Coexistence of Different Histotypes of Renal Carcinoma: Our Experience and Literature Review

2009 ◽  
Vol 76 (2) ◽  
pp. 130-132
Author(s):  
M. Esposito ◽  
V. Varca ◽  
A. Simonato ◽  
C. Toncini ◽  
G. Carmignani ◽  
...  

The coexistence of multiple, synchronous primary tumors of different histology within the same kidney is a rare condition. We report herein a series of five patients with two tumors of different histology involving synchronously the same kidney. Materials and Methods We reviewed the pathology reports of a series of 381 patients who underwent surgery for primary renal tumors at our institution from 2000 to 2007. In the files of all patients with synchronous tumors of different histology, special attention was given to the results of imaging studies. Results Five out of 381 patients (1.37%) had coexistence of two primary tumors of different histology within the same kidney. Four patients had ultrasonography as the first imaging procedure, one patient had ultrasonography as the second imaging procedure; all had preoperative CT of the abdomen. Both lesions were detected by preoperative CT in 4/5 of the cases; in the remaining one, the smaller lesion was not visible, even in retrospect. Conclusions The coexistence of multiple and synchronous primary tumors of different histology within the same kidney has been only rarely described. To the best of our knowledge, in literature there are only case reports with the exception of a case of renal oncocytoma with evolving papillary RCC. We believe that this condition could be more frequent if the radiologist and the anatomopathologist try to find it.

2019 ◽  
Vol 98 (7) ◽  
pp. 291-296

Introduction: Fournier’s gangrene is a rare but fast deteriorating and serious condition with high mortality. In most cases, it is characterized as necrotizing fasciitis of the perineum and external genitals. Amyand’s hernia is a rare condition where the appendix is contained in the sac of an inguinal hernia. Inflammatory alterations in the appendix account only for 0.1 % of the cases when Amyand’s hernia is verified. Fournier’s gangrene as a complication of a late diagnosis of appendicitis located in the inguinal canal is described in the literature as rare case reports. Case report: The case report of a 70-year-old patient with Fournier’s gangrene resulting from gangrenous appendicitis of Amyand’s hernia. Conclusion: Fournier’s gangrene as a complication of Amyand’s hernia is a rare condition. Only sporadic case reports thereof can be found in the literature. Because of the rarity of this pathology and the lack of randomized controlled studies, it is difficult to determine the optimal treatment according to the principles of evidence-based medicine. An appropriate approach for this condition appears to be the combination of guidelines developed in Amyand’s therapy according to Losanoff and Basson, along with the recommended “gold standard” therapy for Fournier’s gangrene. This means early and highly radical surgical debridement, adequate antibiotic therapy and intensive care.


2019 ◽  
Vol 65 (5) ◽  
pp. 756-759
Author(s):  
Mikhail Postolov ◽  
Nadezhda Kovalenko ◽  
K. Babina ◽  
Stanislav Panin ◽  
Yelena Levchenko ◽  
...  

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm characterized by expression of both mela-nocytic and smooth muscle markers. Only 10 primary malignant lung PEComas have been reported up to date. We report a 59-year-old male who presented with a lung neoplasm, found during the routine X-ray examination. Preoperative CT-scan revealed the 3,5-cm-sized mass, located at the border of the upper, middle and lower lobes of the right lung. Patient underwent a thoracotomy, resection of the upper, middle and lower lobes of the right lung accompanied with mediastinal lymphadenectomy. After surgery, chylothorax was revealed. Conservative treatment was unsuccessful, so we performed laparoscopic clipping of the thoracic lymph duct. Patient was dismissed from hospital on the 10-th day after the second operation in good condition. In this report, we intend to increase the limited knowledge relating to natural history and optimal treatment of such a rare condition as a primary malignant lung PEComa.


2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Moutaz Ragab ◽  
Omar Nagy Abdelhakeem ◽  
Omar Mansour ◽  
Mai Gad ◽  
Hesham Anwar Hussein

Abstract Background Fetus in fetu is a rare congenital anomaly. The exact etiology is unclear; one of the mostly accepted theories is the occurrence of an embryological insult occurring in a diamniotic monochorionic twin leading to asymmetrical division of the blastocyst mass. Commonly, they present in the infancy with clinical picture related to their mass effect. About 80% of cases are in the abdomen retroperitoneally. Case presentation We present two cases of this rare condition. The first case was for a 10-year-old girl that presented with anemia and abdominal mass, while the second case was for a 4-month-old boy that was diagnosed antenatally by ultrasound. Both cases had vertebrae, recognizable fetal organs, and skin coverage. Both had a distinct sac. The second case had a vascular connection with the host arising from the superior mesenteric artery. Both cases were intra-abdominal and showed normal levels of alpha-fetoprotein. Histopathological examination revealed elements from the three germ layers without any evidence of immature cells ruling out teratoma as a differential diagnosis. Conclusions Owing to its rarity, fetus in fetu requires a high degree of suspicion and meticulous surgical techniques to avoid either injury of the adjacent vital structures or bleeding from the main blood supply connection to the host. It should be differentiated from mature teratoma.


