomorphous benign and malignant spindle cell tumors of the kidney, such as fibroma, benign fibrous histiocytoma, hemangiopericytoma, inflammatory myofibroblastic (pseudo-)tumor, leiomyoma, angiomyolipoma with predominant spindle cell smooth muscle component, benign peripheral nerve sheath tumors, renal mixed epithelial/stromal tumors, adult type mesoblastic nephroma, fibrous type monophasic synovial sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, and low-grade fibromyxoid sarcoma.- - - - - - - - - - ranking = 0.0039729582569396keywords = nerve (Clic here for more details about this article) |
8/33. radiation-associated synovial sarcoma: clinicopathologic and molecular analysis of two cases.Development of a soft-tissue sarcoma is an infrequent but well-known long-term complication of radiotherapy. Malignant fibrous histiocytomas, extraskeletal osteosarcomas, fibrosarcomas, malignant peripheral nerve sheath tumors, and angiosarcomas are most frequently encountered. radiation-associated synovial sarcomas are exceptional. We report the clinicopathologic, immunohistochemical, and molecular features of two radiation-associated synovial sarcomas. One tumor developed in a 42-year-old female 17 years after external irradiation was given for breast carcinoma; the other occurred in a 34-year-old female who was irradiated at the age of 7 years for a nonneoplastic condition of the left hand. Both lesions showed morphologic features of monophasic spindle cell synovial sarcoma, were immunoreactive for cytokeratins, epithelial membrane antigen, CD99, CD117 (c-kit), and bcl-2 and bore the t(X;18) (SYT-SSX1) translocation. We conclude that synovial sarcoma has to be added to the list of radiation-associated soft-tissue sarcomas.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
9/33. Metastatic synovial sarcoma to the skull base and orbit.PURPOSE: To report a case of metastatic synovial sarcoma to the parasellar area and orbit. DESIGN: Interventional case report. methods: A 43-year-old woman with a history of a right forearm synovial sarcoma treated with excision, radiation, and chemotherapy 16 months previously presented with blurred vision of the right eye, pain with eye movement, epistaxis, and a right superior division cranial nerve III palsy. magnetic resonance imaging showed a right parasellar and right orbital apex mass. RESULTS: Pathologic examination of the parasellar biopsy revealed metastatic synovial sarcoma. At 6-month follow-up, the neuro-ophthalmic examination was unchanged, and repeat magnetic resonance imaging showed no recurrence of the tumor. CONCLUSIONS: Metastatic synovial sarcoma should be added to the differential diagnosis of parasellar and orbital tumors.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
10/33. Cryptic t(X;18), ins(6;18), and SYT-SSX2 gene fusion in a case of intraneural monophasic synovial sarcoma.A 54-year-old male presented with a spontaneous peroneal nerve palsy and a diagnosis of monophasic synovial sarcoma (SS) was rendered by histologic examination. cytogenetic analysis revealed a complex abnormal karyotype without evidence of the typical t(X;18)(p11;q11) associated with SS. Subsequent reverse transcriptase polymerase chain reaction analysis showed the presence of an SYT/SSX2 fusion transcript, confirming the presence of a cyptic t(X;18). In light of -X, -18 and marker chromosomes evident in the G-band karyotype, it was suspected that a cryptic chromosomal rearrangement involving the marker chromosomes would harbor an X;18 fusion. Multi-colored karytotyping (M-FISH) revealed a previously unrecognized t(X;18) and t(5;19) in the marker chromosomes as well as unrecognized ins(6;18) and t(16;20). The addition of M-FISH analysis in this case led to the identification of complex inter-chromosomal rearrangements, thus providing an accurate karyotype.- - - - - - - - - - ranking = 0.0019864791284698keywords = nerve (Clic here for more details about this article) |
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