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Beijing Da Xue Xue Bao. Yi Xue Ban =... Oct 2017Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) in the kidney is a rare but high-grade malignant tumor that affects predominantly elder children and... (Review)
Review
Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) in the kidney is a rare but high-grade malignant tumor that affects predominantly elder children and adolescents. Patients mostly present with nonspecific symptoms such as abdominal pain and gross hematuria. Since EWS/PNET has a rapid clinical progression with early metastasis and death, it is essential to make an accurate and early diagnosis. Once diagnosed, multimodality treatment, including radical surgery combined with adjuvant chemotherapy, and radiotherapy if necessary, is recommended. Unfortunately, there are no characteristic signsthat have been described in ultrasonography or any other imaging modalities so far. The diagnosis of EWS/PNET is now based on a classical histological and immunohistochemical investigation complemented by a demonstration of specific chromosomal changes. Strong immunoreactivity to CD99 is ubiquitous, and t(11;22) translocation is seen in approximately 90% of EWS/PNET. Herein, we report a patient with such condition. The patient was a young woman, and she presented with sudden right flank pain clinically. Ultrasonography revealed a large heterogeneous mass in the lower pole of her right kidney. The tumor compressed the renal pelvis and led to upper pole caliectasis. Color Doppler demonstrated blood flow with a pulsatile arterialized waveform within the mass. The patient received radical nephrectomy with right renal vein and vena cava thrombectomy. A search for other sites of tumor involvement yielded negative results. And six cycles of chemotherapy were sequentially performed. The diagnosis of EWS/PNET was confirmed based on primitive small round cell histology and characteristic immunohistochemical results. She was still alive with no evidence of recurrence five years after initial diagnosis. We would like to point out that ultrasound is still a useful method for initial assessment, and ultrasound-guided fine needle aspiration may play an important role in determining preoperative diagnosis.
Topics: Adult; Female; Humans; Kidney Neoplasms; Neoplasm Recurrence, Local; Nephrectomy; Neuroectodermal Tumors, Primitive; Sarcoma, Ewing
PubMed: 29045981
DOI: No ID Found -
International Journal of Molecular... Aug 2023Endometrial cancer and uterine sarcoma represent the two major types of uterine cancer. In advanced stages, both cancer entities are challenging to treat and correlate... (Review)
Review
Endometrial cancer and uterine sarcoma represent the two major types of uterine cancer. In advanced stages, both cancer entities are challenging to treat and correlate with a meagre survival and prognosis. Hyperthermic Intraperitoneal Chemotherapy (HIPEC) is a form of localized chemotherapy that is heated to improve the chemotherapeutic effect on peritoneal metastases. The aim of the current review is to study the role of HIPEC in the treatment of uterine cancer. A literature review was conducted using the MEDLINE and LIVIVO databases with a view to identifying relevant studies. By employing the search terms "hyperthermic intraperitoneal chemotherapy", "uterine cancer", "endometrial cancer", and/or "uterine sarcoma", we managed to identify 26 studies published between 2004 and 2023. The present work embodies the most up-to-date, comprehensive review of the literature centering on the particular role of HIPEC as treatment modality for peritoneally metastasized uterine cancer. Patients treated with cytoreductive surgery, alongside HIPEC, seem to profit from not only higher survival but also lower recurrence rates. Factors such as the completeness of cytoreductive surgery, the peritoneal cancer index, the histologic subtype, or the applied chemotherapeutic agent, all influence HIPEC therapy effectiveness. In summary, HIPEC seems to represent a promising treatment alternative for aggressive uterine cancer.
Topics: Female; Humans; Combined Modality Therapy; Hyperthermic Intraperitoneal Chemotherapy; Hyperthermia, Induced; Peritoneal Neoplasms; Uterine Neoplasms; Endometrial Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Sarcoma; Survival Rate; Retrospective Studies
PubMed: 37569726
DOI: 10.3390/ijms241512353 -
Journal of Cancer Research and... 2018Synovial sarcomas (SSs) are very rare, poorly studied tumors that generally occurs around joint and muscle tendons. Primary SSs of the kidney are even rarer, accounting... (Review)
Review
Synovial sarcomas (SSs) are very rare, poorly studied tumors that generally occurs around joint and muscle tendons. Primary SSs of the kidney are even rarer, accounting for <2% of all malignant renal tumors. We report the case of a 44-year-old man who was diagnosed with primary renal SS on the basis of imaging, histopathological, and immunohistochemical examination. We also present a comprehensive review of the literature, with a focus on the differential diagnosis and treatment of renal tumors.
