Teratoid Wilms Research Articles

Horseshoe kidney with teratoid type of Wilms tumor: a rare case report

BackgroundHorseshoe kidney is the most common renal fusion anomaly, and Wilms tumor is the most frequent renal malignancy in children. The occurrence of Wilms tumor in association with horseshoe kidney is a scarce anomaly. However, the arising of a teratoid type, which is a rare variant of Wilms tumor in a horseshoe kidney, is exceptionally unique.Case presentationThis report presents a 5-year-old male admitted with horseshoe kidney involved by a large heterogeneous calcified mass that was diagnose on biopsy as Wilms tumor blastemal dominant. According to the local and regional extension and metastatic tumor in the lungs, the patient underwent neoadjuvant chemotherapy and then surgery. Post-operative pathologic findings confirmed the diagnosis of teratoid Wilms tumor.ConclusionsThe occurrence of renal anomalies associated with a malignancy might be more frequent in the clinical environment. There are numerous differential diagnoses for renal tumors and masses, but the possibility of exceptional anomalies should not be denied, and clinicians should be prepared for these occasions. Although studies propose that chemotherapy has a trivial effect on teratoid Wilms tumors, it is essential to evaluate the tumor for any possibility of regression in non-teratoid regions before proceeding to upfront tumoral resection.

Is it a teratoma? is it a nephroblastoma? no, it is a teratoma with nephroblastoma: a rare tumor in pediatric oncology

Mixed extragonadal teratoma with malignant nephroblastoma (TWN) is an exceptionally rare and complex combination of solid tumors which has been referred to as “teratoid Wilms tumor,” “teratoid nephroblastoma,” or “teratoma with nephroblastoma.” Here we report a seven-year-old male who presented with a large retroperitoneal mass outside of the right kidney with lung metastases and diagnosed with a malignant mixed germ cell tumor with mature teratoma and extensive malignant nephroblastoma. Our case report highlights the controversial pathogenesis of this rare tumor and describes the variety of treatment regimens delivered for this type of tumor. In this case report, we described a young child with metastatic TWN who was successfully treated with multimodal approach based on Wilms tumor management including surgery, chemotherapy, and radiation.

Teratoid Wilms Tumor and Classical Wilms Tumor: A Retrospective 10-Year Single-Center Study and Literature Review.

BackgroundOne of the most prevalent forms of renal tumors detected among pediatric patients is the Wilms tumor (WT). Teratoid WT is a rare WT subclassification that is characterized by teratoma-like characteristics that include the features of many diverse tissue categories. Less than 70 teratoid Wilms tumor (TWT) cases have been explained up to now.MethodsBetween 2010 and 2020, patients with classical WT and TWT admitted to our hospital were included in this study. Clinicopathological characteristics, intraoperative findings, histopathological parameters, and prognostic outcomes were then compared between classical WT and TWT. To compare these variables, TWT and WT cases were matched at a 1:3 ratio.ResultsA total of 67 total WT cases, i.e., five diagnosed with TWT, were enrolled. While no significant differences in analyzed variables were detected between these groups, tumor volumes were notably larger in the TWT group relative to the classical WT group (203.30 ± 109.89 vs. 104.30 ± 66.97 cm3) despite similar tumor weight values in both groups (471.00 ± 80.65 vs. 432.67 ± 109.25 g). As for five patients diagnosed with TWT, all were alive during the follow-up, while one of them was diagnosed with pelvic metastasis.ConclusionsThis study is the first to our knowledge to have reported on the incidence of TWT among Chinese children, and our results preliminarily suggest that a combination of surgery and chemotherapy may be appropriate for the treatment of patients with WT, although prognostic outcomes varied substantially among patients with different stages of the disease. TWT tumor density may be lower than classical WT tumor density. Further research regarding the basic biological characteristics of TWT and relevant theranostic markers associated with this tumor type is warranted to better guide the development of individualized treatments for this rare cancer type.

Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level.

Familial Wilms tumor is a rare entity that accounts for only 1-2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tumor volume. Wilms tumor does not usually secrete any specific tumor marker and all teratoid Wilms tumor cases previously reported were sporadic non-secreting neoplasms. Here we describe an infant with familial synchronous bilateral teratoid Wilms tumor whose serum alpha-fetoprotein level was elevated. To our knowledge, this extremely rare type of case is reported for the first time in the literature.

Uncommon renal tumors in children: A single center experience.

