FGF23 Expression Is a Promising Immunohistochemical Diagnostic Marker for Undifferentiated Pleomorphic Sarcoma of Bone (UPSb).

Background: Undifferentiated pleomorphic sarcoma of bone (UPSb) is a rare primary bone sarcoma that lacks a specific line of differentiation. Distinguishing between UPSb and other malignant bone sarcomas, including dedifferentiated chondrosarcoma and osteosarcoma, is challenging due to their overlapping features. We have previously identified that UPSb tumours have elevated mRNA levels of Fibroblast Growth Factor 23 (FGF23) transcripts compared to other sarcomas including osteosarcoma. In the present study, we evaluated the specificity and practicality of FGF23 immunoreactivity as a specific diagnostic tool to differentiate UPSb tumours from osteosarcomas and dedifferentiated chondrosarcomas. Methods: A total of 10 UPSb, 10 osteosarcoma, and 10 dedifferentiated chondrosarcoma cases (all high-grade), were retrieved and immunohistochemistry for FGF23 was performed. Results: FGF23 protein was expressed at high levels in 80-90% of undifferentiated pleomorphic sarcoma of the bone cases, whereas it was expressed at significantly lower levels in dedifferentiated chondrosarcoma and osteosarcoma cases. A semiquantitative analysis, considering the intensity of immunoreactivity, confirmed significantly elevated FGF23 expression levels in UPSb tissues compared to those observed in osteosarcoma and dedifferentiated chondrosarcoma tissues. Conclusions: The results we present here suggest that FGF23 immunohistochemistry may be a useful tool to aid in differentiating UPSb from morphologically similar malignant bone sarcomas, especially in situations where sampling is restricted and there is limited clinical information available.

A novel TMTC2-NTRK3 fusion in undifferentiated high-grade pleomorphic sarcoma.

Undifferentiated high-grade pleomorphic sarcoma (UHPS) is a rare soft tissue sarcoma (STS) originated from mesenchyme. UHPS is mostly advanced, aggressive and has poor prognosis. Patients with UHPS tend to have a lower 5-year survival rate than patients with other types of STS. NTRK fusions are commonly found in rare histological tumor types. Among sarcomas, 90% of infantile fibrosarcomas have NTRK fusions. Many other types of sarcomas have also been studied for NTRK fusions. Targeted therapy with NTRK inhibitors, such as Larotrectinib and Entrectinib, leads to response in most patients with NTRK1/2/3 gene fusion-positive tumors. Herein, we present a 68-years old man with UHPS by pathological diagnosis. Next-generation sequencing (NGS) revealed a novel TMTC2-NTRK3 fusion, which was also detected by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). This report broadens the spectrum of NTRK fusions in UHPS and highlights a new target for treatment.

[Treatment and analysis of prognostic factors of stage â ¡ and â ¢ undifferentiated high-grade pleomorphic sarcoma in extremities and trunk].

Objective: To evaluate the efficacy and prognostic factors of comprehensive treatment of undifferentiated high grade pleomorphic sarcoma (UHGPS) in extremities and trunk, including surgery, radiotherapy and chemotherapy. Methods: A retrospective analysis and follow-up of 131 UHGPS cases with clinical stage Ⅱ or Ⅲ in extremities and trunk soft tissue was performed to analyze the prognostic factors. Survival data were collected through follow-up. The survival rate was calculated with life table method and Kaplan-Meier survival curves were drawn. Survival rate between the two groups was compared using Log rank test. The multivariate analysis was performed using Cox regression model. Results: The median survival time of 131 patients was 41.6 months. The 1-year, 3-year and 5-year survival rates were 95.0%, 82.0%, and 77.0%, respectively. The 5-year recurrence-free survival rate was 81.0%, and the 5-year metastasis-free survival rate was 72.0%. Univariate analysis showed that the tumor size, initial or recurrence, surgical margin, AJCC stage, and with/without standard treatment were associated with overall survival (all P<0.05). Stratification analysis according to the American Joint Committee of Cancer (AJCC) stage showed that 5-year survival rate of stage Ⅱ patients with radiotherapy was 100.0%, which was higher than that of patients without radiotherapy (79.6%) and the difference was statistically significant (P=0.010); but no statistical significance of radiotherapy for stage Ⅲ and chemotherapy for stage Ⅱ or Ⅲ patients (all P>0.05). The multivariate analysis showed surgical margin (HR=4.220, P=0.002), with/without standard treatment (HR=4.040, P=0.030) were independent risk factors associated with prognosis of UHGPS patients. Conclusions: For UHGPS with stage Ⅱ or stage Ⅲ in extremities and trunk soft tissue, patients with complete resection and standard treatment have improved prognosis. Therefore, standard treatment, including extensive resection for the first surgery, should be performed according to expert consensus in order to increase the long-term survival rate. Adjuvant radiotherapy should be performed for stage Ⅱ patients.

Management of undifferentiated high-grade pleomorphic sarcoma of parotid region in elderly women.

Undifferentiated high-grade pleomorphic sarcoma is a slow-growing tumor rarely localized in the head and neck region. The treatment of UHPS should be based on large surgical resections in free margins associated with neck dissection. Postoperative radiotherapy improves local control of the disease and the prognosis quod vitam.

