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British Journal of Cancer Oct 2023Axitinib is an oral vascular endothelial growth factor receptor inhibitor with anti-tumour activity in renal, thyroid, and pancreatic cancer.
BACKGROUND
Axitinib is an oral vascular endothelial growth factor receptor inhibitor with anti-tumour activity in renal, thyroid, and pancreatic cancer.
METHODS
Axi-STS was a pathologically-stratified, non-randomised, open-label, multi-centre, phase II trial of continuous axitinib treatment in patients ≥16 years, performance status ≤2, with pathologically-confirmed advanced/metastatic soft tissue sarcoma (STS). Patients were recruited within four tumour strata, each analysed separately: angiosarcoma, leiomyosarcoma, synovial sarcoma, or other eligible STSs. The primary outcome was progression-free survival at 12 weeks (PFS12). A Simon's two-stage design with activity defined as PFS12 rate of 40% determined a sample size of 33 patients per strata.
RESULTS
Between 31-August-2010 and 29-January-2016, 145 patients were recruited: 38 angiosarcoma, 37 leiomyosarcoma, 36 synovial sarcoma, and 34 other subtypes. PFS12 rate for each stratum analysed was 42% (95% lower confidence interval (LCI); 29), 45% (95% LCI; 32), 57% (95% LCI; 42), and 33% (95% LCI; 21), respectively. There were 74 serious adverse events including two treatment-related deaths of pulmonary haemorrhage and gastrointestinal bleeding. Fatigue and hypertension were the most common grade 3 adverse events.
CONCLUSIONS
Axitinib showed clinical activity in all STS strata investigated. The adverse event profile was acceptable, supporting further investigation in phase III trials.
CLINICAL TRIAL REGISTRATION
ISRCTN 60791336.
Topics: Humans; Axitinib; Leiomyosarcoma; Sarcoma, Synovial; Hemangiosarcoma; Vascular Endothelial Growth Factor A; Sarcoma; Soft Tissue Neoplasms; Angiogenesis Inhibitors; Treatment Outcome
PubMed: 37684354
DOI: 10.1038/s41416-023-02416-6 -
Postgraduate Medical Journal Sep 1973Two cases of liposarcoma of the kidneys were described, these being the thirty-sixth and thirty-seventh cases documented in the world literature. The association between...
Two cases of liposarcoma of the kidneys were described, these being the thirty-sixth and thirty-seventh cases documented in the world literature. The association between renal liposarcoma and tuberous sclerosis was discussed. The presenting symptoms and signs of this condition were noted. A search of the recent world literature has been carried out.
Topics: Female; Humans; Kidney Neoplasms; Liposarcoma; Male; Middle Aged; Tuberous Sclerosis
PubMed: 4788920
DOI: 10.1136/pgmj.49.575.660 -
The Journal of International Medical... Jun 2022Dedifferentiated liposarcoma (DDLPS) is a rare subtype of liposarcoma with a poor prognosis. This current case report describes a rectal DDLPS in a 68-year-old Chinese... (Review)
Review
Dedifferentiated liposarcoma (DDLPS) is a rare subtype of liposarcoma with a poor prognosis. This current case report describes a rectal DDLPS in a 68-year-old Chinese male that presented with lower abdominal pain and weight loss. Computed tomography and magnetic resonance imaging were undertaken to evaluate the tumour. The patient underwent radical resection of the rectal tumour, sigmoid colostomy and partial ureterectomy. The tumour was positive for mouse double minute 2 by immunohistochemistry. The patient healed well but refused chemotherapy postoperatively for economic reasons. The tumour recurred and metastasized 4 weeks after the operation. After relevant treatment, the patient's condition deteriorated and he died of shock, metabolic acidosis, hyperlactataemia and acute renal failure. The case report also reviews the literature in terms of the clinical diagnosis, treatment and pathological characteristics of rectal DDLPS with the aim of improving the level of diagnosis and treatment.