2019 ◽  
Vol 45 (10) ◽  
pp. 3193-3201 ◽  
Author(s):  
Yajuan Li ◽  
Xialing Huang ◽  
Yuwei Xia ◽  
Liling Long

Abstract Purpose To explore the value of CT-enhanced quantitative features combined with machine learning for differential diagnosis of renal chromophobe cell carcinoma (chRCC) and renal oncocytoma (RO). Methods Sixty-one cases of renal tumors (chRCC = 44; RO = 17) that were pathologically confirmed at our hospital between 2008 and 2018 were retrospectively analyzed. All patients had undergone preoperative enhanced CT scans including the corticomedullary (CMP), nephrographic (NP), and excretory phases (EP) of contrast enhancement. Volumes of interest (VOIs), including lesions on the images, were manually delineated using the RadCloud platform. A LASSO regression algorithm was used to screen the image features extracted from all VOIs. Five machine learning classifications were trained to distinguish chRCC from RO by using a fivefold cross-validation strategy. The performance of the classifier was mainly evaluated by areas under the receiver operating characteristic (ROC) curve and accuracy. Results In total, 1029 features were extracted from CMP, NP, and EP. The LASSO regression algorithm was used to screen out the four, four, and six best features, respectively, and eight features were selected when CMP and NP were combined. All five classifiers had good diagnostic performance, with area under the curve (AUC) values greater than 0.850, and support vector machine (SVM) classifier showed a diagnostic accuracy of 0.945 (AUC 0.964 ± 0.054; sensitivity 0.999; specificity 0.800), showing the best performance. Conclusions Accurate preoperative differential diagnosis of chRCC and RO can be facilitated by a combination of CT-enhanced quantitative features and machine learning.


Author(s):  
K. Dhanasekar ◽  
V. Visakan ◽  
F. Tahir ◽  
S. P. Balasubramanian

Abstract Introduction Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre’s records and presents a systematic literature review of composite phaeochromocytomas. Methods In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist. Results There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4–86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5–168) months. Conclusion Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.


Author(s):  
Mateusz Kozłowski ◽  
Katarzyna Nowak ◽  
Agnieszka Kordek ◽  
Aneta Cymbaluk-Płoska

Carcinosarcoma, leiomyosarcoma, melanoma and carcinoid as primary tumors in the ovary are extremely rare. In this paper, the authors reviewed the literature from 2010 to 2021, based on specific criteria, to analyze the treatment of these rare ovarian neoplasms. We also aimed to verify whether modern therapies have been found in recent years. For this article, 80 papers were finally selected. The vast majority of the articles were clinical case reports. Despite single mentions of new potential pharmacological treatments, surgery (radical or fertility-sparing) is definitely the mainstay of treatment. There are currently no treatment guidelines for these tumors. A review of the literature has revealed the use of various adjuvant treatments. We, therefore, believe that a more detailed understanding of the biology of these tumors is necessary in order to find new target points for treatment. We would like to emphasize the importance of creating an international database of rare ovarian tumors which would make it possible to gather data from various oncological centers and enable further research into these neoplasms.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jianxiong Fu ◽  
Jing Ye ◽  
Wenrong Zhu ◽  
Jingtao Wu ◽  
Wenxin Chen ◽  
...  

Abstract Background Benign and malignant renal tumors share similar some imaging findings. Methods Sixty-six patients with clear cell renal cell carcinoma (CCRCC), 13 patients with renal angiomyolipoma with minimal fat (RAMF) and 7 patients with renal oncocytoma (RO) were examined. For diffusion kurtosis imaging (DKI), respiratory triggered echo-planar imaging sequences were acquired in axial plane (3 b-values: 0, 500, 1000s/mm2). Mean Diffusivity (MD), fractional Anisotropy (FA), mean kurtosis (MK), kurtosis anisotropy (KA) and radial kurtosis (RK) were performed. Results For MD, a significant higher value was shown in CCRCC (3.08 ± 0.23) than the rest renal tumors (2.93 ± 0.30 for RO, 1.52 ± 0.24 for AML, P < 0.05). The MD values were higher for RO than for AML (2.93 ± 0.30 vs.1.52 ± 0.24, P < 0.05), while comparable MD values were found between CCRCC and RO (3.08 ± 0.23 vs. 2.93 ± 0.30, P > 0.05). For MK, KA and RK, a significant higher value was shown in AML (1.32 ± 0.16, 1.42 ± 0.23, 1.41 ± 0.29) than CCRCC (0.43 ± 0.08, 0.57 ± 0.16, 0.37 ± 0.11) and RO (0.81 ± 0.08, 0.86 ± 0.16, 0.69 ± 0.08) (P < 0.05). The MK, KA and RK values were higher for RO than for CCRCC (0.81 ± 0.08 vs. 0.43 ± 0.08, 0.86 ± 0.16 vs. 0.57 ± 0.16, 0.69 ± 0.08 vs. 0.37 ± 0.11, P < 0.05). Using MD values of 2.86 as the threshold value for differentiating CCRCC from RO and AML, the best result obtained had a sensitivity of 76.1%, specificity of 72.6%. Using MK, KA and RK values of 1.19,1.13 and 1.11 as the threshold value for differentiating AML from CCRCC and RO, the best result obtained had a sensitivity of 91.2, 86.7, 82.1%, and specificity of 86.7, 83.2, 72.8%. Conclusion DKI can be used as another noninvasive biomarker for benign and malignant renal tumors’ differential diagnosis.