Treatment and analysis of prognostic factors of stage Ⅱ and Ⅲ undifferentiated high-grade pleomorphic sarcoma in extremities and trunk / 中华肿瘤杂志

Objective@#To evaluate the efficacy and prognostic factors of comprehensive treatment of undifferentiated high grade pleomorphic sarcoma (UHGPS) in extremities and trunk, including surgery, radiotherapy and chemotherapy.@*Methods@#A retrospective analysis and follow-up of 131 UHGPS cases with clinical stage Ⅱ or Ⅲ in extremities and trunk soft tissue was performed to analyze the prognostic factors. Survival data were collected through follow-up. The survival rate was calculated with life table method and Kaplan-Meier survival curves were drawn. Survival rate between the two groups was compared using Log rank test. The multivariate analysis was performed using Cox regression model.@*Results@#The median survival time of 131 patients was 41.6 months. The 1-year, 3-year and 5-year survival rates were 95.0%, 82.0%, and 77.0%, respectively. The 5-year recurrence-free survival rate was 81.0%, and the 5-year metastasis-free survival rate was 72.0%. Univariate analysis showed that the tumor size, initial or recurrence, surgical margin, AJCC stage, and with/without standard treatment were associated with overall survival (all P<0.05). Stratification analysis according to the American Joint Committee of Cancer (AJCC) stage showed that 5-year survival rate of stage Ⅱ patients with radiotherapy was 100.0%, which was higher than that of patients without radiotherapy (79.6%) and the difference was statistically significant (P=0.010); but no statistical significance of radiotherapy for stage Ⅲ and chemotherapy for stage Ⅱ or Ⅲ patients (all P>0.05). The multivariate analysis showed surgical margin (HR=4.220, P=0.002), with/without standard treatment (HR=4.040, P=0.030) were independent risk factors associated with prognosis of UHGPS patients.@*Conclusions@#For UHGPS with stage Ⅱ or stage Ⅲ in extremities and trunk soft tissue, patients with complete resection and standard treatment have improved prognosis. Therefore, standard treatment, including extensive resection for the first surgery, should be performed according to expert consensus in order to increase the long-term survival rate. Adjuvant radiotherapy should be performed for stage Ⅱ patients.

A case of primary gastric undifferentiated high-grade pleomorphic sarcoma diagnosed with chief complaint of fever: a case report and literature review.

BACKGROUND: Undifferentiated high-grade pleomorphic sarcoma in gastrointestinal tract is extremely rare, and its prognosis is poor. CASE PRESENTATION: An 82-year-old man visited a previous hospital complaining of fever, general fatigue, and shaking chill, for which he received antibiotics therapy. As the fever continued, he was referred to our hospital, where computed tomography and upper gastrointestinal endoscopy showed a 6-cm gastric tumor. A preoperative biopsy was consistent with a malignant mesenchymal tumor, but could not provide a definitive pathological diagnosis nor prove a cause-and-effect relationship between the chief complaint and the gastric tumor. The gastric tumor had grown to 8 cm in diameter within a month so we performed a partial gastrectomy. The pathological postoperative diagnosis was undifferentiated high-grade pleomorphic sarcoma that produced granulocyte colony-stimulating factor. The patient's fever quickly improved, and he showed a good postoperative course. CONCLUSIONS: We herein report a case of rapidly growing, undifferentiated, high-grade pleomorphic gastric sarcoma, which presented as a chief complaint of fever.

A case of mediastinum undifferentiated high grade pleomorphic sarcoma.

Primary anterior mediastinum undifferentiated high grade pleomorphic sarcoma is rare that most physician including thoracic surgeon would not experience this disease. Huge anterior mediastinum tumor pressed the heart and it invaded into the left pulmonary arterial trunk. We did the mediastinum tumor resection, left pneumonectomy and left phrenic, sympathetic and vagus nerve merger excision. But the recurrence lesions were detected in the right lung, the left thoracic cavity and thoracic vertebrae within one month after operation. We reported a case of aggressively progressed undifferentiated high grade pleomorphic sarcoma originated from the anterior mediastinum.

Cytologic diagnosis of undifferentiated high grade pleomorphic sarcoma of breast presenting with brain metastasis.

Primary sarcoma of breast are rare. Diagnosis by aspiration cytology is difficult due to nonspecific cytomorphologic features. An initial presentation with neurological symptoms due to metastasis of breast sarcoma to the brain has not been previously reported. Here, we describe a case of a 60-year-old female who presented with headache, dizziness and convulsion and was subsequently diagnosed with undifferentiated high grade pleomorphic sarcoma of breast with cerebellar metastasis.

Eight-year Follow-up of Malignant Fibrous Histiocytoma (Undifferentiated High-grade Pleomorphic Sarcoma) of the Maxilla: Case Report and Review of the Literature.

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of late adult life and may also arise as a primary tumor in bone. It is a rare condition that constitutes less than 1% of the malignant tumors of bone and commonly occurs in the mathaphysis of long bones of extremities such as the femur and tibia. The occurrence in the head and neck region is very rare. MFH of the jaws is a highly malignant tumor that recurs, metastasizes, and usually causes death despite aggressive surgical therapy. We present a case of MFH of maxilla with 8 years follow-up. The clinical, pathologic and radi-ographic features as well as the treatment of this case are discussed.