Extrarenal Nephroblastoma Research Articles

Extrarenal Nephroblastoma: A Report of Two Cases

In children, the most common solid tumours is nephroblastoma, but the extrarenal nephroblastoma exists and it is especially rare. It is defined by extrarenal location and histological confirmation. There are many symptoms of extrarenal nephroblastoma and it depends on the location. The diagnosis is based on histology after the resection of the tumor. The treatment of the extrarenal nephroblastoma is similar to the treatment of intrarenal nephroblastoma.

Nephroblastoma Arising from Primary Testicular Germ Cell Tumor: A Case Report and Literature Review.

Adult extrarenal nephroblastoma is a very rare tumor. Nephroblastoma arising from primary testicular germ cell tumor is exceedingly rare. To our knowledge, only three cases have been reported in the English literature. We report a case of a 19-year-old man who presented with a large right testicle. Image studies showed a large retroperitoneal mass along with liver and lung metastases. Orchiectomy demonstrated a mixed germ cell tumor composed of yolk sac tumor, embryonal carcinoma, and mature and immature teratoma with a significant portion of nephroblastoma. The patient received chemotherapy and no recurrence was noted during six months of followup. WT-1 expression was also studied due to the lack of consistency of its expression in testicular nephroblastoma in the literature. We also present a discussion and review of the literature due to its rarity, which indicate an adverse prognosis for patients with nephroblastoma components receiving standard chemotherapeutical regimes for testicular germ cell tumors.

Extrarenal Nephroblastoma

Introduction: As much as the nephroblastoma is almost exclusively known to be a renal tumor, some of the extrarenal locations, although rare, have been described in the literature. The sites of this rare entity (extrarenal nephroblastoma) reported entail the inguinal canals, retro-peritoneum, mediastinum, chest wall, ovaries, cervix, uterus and the prostate gland. Case Report: We report a case of a 13-month-old boy who was referred from the peripheral hospital presenting with severe abdominal distention of three months duration. The patient was critical ill on arrival and had to be actively resuscitated. The patient was emaciated. No known past medical history reported and the patient had normal developmental milestones. On physical examination a large abdominopelvic mass was palpated. The patient also had sepsis confirmed with blood culture and hypoglycemia with a blood glucose measurement of 1.4 mmol/l saturation was 96% in room air and had episodes of gasping respiration, the heart beat was ranging between 110–140 bpm. The patient had pallor with hemoglobin 8.4 g/dL and had cold peripheries. Computed tomography scan and biopsy were performed and the diagnosis of an extrarenal large abdominopelvic nephroblastoma was made. The patient was later, after stabilization, treated with chemotherapy. The patient now remains in a critical general condition while on chemotherapy. Conclusion: A nephroblastoma may present in some extrarenal sites which is quite an uncommon encounter, the most common of these rare sites being the retro-peritoneum. The final diagnosis is mainly histopathological. There are no specific management protocols and as a result the extrarenal tumors are managed in the same manner in which the intra-renal tumors are managed with a general good prognosis.

Extrarenal nephroblastoma.

IntroductionNephroblastoma is one of the most common solid tumours in children. The occurrence of extrarenal nephroblastoma is exceedingly rare. What can be defined as extrarenal Wilms’ tumor must satisfy the following criteria: histologically confirmed nephroblastoma and extrarenal location.Material and methodsCurrent data on extrarenal nephroblastoma based on a selective review of the literature.ResultsThe retroperineal location is reported to be typical in males, whereas the inguinal region is believed to predominate in females. There are no characteristic manifestations of extrarenal nephroblastoma. The symptoms depend on the location and stage upon diagnosis. US, CT and MRI are used to detect tumours in the retroperiteneal space, inguinal, sacro-coccal and scrotal area. However, extrarenal nephroblastoma does not show characteristic radiological features.ConclusionsIn absence of typical clinical presentation or conclusive imaging tests, the diagnosis is based on histology after the resection of the tumor. The recommended management of the extrarenal nephroblastoma is similar to the treatment of intrarenal nephroblastoma.

