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Mesoblastic nephroma is a benign mesenchymal tumor of the kidney generally diagnosed during the first 3 months of life. A case of the cellular variant of mesoblastic nephroma associated with prominent cystic change and renal vein invasion arising in an 8-month-old female is described. This unusual case expands the morphologic spectrum of mesoblastic nephroma.
Mesoblastic nephroma is a rare renal tumour mainly observed in neonates and young infants. The authors report a case in a 5-day-old new-born infant presenting with an isolated abdominal mass occupying the left hypochondrium and flank. Ultrasonography demonstrated a 4 cm heterogeneous mass in the lower pole and middle of the left kidney. CT showed an intensely contrast-enhanced heterogeneous mass. The patient was treated by left radical nephrectomy with an uneventful postoperative course. The outcome was favourable without recurrence with a follow-up of one year. Mesoblastic nephroma is considered to be a benign tumour. The diagnosis is suggested by clinical, ultrasonographic and CT findings. Treatment is based on radical nephrectomy. In the light of this case, the authors discuss the clinical, radiological and therapeutic aspects of this tumour.
Mesoblastic nephroma is the most common solid renal neoplasm in the first few months of life. It has been recognized only recently as a distinct entity, having been classified previously as Wilms tumor. There are relatively few ultrasonic descriptions of this entity in the literature. We present an additional case featuring a large peripheral cystic component with a solid central core demonstrated on ultrasonograhy. This feature is not specific for mesoblastic nephroma and occurs in cystic Wilms tumor.
Mesoblastic nephroma (MN) is the most common renal tumor diagnosed in infancy. Histologically, MNs are designated as classic, cellular, or mixed type based upon variations in cellularity. Recent karyotypic reports have suggested that extra copies of chromosome 11 are a nonrandom occurrence in MNs. We analyzed nuclear suspensions prepared from a group of 17 formalin-fixed, paraffin-embedded tumors to determine the possible role of chromosome 11 copy number in the genesis of MN. Extra copies of D11Z1 (a probe for the centromeric region of chromosome 11) were detected in seven out of 10 MNs with cellular or mixed histology, whereas each of six classic histology MNs were disomic for D11Z1 (P < 0.05). Additional fluorescence in situ hybridization studies utilizing the probes for the alpha satellite, centromeric regions of chromosomes 7, 8, 9, 12, 17, and 20 were then carried out on all cases with cellular or mixed histology. Five out of 10 cellular or mixed MNs had extra copies of D8Z1, and four out of 10 had extra copies of D17Z1, suggesting that gains of chromosomes 8 and 17 may be additional nonrandom cytogenetic events associated with the evolution of MNs. DNA aneuploidy, as determined by image analysis, was detected in three tumors: all had greater than four chromosomal aberrations documented by fluorescence in situ hybridization.
Atypical Mesoblastic Nephroma (AMN) is a rare infantile renal tumour which may behave aggressively, in contrast to conventional Mesoblastic nephroma. During a 10 year period we encountered four cases of AMN. The mean age of presentation was three months. Size varied from 9-18 cms. All were soft and fleshy with a weight varying from 290-1200 gms. Three cases revealed cystic changes. Hemorrhage and necrosis was seen in all four cases. One case had associated Nephroblastomatosis Complex. The capsule and ureter were involved in one case each. Two of the four patients died of distant metastasis. Thus a close periodic follow up by non-invasive imaging techniques is indicated for early detection of recurrence and or metastasis in cases of AMN.
The mesoblastic nephroma is the most common renal tumor among newborn and young infants. The tumor is benign, and metastatic growth has only been described in individual cases. Three patients suffering from histologically proven mesoblastic nephroma were examined with an ultrasonic device. Sonographically, the mesoblastic nephroma is manifest as solid and homogeneous tumor of a fine to medium coarse basic texture and of a medium echogenity which may show individual small cystic areas. The tumor cannot be delimited sharply from the healthy renal tissue although uneventful tissue which often caps the kidney is proved. A secure differential diagnosis against a Wilms' tumor is not possible if metastases cannot be detected sonographically or by means of an excretory urogram. The echopattern and the relation of the mesoblastic nephroma to the kidney in combination with an excretory urogram should, however, make the delimitation of a neuroblastoma possible.
Congenital mesoblastic nephroma is a relatively rare infantile renal tumor. It comprises 3-6% of renal masses in childhood and 50% during the neonatal period. Most mesoblastic nephroma occur in the newborn period, with 80% of the cases being reported within the first month of life. Macroscopically the tumor is composed of a solid mass of different sizes tending to invade the surrounding structures and renal parenchyma. The authors report a case of cystic mesoblastic nephroma of the cellular subtype, with diffuse areas of hemorrhage and necrosis. The tumor was treated by surgical excision with radical nephrectomy and the child is doing well 4 years after the operation.
