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1.  Rare case of blastemal predominant adult Wilms’ tumor with skeletal metastasis case report and brief review of literature 
Wilms’ tumor (nephroblastoma) is extremely rare in adults, skeletal metastasis being still rarer. The clinical course of adult Wilms’ tumor is very aggressive. The present case is a rare blastemal predominant adult Wilms’ tumor presenting with skeletal metastasis. We report a case of 19-year-old female presented with severe low backache and colicky left loin pain of 3 months and progressive weakness of 15 days duration. Magnetic resonance image (MRI) of lumbosacral spine was reported as spinal metastasis with right renal mass. The patient underwent right radical nephrectomy and the tumor was histopathologically confirmed as adult Wilms’ tumor. In case of adult Wilms’ tumor, distant metastasis may be the first presentation and this possibility should be considered when an adult patient presents with flank pain and a renal mass.
doi:10.4103/0970-1591.105772
PMCID: PMC3579131  PMID: 23450214
Adult Wilms’ tumor; renal tumor; skeletal metastasis
2.  Pattern of computed tomography scan findings in children with Wilms’ tumor in a tertiary hospital in Lagos, Nigeria 
Context:
Abnormal abdominal distension in a child is usually a source of great concern and apprehension in both the parents and the pediatric surgeon. Although Wilms’ tumor is the most common malignant abdominal childhood tumor, usually causing abdominal distension, many other less common causes can also result in abdominal distension. Therefore, early diagnosis of Wilms’ tumor and distinguishing it from all these other causes is essential.
Aims:
We intend to document the pattern of computed tomography (CT) scan findings in patients with Wilms’ tumor and the peculiarity of its radiological presentation in this environment, highlighting its accuracy and advantages over other imaging modalities.
Settings and Design:
This is a prospective descriptive study conducted between January, 2011 to January 2012, where all infants, 6-96 months of age, who were suspected to have Wilms’ tumor were made to undergo CT scan examination as part of their management work-up. It was carried out in a teaching hospital.
Subjects and Methods:
The study performed on 12 children age range of 6-96 months old underwent abdominal CT scans as part of their clinical work-up using a GE Hi-speed dual CT scanner®. Pre- and post-contrast images were acquired and assessed by the radiologists. They were operated and their radiological and histopathology results were correlated.
Statistical Analysis:
All data was entered into a Microsoft word excel spread sheet and analyzed by simple analysis. Percentages and proportions were determined for categorical variables.
Results:
Of the 12 patients seen, nearly 75% were males, with a male to female ratio of 3:1 and an average age of 37 months. The right sided tumors were slightly more than the left and nearly 25% were bilateral. Approximately 17% showed punctuate calcifications. Heterogeneous contrast enhancement was seen in all the cases, vascular encasement in three and lymphadenopathy in six cases. There was no bony involvement in any of them but 75% of the masses seen, crossed the midline. Histological confirmation of Wilms’ tumor was obtained in all cases.
Conclusions:
Majority of the tumors seen in this study crossed the midline and a few of them showed calcification. CT scan is relevant and accurate in investigating children with Wilms’ tumor as it reveals concomitant lesions with involvement of the abdominal vessels and lymph nodes.
doi:10.4103/0971-5851.133713
PMCID: PMC4080660  PMID: 25006281
Children; computed tomography scan; Nigeria; Wilms’ tumor
3.  Botryoid Wilms’ tumor: a case report and review of the literature 
Here, we report a new case of botryoid Wilms’ tumor, a 4-year-old boy, who was referred to us with a chief complaint of dysuria and gross hematuria. The computed tomography and radical nephroureterectomy showed that a botryoid sarcoma-like appearance occupied the right renal pelvis and extended into the bladder. Histologic examination further confirmed this case was a mixed type of Wilms’ tumor. In a word, we demonstrated a rare case of botryoid Wilms’ tumor, which extended from the renal pelvis into the ureter and bladder, then some degenerative and necrotic tissue with calcification discharged from urethra. Postoperative adjuvant chemotherapy was executed. At 24-month follow-up, there was no evidence of recurrence.
doi:10.1186/1477-7819-11-102
PMCID: PMC3661349  PMID: 23687909
Wilms’ tumor; Botyroid Wilms’ tumor; Renal; Computed tomography; Diagnosis
4.  Wilms' Tumor with Long-delayed Recurrence: 25 Years after Initial Treatment 
Korean Journal of Urology  2012;53(4):288-292.