Topics: Aged; China; Fatal Outcome; Humans; Liposarcoma; Magnetic Resonance Imaging; Male; Neoplasm Recurrence, Local; Rectal Neoplasms; Tomography, X-Ray Computed
PubMed: 35751419
DOI: 10.1177/03000605221102081 -
Bioscience Reports Jun 2022In the last couple of decades, biomarkers have been on the rise for diagnostic and predictive value. There has been a rush to identify new markers using new technologies...
In the last couple of decades, biomarkers have been on the rise for diagnostic and predictive value. There has been a rush to identify new markers using new technologies and drug repurposing approaches. SMARCB1 acronym arises from the SWI/SNF (SWItch/Sucrose Non-Fermentable)-related Matrix-associated Actin-dependent Regulator of Chromatin subfamily B member 1 (SMARCB1). It is a molecule, whose role is associated with the sucrose metabolism. SMARCB1 is also called INI1 (Integrase Interactor 1). The molecule was discovered in the mid-1990s. Its role as a loss-of-function marker for malignant rhabdoid tumors (MRT) of renal and extrarenal origin has enormously expanded the spectrum of involved neoplasms since that time. Several tumors have been characterized by genetic aberrations in the SMARCB1 gene. They include reduction in expression, loss of expression, and mosaic expression. Most of the tumors are sarcomas, but a variegated group of tumors with mixed phenotypes has also been delineated. It is well known that the outcome of patients harboring genetic aberrations in the SMARCB1 gene has been poor. Guo et al. reported that reduced SMARCB1 expression occurred in 70% of osteosarcomas. Their data significantly correlated with poor neoadjuvant response. These authors emphasize a shorter progression-free and overall survival of the patients demonstrating an altered expression of this gene. Interestingly, mRNA in silico analysis established that SMARCB1 expression correlates with the response to chemotherapy of osteosarcoma patients, but there was no reliable correlation between SMARCB1 expression level and metastasis, response to neoadjuvant therapy, overall survival, and progression-free survival. The study involved a tissue microarray (TMA) on bone tumors that may limit the full evaluation of the gene expression. Nevertheless, Guo et al.'s study is remarkable. It expands the list of the tumors harboring an altered SMARCB1 gene expression and suggests that this marker should be investigated in every pathology workup for potential predictive value. On the other side, much work needs to be done if we hope that we strive to provide additional therapeutic strategies for osteosarcoma patients with altered SMARCB1 gene expression.
Topics: Biomarkers, Tumor; Bone Neoplasms; Humans; Osteosarcoma; Prognosis; Rhabdoid Tumor; SMARCB1 Protein; Sucrose
PubMed: 35583077
DOI: 10.1042/BSR20220040 -
Current Oncology (Toronto, Ont.) Aug 2023Sarcomas are a rare tumor of mesenchymal origin. The liposarcoma is the most common sarcoma of the retroperitoneum. Liposarcomas are typically low grade, and present at...
Sarcomas are a rare tumor of mesenchymal origin. The liposarcoma is the most common sarcoma of the retroperitoneum. Liposarcomas are typically low grade, and present at an advanced stage and a large size. We report a case of a large retroperitoneal liposarcoma, approximately 50 kg, encasing both kidneys, which was managed via a two-stage resection and staged renal auto-transplantation into the intra-peritoneal pelvis. The patient maintained normal renal function throughout, and remains disease free two years post-resection. Renal auto-transplantation with pelvic placement may facilitate improved margin-free resection. Renal relocation may allow the use of curative-intent ablative therapies such as radiofrequency ablation and radiation in cases of retroperitoneal recurrence.
Topics: Humans; Retroperitoneal Neoplasms; Sarcoma; Liposarcoma; Soft Tissue Neoplasms; Pelvis
PubMed: 37623033
DOI: 10.3390/curroncol30080552 -
Scientific Reports Feb 2023The safety and effectiveness of pazopanib are related to plasma trough concentrations in renal cell carcinoma (RCC); however, data on pazopanib plasma trough...
