Fibroma Of Tendon Sheath Research Articles

Fibroma of tendon sheath: a clinicopathological and genetic analysis of 134 cases

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of fibroma of tendon sheath (FTS). Methods: One hundred and thirty-four cases of FTS or tenosynovial fibroma diagnosed in the Department of Pathology, West China Hospital, Sichuan University, Chengdu, China from January 2008 to April 2019 were selected. The clinical and histologic features of these cases were retrospectively reviewed. Immunohistochemistry, fluorescence in situ hybridization (FISH) and reverse transcription-polymerase chain reaction (RT-PCR) were performed on the above cases. Results: There were a total of 134 cases of FTS, including 67 males and 67 females. The patients' median age was 38 years (ranged from 2 to 85 years). The median tumor size was 1.8 cm (ranged from 0.1 to 6.8 cm). The most common site was the upper extremity (76/134, 57%). Follow-up data was available in 28 cases and there was no detectable recurrence. Classic FTS (114 cases) were well-defined and hypocellular. A few spindle-shaped fibroblasts were scattered in the dense collagenous sclerotic stroma. Characteristically elongated slit-like spaces or thin-walled vessels were observed. Most of cellular FTSs (20 cases) were well-defined and the area with increased cellularity of the spindle cells coexisted with classic FTS. There were occasional mitotic figures, but no atypical mitotic figures. Immunohistochemistry was performed in 8 cases of classic FTS and most cases were positive for SMA (5/8). Immunohistochemistry was also performed in 13 cases of cellular FTS and showed 100% positive rate for SMA. FISH was conducted on 20 cases of cellular FTS and 32 cases of classical FTS. USP6 gene rearrangement was found in 11/20 of cellular FTS. Among 12 cases of CFTS with nodular fasciitis (NF)-like morphological feature, 7 cases showed USP6 gene rearrangement. The rearrangement proportion of USP6 gene in cellular FTS without NF-like morphological features was 4/8. By contrast, 3% (1/32) of the classic FTS showed USP6 gene rearrangement. RT-PCR was performed in those cases with detected USP6 gene rearrangement and sufficient tissue samples for RT-PCR. The MYH9-USP6 fusion gene was detected in 1 case (1/8) of the cellular FTSs, while no target fusion partner was detected in the classic FTS. Conclusions: FTS is a relatively rare benign fibroblastic or myofibroblastic tumor. Our study and recent literature find that some of the classic FTS also show USP6 gene rearrangements, suggesting that classical FTS and cellular FTS are likely to be at different stages of the same disease (spectrum). FISH for USP6 gene rearrangement may be used as an important auxiliary diagnostic tool in distinguishing FTS from other tumors.

Recurrent FOSL1 rearrangements in desmoplastic fibroblastoma.

The FOS gene family has been implicated in tumourigenesis across several tumour types, particularly mesenchymal tumours. The rare fibrous tumour desmoplastic fibroblastoma is characterised by overexpression of FOSL1. However, previous studies using cytogenetic and molecular techniques did not identify an underlying somatic change involving the FOSL1 gene to explain this finding. Prompted by an unusual index case, we report the discovery of a novel FOSL1 rearrangement in desmoplastic fibroblastoma using whole-genome and targeted RNA sequencing. We investigated 15 desmoplastic fibroblastomas and 15 fibromas of tendon sheath using immunohistochemistry, in situ hybridisation and targeted RNA sequencing. Rearrangements in FOSL1 and FOS were identified in 10/15 and 2/15 desmoplastic fibroblastomas respectively, which mirrors the pattern of FOS rearrangements observed in benign bone and vascular tumours. Fibroma of tendon sheath, which shares histological features with desmoplastic fibroblastoma, harboured USP6 rearrangements in 9/15 cases and did not demonstrate rearrangements in any of the four FOS genes. The overall concordance between FOSL1 immunohistochemistry and RNA sequencing results was 90%. These findings illustrate that FOSL1 and FOS rearrangements are a recurrent event in desmoplastic fibroblastoma, establishing this finding as a useful diagnostic adjunct and expanding the spectrum of tumours driven by FOS gene family alterations. © 2022 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Histopathogenesis of bone- and soft-tissue tumor spectrum with USP6 gene rearrangement: multiple partners involved in the tissue repair process.