Wilms' tumor is one of the most frequent malignant neoplasms in childhood. Advances in treatment modalities such as the combination of chemoradiation therapy with surgery have enhanced overall survival. However, recurrence of Wilms' tumor is still a problem. In this case, a 28-year-old female had experienced intermittent abdominal pain, and the computed tomography scan showed a huge pelvic mass. The patient had a history of radical nephrectomy for Wilms' tumor with concurrent chemotherapy at the age of three. The pelvic mass was resected in February 2010 and was confirmed to be a recurrent Wilms' tumor. The recurrent tumor showed a classic triphasic Wilms' tumor growth pattern with frequent mitoses and tumor necrosis. Our case is an extraordinary case of a long-delayed recurrent Wilms' tumor after 25 years, which is the longest disease-free interval ever reported. The possible effects of chemotherapy as well as some other mechanisms of this late relapse are discussed.
doi:10.4111/kju.2012.53.4.288
PMCID: PMC3332143  PMID: 22536475
Drug therapy; Late recurrence; Therapeutics; Wilms tumor
5.  Thyroid-like follicular carcinoma of the kidney: A report of two cases and literature review 
Oncology Letters  2014;7(6):1796-1802.
There have only been a few reports of thyroid-like follicular carcinoma of the kidney (TLFCK) to date. In the present study, two patients with TLFCK are reported. Patient 1 was a 65-year-old male exhibiting repeated hematuria and right back pain. No tumors were located in the patient’s thyroid or lungs. The physical examination revealed percussion tenderness over the right kidney region was noticed. Enhanced computed tomography (CT) indicated a right renal pelvic carcinoma, for which the patient underwent a radical right nephrectomy. Patient 2 was a 59-year-old male with a mass in the right kidney, located during a health examination and who exhibited no obvious clinical symptoms. The patient was clinically diagnosed with right renal carcinoma, confirmed by an enhanced CT. The patient underwent a radical right nephrectomy. The clinical features, imaging results, pathology, immune phenotypes, treatment and prognosis were analyzed. The associated literature was also reviewed. The cut surface of each tumor showed gray-white material with a central solid area, including scattered gray-brown necrotic and gray hemorrhagic areas and small cystic cavities. Microscopically, the arrangement of the tumor cells mimicked thyroid follicles with red-stained colloid-like material in the lumen. No renal hilar lymph node involvement was noted. The tumor tissue of patient 1 was immunohistochemically positive for vimentin, epithelial membrane antigen (EMA), cytokeratin (CK), CK7, and neuron specific enolase; and negative for CK34BE12, synapsin (Syn), CK20, cluster of differentiation 56 (CD56), CD10, Wilm’s tumor-1 (WT-1), CD34, CD57, P53, CD99, thyroid transcription factor-1 (TTF-1), CD15 and thyroglobulin (TG); with a Ki-67 labeling index (LI) of 30%. The tumor tissue of patient 2 was immunohistochemically positive for vimentin, EMA, CK7 and CK20; and negative for CD56, CD10, WT-1, CD34, CD57, P53, CD117, TTF-1, CD15, CD99, TG, chromogranin A and Syn; with a Ki-67 LI of 20%. TLFCK is a rare renal tumor with low malignancy but medium invasiveness. It morphologically resembles thyroid follicular carcinoma but does not express TTF-1 or TG. Radical nephrectomy can achieve good patient outcomes.
doi:10.3892/ol.2014.2027
PMCID: PMC4049768  PMID: 24932236
thyroid-like follicular carcinoma of the kidney; features; treatment; prognosis
6.  Adult Wilms' tumour: a case report with review of literature 
Diagnostic Pathology  2006;1:46.
Background
Wilms' tumor is the commonest primary malignant renal tumor in childhood. Rarely, it may present in the adult age group.
Case presentation
We report a 48-year-old male presenting with flank pain and haematuria. Abdominal ultrasound revealed a right renal mass measuring 11 × 10 cms, and a clinical diagnosis of renal cell carcinoma was made. Nephrectomy was performed, and a final diagnosis of adult Wilms' tumor was made based on the criteria proposed by Kilton et al.
Conclusion
The possibility of an adult Wilms' tumor should be considered when a patient presents with pain in the flank and a renal mass. Rarity of the tumor favors documentation in literature.
doi:10.1186/1746-1596-1-46
PMCID: PMC1702367  PMID: 17144931
7.  RETROPERITONEAL TUMORS IN CHILDREN—Roentgen Diagnosis 
California Medicine  1953;78(4):263-266.
A study was undertaken to determine whether there are any features of retroperitoneal tumors in children that might be demonstrated on roentgenograms to aid in identifying them preoperatively. Study was limited to Wilms' tumor of the kidney and neuroblastoma.
Calcification was found in 57 per cent of the neuroblastomas and in only 12 per cent of Wilms' tumors. Calcifications in neuroblastomas differed from those in Wilms' tumors. Calcification in neuroblastoma was more frequent in older children than in the younger ones.
The kidney was frequently displaced by both types of tumor. However, the neuroblastoma always displaced the kidney downward, or downward and slightly outward.
In most instances, the Wilms' tumor also displaced the kidney downward and outward, but in some instances upward and medially. This, of course, depended upon the site of origin of the tumor.