The safety and effectiveness of pazopanib are related to plasma trough concentrations in renal cell carcinoma (RCC); however, data on pazopanib plasma trough concentrations with soft tissue sarcoma (STS) are limited. This study investigated the relationship between plasma trough concentrations and pazopanib safety in 45 Japanese patients with RCC or STS. Among the 33 patients included, the median pazopanib trough concentration was 37.5 (range, 12.1-67.6) µg/mL, which was not significantly different between Japanese RCC and STS patients. The plasma trough concentrations showed significant and positive correlations with aspartate aminotransferase and alanine aminotransferase values in blood samples taken for pharmacokinetic measurements after the administration. The incidence of pazopanib treatment discontinuation were significantly higher in RCC patients (p = 0.027). The primary reason for treatment discontinuation was hepatic dysfunction (5/6, 83.3%). Furthermore, this study revealed that pazopanib trough concentration was affected significantly by proton pump inhibitors but not by histamine 2-receptor blockers. In conclusion, the observed pazopanib trough levels and their safety in the Japanese RCC and STS populations in this study were similar to those of the global population. This is the first study to correlate the hepatotoxicity and pharmacokinetic property of pazopanib plasma trough levels by comparing Japanese patients with RCC or STS.
Topics: Humans; Carcinoma, Renal Cell; East Asian People; Sarcoma; Indazoles; Soft Tissue Neoplasms; Kidney Neoplasms; Angiogenesis Inhibitors
PubMed: 36746987
DOI: 10.1038/s41598-023-28688-9 -
Scientific Reports May 2024To assess epidemiology, clinical presentation, treatment and overall survival of adult patients with renal sarcomas, the 2004-2016 SEER and NCDB databases were queried...
To assess epidemiology, clinical presentation, treatment and overall survival of adult patients with renal sarcomas, the 2004-2016 SEER and NCDB databases were queried for adult patients diagnosed with renal sarcoma, calculating average annual age-adjusted incidence rates (AAIR) and average annual percentage change (AAPC) as well as overall survival (OS). In n = 1279 included renal sarcoma patients, AAIR remained constant over the study period (average 0.53 cases/1million; AAPC = 0.7, p = 0.6). Leiomyosarcoma (AAIR 0.14 cases/1 million) and malignant rhabdoid tumors (0.06 cases/1 million) were most common. Sarcoma histiotypes demonstrated considerable heterogeneity regarding demographic and cancer-related variables. Patients presented with advanced local extent (T3 33.3%; T4 14.2%) or distant metastases (29.1%) and commonly underwent surgical resection (81.6%). Longer OS was independently associated with younger age, female sex, lower comorbidity index, low T stage, negative surgical margins, absence of tumor necrosis or distant metastases and leiomyosarcoma histiotype (multivariable p < 0.05 each). Treatment efficacy varied according to sarcoma histiotype (interaction p < 0.001). Accounting for 0.25% of renal malignancies, renal sarcomas include 43 histiotypes with distinct epidemiology, clinical presentation, outcomes and sensitivity to systemic therapy, thereby reflecting soft-tissue sarcoma behavior. Renal sarcoma treatment patterns follow recommendations by renal cancer guidelines with surgical resection as the cornerstone of therapy.
Topics: Humans; Male; Female; Middle Aged; Sarcoma; Kidney Neoplasms; Aged; Adult; Treatment Outcome; Incidence; SEER Program; Aged, 80 and over
PubMed: 38693188
DOI: 10.1038/s41598-024-60174-8 -
Modern Pathology : An Official Journal... Jun 2008Schwannomas of the kidney are rare, with only a few reported cases. We report three additional cases with immunohistochemical analysis. All three tumors were from...
Schwannomas of the kidney are rare, with only a few reported cases. We report three additional cases with immunohistochemical analysis. All three tumors were from females (aged 27, 35, and 59 years) and ranged from 4.8 to 8 cm in diameter. All of the patients underwent nephrectomy. The tumors were totally or partially encapsulated; two were in the hilum and one was centered in the renal cortex. All tumors were diffusely positive for S100 protein. Two were positive for neuron-specific enolase. Immunostaining for neurofilament, HMB45, microphthalmia transcription factor, smooth muscle actin, CD34, cytokeratin AE1/3, cytokeratin 7, and CD10 were negative. Follow-up data were available for two patients; neither had tumor recurrence or metastasis. In conclusion, renal schwannoma is rare, usually arises centrally, impinging on the hilum or the pelvis, and is cured by resection. Sarcomatoid carcinoma and other spindle cell tumors should be considered in the differential diagnosis.