Primary aneurysmal bone cyst, nodular fasciitis, myositis ossificans and related lesions as well as fibroma of tendon sheath are benign tumors that share common histological features and a chromosomal rearrangement involving the ubiquitin-specific peptidase 6 (USP6) gene. The tumorigenesis of this tumor spectrum has become complex with the identification of an increasing number of new partners involved in USP6 rearrangements. Because traumatic involvement has long been mentioned in the histogenesis of most lesions in the USP6 spectrum and they morphologically resemble granulation tissue or callus, we attempted to shed light on the function and role USP6 partners play in tissue remodelling and the repair process and, to a lesser extent, bone metabolism.

Ulnar Wrist Mass, Large But Not Giant

HISTORY: An active 60 year old complained of a mass on the volar ulnar side of the right wrist. First appeared seven months prior and continued to grow in size. Initially symptoms were aching pain, decreased grip strength and reduced wrist flexion interfering with physical activity and work. One month later they developed ulnar side numbness. PHYSICAL EXAM: Soft non discrete, non pulsatile mass off the volar ulnar side of the wrist deep to the FCU tendon. No atrophy. Able to fully flex and extend fingers. Weakness with wrist extension and little finger extension. TFCC load test negative. Froment’s sign negative. Light touch sensation grossly intact. DIFFERENTIAL DIAGNOSIS: Triangular fibrocartilage complex cyst, Ganglion Cyst, Tenosynovial giant cell, tumorfibroblastic/myofibroblastic lesion. TEST AND RESULTS: Wrist frontal, lateral and oblique radiographs: No underlying osseous abnormalities. Focal soft tissue bulge along ulnar aspect of the wrist. No underlying erosion. No gas or radiopaque foreign body. Wrist MRI: Mass within the volar and medial soft tissues of the wrist from the level of the distal ulnar epiphysis to the proximal aspect of the pisotriquetral articulation causing a mass effect and displacement of the adjacent ulnar neurovascular bundle. The mass demonstrates low signal intensity on T2 FS and T1. Excisional Biopsy: benign paucicellular fibrocollagenous proliferation. FINAL DIAGNOSIS: Benign fibroma of tendon sheath. TREATMENT AND OUTCOMES: Surgical excision of the volar ulnar wrist mass. The ulnar neurovascular bundle was displaced radially due to the mass. The homogeneous mass measuring 3 x 2.8 x 1.5 cm was removed in one piece and sent to pathology. At the 2 week follow up, the patient was fitted with a customized thermoplastic volar hand, wrist and forearm based orthosis since OTC DME splint would not fit with the proximal end being over the surgical site. At 4 weeks post op, the patient was doing very well and beginning to wean out of the wrist brace.

Multimodality imaging features of USP6-associated neoplasms.

Since the discovery of USP6 gene rearrangements in aneurysmal bone cysts nearly 20years ago, we have come to recognize that there is a family of USP6-driven mesenchymal neoplasms with overlapping clinical, morphologic, and imaging features. This family of neoplasms now includes myositis ossificans, aneurysmal bone cyst, nodular fasciitis, fibroma of tendon sheath, fibro-osseous pseudotumor of digits, and their associated variants. While generally benign and in many cases self-limiting, these lesions may undergo rapid growth, and be confused with malignant bone and soft tissue lesions, both clinically and on imaging. The purpose of this article is to review the imaging characteristics of the spectrum of USP6-driven neoplasms, highlight key features that allow distinction from malignant bone or soft tissue lesions, and discuss the role of imaging and molecular analysis in diagnosis.

Trigger wrist in adult. A case report.