Topics: Adult; Biomarkers; Diagnosis, Differential; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Kidney Neoplasms; Male; Sarcoma, Synovial; Tomography, X-Ray Computed
PubMed: 29578187
DOI: 10.4103/0973-1482.181170 -
Cold Spring Harbor Molecular Case... Oct 2021Sclerosing epithelioid fibrosarcoma (SEF) is a rare and aggressive soft-tissue sarcoma thought to originate in fibroblasts of the tissues comprising tendons, ligaments,... (Review)
Review
Sclerosing epithelioid fibrosarcoma (SEF) is a rare and aggressive soft-tissue sarcoma thought to originate in fibroblasts of the tissues comprising tendons, ligaments, and muscles. Minimally responsive to conventional cytotoxic chemotherapies, >50% of SEF patients experience local recurrence and/or metastatic disease. SEF is most commonly discovered in middle-aged and elderly adults, but also rarely in children. A common gene fusion occurring between the and genes has been observed in 80%-90% of SEF cases. We describe here the youngest SEF patient reported to date (a 3-yr-old Caucasian male) who presented with numerous bony and lung metastases. Additionally, we perform a comprehensive literature review of all SEF-related articles published since the disease was first characterized. Finally, we describe the generation of an SEF primary cell line, the first such culture to be reported. The patient described here experienced persistent disease progression despite aggressive treatment including multiple resections, radiotherapy, and numerous chemotherapies and targeted therapeutics. Untreated and locally recurrent tumor and metastatic tissue were sequenced by whole-genome, whole-exome, and deep-transcriptome next-generation sequencing with comparison to a patient-matched normal blood sample. Consistent across all sequencing analyses was the disease-defining - fusion as a single feature consensus. We provide an analysis of our genomic findings and discuss potential therapeutic strategies for SEF.
Topics: Biomarkers, Tumor; Child, Preschool; Fibrosarcoma; Gene Fusion; Gene Rearrangement; Humans; Male; Soft Tissue Neoplasms
PubMed: 34362827
DOI: 10.1101/mcs.a006093 -
Medicine Dec 2022To present the clinical experience of primary renal Ewing's sarcoma/primitive neuroectodermal tumors (rEWs/PNET) admitted to our hospital and systematically review the... (Review)
Review
To present the clinical experience of primary renal Ewing's sarcoma/primitive neuroectodermal tumors (rEWs/PNET) admitted to our hospital and systematically review the published literature. A retrospective analysis was performed on patients with pathologically confirmed renal EWs/PNET (rEWs) in our hospital, and the literature on rEWs published in PubMed and Embase databases before March 1, 2022 was searched for analysis. A total of 337 rEWs were included in the statistical analysis, including 6 cases of our patients and 331 cases published in the literature. The common clinical symptoms of rEWs are abdominal pain, hematuria, abdominal mass and so on. computed tomography (CT) plays an important role in the diagnosis of rEWs, and the typical manifestation is a large heterogeneous soft tissue density mass, with a specific "septum-like" enhancement in contrast-enhanced scan. The 2-year overall survival rate of rEWs was 48%, with a median survival time of 18 months. "Septum-like" enhancement on CT can be used as a relatively specific sign for the differential diagnosis of rEWs from Wilms tumor and neuroblastoma. The maximum diameter of the rEWs was usually greater than 10 cm, the clinical symptoms of weight loss, metastasis at diagnosis, tumor thrombogenesis of renal vein or/and inferior vena cava tumor, and the failure to undergo radical nephrectomy were the factors of poor prognosis. The incidence of primary rEWs is low and the prognosis is poor. Early diagnosis and radical nephrectomy combined with chemotherapy is the key to improve the prognosis of patients, and CT plays an important role in early diagnosis.
Topics: Humans; Sarcoma, Ewing; Retrospective Studies; Kidney Neoplasms; Neuroectodermal Tumors, Primitive; Tomography, X-Ray Computed
PubMed: 36626543
DOI: 10.1097/MD.0000000000032189 -
Frontiers in Public Health 2022Renal sarcoma (RS) is rarely seen in clinical practice. The purpose of this study was to develop a prognostic nomogram model, which could predict the probability of... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Renal sarcoma (RS) is rarely seen in clinical practice. The purpose of this study was to develop a prognostic nomogram model, which could predict the probability of overall survival (OS) and cancer-specific survival (CSS) in adult patients with RS.
METHODS
Patients diagnosed with RS were recruited from the SEER database between 2004 and 2015, and randomized to two cohorts: the training cohort and the validation cohort. Uni- and multivariate Cox regression analyses in the training cohort were used to screen independent prognostic factors for OS and CSS. Prognostic nomograms for OS and CSS were created separately for adult RS patients based on independent risk factors. The area under the receiver operating characteristic (ROC) curves, calibration curves, and decision curve analysis (DCA) were used to validate the nomograms.
RESULTS
A total of 232 eligible patients were recruited, including 162 in the training cohort and 70 in the validation cohort. Sex, histological type, SEER stage, and surgery were independent prognostic factors for OS, while histological type, SEER stage, surgery, chemotherapy were independent prognostic factors for CSS. Based on the above independent prognostic factors, prognostic nomograms for OS and CSS were created respectively. In the training cohort, the AUCs of the nomograms for OS and CSS were 0.742 and 0.733, respectively. In the validation cohort, the AUCs of the nomograms for OS and CSS were 0.837 and 0.758, respectively. The calibration curves of the nomograms showed high consistencies between the predicted and actual survival rates. Finally, the DCA demonstrated that the nomograms in the wide high-risk threshold had a higher net benefit than the SEER stage.
CONCLUSION
A prognostic nomogram for renal sarcoma was created and validated for reliability and usefulness in our study, which assisted urologists in accurately assessing the prognosis of adult RS patients.
Topics: Adult; Humans; Neoplasm Staging; Nomograms; Prognosis; Reproducibility of Results; Retrospective Studies; Risk Factors; SEER Program; Sarcoma
PubMed: 36187680
DOI: 10.3389/fpubh.2022.942608 -
American Journal of Kidney Diseases :... Aug 1986Renal angiosarcoma, an uncommon vascular sarcoma, is primarily treated surgically. We present a patient who, at nephrectomy, had tumor adherent to the diaphragm and...
Renal angiosarcoma, an uncommon vascular sarcoma, is primarily treated surgically. We present a patient who, at nephrectomy, had tumor adherent to the diaphragm and renal artery. Postoperative radiation was given to the renal fossa. Despite the subsequent development of bone and liver metastases, disease in the renal fossa was apparently controlled. Symptomatic bone lesions were effectively palliated with radiotherapy. This report confirms those of others suggesting that angiosarcoma does respond to radiation.
Topics: Bone Neoplasms; Combined Modality Therapy; Hemangiosarcoma; Humans; Kidney; Kidney Neoplasms; Liver Neoplasms; Male; Middle Aged; Nephrectomy
PubMed: 3740062
DOI: 10.1016/s0272-6386(86)80126-3 -
International Braz J Urol : Official... 2015To investigate the clinical characteristics, prognosis, survival and diagnosis of high-grade primary renal leiomyosarcoma.
OBJECTIVE
To investigate the clinical characteristics, prognosis, survival and diagnosis of high-grade primary renal leiomyosarcoma.
MATERIALS AND METHODS
From January 2003 to April 2013, 10 cases of high-grade primary renal leiomyosarcoma were retrospectively reviewed. We analyzed clinical manifestations, treatment and prognosis of our group and correlated to the literature.
RESULTS
Ten cases (five male and five female patients; age range 43-77 years, mean = 57 ± std d:12.3 ) were enrolled. The mean diameter of the tumor masses was 9.35 ± 4.5 cm (range 3-18 cm). 40% of the patients were asymptomatic while the major symptom of 60% patients was lumbar pain. Nephrectomy was performed in 90% of patients. Partial nephrectomy surgery was preferred for only one patient. Pleomorphism and necrosis with high-grade, pink spindle cell cytoplasm were viewed in all patients. All patients were high-grade, pink spindle cell cytoplasm and pleomorfism and necrosis were observed in all. In an immunohistochemical examination, vimentin was seen in 100%, desmin in 90% and smooth muscle actin in 80% of the patients. CD117 was negative in all patients. All of the cases were followed-up, and the time of survival varied from 6 to 68 months (mean 23.9 ± std d:20.1). No patient received adjuvant CTx and/or RTx.
CONCLUSION
High-grade primary renal leiomyosarcomas (LMSs) are rare and highly malignant and the prognosis is poor. Early diagnosis and radical nephrectomy can prolong the patient's life. Surgery is the main treatment modality for renal (leiomyosarcoma) LMS.
Topics: Adult; Aged; Female; Humans; Immunohistochemistry; Kidney; Kidney Neoplasms; Leiomyosarcoma; Magnetic Resonance Imaging; Male; Middle Aged; Necrosis; Nephrectomy; Prognosis; Retrospective Studies; Tumor Burden
PubMed: 26005972
DOI: 10.1590/S1677-5538.IBJU.2015.02.17 -
International Braz J Urol : Official... 2006Primary renal Angiosarcoma is a rare neoplasm and only 24 cases have been reported in specialized literature. We describe a case of primary renal angiosarcoma in a...
Primary renal Angiosarcoma is a rare neoplasm and only 24 cases have been reported in specialized literature. We describe a case of primary renal angiosarcoma in a patient presenting with hematuria, palpable abdominal mass, left flank pain and anemia. A computerized tomography of the abdomen with contrast medium showed a tumor with 15 cm diameter, in the upper pole of the left kidney, with a low-density central area, suggesting necrosis or hemorrhage. Diagnosis was given in a morphologic base and proven by an immunohistochemical study. Primary renal angiosarcoma should be included among differential diagnosis of retroperitoneal hematoma and hemorrhagic renal tumors.
Topics: Aged; Fatal Outcome; Hemangiosarcoma; Humans; Immunohistochemistry; Kidney Neoplasms; Male; Nephrectomy; Tomography, X-Ray Computed
PubMed: 16953913
DOI: 10.1590/s1677-55382006000400011 -
Iranian Journal of Kidney Diseases Sep 2021No. Abstract. DOI: 10.52547/ijkd.6601.
No. Abstract. DOI: 10.52547/ijkd.6601.
Topics: Humans; Kidney Neoplasms; Sarcoma, Ewing
PubMed: 34582366
DOI: No ID Found