Dataset Information

Clinical outcome and prognostic factors in renal medullary carcinoma: A pooled analysis from 18 years of medical literature.

ABSTRACT: We describe clinical features and prognostic factors of renal medullary carcinoma (RMC) by performing a pooled analysis of all reported cases since 1995.A systematic search was performed to identify all articles describing patients with medullary renal cancer until February 2013. Survivals were estimated using Kaplan-Meier method with 95% confidence intervals and compared across the groups using the log-rank test. The following factors were evaluated using the Cox proportional hazards model: association of extension of disease at diagnosis, response to therapy, and surgical treatment of primary tumour with overall.A total 47 articles were selected; these described 165 patients with RMC plus 1 from our centre. The median age was 21 years and 98% of cases had the sickle cell trait. The mean size of the primary tumours was 6.0 cm, with an involvement of loco-regional lymph nodes in 71% of cases. The overall survival at diagnosis was 4.0 months in metastatic patients and 17.0 months in non-metastatic patients. Patients who received platinum-paclitaxel-gemcitabine had longer control of the disease when compared to topoisomerase inhibitors or targeted therapies. The multivariate analysis confirmed that the advanced stage at diagnosis increased the risk of death of about threefold.RMC is a tumour with poorer prognosis; based on these results, platinum-based chemotherapy is the preferred systemic treatment. Even if radical nephrectomy as an up-front strategy did not report a survival benefit, it may be considered to palliate local symptoms and to perform a correct diagnosis.

SUBMITTER: Iacovelli R

PROVIDER: S-EPMC4455635 | biostudies-other | 2015 Mar-Apr

REPOSITORIES: biostudies-other

ACCESS DATA

Publications

Clinical outcome and prognostic factors in renal medullary carcinoma: A pooled analysis from 18 years of medical literature.

Iacovelli Roberto R Modica Daniela D Palazzo Antonella A Trenta Patrizia P Piesco Gabriele G Cortesi Enrico E

Canadian Urological Association journal = Journal de l'Association des urologues du Canada 20150301 3-4

<h4>Introduction</h4>We describe clinical features and prognostic factors of renal medullary carcinoma (RMC) by performing a pooled analysis of all reported cases since 1995.<h4>Methods</h4>A systematic search was performed to identify all articles describing patients with medullary renal cancer until February 2013. Survivals were estimated using Kaplan-Meier method with 95% confidence intervals and compared across the groups using the log-rank test. The following factors were evaluated using th ...[more]

PMID: 26085875

Similar Datasets

Project description:Renal medullary carcinomas (RMCs) and collecting duct carcinomas (CDCs) are rare subsets of lethal high-stage, high-grade distal nephron-related adenocarcinomas with a predilection for the renal medullary region. Recent findings have established an emerging group of fumarate hydratase (FH)-deficient tumors related to hereditary leiomyomatosis and renal cell carcinoma (HLRCC-RCCs) syndrome within this morphologic spectrum. Recently developed, reliable ancillary testing has enabled consistent separation between these tumor types. Here, we present the clinicopathologic features and differences in the morphologic patterns between RMC, CDC, and FH-deficient RCC in consequence of these recent developments. This study included a total of 100 cases classified using contemporary criteria and ancillary tests. Thirty-three RMCs (SMARCB1/INI1-deficient, hemoglobinopathy), 38 CDCs (SMARCB1/INI1-retained), and 29 RCCs defined by the FH-deficient phenotype (FH/2SC or FH/2SC with FH mutation, regardless of HLRCC syndromic stigmata/history) were selected. The spectrum of morphologic patterns was critically evaluated, and the differences between the morphologic patterns present in the 3 groups were analyzed statistically. Twenty-five percent of cases initially diagnosed as CDC were reclassified as FH-deficient RCC on the basis of our contemporary diagnostic approach. Among the different overlapping morphologic patterns, sieve-like/cribriform and reticular/yolk sac tumor-like patterns favored RMCs, whereas intracystic papillary and tubulocystic patterns favored FH-deficient RCC. The tubulopapillary pattern favored both CDCs and FH-deficient RCCs, and the multinodular infiltrating papillary pattern favored CDCs. Infiltrating glandular and solid sheets/cords/nested patterns were not statistically different among the 3 groups. Viral inclusion-like macronucleoli, considered as a hallmark of HLRCC-RCCs, were observed significantly more frequently in FH-deficient RCCs. Despite the overlapping morphology found among these clinically aggressive infiltrating high-grade adenocarcinomas of the kidney, reproducible differences in morphology emerged between these categories after rigorous characterization. Finally, we recommend that definitive diagnosis of CDC should only be made if RMC and FH-deficient RCC are excluded.