Background: One of common disorders in hand is trigger finger, but trigger wrist is an uncommon condition. Eibel in 1961 reported the trigger wrist. This condition gets obvious with finger or wrist flexion and extension and painful clicking or triggering of finger around carpal tunnel and can be associated with neuropathy and space occupying lesion or any other underlying condition the Objective of treatment is to achieve complete cure with surgical treatment in terms of functional and aesthetic improvement. Case: A 35 years old right handed housewife, presented to us in OPD with 1 year history of triggering at left wrist level. She had history of pain; with off and on clicking during flexion/extension of index finger of left hand. Pain had been increasing over the last 6 months. She underwent standard trigger finger surgery and release of left index finger A1 pulley four month ago but symptom was still present. Pre-operative ultrasonography showed irregular hypo echo mass at wrist that caused adhesion and inflammation at this area. After detail discussion, written informed consent was obtained and she underwent surgical treatment with zigzag incision under axillary block. Median never and all flexor tendons were identified. We found mass over FDP of left index finger that was stuck in carpal tunnel and caused snapping and clicking of left index finger. The other tendons were normal. After resection of occupying lesion of left index finger FDP and carpal tunnel release, there was free gliding of tendons at wrist level. Histopathological evaluation of the lesion turned out as fibroma of tendon sheath without evidence of inflammation and malignancy. After the discharge, physiotherapy was advised and patient was followed-up on weekly basis for 1st month then 2 weekly for 6 months. After the surgical treatment, there was free gliding of tendons at wrist level, under carpal tunnel, with complete functional and aesthetic improvement. Conclusion: Trigger wrist is an uncommon condition caused by space occupying lesion or any other associated pathology. Surgery is the mainstay of treatment that provides complete resolution of symptoms with functional and aesthetic improvement.

Rare case of fibroma of the tendon sheath in a pediatric patient

Fibroma of tendon sheath (FTS) is a benign uncommon soft tissue tumor that presents as a solitary, firm, slow-growing painless nodule. This tumor arises from the synovium of the tendon sheath that occurs mostly around small joints such as the fingers, hands, and wrist. This soft tissue tumor rarely arises around large joints such as the knees, shoulders, elbows, and ankles with intra-articular or extra-articular involvement. It most commonly presents as a painless, slow-growing tumor with small nodular swelling and is often seen in adults 20-50. Our report reviews a unique case of fibroma of the tendon sheath in a pediatric patient. A 12-year old right-hand dominant female presented to the clinic with a mass located on the volar region of the proximal phalanx of the right long finger. This mass was present for 6 months and progressively increased in size. It caused intermittent pain, with prolonged activity. An ultrasound displayed a 1.3 cm mass around the proximal portion of the right long finger, which was consistent with a giant cell tumor versus a neuroma. Surgical dissection was performed and the mass was about 1 cm in diameter and emanated deep to the flexor tendons in the flexor sheath. When the patient followed up in the clinic postoperatively, there was no tenderness of the hand and mild tenderness over the volar portion of the right long finger. There was no palpable mass or angular deformity noted and the patient was neurovascularly intact. Clinical presentation, surgical management, recurrence, differential diagnosis, progression, and growth of the mass make this case unique. Typically, this is seen in the young and middle-aged demographic and reported in a 3:1 male to female ratio. The differential diagnosis for FTS includes giant cell tumor, mucinous cyst, lipoma, epidermal cyst and leiomyoma. Future studies should investigate other forms of management for Fibroma of the tendon sheath.

Fibroma of tendon sheath is defined by a USP6 gene fusion—morphologic and molecular reappraisal of the entity

Fibroma of tendon sheath (FTS) is an uncommon benign myofibroblastic neoplasm that arises in association with tenosynovial tissue. Fusions of the USP6 gene have been recently documented in a proportion of so-called “cellular FTS” but not in “classic FTS”. It remains unknown whether FTS can be defined by a USP6 fusion, regardless of cellularity, and what are USP6 fusion-negative “cellular FTS”. Furthermore, FTS with low cellularity seems to be frequently confused with desmoplastic fibroblastoma. We performed a comprehensive analysis, including targeted RNA sequencing, of 58 consecutive cases originally diagnosed as FTS (n = 49), desmoplastic fibroblastoma (n = 6), or nodular fasciitis (n = 3); the latter two at the predilection sites for FTS. After review of the original slides, 28 lesions were morphologically classified as FTS (13 “classic” and 15 “cellular”) and 23 as desmoplastic fibroblastoma. Among originally diagnosed FTS at the more cellular end of the spectrum, we identified seven lesions that shared many morphologic features of FTS but, in addition, showed several distinct morphologic features consistent with myofibroma, such as myoid appearance, branching thin-walled vessels, and perivascular growth. Targeted RNA sequencing showed a USP6 fusion in 17 of 18 analyzed FTS, regardless of cellularity, 0 of 5 desmoplastic fibroblastomas and 0 of 4 myofibromas. MYH9, COL1A1, and ASPN were identified as fusion partners in three cases each, and MIR22HG, CTNNB1, SPARC, CAP1, EMP1, LINC00152, NR1D1, and RAB1A in a single case each. FTS, regardless of cellularity, can be defined by a USP6 fusion with a variety of fusion partners. More cellular lesions exhibiting some morphologic features of FTS but lacking a USP6 fusion tend to be myofibromas.

Fibroma of extensor digitorum tendon sheath in 27yr old male patient: A case report

Fibroma of tendon sheath was first described by Geschickter and Copeland in 1949 (1), but was not formally documented as a clinicopathologic entity until Chung and Enzinger's superb series of 138 cases was published in 1979 (2). These lesions not infrequently present to the dermatologist as small subcutaneous nodules. This tumor has received little attention in the dermatological literature anti as dermatologists play an increasingly important role in the surgical management of cutaneous and subcutaneous tumors the entity deserves wider recognition. The importance of suggesting the diagnosis clinically is that although most are histologically bland ant obviously benign, more cellular lesions may he misinterpreted as malignant. The differential diagnosis is briefly discussed.

The Unusual Case of Fibroma of Tendon Sheath in a Young Girl with Turner Syndrome Undergoing Growth Hormone Treatment

Fibroma of tendon sheath (FTS) is an uncommon mass that arises from the tendon sheath of extremities. The tumor typically affects adults between ages 20 and 50 years with a predominance in males. To date, growth hormone (GH) treatment is safe for children with Turner syndrome without risk factors and is accepted worldwide. This article reports the case of a nine-year-old female patient with Turner syndrome and FTS during GH treatment. She had been treated with daily subcutaneous GH to improve growth failure with a mean dose of 0.28 mg/kg/week and the level of insulin-like growth factor-1 was within the normal range. During the follow-up period, she complained about a mass in her hand, subsequently diagnosed as FTS. This report illustrates the clinical impact of Turner syndrome and GH treatments on the occurrence of this tumor through literature reviews. Further studies are needed to highlight the association between FTS and GH treatment, especially in Turner syndrome.

Ubiquitin-specific Peptidase 6 (USP6)-associated Fibroblastic/Myofibroblastic Tumors: Evolving Concepts.

Ubiquitin-specific peptidase 6 (USP6) is a hominoid-specific gene residing on chromosome 17p13 and serves as a deubiquitinating enzyme with a diverse set of functions including intracellular trafficking, inflammatory signaling, cell transformation and protein turnover. USP6 rearrangements were first identified in aneurysmal bone cysts, resulting in promoter swapping and over-expression of wild type USP6. Several morphologically overlapping fibroblastic/myofibroblastic tumors are known to harbor USP6 rearrangements, including nodular fasciitis, cellular fibroma of tendon sheath, myositis ossificans and fibro-osseous pseudotumor of digits. Over the past few years, fusions involving the USP6 gene and various partner genes have been described in these neoplasms. The current World Health Organization Classification of Tumors of Soft Tissue suggests that USP6-rearranged lesions are typically benign and usually self-limited in their growth. This review provides an updated overview of the clinical, histological and molecular genetic features of USP6-associated fibroblastic/myofibroblastic tumors and discusses how these lesions should be best classified.