Extrarenal nephroblastoma in a 7 year old child: a rare case report with review of literature

Nephroblastoma or Wilms’ tumors are a tumour of the kidney and is the commonest primary malignant tumour occurring in childhood with classic histopathological features. Majority of these tumors present as renal masses and atypical presentations like extrarenal masses have also been known. Extrarenal Wilms’ tumor or Nephroblastoma (ERWT), excludes primary tumor in the kidney, is extremely rare and occurs predominantly in children. Most of the cases that have been reported involved the retroperitoneum. It is generally located anywhere in the retroperitoneum from the nephric region down through lumbar, iliac, pelvic region to inguinal canal. Though there are reports regarding its prevalence, its occurrence is very rare in childhood. We are reporting a case of ERWT in a 7 year old child arising from the retroperitoneum, adherent to the ascending colon, second and third part of duodenum, right kidney and ureter.

Rare localization of extrarenal nephroblastoma in 1-month-old female infant

Extrarenal occurrence of Wilms' tumor is exceptional and the diagnosis is almost always made after surgery. The exact mechanism whereby a Wilms' tumor occurs in extrarenal tissue is unknown. The tumor is most commonly located in the retroperitoneum or inguinal region. Localization in subcutaneous tissue is extremely rare. In this paper, the case of a 1-month-old female infant with an extrarenal Wilms' tumor located in the lumbosacral region is presented. Surgical excision is the treatment of choice, and the same general therapeutic rules should be followed as when the kidney is affected.

Extrarenal adult nephroblastoma.

Ga-67 scintigraphy, abdominal computed tomography (CT), and pelvic magnetic resonance imaging (MRI) were performed in a patient with extrarenal adult nephroblastoma. CT showed a large intra-abdominal cystic tumor displacing the liver and the right kidney. MRI revealed a pelvic tumor with cystic changes. Ga-67 scintigraphy revealed multiple sites of tumor localization in the abdomen. These results suggest that Ga-67 scintigraphy may be a useful imaging method in the detection of primary and metastatic lesions in the setting of extrarenal adult nephroblastoma.

Immunohistochemical demonstration of wilms tumour gene product WT1 in a canine “Neuroepithelioma” providing evidence for its classification as an extrarenal nephroblastoma

An intradural extramedullary tumour, surgically removed from the spinal canal of a young dog with paraplegia, had the histological appearance of a nephroblastoma. Subsequent necropsy revealed no evidence of a renal primary tumour or of any other tumour. Similar tumours of the spinal canal have been described previously under a variety of names, in particular neuroepithelioma. With an antibody to the human Wilms tumour (nephroblastoma) gene product WT1, labelling of glomeruloid bodies, similar to glomerular podocytes in human fetal kidney, was demonstrated in the tumour. This finding strengthened the suggestion that it was a nephroblastoma.

Extrarenal Wilms' Tumor with Cerebellar Metastasis in a Four-Year-Old Girl with Spina Bifida

This report documents the occurrence of an extrarenal nephroblastoma from which a cerebellar metastasis developed in a four-year-old girl with spina bifida. The second tumor became symptomatic two years after the resection of the primary, suggesting a treatment effect as a factor for the delay in the growing of the metastatic neoplasm. Histologic and ultrastructural features of the metastasis were similar to those described in Wilms' tumors of the kidney. The pathogenesis of this exceptional association, including malformation, malignancy, and unusual site of metastasis, is discussed.

Extrarenal nephroblastomas

The cases of extrarenal nephroblastoma published in the world literature have been tabulated and reviewed, and are discussed from a historical point of view.

Extrarenal Wilms' tumor

The extrarenal location of Wilms' tumor is extremely rare. These tumors can arise from other neoplasms, generally teratomas, or they can present without any associated teratomatous elements. We encountered only 19 well-documented cases of isolated extrarenal nephroblastoma, and we describe three previously unreported patients with this disease. Two of the three children presented also with horseshoe kidneys, an association that may have clinical and embryologic significance. The presence of tumor cephalad to a horseshoe kidney is easier to explain when we consider that they originate from primitive mesodermal tissue, probably mesonephric rests, and not from metanephric remnants. Also, this association should alert physicians to the possible diagnosis of extrarenal nephroblastoma in patients with a retroperitoneal mass and horseshoe kidneys. These patients should be treated according to the same protocols as those for patients with intrarenal Wilms' tumor.