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Anatomical Record (Hoboken, N.J. : 2007) Oct 2020Despite the rarity of renal tumors in children, many different types of malignant and nonmalignant renal neoplasms have been described. Therefore, the correct diagnosis... (Review)
Review
Despite the rarity of renal tumors in children, many different types of malignant and nonmalignant renal neoplasms have been described. Therefore, the correct diagnosis and clinical management of these patients can represent a challenge. Here we provide a comprehensive review of the commonly diagnosed pediatric renal malignancies, including nephroblastoma (commonly known as Wilms tumor), clear cell sarcoma of the kidney, rhabdoid tumor of the kidney, several subtypes of renal cell tumors (often collectively termed renal cell carcinoma), and congenital mesoblastic nephroma. The epidemiology, pathology, treatments, underlying genetic and molecular mechanisms, and proposed developmental origins are discussed in detail, highlighting differential features and potential improved therapeutic strategies for affected individuals.
Topics: Carcinoma, Renal Cell; Child; Humans; Kidney; Kidney Neoplasms; Sarcoma, Clear Cell; Wilms Tumor
PubMed: 31738020
DOI: 10.1002/ar.24315 -
Archives of Pathology & Laboratory... Feb 2020Anastomosing hemangioma is a rare neoplastic vascular lesion, histologically mimicking angiosarcoma. It is predominantly composed of irregularly anastomosing... (Review)
Review
Anastomosing hemangioma is a rare neoplastic vascular lesion, histologically mimicking angiosarcoma. It is predominantly composed of irregularly anastomosing sinusoidal-like spaces lined by endothelial cells with minimal atypia, a frequently hobnail morphology, and minimally invasive margins. Since its original description in the genitourinary system, an increasing number of anastomosing hemangiomas have been reported, localized deep in the body in various organs, always with similar histologic features. It is more frequently asymptomatic, often discovered incidentally by imaging studies, owing to coexisting benign or malignant tumors. In renal cases, the most frequent clinical context is end-stage renal disease. There is overwhelming evidence of the benign nature of the lesion and an accurate diagnosis could prevent overtreatment. The differential diagnosis includes other benign vascular tumors, well-differentiated angiosarcoma, Kaposi sarcoma, and other vascular-rich neoplasms. We review the clinical and histopathologic characteristics of this peculiar lesion with an emphasis on the differential diagnosis.
Topics: Diagnosis, Differential; Hemangioma; Hemangiosarcoma; Humans; Kidney Neoplasms
PubMed: 30958692
DOI: 10.5858/arpa.2018-0264-RS -
Journal of Pediatric Hematology/oncology Nov 2020The Ewing sarcoma family of tumors (ESFT) are high-grade small round blue cell malignancies traditionally presenting in children and adolescents. The most common site of...
The Ewing sarcoma family of tumors (ESFT) are high-grade small round blue cell malignancies traditionally presenting in children and adolescents. The most common site of primary disease is bone, though extraskeletal primary sites are well-recognized. We present 6 cases of primary ESFT of the kidney and 1 case of the adrenal gland. Patients were 11 to 18 years of age at diagnosis. Metastases at diagnosis were present in most cases (n=6). All patients underwent surgery, and most received radiation (n=5). Five patients relapsed after initial remission. Comprehensive review of the primary renal ESFT literature was used to analyze various factors, including age, sex, disease metrics, metastases at diagnoses, and overall survival in a total of 362 cases. Notably, while the general ESFT population has reported rates of metastasis at diagnosis of 20% to 25%, this rate in the renal ESFT population was 53% with a rate of 59% in adolescent and young-adult patients (11 to 24 y). Nodal disease at diagnosis was present in 24% of renal ESFT cases compared with 3.2% in patients with primary skeletal ESFT. While this malignant process may share histologic and molecular features with its bone and soft tissue counterparts, primary renal ESFT presentations seem to be more aggressive and have worse outcomes.
Topics: Adolescent; Child; Female; Humans; Kidney Neoplasms; Male; Sarcoma, Ewing
PubMed: 32282650
DOI: 10.1097/MPH.0000000000001804 -
Thoracic Cancer Feb 2018Tumor metastases are the basic biological characteristics of malignant tumors, and the lungs are the second most prominent metastatic organs in which these develop after... (Review)
Review
Tumor metastases are the basic biological characteristics of malignant tumors, and the lungs are the second most prominent metastatic organs in which these develop after the liver. Currently, with the rapid development of ablation technology, ablation therapy as a local treatment is playing an increasingly important role in the treatment of lung metastases. Whether alone or in combination with other treatments, ablation therapy has achieved good therapeutic effects for the treatment of partial lung metastases. This article briefly summarizes the results of current and previous ablation treatments for lung metastases, and focuses on the value of ablation therapy for different kinds of lung metastases.