Project description:Medullary carcinoma of the pancreas (MCP) is a rare malignant pancreatic tumor. The World Health Organization classification defines the tumor as a subtype of pancreatic ductal carcinomas. MCP is histologically characterized as having highly pleomorphic cells with syncytial morphology, expansive tumor growth, and necrosis. The pathogenesis and clinical course of MCP are largely unknown. Herein, we report an unusual case of MCP that was radiologically followed up for 9 years prior to surgical intervention.A 73-year-old Japanese woman with a nonspecific disease history was found to have an asymptomatic cyst in the pancreatic duct by abdominal ultrasonography. Thorough radiological investigation suggested it was not an aggressive neoplasm, and she received periodic check-ups under a clinical diagnosis of "a cystic pancreatic lesion of uncertain malignancy." Nine years after initial presentation, she experienced acute pancreatitis and underwent thorough re-evaluation. Dynamic computed tomography revealed no cyst; rather, a solid tumor was detected. Cytology of the pancreatic duct suggested adenocarcinoma. Pancreatoduodenectomy with D2 lymph node dissection was performed. The resected tumor was a non-mucinous, solid mass measuring 22?×?10 mm. Microscopically, the tumor had a well-demarcated pushing border. Lymphocytic infiltration was abundant, and stromal component was sparse. The tumor cells were composed of highly pleomorphic cells, proliferating in sheets without glandular formation. Neither lymphovascular invasion nor lymph node metastasis was detected. The histopathologic diagnosis was MCP, pT1aN0M0. The tumor carried a KRAS mutation, and MLH-1, MSH-2, MSH-6, and PMS-2 immunostaining results were normal, suggesting microsatellite stability. The patient has remained free of disease for 29 months following surgical intervention.A review of 20 previously reported cases plus the present case suggests that subsets of MCPs have genetic aberrations such as KRAS mutation and high microsatellite instability. MCP has been suggested to have a better prognosis than common ductal adenocarcinoma; however, 15 of 20 previously reported cases died from disease. Whether the asymptomatic cyst observed over 9 years contributed to the development of MCP in this patient is a subject for future study.

Project description:The purpose of the study was to identify a comprehensive prognostic system of pretreatment clinical parameters in 425 patients (pts) with metastatic renal-cell carcinoma treated with different subcutaneous (s.c.) recombinant cytokine-based home therapies in consecutive trials. Treatment consisted of (A) s.c. interferon-alpha 2a (INF-alpha), s.c. interleukin-2 (IL-2) (n=102 pts), (B) s.c. IFN-alpha 2a, s.c. IL-2, and i.v. 5-fluorouracil (5-FU) (n=235 pts) or (C) s.c. IFN-alpha 2a, s.c. IL-2, and i.v. 5-FU combined with p.o. 13-cis-retinoic acid (13cRA) (n=88 pts). Kaplan-Meier survival analysis, log-rank statistics, and Cox regression analysis were employed to identify risk factors and to create a multiple risk factor model. The following pretreatment risk factors were identified by univariate analysis: (1) three and more metastatic sites, (2) presence of liver, lymph node or bone metastases, (3) neutrophil count > or = 6500 cells microl(-1), (4) serum lactate dehydrogenase level (LDH) > or = 220 U l(-1), and (5) serum C-reactive protein level (CRP) > or = 11 mg l(-1). Cox regression analysis with forward stepwise variable selection identified neutrophil count as the major prognostic factor (hazard ratio=1.9, P<0.001), while serum levels of LDH and CRP, time between diagnosis of tumour and onset of metastatic disease, number of metastatic sites, and bone metastases were significant but somewhat less important prognostic variables within the multiple risk factor model (hazard ratio < or = 1.5). Patients were assigned to one of the three risk groups according to cumulative risk defined as the sum of simplified risk s.c.ores for six pretreatment variables. Low-, intermediate-, and high-risk patients achieved a median overall survival of 32+ months (95% CI 24, 43; 5-year survival of 27%), 18+ months (95% CI 15, 20; 5-year survival of 11%), and 8+ months (95% CI 6, 10; 5-year survival of 5%), respectively. These prognostic categories are helpful both in individual patient care and in the assessment of patients entering prospective clinical trials.

Dataset Information

Clinical outcome and prognostic factors in renal medullary carcinoma: A pooled analysis from 18 years of medical literature.

Publications

Clinical outcome and prognostic factors in renal medullary carcinoma: A pooled analysis from 18 years of medical literature.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure