This paper reported a case of juvenile trabecular ossifying fibroma（JTOF） originating from the uncinate process. The main clinical manifestation was nasal obstruction and epiphora. Contrast-enhanced sinus CT revealed an irregular heterogeneous soft tissue mass centered in the right uncinate process, with involvement of the right anterior ethmoid sinus, maxillary sinus ostium, frontal process of the maxilla, and partial nasolacrimal duct. The solid components of the tumor demonstrated enhancement on contrast imaging. The patient underwent endoscopic resection of the right sinonasal tumor under general anesthesia. Postoperative pathological examination confirmed the diagnosis of JTOF. No tumor recurrence was observed during the 3-month follow-up period.
Humans ; Ethmoid Bone/pathology* ; Fibroma, Ossifying

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.
Male ; Female ; Humans ; Gene Rearrangement ; In Situ Hybridization, Fluorescence ; Retrospective Studies ; Fibroma/pathology* ; Fasciitis/genetics* ; Ubiquitin Thiolesterase ; Tendons/pathology*

Humans ; Fasciitis/pathology* ; Fibroma/pathology* ; Gene Rearrangement ; Proto-Oncogene Proteins/genetics* ; Ubiquitin Thiolesterase

Fibro-osseous lesions is a class of diseases with obvious similarities in clinical manifestations and pathological features, which has been attracting the attention of clinicians and pathologists. The latest WHO 2022 Classification (5th edition) included six of these diseases (cemento-osseous dysplasia, segmental odontomaxillary dysplasia, fibrous dysplasia, juvenile trabecular ossifying fibroma, psammomatoid ossifying fibroma and familial gigantiform cementoma) in the " fibro-osseous tumours and dysplasias ", and put forward new ideas on the diagnosis and treatment of these diseases. According to the latest WHO 2022 Classification (5th edition), the clinical and pathological features, diagnosis and differential diagnosis of these six diseases were described.
Humans ; Fibroma, Ossifying/pathology* ; Diagnosis, Differential ; Cementoma/pathology* ; Jaw Neoplasms ; Facial Bones

PURPOSE: Desmoid tumors are locally aggressive tumors with no known potential for metastasis. They tend to recur even after complete excision. Sometimes it is not easy to differentiate between intra-abdominal desmoid and tumor recurrence, especially after gastrointestinal (GI) tumor resection. The current study aims to review the characteristics, management, and outcomes of patients with intra-abdominal desmoid tumor post GI resection.METHODS: During the period between 2007 and 2018, after a retrospective review of patients' clinical data, 10 patients were finally included. Medical records were screened for demographic, clinical, pathological data, management strategy, postoperative morbidity, mortality, recurrence rate and follow-up.RESULTS: The study comprised 10 patients (8 males). The median age was 53.5 years (range, 35–68 years). Two patients diagnosed as familial adenomatous polyposis (FAP). All the patients underwent previous GI resection: three (30%) for colon cancer, three (30%) gastrectomy, two (20%) total proctocolectomy with ileal pouch-anal anastomosis (TPC+IPAA) for FAP, one (10%) low anterior resection (three rectal cancers) and one (10%) distal pancreatectomy. The tumor was found to be in bowel mesentery in eight cases (80%). The median tumor size was 5.3 cm (range, 2.6–19.0 cm). Six patients (60%) underwent open resection, while four patients (40%) underwent laparoscopic surgery. Complications occurred in five cases (50%) and ranged from Clavien-Dindo (II-III). The median follow-up period was 16.5 months (1.5–136.0 months) with recurrence in one case (10%). Pathology came out to be desmoid tumor fibromatosis in all cases.CONCLUSION: When a mass develops after surgical resection for abdominal GI malignancy and tends to be large in size, located in the bowel mesentery and away from previous primary tumor site, most probably it is desmoid rather than tumor recurrence.
Adenomatous Polyposis Coli ; Colonic Neoplasms ; Fibroma ; Fibromatosis, Aggressive ; Follow-Up Studies ; Gastrectomy ; Humans ; Laparoscopy ; Medical Records ; Mesentery ; Mortality ; Neoplasm Metastasis ; Pancreatectomy ; Pathology ; Recurrence ; Retrospective Studies

OBJECTIVETo explore the clinicopathologic features and imaging diagnosis of 17 cases of liposclerosing myxofibrous tumor (LSMFT) and to discuss the mechanism of the disease.

METHODSCases of LSMFT diagnosed in 2014 were included in this retrospective study. The clinicopathologic features and imaging findings were evaluated.

RESULTSThere were 17 cases of LSMFT, occurring in 11 men and 6 women with a mean age of 46 years (range, 26-67 years). Patients were asymptomatic or presented with pain localized over the lesions. Most (13/17) lesions were located in the intertrochanteric region. Radiographs showed well-defined and often extensively sclerotic margin. MRI showed the lesions to be relatively heterogeneous on T1W, and heterogeneous with high signal intensity on T2W with fat suppression. Microscopically, LSMFT was characterized by a complex mixture of histologic elements, including myxofibrous and collagen tissues, lipomatous areas, xanthoma cells, calcification, irregular ossification and pseudo-Paget's bone.

CONCLUSIONSLSMFT is a benign fibro-ossesous lesion with unique imaging characteristics and histologic features, occurring preferentially in some locations. It might represent end-stage degenerative changes in other benign bone lesions such as fibrous dysplasia, simple bone cyst and intraosseous lipoma secondary to trauma from forces and ischemic bone injury exerted on the intertrochanteric region of the femur.

Adult ; Aged ; Bone Neoplasms ; diagnosis ; pathology ; Calcinosis ; Female ; Femur ; pathology ; Fibroma ; diagnosis ; pathology ; Hip Joint ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Retrospective Studies

Adult ; Facial Neoplasms ; diagnosis ; etiology ; pathology ; Fibroma ; diagnosis ; etiology ; pathology ; Forehead ; Hamartoma Syndrome, Multiple ; complications ; diagnosis ; pathology ; Humans ; Male ; Mouth Neoplasms ; diagnosis ; etiology ; Papilloma ; diagnosis ; etiology ; Skin Neoplasms ; diagnosis ; etiology ; pathology

Iliotibial band (ITB) friction syndrome is a common overuse injury typically seen in the active athlete population. A nodular lesion on the inner side of the ITB as an etiology or an accompanying lesion from friction syndrome has been rarely reported. A 45-year-old male presented with recurrent pain and a movable nodule at the lateral joint area, diagnosed as ITB friction syndrome. The nodule was confirmed as a rare intra-articular fibroma of the tendon sheath (FTS) on the basis of histopathologic findings. We describe the MRI findings, arthroscopic and pathologic features, in this case of intra-articular FTS presenting with ITB friction syndrome.
Cumulative Trauma Disorders/complications/*diagnosis ; Fibroma/*etiology ; Friction ; Humans ; Iliotibial Band Syndrome/complications/*diagnosis ; Knee Joint/*pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pain/etiology ; Tendons/*pathology

Bone Neoplasms ; diagnosis ; pathology ; surgery ; Child ; Diagnosis, Differential ; Femur ; pathology ; Fibroma ; diagnosis ; pathology ; surgery ; Humans ; Male

Calcinosis ; pathology ; Fibroma ; pathology ; Humans ; Infant ; Male ; Shoulder ; Soft Tissue Neoplasms ; pathology