EWSR1-SMAD3 positive fibroblastic tumor: a clinicopathological analysis / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of EWSR1-SMAD3 positive fibroblastic tumor (ESFT) with an emphasis on differential diagnosis. Methods: The clinicopathological data, immunohistochemical profiles and molecular profiles of 3 ESFT cases diagnosed at the Department of Pathology, Fudan University Shanghai Cancer Center from 2018 to 2021were analyzed. The related literature was also reviewed. Results: There were two males and one female. The patients were 24, 12 and 36 years old, respectively. All three tumors occurred in the subcutis of the foot with the disease duration of 6 months to 2 years. The tumors were presented with a slowly growing mass or nodule, accompanied with pain in 1 patient. The tumors ranged in size from 0.1 to 1.6 cm (mean, 1.0 cm). Microscopically, the tumors were located in the subcutaneous tissue with a nodular or plexiform growth pattern. They were composed of cellular fascicles of bland spindle cells with elongated nuclei and fine chromatin. One of the tumors infiltrated into adjacent adipose tissue. There was no nuclear atypia or mitotic activities. All three tumors showed prominent stromal hyalinization with zonal pattern present in one case. Focal punctate calcification was noted in two cases. The immunohistochemical studies showed that tumor cells were diffusely positive for ERG and negative for CD31 and CD34, with Ki-67 index less than 2%. Fluorescence in situ hybridization on the two tested cases identified EWSR1 gene rearrangement. The next generation sequencing analysis demonstrated EWSR1-SMAD3 fusion in all three cases. During the follow up, one patient developed local recurrence 24 months after the surgery. Conclusions: ESFT is a benign fibroblastic neoplasm and has a predilection for the foot, characterized by ERG immunoreactivity and EWSR1-SMAD3 fusion. Local recurrence might occur when incompletely excised. Familiarity with its clinicopathological features is helpful in distinguishing it from other spindle cell neoplasms that tend to occur at acral sites.

Agreement Between Clinical-Radiographic and Histopathological Diagnoses in Maxillofacial Fibro-Osseous Lesions

ABSTRACT Objective: To compare the agreement of clinical and radiographic diagnosis with the histopathological diagnosis in fibro-osseous lesions of the jaws. Material and Methods: An analytical and exploratory study was made based on systematic collected data, carried out in the laboratory of surgical pathology of a public Dental School. There were evaluated cases of fibrous dysplasia (FD), cemento-osseous dysplasia (COD) and ossifyng fibroma (OF), diagnosed by clinical, radiographic (panoramic and periapical radiography), and histopathological analysis, in a period of 12 years (from March 2001 to June 2013). Descriptive and inferential statistics (Fisher's exact test) were obtained. Results: Ninety-six cases of FOLs were evaluated. The radiographic aspects of the FOLs studied did not differ significantly (p=0.09). Radiolucent lesions were the least frequent, corresponding to approximately 13.5% of radiographic findings. Mixed lesions and radiopaques were more present, how they were COD and FD, respectively. The more aggressive variation of OF (Juvenile Ossifying Fibroma - JOF) was less frequent among the pathologies evaluated. In approximately 61.46% of the cases clinical and radiographic diagnosis were confirmed by histopathological diagnosis of FOLs. The highest agreement and the highest disagreement were observed in COD cases (40.7% and 62.2%, respectively). Conclusion: FOLs of the maxillaries represent a group of lesions in which the establishment of the clinical and radiographic diagnosis supported by the histopathological confirmation is critical and challenging.

Local recurrences of meningeal solitary fibrous tumour with loss of CD34 expression: a diagnostic challenge with prognostic relevance during a 9-year follow-up and literature review

We report the case of a 41-year-old man suffering for nine years from solitary fibrous tumour [SFT] of the tentorium cerebella. The tumour recurred twice, being characterized by a stepwise loss of antigenic profile and an enhanced proliferative activity. Meningeal SFTs are seldom described in literature. Malignant variants occur at peripheral sites but are not known within the central nervous system. Our case can provide deeper information about the tumour's behavior during long-term follow-up

Solitary Fibrous Tumor Arising from Stomach: CT Findings

Solitary fibrous tumors are spindle-cell neoplasms that usually develop in the pleura and peritoneum, and rarely arise in the stomach. To our knowledge, there is only one case reporting a solitary fibrous tumor arising from stomach in the English literature. Here we report the case of a 26-year-old man with a large solitary fibrous tumor arising from the stomach which involved the submucosa and muscular layer and resembled a gastrointestinal stromal tumor in the stomach, based on what was seen during abdominal computed tomography. A solitary fibrous tumor arising from the stomach, although rare, could be considered as a diagnostic possibility for gastric submucosal tumors.

Solitary fibrous tumor of the thyroid gland

A solitary fibrous tumor is a mesenchymal neoplasm originally described in the pleura. Subsequently, it was found to exist in many extra-pleural sites including the thyroid gland. Herein, we report a case of solitary fibrous tumor of the thyroid gland associated with symptoms of hoarseness of voice in a 45-year-old man. In this report we discuss and illustrate various aspects of this rare tumor including, the gross macroscopic appearance, the histological findings, the immunohistochemical staining properties, the differential diagnosis, and the outcome of our experience regarding fine needle aspiration technique in this particular tumor

Solitary Fibrous Tumor of the Urinary Bladder

Solitary fibrous tumor (SFT) is a distinct spindle cell tumor arising mainly in the pleura, however, SFT of extra-pleural sites had been reported. To our knowledge, five cases of the urinary bladder SFT had been reported. We have recently experienced a case of SFT of the urinary bladder in a 56-year- old man who was admitted to the hospital with a 4-month history of voiding difficulty, frequency, and residual urine sensation. A computerized tomography scan revealed a 12cm sized intravesical mass. The mass was marginally excised under the impression of it being a benign spindle cell tumor by pre-operative needle biopsy. On gross examination, the mass was multinodular, creamy white, and the cut surface was fibrotic with a rubbery consistency. Microscopic examination revealed haphazardly arranged spindle cells with a lace-like deposition of inter- and pericellular collagen. Areas of increased cellularity show nuclear atypia and occasional mitoses (2-3/10HPF). The immunohistochemical staining revealed an intense CD34 and bcl-2 immunoreactivity. Although the exact biologic behavior of these tumors has not been clarified, most of the reported cases have undergone a benign clinical course.