Aims:Scrutiny over the clinical behaviors, management, and the final outcome of some rare renal neoplasm in order to find out some hidden facts about these tumors which are playing an important role in the disease course and its management.Materials and Methods:Retrospective evaluation of uncommon (non-Wilms’) renal neoplasm in the pediatric population in a tertiary care center. Fifteen cases of uncommon renal tumors were treated in our institution over the last 5 years (January 2008 to December 2012). The cases were tabulated in the form of age, sex, mode of presentation, preoperative investigations, intraoperative grading, pathological type, postoperative management and the final outcome. The patients were followed up for 2 years (clinically every 3 months and ultrasonography abdomen in every 6 months for first 2 years) in order to see any evidence of recurrence and complications related to postoperative chemotherapy.Results:Out of 15 cases, four cases were clear cell sarcoma (CCS) (26.6%), three cases were rhabdoid tumor (20%), three cases were congenital mesoblastic nephroma (20%), two cases were multilocular cystic nephroma (13.3%), two cases were renal teratoma (13.3%), and one case of teratoid Wilms’ tumor (6.6%). There were two deaths (one CCS and one rhabdoid tumor) due to chemotherapy-related toxicity but no recurrence. Three patients were lost during postoperative follow-up; ten patients are doing well and getting a regular visit in the follow-up clinic.Conclusion:The clinical presentations of these uncommon renal tumors are similar to that of Wilms’ tumor. Thus, preoperative diagnosis is difficult even with modern imaging techniques. Some of these tumors (CCS, rhabdoid tumor) are rapidly progressing and have a poor outcome. Hence, early intervention in the form of complete surgical resection of the tumor (whenever possible) and postoperative chemo/radiotherapy are imperative for fruitful outcome.

Teratoid Wilms tumour with chemotherapy resistance

We present a case of Teratoid Wilms tumour (a rare histologic variant) in a 4 year old male who presented with an abdominal lump. Wilms Tumour with paracaval lymphadenopathy and tumour thrombi in right renal vein and inferior vena cava was made radiologically. FNAC report was suggestive of Wilms tumour and patient was subjected to 6 cycles of chemotherapy with not much reduction in size. Post nephrectomy histological diagnosis of Teratoid Wilms tumour was established. Resistance to chemotherapy and radiotherapy is thought to be due to presence of well differentiated histologic appearance. Teratoid Wilms tumour is usually not an aggressive neoplasm and prognosis is comparatively neoplasm and prognosis is comparatively good if the tumour is excised completely thus surgery being the best treatment.

Adult teratoid Wilms’ tumor: A case report of rare variant with predominant neuroepithelium

Teratoid Wilms' tumor (WT) is a rare variant of nephroblastoma which had been only reported in pediatric patients. Herein, we report a 32-years-old female patient with teratoid WT with predominant heterologous neuroepithelial component of the right kidney. Histologically, the tumor showed the typical histological features of a teratoid WT formed of triphasic pattern of blastema, epithelium and stroma with heterologous elements which comprised more than 50% of the tumor area. The heterologous elements were mainly composed of neuroepithelial tissue including ganglion cells, differentiating neuroblasts, true and pseudorosettes, ganglioneuromatous differentiation, schwannianstroma, abundant neuropils admixed with mature types of epithelium and rhabdomyoblasts. This was the second reported case of adult teratoid WT with extensive neuroepithelial differentiation.

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.

Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level

Familial Wilms tumor is a rare entity that accounts for only 1–2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tumor volume. Wilms tumor does not usually secrete any specific tumor marker and all teratoid Wilms tumor cases previously reported were sporadic non-secreting neoplasms. Here we describe an infant with familial synchronous bilateral teratoid Wilms tumor whose serum alpha-fetoprotein level was elevated. To our knowledge, this extremely rare type of case is reported for the first time in the literature.

Extrarenal Teratoid Wilms’ Tumor in Association with Horseshoe Kidney

Extrarenal teratoid Wilms' tumor is a rare variant of Wilms' tumor. Literature shows only 17 reported cases. We present this right-sided teratoid Wilms' tumor in a 3-year-old boy for its rarity in association with the horseshoe kidney. The tumor was found in the anterior aspect of the right kidney and consisted of triphasic Wilms' tumor element along with the presence of heterologous components.

Squamous Predominant Teratoid Wilms’ Tumor

Teratoid Wilms tumor is an unusual histological variant of nephroblastoma with predominant heterologous component. Frequently present components include adipose tissue, glial tissue, muscle, cartilage or bone. The presence of squamous epithelial component on the other hand is rarely reported. We describe a case of unilateral teratoid Wilms’ tumor in a 2-year-old boy with lung metastasis. In this case, tumor showed the familiar triphasic histologic pattern of nephroblastoma along with extensive squamous epithelial component.

A Case of Retroperitoneal Immature Teratoma With Nephroblastic Components

The presence of nephroblastic components is extremely rare in retroperitoneal teratomas. We described an unusual case of right retroperitoneal immature teratoma with nephroblastic components in a 2-month-old Japanese girl. Approximately 15% of tumor was composed of nephroblastic components within immature teratoma components. Although overlap of histopathological findings between teratomas with nephroblastic components and teratoid Wilms tumors, which have heterologous elements in >50% of the tumor, occasionally exist, the most distinct feature of teratoma is the presence of organoid arrangement. The prognosis of retroperitoneal teratomas with nephroblastic components has not yet been established, therefore, additional case studies are needed.

Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy

Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Fewer than 30 cases have been described. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. The patient continued to survive despite cessation of treatment. The overall predominance of differentiated stromal elements in this subtype of Wilms' tumor might explain the poor response to chemotherapy yet generally favorable outcome. Recognition of this subtype on biopsy might justify earlier surgical intervention that, for bilateral tumors, might allow for greater nephron sparing.

Teratoid Wilms′ tumor - A rare renal tumor

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.

Distinct features of teratoid Wilms tumor

Teratoid Wilms tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. Typically, histopathologic examination shows a mixture of mature squamous and mucus-producing columnar epithelium and a prominent component of mature adipose tissue. The treatment of this tumor has not been established because of its rarity with some reports doubting the value of chemotherapy. We report 3 new cases and review the 23 previously reported cases that we were able to identify. The first case was a 2-year-old boy with a huge tumor that occupied most of the abdominal cavity. The second case was a 5-year-old girl with a metastatic tumor in association with horseshoe kidney and is the first case of teratoid Wilms tumor with brain metastasis. The third case was an 11-month-old girl with bilateral involvement. Imaging studies of our patients were similar to those reported in the literature with areas of fat attenuation and calcification. Failure to respond to preoperative chemotherapy was seen in our patients and in most previously reported cases that were treated with preoperative chemotherapy. We recommend treating this entity with protocols designed for Wilms tumor and to conduct further basic research to understand its biologic nature.

Extrarenal teratoid Wilms' tumor: Two cases in unusual locations, one associated with elevated serum AFP

Teratoid Wilms' tumor is an unusual morphological entity characterized by a classic triphasic malignancy with predominantly heterologous tissue. The authors describe two cases of teratoid Wilms' tumor with an extrarenal site: one in a 13-year-old girl with vaginal spotting (patient 1) and another in a 1-day-old girl with a sacrococcygeal mass (patient 2). The tumors were located in the vagina and coccyx, respectively. Under the initial clinical diagnosis of sarcoma botryoides in patient 1 and teratoma in patient 2, the masses were removed. Microscopically, both tumors were composed of typical triphasic Wilms' tumor tissue with primitive cartilage and skeletal muscle, and squamous and columnar mucinous epithelia. The patient with sacrococcygeal mass (patient 2) had an elevated serum AFP level. The patients were given chemotherapy and have now remained disease free for 7 years 1 month, and 2 years 5 months after surgery, respectively. Familiarity with this rare variant of Wilms' tumor might be important in arriving at a correct diagnosis.

Teratoid Wilms’ Tumor with Hypertension Treated with Partial Nephrectomy: Case Report with Literature Review

Teratoid Wilms’ Tumor with Hypertension Treated with Partial Nephrectomy: Case Report with Literature Review

Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation

Teratoid Wilms' tumor is a rare variant of Wilms' tumor (WT) that has been reported exclusively in pediatric patients. The present paper describes a teratoid WT in a 50-year-old Korean man with a giant right renal mass. Radical nephrectomy was performed under the impression that the mass was a renal cell carcinoma. Grossly, the removed kidney contained a giant well-encapsulated mass measuring 24 x 18 x 10 cm with cystic changes, necrosis, and hemorrhage. On microscopy triphasic patterns of WT and prominent heterologous components were seen, including rhabdomyoblasts, neuroepithelial tissue, fat, cartilage, and various types of mature epithelium. This is the first reported case of a teratoid WT occurring in an adult kidney.

Adult extrarenal Wilms tumor of the uterus with teratoid features

The present article reports for the first time a case of an extrarenal teratoid Wilms tumor in the uterus of a 62-year-old woman. It had triphasic histology with epithelial areas composed of metanephric tubules harboring glomerular structures, adamantine patterns, neural type rosettes, blastema, and a primitive, myxoid type stroma. Abundant heterologous elements such as cartilage, striated muscle, squamous epithelium, and an alpha fetoprotein and TTF1-positive early endodermal epithelium were also present. Immunohistochemistry in Wilms tumor areas showed positivity for markers also indicative of peripheral primitive neuroectodermal tumors such as neuron-specific enolase, CD99, and CD56. However, nuclear positivity for Wilms tumor antigen together with the presence of glomeruli and the absence of endometrioid tumor areas and the organoid arrangement of tissues excluded peripheral primitive neuroectodermal tumors, carcinosarcoma, and teratoma, respectively. Although the diagnosis of female genital tract Wilms tumors is difficult in cases where glomerular structures are lacking, it should be considered because these neoplasms have a better therapeutic response than peripheral primitive neuroectodermal tumors and carcinosarcoma.

Teratoid Wilms’ tumor in a child

Teratoid Wilms’ tumor in a child