Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0403608 (ureter)
9,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

WAGR syndrome is caused by an 11p13 deletion and includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation. We encountered a case of a dysgerminoma originating in an ectopic ovary in a woman with WAGR syndrome. Our patient was a 24-year-old nulliparous woman who was diagnosed with WAGR syndrome. The patient had undergone left nephrectomy for a Wilms' tumor and postoperative chemotherapy at the age of 7 months. She also had a history of glaucoma surgery in both eyes, and was followed up at the Department of Pediatrics for diabetes mellitus, hypertension, liver dysfunction and hyperuricemia. The patient was investigated for oliguria and had elevated levels of blood urea nitrogen (45 mg/dl) and creatinine (5.4 mg/dl); she was admitted to the hospital with acute renal failure and a computed tomography scan revealed a pelvic tumor with a long axis of 10 cm that was obstructing the right ureter. Following insertion of a ureteral stent, the tumor was removed. The tumor had developed in the retroperitoneal space independent of the ovaries. The right adnexa were normal. The tumor was histopathologically diagnosed as dysgerminoma. Follicles were found in part of the tumor; it was thus hypothesized that the tumor developed from an ectopic ovary. The patient was administered etoposide after surgery, and has been recurrence-free for 4 years since treatment.
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PMID:Dysgerminoma developing from an ectopic ovary in a patient with WAGR syndrome: A case report. 2788 34

Wilms tumor is the most common renal tumor in children. However, tumor extension into the ureter is exceedingly rare. We present a case of bilateral Wilms tumor with unilateral ureteral extension into the bladder. This case illustrates the importance of thoughtful diagnostic evaluation and surgical planning to obtain a good oncologic outcome while preserving renal function.
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PMID:Bilateral Wilms Tumor With Ureteral Extension. 2806 84

Ureteral extension of Wilms tumor (WT) is a rare occasion. The association of duplex collecting system and WT is extremely rare. Ureteral extension of WT in a duplex collecting system is an unreported entity to date. A 10-year-old girl presenting with WT extending into upper pole ureter of duplex system is reported to emphasize the importance of preoperative diagnosis to plan step by step surgery in this rare coincidental situation.
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PMID:Wilms Tumor Extension Into Duplex Ureter in a 10-Year-Old Girl. 2833 38

Wilms' tumor manifesting as an obstructing ureteral mass is extremely rare. Herein, we report an unusual case in which a child presented with a clinical picture concerning for and suggestive of ureteropelvic junction obstruction (UPJO), but was instead found to have an intrapelvic pedunculated Wilms' tumor with extension into the proximal ureter. We discuss the patient's diagnostic workup, radiographic, operative and pathologic findings, as well as important lessons learned from this unusual case.
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PMID:Pedunculated and obstructive Wilms' tumor: A rare presentation in a 2 year-old male. 2992 91

Wilms' tumor recurrences are notably encountered in the tumor bed, less commonly in the lungs. Metastatic involvement ureter markedly uncommon, though extension of the tumor at the presentation of primary tumor is known. We are documenting the metastasis to the ureteric stump which occurred in our patient nearly 2 years after completion of treatment for Wilms' tumor.
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PMID:Can Wilms' tumor recur in the ureteric stump? 3129 Nov 89

Wilms' tumor is the most common primary renal malignancy in children (80%) and the less common tumors include renal cell carcinoma, rhabdoid tumor, clear cell sarcoma, cellular congenital mesoblastic nephroma and medullary carcinoma, all of which originate from renal parenchyma. The tumors originating from renal pelvis are rare. The immunohistochemistry (IHC) showed INI1 deletion with the WT1 positive which has not been reported as we know. A 3-year-old boy was admitted to hospital for vomiting. An ultrasonography examination revealed a mass in the right kidney, medium echo, as well as hydronephrosis with collecting system separation of 3.5 cm. The computed tomography and the magnetic resonance (MR) radical showed that the tumor occupied the right renal pelvis and extended into the ureter. A radical nephroureterectomy was accomplished through a transabdominal approach. The pathologic diagnosis was malignant renal tumor with INI1 deficiency which was atypical in morphology and immunophenotype, but according to immunophenotype renal rhabdomyoid tumor could not be excluded. The patient was treated with carboplatin, etoposide and cyclophosphamide chemotherapy for 6 months. Follow-up studies of the patient showed no indication of recurrence or metastasis 22 months after nephrectomy. The novel findings may expand the spectrum of pediatric renal tumors to include the special malignancy.
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PMID:Renal malignant tumor with the loss of INI1 expression and WT1 positive in a 3-year-old boy: a case report. 3320 93


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