2021 ◽  
Vol 81 ◽  
pp. 105720
Author(s):  
Youssef Oukessou ◽  
Yassir Hammouda ◽  
Khadija El Bouhmadi ◽  
Redallah Larbi Abada ◽  
Mohamed Roubal ◽  
...  

2020 ◽  
Vol 8 (Suppl 3) ◽  
pp. A843-A843
Author(s):  
Michael Wagner ◽  
Megan Othus ◽  
Sandip Patel ◽  
Christopher Ryan ◽  
Ashish Sangal ◽  
...  

BackgroundAngiosarcoma is a rare cancer of endothelial cells that can be aggressive and carries a high mortality. A subset of angiosarcomas are characterized by high tumor mutational burden (TMB) and UV light exposure DNA mutational signature. Isolated case reports have suggested clinical efficacy of immune checkpoint blockade in angiosarcoma; no prospective studies of immune checkpoint inhibition in angiosarcoma have been reported. We report efficacy analysis results for patients with advanced or unresectable angiosarcoma treated with ipilimumab and nivolumab as a cohort of an ongoing phase II study for rare cancers (NCT02834013).MethodsThis is a prospective, open-label, multicenter phase II clinical trial of ipilimumab (1mg/kg IV q6weeks) plus nivolumab (240mg IV q2weeks) for patients with metastatic or unresectable angiosarcoma. Primary endpoint is objective response rate as assessed by RECIST v1.1, including measurable cutaneous disease that can be followed by photography. Secondary endpoints include PFS, OS, stable disease at six months, and toxicity. A two-stage design is used with six patients in the first stage and an additional ten patients in the second stage.ResultsAt data cutoff, 16 patients with angiosarcoma were enrolled. Median age was 68 years (25-81 years). Median number of prior lines of therapy was 2 (0-5). 9 patients had cutaneous primary tumors of any cutaneous site, 7 had non-cutaneous primary tumors. ORR for all patients was 25% (4/16, table 1, figure 1). Subgroup analysis revealed that 60% (3/5) of patients with primary cutaneous tumors of the scalp or face had a confirmed objective response. 6-month PFS was 38%. 75% of patients experienced an adverse event (AE), and 25% experienced a grade 3-4 AE. 68.8% experienced an immune related AE (irAE), and 2 (12.5%) developed grade 3 or 4 irAEs. Grade 3-4 irAEs were ALT and AST increase and diarrhea. There were no grade 5 toxicities.ConclusionsThe combination of ipilimumab and nivolumab was well tolerated and had an ORR of 25% in angiosarcoma regardless of primary site, with 3 of 5 patients with cutaneous tumors of the scalp or face responding. Ipilimumab and nivolumab warrant further investigation in angiosarcoma.AcknowledgementsFunding: National Institutes of Health/National Cancer Institute grant awards CA180888, CA180819, CA180868; and in part by Bristol-Myers Squibb CompanyTrial RegistrationNCT02834013Ethics ApprovalThis study was approved by the NCI CIRB.


2016 ◽  
Vol 88 (2) ◽  
pp. 150 ◽  
Author(s):  
Francesco De Luca ◽  
Evangelos Zacharakis ◽  
Majed Shabbir ◽  
Angela Maurizi ◽  
Emy Manzi ◽  
...  

Malignant priapism secondary to penile metastases is a rare condition. This term was originally used by Peacock in 1938 to describe a condition of painful induration and erection of the penis due to metastatic infiltration by a neoplasm. In the current literature there are 512 case reports. The primary tumor sites are bladder, prostate and rectum. The treatment has only palliative intent and consists of local tumor excision, penectomy, radiotherapy and chemotherapy. We present one case of malignant priapism originated from prostate cancer, and two from urothelial carcinoma of the bladder. Different approaches in diagnosis and therapy were performed. The entire three patient reported a relief of the pain following the treatment, with an improvement of their quality of life, even though it was only temporary as a palliative. Malignant priapism is a rare medical emergency. Penile/pelvis magnetic resonance imaging (MRI) scan and corporal biopsies are considered an effective method of diagnosis of the primary organ site.


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