Topics: Catheter Ablation; Colorectal Neoplasms; Disease-Free Survival; Humans; Kidney Neoplasms; Lung Neoplasms; Neoplasm Metastasis; Sarcoma; Treatment Outcome
PubMed: 29193688
DOI: 10.1111/1759-7714.12567 -
Laboratory Investigation; a Journal of... Jan 2018Sarcomas are a rare group of tumors of mesenchymal origin. Metastatic sarcomas are often difficult to treat and unresponsive to standard radio- and chemotherapy,... (Review)
Review
Sarcomas are a rare group of tumors of mesenchymal origin. Metastatic sarcomas are often difficult to treat and unresponsive to standard radio- and chemotherapy, resulting in a poor survival rate for patients. Novel treatments with immune checkpoint inhibitors have been proven to prolong survival of patients with a variety of cancers, including metastatic melanoma, lung, and renal cell carcinoma. Since immune checkpoint inhibitors could provide a novel treatment option for patients with sarcomas, clinical trials investigating their efficacy in these group of tumors are ongoing. However, the discrimination of patients that are the most likely to respond to these treatments is still an obstacle in the design of clinical trials. In this review, we provide a brief overview of the mechanisms of action of immune checkpoint inhibitors and discuss the proposed biomarkers of therapy response, such as lymphocytic infiltration, intratumoral PD-L1 expression, and mutational load in sarcomas.
Topics: Animals; Antibodies, Blocking; Antineoplastic Agents; Biomarkers, Tumor; Biomedical Research; Chemotaxis, Leukocyte; Costimulatory and Inhibitory T-Cell Receptors; DNA Mismatch Repair; Drug Resistance, Neoplasm; Drugs, Investigational; Gene Expression Regulation, Neoplastic; Humans; Immunotherapy; Lymphocyte Activation; Sarcoma; T-Lymphocytes
PubMed: 29155424
DOI: 10.1038/labinvest.2017.128 -
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 Journal of Cancer Dec 2020Malignant renal tumours represent 5% of childhood cancers and include types with likely different aetiology: Wilms tumour (WT), rhabdoid renal tumour, kidney sarcomas...
Malignant renal tumours represent 5% of childhood cancers and include types with likely different aetiology: Wilms tumour (WT), rhabdoid renal tumour, kidney sarcomas and renal carcinomas. WT is the most common renal tumour in children, previously shown to vary internationally and with ethnicity. Using the comprehensive database of the International Incidence of Childhood Cancer study (IICC), we analysed global variations and time trends in incidence of renal tumour types in children (age 0-14 years) and adolescents (age 15-19 years). The results were presented by 14 world regions, and five ethnic groups in the US. We included 15 320 renal tumours in children and 800 in adolescents reported to the 163 contributing registries during 2001-2010. In children, age-standardised incidence rate (ASR) of renal tumours was 8.3 per million (95% confidence interval, CI = 8.1, 8.4); it was the highest in North America and Europe (9-10 per million) and the lowest in most Asian regions (4-5 per million). In the US, Blacks had the highest ASR (10.9 per million, 95% CI = 10.2, 11.6) and Asian and Pacific Islanders the lowest (4.4 per million, 95% CI = 3.6, 5.1). In adolescents, age-specific incidence rate of renal tumours was 1.4 per million (95% CI = 1.3, 1.5). WT accounted for over 90% of all renal tumours in each age from 1 to 7 years and the proportion of renal carcinomas increased gradually with age. From 1996 to 2010, incidence remained mostly stable for WT (average annual percent change, AAPC = 0.1) and increased for renal carcinomas in children (AAPC = 3.7) and adolescents (AAPC = 3.2). Our findings warrant further monitoring.
Topics: Adolescent; Child; Child, Preschool; Female; Global Health; Humans; Incidence; Infant; Infant, Newborn; Kidney Neoplasms; Male; Registries; Rhabdoid Tumor; Sarcoma; Wilms Tumor
PubMed: 32902866
DOI: 10.1002/ijc.33147 -
PloS One 2022Linear and parallel are the two leading models of metastatic progression. In this study we propose a simple way to differentiate between them. While the linear model...
BACKGROUND
Linear and parallel are the two leading models of metastatic progression. In this study we propose a simple way to differentiate between them. While the linear model predicts accumulation of genetic and epigenetic alterations within the primary tumor by founder cells before spreading as waves of metastases, the parallel model suggests preclinical distribution of less advanced disseminated tumor cells with independent selection and expansion at the ectopic sites. Due to identical clonal origin and time of dispatching, linear metastases are expected to have comparable diameters in any specific organ while parallel metastases are expected to appear in variable sizes.
METHODS AND FINDINGS
Retrospective revision of chest CT of oncological patients with lung metastases was performed. Metastasis number and largest diameters were recorded. The sum number of metastases with a similar diameter (c) and those without (i) was counted and the linear/parallel ratio (LPR) was calculated for each patient using the formula (∑c-∑i)/(∑c+∑i). A LPR ratio of 1 implies pure linear progression pattern and -1 pure parallel. 12,887 metastases were measured in 503 patients with nine malignancy types. The median LPR of the entire group was 0.71 (IQR 0.14-0.93). In carcinomas of the pancreas, prostate, and thyroid the median LPR was 1. Median LPRs were 0.91, 0.65, 0.60, 0.58, 0.50 and 0.43 in renal cell carcinomas, melanomas, colorectal, breast, bladder, and sarcomas, respectively.
CONCLUSIONS
Metastatic spread of thyroid, pancreas, and prostate tumors is almost exclusively by a linear route. The spread of kidney, melanoma, colorectal, breast, bladder and sarcoma is both linear and parallel with increasing dominance of the parallel route in this order. These findings can explain and predict the clinical and genomic features of these tumors and can potentially be used for evaluation of metastatic origin in the individual patient.
Topics: Carcinoma, Renal Cell; Colorectal Neoplasms; Humans; Kidney Neoplasms; Male; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms
PubMed: 36129954
DOI: 10.1371/journal.pone.0274942 -
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 -
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