There was a distortion of the intrarenal structures in 75 per cent of the cases of neuroblastoma and in 71 per cent of the cases of Wilms' tumor.
Images
PMCID: PMC1521811  PMID: 13042655
8.  Differential diagnosis of renal tumors with tubulopapillary architecture in children and young adults: a case report and review of literature 
Background: Tumors of the kidney are uncommon in children and young adults. Accurate classification is crucial for both prognostication and therapeutic intervention. However, majority of the tumors in this age group have unusual morphology that renders classification challenging. Tubulopapillary architecture is one of the most common morphological patterns observed in renal tumors of children and young adults. Methods: A patient with epithelial predominant Wilms tumor was reported. Differential diagnosis of renal tumors with tubulopapillary morphology was discussed with an emphasis on the histological and immunohistochemical features, and the literature was reviewed. Results: A 25 year-old female patient presented with bilateral multilocular cystic masses. She underwent right radical nephrectomy and left partial nephrectomy. The pathological examination revealed a tumor with tubulopapillary architecture which was lined with low columnar epithelial cells. During the work-up of this case, several entities were considered and ruled out by careful gross, microscopic examination and prudent use of immunohistochemistry. The tumor cells were positive for WT-1, and variably positive for cytokeratin AE1/3, CD56, CD57, and negative for cytokeratin 7 and EMA. Fluorescent in-situ hybridization revealed no gain of chromosome 7 and 17. A diagnosis of epithelial predominant adult Wilms tumors was rendered for both kidneys. The patient received systemic chemotherapy and radiation to the remnant left kidney and was free of disease three years after the initial surgery. Conclusion: The differential diagnosis of renal tumors with tubulopapillary features in children and young adults include papillary renal cell carcinoma, metanephric adenoma, epithelial predominant Wilms tumor, translocation renal cell carcinoma and metastatic adenocarcinoma to the kidney. An accurate classification relies on careful examination of clinical and pathological features and immunohistochemical characteristics.
PMCID: PMC4219309  PMID: 25374929
Kidney; renal tumor; children; young adults; tubulopapillary; differential diagnosis; Wilms tumor; papillary renal cell carcinoma; metanephric adenoma
9.  Calcified, minimally Fat-contained angiomyolipoma clinically indistinguishable from a renal cell carcinoma 
BMC Nephrology  2013;14:160.
Background
Angiomyolipomas are benign tumors of the kidney. Typical angiomyolipomas are usually recognized by identifying fat components before any intervention. On the contrary, solid renal masses without evident fatty components but containing calcifications on the computed tomography scan are suspicious for malignancy. However, as in this rare case, rules of diagnostic imaging are of exceptions.
Case presentation
A 40-year-old man presented with left flank pain. The plain X-ray showed multiple coarse calcifications of 4.0 x 3.2 cm in diameter on the left upper quadrant abdomen. Computed tomography scan further revealed a solid renal mass and inside the mass there were calcifications. The size of the tumor was 5.6 × 5.5 × 6.3 cm. We performed a radical nephrectomy, and the histopathology showed a minimally fat-contained angiomyolipoma of multiple calcifications. The patient was free of recurrence or metastases after a follow-up period of 3 years.
Conclusion
An angiomyolipoma containing calcification is rare. An angiomyolipoma with minimal fat concomitant with calcifications is an even rarer presentation. It is very difficult to differentiate a minimal-fat angiomyolipoma with calcifications from a renal cell carcinoma preoperatively. In such a circumstance, a well-planned partial nephrectomy may be optimal for the patient, regardless of the tumor size.
doi:10.1186/1471-2369-14-160
PMCID: PMC3734114  PMID: 23876081
Angiomyolipoma; Calcifications; Radical nephrectomy; Renal cell carcinoma
10.  Peripheral primitive neuroectodermal tumor of the kidney in a 51-year-old female following breast cancer: A case report and review of the literature 
Oncology Letters  2014;9(1):108-112.
Peripheral primitive neuroectodermal tumor/Ewing’s sarcoma (pPNET/EWS) is an aggressive type of sarcoma that is rarely observed in the kidney. pPNET of the kidney principally occurs in young patients (<50 years old) and is very rare in older patients (≥50 years old). Additionally, only six cases of pPNET of the kidney have been reported in the literature in older patients (≥50 years old), and pPNET as a secondary primary tumor has rarely been reported. The current study presents a case of renal pPNET in a 51-year-old female who had been surgically treated for breast carcinoma and administered with adjuvant chemotherapy five years prior to hospitalization for pPNET. A computed tomography scan identified a tumor in the lower pole of the right kidney, which was treated by nephrectomy. Immunohistochemistry demonstrated diffuse, strong membranous positivity for cluster of differentiation (CD)99, positive nuclear staining for friend leukemia integration 1, and negative staining for Wilms’ tumor 1 and other markers. Fluorescence in situ hybridization (FISH) analysis of the EWS breakpoint region 1 (EWSR1) demonstrated the characteristic EWSR1 translocation. The patient declined chemotherapy or radiotherapy but accepted traditional Chinese medicine. No evidence of recurrence was observed eight months after diagnosis. Only two cases of renal pPNET with a history of an earlier or synchronous primary cancer were reported in the literature from the USA and Germany, respectively. To the best of our knowledge, the present case is the first FISH-confirmed renal pPNET in an older patient following breast adenocarcinoma.
doi:10.3892/ol.2014.2695
PMCID: PMC4246623  PMID: 25435942
renal peripheral primitive neuroectodermal tumor; breast adenocarcinoma; immunohistochemistry; fluorescent in situ hybridization; Ewing sarcoma breakpoint region 1; adjuvant therapy
11.  Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant 
Background: The multilocular cystic nephroma (MLCN) is a unilateral cystic neoplasm of the kidney exhibiting benign biological behavior. The etiology and histopathogenesis of the disease is controversial (dysplastic/hamartomous/neoplastic). MLCNs show bimodal age distribution, with peak incidence occurring at 2-4 years of age and between the fourth and sixth decades. The male to female ratio in patients aged below 4 years is 3:1, which reverses to 1:8 between the fourth and sixth decades. Patients and methods: A 59-year-old female patient presented with left flank pain and abdominal pain. Ultrasound (US) revealed 220×109×82 mm multiple septated hyperechoic kidney cysts with a semi-solid appearance. MRI showed a 245×119×98 mm multilocular cystic renal mass in the left kidney with hypointense appearance in T1-weighted images and hyperintense in T2-weighted images, and multicystic appearance in ureter projection, the largest portion measuring 17 mm in diameter. Radical nephrectomy was planned with the pre-diagnosis of multilocular cystic nephroma or multicystic renal cell carcinoma. Results: The patient underwent transperitoneal radical nephroureterectomy. The immunohistopathological examination revealed MLCN with ureteral invagination. Conclusion: The etiology, pathogenesis, and genetic basis of multilocular cystic nephroma are currently unknown. This tumor is confused with cystic partially differentiated nephroblastoma and cystic Wilms tumor in childhood, and multilocular cystic renal cell carcinoma, clear cell papillary renal cell carcinoma, and tubulocystic carcinoma in adults. The association of this tumor with pleuropulmonary blastoma in children exhibits genetic inheritance. US control is particularly recommended in siblings of these children. Albeit rare, the disease can occur as a bilateral synchronous or metachronous lesion. There are four reports of cases with recurrence in the literature. The laparoscopic partial nephrectomy is the recommended treatment method in patients with sufficient renal reserve that are found to be free of malignancy in the frozen section examination. The symptoms of hematuria and flank pain can be associated with invagination of the cysts into the pelvis and intrarenal rupture of the cysts. The invagination of cysts into the pelvis has been previously described. The authors consider that this was the first case of MLCN in the literature exhibiting invagination into the ureter.
PMCID: PMC4152099  PMID: 25197409
Multilocular cystic nephroma; renal cystic mass; multilocular cystic renal tumors; bosniak classification of renal cysts
12.  Monitoring Therapy with MEK Inhibitor U0126 in a Novel Wilms Tumor Model in Wt1 Knockout Igf2 Transgenic Mice Using 18F-FDG PET with Dual-Contrast Enhanced CT and MRI: Early Metabolic Response Without Inhibition of Tumor Growth 
Molecular Imaging and Biology  2012;15(2):175-185.
Purpose
The understanding of the role of genetic alterations in Wilms tumor development could be greatly advanced using a genetically engineered mouse models that can replicate the development and progression of this disease in human patients and can be monitored using non-invasive structural and molecular imaging optimized for renal tumors.
Procedures
Repetitive dual-contrast computed tomography (CT; intravenous and intraperitoneal contrast), T2-weighted magnetic resonance imaging (MRI), and delayed 2-deoxy-2-[18F]fluoro-d-glucose (18F-FDG) positron emission tomography (PET) were utilized for characterization of Igf2 biallelic expression/Wt1 knockout mouse model of Wilms tumor. For CT imaging, Ioversol 678 mg/ml in 200 μl was administered i.p. followed by 100 μl injected intravenously at 20 and 15 min prior to imaging, respectively. Static PET imaging studies were acquired at 1, 2, and 3 h after i.v. administration of 18F-FDG (400 μCi). Coronal and sagittal T1-weighted images (TE/TR 8.5/620 ms) were acquired before and immediately after i.v. injection of 0.4 ml/kg gadopentetate dimeglumine followed by T2-weighted images (TE/TR 60/300 ms). Tumor tissue samples were characterized by histopathology and immunohistochemistry for Glut1, FASN, Ki67, and CD34. In addition, six Wt1-Igf2 mice were treated with a mitogen-activated protein kinase (MEK) inhibitor U0126 (50 μmol/kg i.p.) every 4 days for 6 weeks. 18F-FDG PET/CT imaging was repeated at different days after initiation of therapy with U0126. The percent change of initial tumor volume and SUV was compared to non-treated historic control animals.
Results
Overall, the best tumor-to-adjacent kidney contrast as well as soft tissue contrast for other abdominal organs was achieved using T2-weighted MRI. Delayed 18F-FDG PET (3-h post 18F-FDG administration) and dual-contrast CT (intravenous and intraperitoneal contrast) provided a more accurate anatomic and metabolic characterization of Wilms tumors in Wt1-Igf2 mice during early development and progression of renal tumors. Over the 8-month period, 46 Wt1-Igf2 mice and 8 littermate control mice were studied. Renal tumors were identified in 54.3 % of Wt1-Igf2 mice between post-natal 50–100 days. In 35.6 % of Wt1-Igf2 mice, tumors were localized in the right kidney; in 24 %, in the left kidney, while 40.4 % of Wt1-Igf2 mice had bilateral kidney tumors. Metastatic lesions were identified in 15.4 % of Wt1-Igf2 mice. Increased levels of Glut1 and IGF1R expression, high Ki67 labeling index, and a dense network of CD34+ microvessels in renal tumors was consistent with increased 18F-FDG accumulation. Treatment with a MEK 1/2 inhibitor U0126 did not cause the inhibition of tumor growth as compared to untreated animals. However, after the first three to four doses (~2 weeks of treatment), a decrease in 18F-FDG SUV was observed, as compared to pre-treatment levels (p < 0.05, paired Student t test), which constitutes a metabolic response. Six weeks later, despite continuing therapy, the 18F-FDG SUV increased again to previous levels.
Conclusions
The optimized dual contrast PET/CT imaging with early post i.v. and i.p. contrast CT and 3 h delayed PET imaging after 18F-FDG administration provides a sensitive and reliable method for detecting early tumor lesions in this endogenous mouse model of Wilms tumor and for monitoring their growth in response to targeted therapies. Therapy with MEK inhibitor U0126 produces only a transient inhibition of tumor glycolytic activity but does not inhibit tumor growth, which is due to continuing IGF2-induced signaling from IGF1R through the PI3K-AKT-mTOR pathway.
doi:10.1007/s11307-012-0588-5
PMCID: PMC3591528  PMID: 22875335
Transgenic mice; Wilms tumor; 18F-FDG; PET/CT; CT; MRI
13.  Diffuse Hepatic Calcifications in a Transfusion-Dependent Patient with Beta-Thalassemia: A Case Report 
Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusion-dependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain. Computed tomography scan of the abdomen revealed diffuse hepatic calcification in the right, left, and caudate lobes of the liver. Her medical history disclosed hypoparathyroidism as well as chronic hepatitis C virus infection, which was successfully treated but led to early micronodular cirrhosis on liver biopsy. Other studies done to search for the cause of hepatic calcification failed to reveal any abnormalities. We suspect that hypoparathyroidism caused liver calcification, and should be, therefore, considered in the differential diagnosis of hepatic calcification if other causative factors have been ruled out.
PMCID: PMC3808953  PMID: 24174700
Beta-Thalassemia major; Hepatic; Calcification; Hypoparathyroidism
14.  AVOIDING MISDIAGNOSING NEUROBLASTOMA AS WILMS TUMOR 
Journal of pediatric surgery  2008;43(6):1159-1163.
Purpose
Although occasionally difficult, distinguishing abdominal neuroblastoma (NBL) from Wilms tumor (WT) at presentation is important, as surgical management differs significantly. We reviewed our 20 year experience (1987–2006) treating patients with NBL, focusing on those with an initial diagnosis of WT, to determine presenting features that would have suggested the correct pre-operative diagnosis.
Methods
Retrospective case cohort study reviewing charts and imaging of patients with NBL initially diagnosed clinically with WT. Preoperative symptoms, laboratory studies, and imaging were evaluated. Similar variables were assessed in the 20 patients with WT most recently treated at our institution.
Results
Nine patients with NBL were identified who had an exploratory laparotomy with a pre-operative diagnosis of WT;eight underwent nephrectomy at exploration. Children with NBL had symptoms such as fever and weight loss at presentation (67%) more often than patients with WT (20%). Pre-operative computed tomography (CT) demonstrated intratumoral calcifications, vascular encasement, or both in 78% of patients with NBL but were never seen in WT patients. Of interest, pre-operative urinary catecholamines were elevated in five patients ultimately diagnosed with NBL.
Conclusion
Although NBL can be mistaken for WT at presentation, the presence of constitutional symptoms, or intratumoral calcification or vascular encasement on preoperative imaging should heighten suspicion for NBL. In addition, laboratory evaluation, including urinary catecholamines, should be completed prior to surgery when the etiology of an abdominal tumor is uncertain.
doi:10.1016/j.jpedsurg.2008.02.047
PMCID: PMC3214966  PMID: 18558200
Neuroblastoma; Wilms tumor; Nephrectomy
15.  Synchronous bilateral Wilms tumor with complete radiographic response managed without surgical resection: A Report from the National Wilms Tumor Study-4 
Journal of pediatric surgery  2008;43(11):1982-1984.
Purpose
We reviewed the long-term local tumor control in patients with bilateral Wilms tumor (BWT) who received no definitive surgical therapy to one kidney after complete radiographic resolution after initial chemotherapy.
Methods
National Wilms Tumor Study-4 (NWTS-4) enrolled 3,335 patients (pts) during the period August 1986 through August 1994. There were 188 pts with BWT or 5.6% of the total enrolled. The treatment records and imaging reports were reviewed to ascertain those children who had documented tumors without definitive surgical therapy after initial treatment. Patients who did not have renal surgery due to progression of tumor were excluded from this study.
Results
Eleven children had no definitive surgical treatment for renal lesions in one kidney (right - 6, left -5) after initial treatment with chemotherapy and or radiation therapy. The pretreatment size of the lesions were <3cm (4pts), 3–6 cm (5pts), >6cm (1pt) and unknown (1 pt). Prechemotherapy biopsy was performed in 6 of 11 patients. Lesions <3cm (1pt), 3–6cm (3pt), >6cm (1pt) 1 unknown. Four biopsies showed favorable Wilms histology. One lesion (4cm) showed an intralobar nephrogenic rest, another lesion of unknown size was read as favorable histology vs. perilobar nephrogenic rest. Five lesions were not biopsied. (4pts <1 cm, 1 pt = 3cm.)
Only two children in this study received radiation treatment. One child received 1050 cGy whole abdominal radiation and one child received 1060 cGy to the left flank post nephrectomy. Radiation therapy was not given to any patient due to failure of the tumor to respond to chemotherapy.
Five patients received treatment regimen EE-4A, dactinomycin and vincristine. The duration of therapy ranged from 24 –102 weeks for an average of 55.6 weeks. Three patients received treatment regimen DD-4A, dactinomycin, vincristine and doxorubicin for 28, 52 and 52 weeks respectively. Three patients received two separate regimens of chemotherapy. One child was treated with dactinomycin, vincristine and cyclophosphamide for 60 weeks and then received regimen EE-4A for 24 weeks Another patient received regimen EE-4A for 16 weeks and then regimen DD4-A for 36 weeks. One child received regimen EE-4-A for 12 weeks and then regimen DD4A for 40 weeks. Management of the contralateral kidney was complete nephrectomy in all eleven patients. There were no local relapses in the renal tumor bed. All patients were alive at a median follow-up of 9 years (range 9 mo–15 yrs).
Conclusion
Children with synchronous BWT or Wilms tumor and contralateral nephrogenic rests that have radiographic resolution, after initial treatment have a low risk for local relapse. These children should be followed by serial imaging.
doi:10.1016/j.jpedsurg.2008.05.037
PMCID: PMC2801881  PMID: 18970928
Wilms-Bilateral
16.  Primary leiomyosarcoma of a horseshoe kidney in a woman with Turner syndrome: a case report 
BMC Research Notes  2014;7:491.
Background
Turner syndrome is characterized by complete or partial X-chromosome monosomy and has various clinical features, including horseshoe kidney. Leiomyosarcoma is an extremely rare tumor that accounts for only 0.1% of all invasive renal tumors.
Case presentation
A 50-year-old Japanese woman presented at a community hospital with a chief complaint of abdominal pain. Computed tomography revealed a horseshoe kidney with a hypovascular tumor (size, 9 × 7 cm) showing calcification in the upper pole of the right kidney. Open right heminephrectomy and division of the isthmus were performed. Histological examination revealed alternating fascicles of spindle cells with blunt ended non-tapering nuclei and eosinophilic cytoplasm. The tumor had high mitotic activity with a mitotic count of 8 mitoses/10 high-power fields. On the basis of these findings, we diagnosed the patient as having leiomyosarcoma.
Conclusion
Primary leiomyosarcoma of the horseshoe kidney in a patient with Turner syndrome is a very rare occurrence.
doi:10.1186/1756-0500-7-491
PMCID: PMC4124479  PMID: 25090932
Leiomyosarcoma; Turner syndrome; Horseshoe kidney
17.  Synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney: a unique case report and review of the literature 
Diagnostic Pathology  2009;4:17.
Background
Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma.
Methods
Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy.
Results
Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery.
Conclusion
This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms.
doi:10.1186/1746-1596-4-17
PMCID: PMC2704177  PMID: 19523243
18.  Sunitinib treatment enabling resection of massive liver metastasis: a case report 
Introduction
Sunitinib was developed as a molecular-targeted drug to treat advanced renal cell carcinoma. It is not yet known whether liver damage occurs in patients with liver metastases of renal cell carcinoma after sunitinib administration. Here, we report the case of a patient with an inoperable massive liver metastasis of renal cell carcinoma for whom sunitinib administration was dramatically effective with no obvious evidence of liver damage. As a result, the liver metastasis could be resected. We emphasize the dramatic reduction in liver metastasis with sunitinib treatment, and the histopathological effects of sunitinib on the non-tumorous liver parenchyma.
Case presentation
A 54-year-old Japanese woman was diagnosed with right renal cell carcinoma and underwent right nephrectomy 12 years earlier. She presented to a local clinic with right abdominal pain. A computed tomography scan showed a massive liver metastasis occupying her right hepatic lobe, and she was referred to our hospital for treatment. The diagnosis was not only liver metastasis, but also left renal metastasis. Oral administration of tyrosine kinase inhibitor sunitinib was started. Adverse events due to sunitinib included liver dysfunction, thrombocytopenia, and decreased hemoglobin, but she completed eight courses with the help of drug holidays and dose adjustments. Post-treatment computed tomography showed a dramatic reduction in size of her liver metastasis, enabling right lobectomy of her liver. Histopathological findings showed no obvious liver damage due to chemotherapy in non-cancerous parenchymal areas.
Conclusions
With the availability of sunitinib, some patients with potentially unresectable massive liver metastases of renal cell carcinoma may be able to undergo major hepatectomy curatively and safely with little histopathological damage to non-tumorous liver parenchyma, thus improving their prognosis.
doi:10.1186/1752-1947-7-234
PMCID: PMC3874753  PMID: 24090151
Chemotherapy; Liver damage; Liver metastasectomy; Liver metastasis; Renal cell carcinoma; Sunitinib
19.  Pattern of malignant solid tumors and lymphomas in children in the east delta of Egypt: A five-year study 
Oncology Letters  2014;8(5):2328-2332.
Worldwide, the incidence and mortality rates of childhood cancers differ. The study of incidence patterns and survival rates in childhood malignancies is important in aiding in the planning of treatment centers and in obtaining further information with regard to the etiology. Few studies have investigated the survival in cases of childhood solid tumors in Egypt. The aim of the current study was to evaluate the patterns, frequency and outcome of solid tumors and lymphomas in children admitted to and followed up at the Pediatric Oncology Department of Zagazig University Hospital (Zagazig, Egypt) over a duration of 5 years (January 2004 to December 2008). A retrospective study was conducted, which included 155 children with solid tumors and lymphomas. The medical records were reviewed and the relevant data collected, in particular, those concerning demographic, clinical, histopathological, laboratory and imaging data as well as the treatment plans and outcomes. The mean age of patients was 5.6±3.04 years at diagnosis. The patients comprised 94 males and 61 females. Non-Hodgkin lymphoma (NHL) was the most common tumor type, followed by neuroblastoma (31.0 and 29.0%, respectively). When patients were stratified in terms of age (<5, ≥5 but <10, and ≥10 years), the <5-years-of-age group exhibited the greatest number of patients. Fever, pallor and pain were the most frequent initial clinical presentations among the patients and stage II was the most common stage (39.1%) followed by stage IV, III and I (35.0, 20.3 and 5.6% respectively). The overall 5-year survival rate in the study group was 66.7%. The survival rate was significantly higher in patients with Wilm’s tumor and Hodgkin lymphoma, followed by NHL (92.0, 88.0 and 72.0%, respectively; P<0.001), while the mortality rate was significantly higher in patients with neuroblastoma (P<0.001). In conclusion, NHL and neuroblastoma were the most common tumors; the survival rates were higher in patients with Wilm’s tumor and Hodgkin lymphoma and lower in patients with neuroblastoma. A larger multicenter study is required to further investigate the conclusions drawn from this study.
doi:10.3892/ol.2014.2501
PMCID: PMC4186620  PMID: 25295119
solid tumors; lymphoma; epidemiology; children
20.  Teratoid Wilms’ tumor in a child: A report of a rare case 
Teratoid Wilms’ tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. We report a case of teratoid Wilms’ tumor in a 2-year-old male. Right sided abdominal mass was the presenting complaint. Ultrasonography of the abdomen showed a mass in the right kidney. Histopathological examination revealed blastemal, epithelial, and mesenchymal components along with areas presenting heterologous elements. More than 75% predominance of squamous differentiation with the keratin pearl formation was observed. The patient underwent nephrectomy and was followed post-operatively for 1 year and was normal.
doi:10.4103/2229-516X.112248
PMCID: PMC3678687  PMID: 23776845
Nephroblastoma; squamous differentiation; teratoid Wilms’ tumor
21.  Chromophobe renal cell carcinoma with concomitant sarcomatoid transformation and osseous metaplasia: a case report 
BMC Urology  2013;13:72.