Topics: Adult; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Kidney Neoplasms; Middle Aged; Nephrectomy; Neurilemmoma; Sarcoma
PubMed: 18391921
DOI: 10.1038/modpathol.2008.52 -
European Journal of Medical Research Jun 2022Leiomyosarcoma originating from the renal vein (RVLMS) is extremely rare. RVLMS lacks specific clinical manifestations and specific imaging features. This article...
BACKGROUND
Leiomyosarcoma originating from the renal vein (RVLMS) is extremely rare. RVLMS lacks specific clinical manifestations and specific imaging features. This article discusses the epidemiological characteristics and diagnostic difficulties of RVLMS, as well as imaging features, differential diagnosis, treatment strategy, and prognostic factors of this disease.
METHOD
A case of RVLMS at our center, and 55 cases from the literature based on the PubMed search.
RESULTS
Total operation time was 224 min, and total blood loss during the surgery was 200 ml. Resected tumor was irregular in shape, with negative margins. On the 6th day after the operation, the drainage tube was removed, and the patient was discharged from the hospital. Postoperative pathological results confirmed the renal vein leiomyosarcoma: spindle cell sarcoma, diffuse severe atypia, S-100 (-), SMA ( +), desmin ( +), CD34 (-), CD99 ( +). Twenty-seven months after the surgery, the patient is alive, and without local recurrence or distant metastases.
CONCLUSION
Unspecific clinical manifestations and imaging features make the diagnosis of RVLMS difficult. Most patients are diagnosed intra-operatively or following postoperative pathology. Differential diagnosis with paraganglioma (PG) and retroperitoneal sarcoma (RPS) should be made. Early and complete resection is considered as the first choice of treatment, and whether to preserve the kidney is based on the patient's condition. RVLMS is highly malignant, and may recur locally or metastasize to distant locations; therefore, adjuvant therapy and regular follow-up should be carried out after surgery.
Topics: Humans; Leiomyosarcoma; Neoplasm Recurrence, Local; Renal Veins; Retroperitoneal Neoplasms; Sarcoma
PubMed: 35761392
DOI: 10.1186/s40001-022-00721-z -
Archivos Espanoles de Urologia Sep 2017Primary undifferentiated pleomorphic sarcoma (UPS) of the testicular tunics is rare, and synchronism with other malignancies of the urinary tract is uncommon, and may... (Review)
Review
OBJETIVE
Primary undifferentiated pleomorphic sarcoma (UPS) of the testicular tunics is rare, and synchronism with other malignancies of the urinary tract is uncommon, and may complicate the staging and therapeutic approach. We report the case of a patient diagnosed with primary paratesticular UPS with synchronous Renal Carcinoma.
METHODS
Patient presenting with intrascrotal tumor who underwent left radical orchiectomy. In staging work up studies a second urologic neoplasia was found in the kidney. Histological diagnosis using immunohistochemical techniques for adequate characterization was performed. Adjuvant treatment options were evaluated. We review the literature and discuss the case.
RESULTS
The UPS diagnosis was performed with immunohistochemistry. Paratesticular origin from testicular tunics was evident in the macroscopic evaluation. After finding the synchronic ipsilateral renal tumor, he underwent conventional left radical nephrectomy, ipsilateral adrenalectomy, excision of remnant left cord and para-aortic and pelvic lymphadenectomy. He received chemotherapy and adjuvant radiotherapy. Currently, after twelve months of follow-up there is no evidence of disease.
CONCLUSIONS
The UPS is a heterogeneous group with an exclusion immunohistochemical diagnosis. Its management requires a multidisciplinary approach; the initial surgical treatment is accepted for the paratesticular mass with high section radical orchiectomy. Although there is insufficient evidence of the efficacy of adjunctive lymphadenectomy, chemotherapy and/or radiotherapy.
Topics: Carcinoma, Renal Cell; Humans; Kidney Neoplasms; Male; Middle Aged; Neoplasms, Multiple Primary; Sarcoma; Testicular Neoplasms
PubMed: 28891799
DOI: No ID Found