Characterization of novel USP6 gene rearrangements in a subset of so-called cellular fibroma of tendon sheath

Fibroma of tendon sheath (FTS) is an uncommon benign fibroblastic/myofibroblastic neoplasm that typically arises in the tenosynovial tissue of the distal extremities. Histologically, it is a well-circumscribed proliferation of spindle cells within collagenous stroma with peripheral slit-like vessels. Most examples are relatively hypocellular and more densely collagenous than nodular fasciitis; however, a cellular variant has been described, which has considerable morphologic overlap with nodular fasciitis and has been shown to harbor USP6 translocations in a subset of cases. The incidence of these rearrangements and the identity of the USP6 fusion partners have not been described in detail. In this study we evaluate 13 cases of cellular fibroma of tendon sheath by anchored multiplex PCR/next generation sequencing in order to detect potential gene fusions. Nucleic acids of adequate quality were obtained in 11 cases, demonstrating gene fusions in 7/11 (64%), all of which involve USP6 with a variety of partners, including PKM, RCC1, ASPN, COL1A1, COL3A1, and MYH9. Some unusual histomorphologic findings were present in a subset of cases including palisading growth pattern, epithelioid cells, and osteoclast-like multinucleated giant cells, particularly in the tumors with PKM and ASPN gene partners. Overall, the findings support a biologic relationship between cellular fibroma of tendon sheath and other lesions within the spectrum of USP6-rearranged neoplasms, particularly nodular fasciitis.

An Update on Clinicopathological, Imaging and Genetic Features of Desmoplastic Fibroblastoma (Collagenous Fibroma).

Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous tissue of upper extremity. Magnetic resonance imaging reveals a well-defined mass in intimate association with dense connective tissue and prominent low signal intensity on all pulse sequences. Peripheral and septal enhancement is usually seen after intravenous contrast. Histologically, the lesion is paucicellular and consists of spindle to stellate-shaped cells embedded in a collagenous or myxocollagenous stroma with low vascularity. Diffuse and strong nuclear immunoreactivity for FOS-like antigen 1 seems to be characteristic of desmoplastic fibroblastoma. Cytogenetic studies have demonstrated the presence of 11q12 rearrangements and an identical t(2;11)(q31;q12) translocation. This review provides an updated overview of the clinical, radiological, histological, cytogenetic and molecular genetic features of desmoplastic fibroblastoma and discusses the relationship to fibroma of tendon sheath.

Differential diagnosis of fibroma of tendon sheath and giant cell tumor of tendon sheath in the finger using signal intensity on T2 magnetic resonance imaging.

The differential diagnosis of fibroma of tendon sheath (FTS) and giant cell tumor of tendon sheath (GCTTS) on the basis of clinical and radiographic characteristics remains difficult. To evaluate the quantitative measurement of signal intensity (SI) obtained by magnetic resonance imaging (MRI) for the differential diagnosis of FTS and GCTTS in just the finger. We retrospectively identified patients with FTS (n = 6) and GCTTS (n = 22) of the finger who were treated at our hospitals between April 2011 and August 2019. Two researchers independently reviewed the MRIs and measured the regions of interest (ROIs) in the tumor and flexor tendon from the same image. The SI ratio obtained for the tumor and tendon ROIs was measured and compared using receiver-operating characteristic curve analyses. Sensitivity and specificity analyses were performed. The SI ratios (mean ± SD) of FTS and GCTTS were 1.83 ± 0.64 and 6.34 ± 3.16 for researcher 1 and 1.82 ± 0.60 and 6.10 ± 3.22 for researcher 2, respectively. The areas under the curve were 0.970 and 0.970 for researchers 1 and 2, respectively. The cut-off values of the SI ratio as determined by researchers 1 and 2 for differentiating FTS from GCTTS were 3.00 and 3.00, respectively (sensitivity = 95.5%, specificity = 100%). The SI ratio is useful for differentiating FTS from GCTTS independent of a combination of tumor signal and shape.

MRI evaluation of solid soft tissue masses of the fingers with pathology correlation

ObjectiveSpace occupying lesions of the fingers are commonly encountered in clinical and radiology practice. The objective of this study was to determine the characteristics of these lesions on MRI and to correlate with surgical pathology results. Material and methodsThis IRB-approved HIPAA-compliant study retrospectively evaluated the clinical, imaging and pathology findings of 100 consecutive patients referred for evaluation of solid soft tissue masses of the fingers. Only solid lesions with MR imaging prior to surgery were included in this study. MR images and when available corresponding radiographs were evaluated by two radiologist in regard to signal characteristics, relation to surrounding structures and enhancement. All masses were classified into different groups based on pathology results. ResultsTenosynovial giant cell tumor (TSGCT) and fibroma of the tendon sheath were the most common solid tumors (36 %) and malignant tumors represent only 5% of the cohort. 70 % of masses with low T2 signal were TSGCT and fibroma of tendon sheath; 100 % of masses with peripheral high T2 signal and central low T2 signal/thrombus were vascular lesions, representing 41 % of the total vascular lesions in the cohort. Additionally, 100 % of serpiginous/tubular shaped masses were vascular lesions and 67 % of masses with infiltrative borders were malignant masses. Enhancement was important in identifying solid lesions but there was no statistical difference between groups related to enhancement pattern. There was a good agreement for all analyses between both readers. ConclusionTypical MR findings can help to narrow the differential diagnosis in the evaluation of finger masses and provide crucial information to guide further management.

Clinicopathological and molecular characterisation of USP6-rearranged soft tissue neoplasms: the evidence of genetic relatedness indicates an expanding family with variable bone-forming capacity.

USP6 rearrangement underpins self-limiting fibroblastic/myofibroblastic neoplasms, including nodular fasciitis (NF), myositis ossificans (MO), aneurysmal bone cyst (ABC), and related variants. The aim of this study was to characterise UPS6 and fusion partners in order to delineate the clinicopathological, genetic and bone-forming features in such lesions of soft tissue (ST). Break-apart fluorescence in-situ hybridisation (FISH) validated USP6 rearrangement in 31 of 35 NF [comprising three of three fasciitis ossificans (FO) cases, seven of eight cellular variant of fibroma of tendon sheath (C-FTS), four of six MO, three of three ST-ABC, and two of two fibro-osseous pseudotumours of digits (FOPD)]. As determined with FISH and reverse transcription polymerase chain reaction, MYH9-USP6 was the commonest fusion in four C-FTS and 20 NF, including one intravascular case and two infantile (one retroperitoneal) cases. The presence of MYH9-USP6 confirmed the diagnosis of two NFs>50mm with prominent ischaemic necrosis. COL1A1-USP6 was predominant in ossifying lesions, including all FO, MO, ST-ABC and FOPD with identified partner genes, and was also present in non-ossifying head and neck NF (HN-NF) and C-FTS in two cases each. A cervical NF of a 14-month-old girl harboured the novel COL1A2-USP6. Ossifying lesions showed considerable genetic and morphological overlaps. Sharing COL1A1-USP6, FO and FOPD showed similar central or haphazard bone matrix deposition. Besides zonation of outward bone maturation, four COL1A1-USP6-positive MO had incipient to sieve-like pseudocysts reminiscent of ST-ABC. MYH9-USP6 is present in some C-FTS and most NF, including rare variants, but is unrelated to bone formation. All bone-forming USP6-rearranged lesions adopt COL1A1 as the 5' partner, indicating close genetic kinships. However, COL1A1/COL1A2 also contributes to the pathogenesis of minor subsets of non-ossifying USP6-rearranged HN-NF and C-FTS.

Fibroma of tendon sheath of the hand in a 3-year-old boy: a case report

BackgroundFibroma of tendon sheath (FTS) is a rare benign soft tissue tumor that often occurs in the upper extremities. It manifests as a slow-growing mass, often without tenderness or spontaneous pain. FTS occurs most commonly in people aged 20–40 years and is extremely rare in young children. Because FTS presents with atypical physical and imaging findings, it might be misdiagnosed as another soft tissue tumor such as a ganglion cyst or tenosynovial giant cell tumor (TSGCT). Although marginal resection is usually performed, a high rate of local recurrence is reported.Case presentationA boy aged 3 years and 1 month visited our outpatient clinic with a complaint of a mass of the left hand. An elastic hard mass approximately 20 mm in diameter could be palpated on the volar side of his left little finger. This mass was initially diagnosed as a ganglion cyst at another hospital. Ultrasonography revealed a well-circumscribed hypoechoic mass with internal heterogeneity on the flexor tendon. On magnetic resonance imaging (MRI), the mass showed iso signal intensity to muscle on T1-weighted images, and homogeneously low signal intensity to muscle on T2-weighted images. The mass was peripherally enhanced after contrast administration. FTS was initially suspected as the diagnosis on the basis of these imaging features. Because of the limited range of motion of his little finger, surgery was performed when he was 4 years old. Histopathological findings indicated the mass was well-circumscribed and contained scattered spindle cells embedded in a prominent collagenous matrix. The spindle cells contained elongated and cytologically bland nuclei with a fine chromatin pattern. Nuclear pleomorphism and multinucleated giant cells were not observed. On the basis of these findings, we made a diagnosis of FTS. One year after surgery, no signs of local recurrence were observed.ConclusionsWe experienced an extremely rare case of FTS in the hand of a 3-year-old child. We especially recommend ultrasonography for hand tumors of young children to diagnose or eliminate ganglion cysts. MRI helped differentially diagnose FTS from TSGCT. Although marginal resection can be performed as a treatment, great care should be taken postoperatively because FTS has a high possibility of local recurrence.

The Tendinous Impaction- Fibroma of Tendon Sheath

Preface Fibroma of tendon sheath (FTS) was initially scripted by Geschickter et al in 1949. Subsequently, Chung and Enzinger elaborated upon cogent clinical and pathological manifestations of the neoplasm. Fibroma of tendon sheath is denominated as a benign, fibroblastic, nodular neoplasm which emerges from synovium of tendon sheath. As fibroma of tendon sheath characteristically originates from adherent tendons and tendon sheaths, it predominantly incriminates small joints of upper limb as fingers, hands and wrists. Infrequently, the toe, foot, ankle, leg, knee, shoulder, elbow, forearm, chest, back and temporomandibular joints are implicated.

USP6-Associated Neoplasms: A Rapidly Expanding Family of Lesions.

Nearly 20 years ago, the first description of a translocation involving chromosome 17 on which USP6 resides was described. Since then, not only the culprit gene but also many fusion partners, leading to transcriptional activation of USP6, have been detected. The first neoplasm known to harbor USP6 rearrangements was aneurysmal bone cyst. Since then, other entities like nodular fasciitis, myositis ossificans, fibro-osseous pseudotumor of digits, and a subgroup of fibromas of tendon sheath, probably representing tenosynovial nodular fasciitis, have been added to the list of USP6-rearranged lesions. Remarkably, all of them share clinical as well as morphological characteristics, and authors have suggested that these entities actually belong to the same spectrum. This review summarizes the current knowledge regarding USP6-rearranged lesions and further elaborates on how these neoplasms relate to one another. We propose to call these lesions UAN (Usp6-associated neoplasm).

肘関節周囲に生じた Fibroma of tendon sheath の一例

肘関節周囲に生じた Fibroma of tendon sheath の一例