Background
Chromophobe renal cell carcinoma is the third most common form of adult renal epithelial neoplasm. A sarcomatoid component occurs in approximately 8% of all chromophobe renal cell carcinoma cases, while metaplastic bone formation is extremely rare.
Case presentation
An abdominal computed tomography scan revealed a hypovascular tumor with focal calcification, measuring 2.5 × 2.3 cm, in the upper pole of the right kidney. The tumor was clinically diagnosed as a right renal cell carcinoma that showed signs of calcification, and a laparoscopic right radical nephrectomy was performed. The cut surface of the tumor was beige in color and indicated that the tumor was an extensively ossified mass. Histological analysis revealed three distinct morphological components of the tumor. The chromophobe renal cell carcinoma consisted of compact epithelial cells arranged in a nested pattern, and these were mixed with extensive areas of sarcomatoid spindle cells with marked nuclear pleomorphism and brisk mitotic activity. The tumor also contained multiple foci of metaplastic ossification.
Conclusion
Chromophobe renal cell carcinoma with concomitant osseous metaplasia and sarcomatoid transformation is a very rare occurrence.
doi:10.1186/1471-2490-13-72
PMCID: PMC3883464  PMID: 24330625
Chromophobe renal cell carcinoma; Sarcomatoid change; Osseous metaplasia
22.  Delayed tumor resection in a 5-year-old child with bilateral Wilms tumor 
Journal of Surgical Case Reports  2012;2013(3):rjt012.
We describe the case of a 5-year-old girl whose abdominal pain and distension were caused by Wilms tumor of the kidney. Because of the bilateral nature of her disease, she was spared biopsy or initial nephrectomy as part of her treatment course. Rather, she was treated presumptively for Wilms tumor based primarily on radiologic findings. Neoadjuvant chemotherapy consisting of vincristine, dactinomycin and doxorubicin was given to facilitate nephron-sparing surgery for tumor resection. Her initial chemotherapeutic course was complicated by tumor lysis syndrome manifested by elevated serum uric acid and was treated effectively with hyperhydration and alkalization of intravenous fluids. The patient's disease responded well to chemotherapy, and she underwent successful tumor excision after 12 weeks of chemotherapy. The resected tumor was identified as anaplastic Wilms tumor, illustrating that pathologic identification of Wilms tumor is possible even after multiple cycles of neoadjuvant chemotherapy and marked tumor shrinkage.
doi:10.1093/jscr/rjt012
PMCID: PMC3635223  PMID: 24964423
23.  Wilms’ Tumor in a 37-Year-Old 
Wilms’ tumor is rare in adults. Though the approach to diagnosis and treatment of adult Wilms’ tumor (AWT) is closely modeled on recommendations for childhood Wilms’ tumor, views differ on how aggressive the treatment should be. We report a case of a 37-year-old with Stage III favorable histology AWT. A radical nephrectomy was performed and the patient was due for chemotherapy. Recent advances, controversies and current recommendations in the treatment of AWT are discussed.
Keywords
Adult; Wilms’ tumor; Kidney
doi:10.4021/jocmr377w
PMCID: PMC3104656  PMID: 21629539
24.  Teratoid Wilms’ tumor – A rare renal tumor 
Urology Annals  2011;3(3):155-157.
Teratoid Wilms’ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms’ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms’ tumor.
doi:10.4103/0974-7796.84959
PMCID: PMC3183709  PMID: 21976930
Chemotherapy; radical nephrectomy; teratoid Wilms’ tumor; teratoma; unilateral; Wilms’ tumor
25.  Dyspnea, Tachycardia, and New Onset Seizure as a Presentation of Wilms Tumor: A Case Report 
Wilms tumor is found in 1 in 10,000 children and most commonly presents in asymptomatic toddlers whose care givers notice a nontender abdominal mass in the right upper quadrant. This case of Wilms tumor presented as a critically ill eleven-year old with significant tachypnea, dyspnea, vague abdominal pain, intermittent emesis, new onset seizure, metabolic acidosis, and hypoxemia. This is the first case in the literature of Wilms Tumor with cavoatrial involvement and seizure and pulmonary embolism resulting in aggressive resuscitation and treatment. Treatment included anticoagulation, chemotherapy, nephrectomy, and surgical resection of thrombi, followed by adjunctive chemotherapy with pulmonary radiation.
doi:10.1155/2014/562672
PMCID: PMC4006574  PMID: 24851188

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