A case of an aggressive variant of conventional congenital mesoblastic nephroma (CMN) occurred in a 10-month-old male infant. The tumor proved to be fatal, with two abdominal recurrences, but no metastases were found. Of the various terms used to designate the tumor, atypical mesoblastic nephroma (AMN) is preferred. We reviewed 17 previously reported cases to attempt pathologic characterization of AMN. The features that distinguish AMN from CMN are as follows: (1) atypical gross features consisting of one or more of the following: fleshy areas, foci of hemorrhage, necrosis, involvement of adjacent structures other than connective tissue; and (2) high cellularity and mitotic index. Aggressive behavior was noted at surgery or on follow-up in seven of the 18 cases. It was characterized by one or more of the following: (1) invasion of adjacent structures, such as adrenal gland, spleen, colon, and diaphragm (three cases); (2) one or more recurrences (four cases); and (3) metastasis (two cases). Atypical mesoblastic nephroma should be recognized as a potentially aggressive lesion separate from CMN.
Congenital mesoblastic nephroma (CMN) is a benign, but locally aggressive, renal tumor of early infancy. Few metastases have been reported, but local recurrence is well documented. CMN is histologically distinct from Wilms' tumor and other childhood renal tumors, and is typically treated by surgical excision without adjuvant therapy. Cytogenetic abnormalities in these tumors have been described and are often compared with abnormalities seen in leiomyomas, fibromatoses, and infantile fibrosarcomas. In particular, trisomy 11 has been suggested as a nonrandom occurrence in CMNs. We describe a case of CMN in a 4-month-old female infant. The diagnostic histologic features included a monophasic mesenchymal appearance, prominent staghorn vascular spaces, and extensive infiltration of the adjacent kidney. Cytogenetic analysis showed a hyperdiploid chromosome number and a single structural abnormality involving a translocation between chromosomes 12 and 15. The composite karyotypic interpretation was: 46-47,XX,-X, +8, +11,t(12;15)(p12;q25), +17, +20[cp14]. The significance of karyotypic changes in this tumor is currently unknown. A genetic basis for histologic variability and progression may exist if certain cytogenetic abnormalities, such as trisomy 11 or specific translocations, confer a proliferative advantage. Additional cases are required to correlate cytogenetic findings with the biologic behaviors of CMN. We present this case as a contribution to existing literature describing these relatively rare and interesting renal tumors.
Congenital mesoblastic nephroma is a relatively rare tumor predominantly of childhood. Occurrence in adults is exceedingly rare and, to my knowledge, only two cases have been reported to date. This article pertains to a mesoblastic nephroma in a 41-year-old woman. The tumor was composed mainly of compact fibrocollagenous elements interspersed with areas containing immature tubules and occasionally glomeruloid structures. There was no evidence of capsular or renal invasion or cytological malignant features. It has been postulated that this neoplasm may represent a form of mature Wilms' tumor with a benign clinical course.
Congenital mesoblastic nephromas are infiltrating tumors that can contain cystic spaces lined with epithelium similar to that of dysplastic tubules and glomeruli. Urographic contrast material may enter these cystic spaces and suggest the diagnosis.
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AIM: Congenital mesoblastic nephroma (CMN) is the commonest renal tumour of infancy, with classical, cellular and mixed histological subtypes described. A specific ETV6-NTRK3 fusion-gene product is reported in association with the cellular variant. The aim was to investigate the relationship between the presence of this product and morphological phenotype using paraffin-embedded archival material. METHODS AND RESULTS: Cases of CMN from a single centre during a 15-year period (1989-1994) were identified, anonymized and blindly classified using morphological criteria. RNA was extracted from frozen and paraffin sections for both conventional reverse transcriptase-polymerase chain reaction (RT-PCR) and quantitative real-time RT-PCR. Fifteen samples were analysed; two were non-informative and three expressed ETV6-NTRK3 using both techniques, two showing similar expression, whilst one showed expression two orders of magnitude lower, from a cellular tumour. All fusion positive cases were previously classified as cellular subtype. Six patients had mixed-subtype tumours in which the cellular components, morphologically indistinguishable from cellular tumours, were fusion negative, as were all classical cases. CONCLUSIONS: Real-time PCR Taqman assays, using both fixed and frozen tissue, provide highly reproducible detection and quantification of fusion transcript expression. Differences in expression levels may explain previous conflicting data on fusion gene detection in these tumours.
Four children with congenital mesoblastic nephroma were treated at the Children's University Hospital in Belgrade, between 1979 and 1990. In relation to the total number of children cured from renal tumours (44), diagnosis of congenital mesoblastic nephroma was confirmed in 9% of all cases with renal tumours. The age of all diagnosed patients was under one year. Three patients developed unilateral and one patient bilateral congenital mesoblastic nephroma. The authors also stress the importance of differential diagnosis between congenital mesoblastic nephroma and other renal tumours, since therapy and prognosis of these tumours are different. In a case of bilateral congenital mesoblastic nephroma that later relapsed with malignant alteration, it is questionable whether bilateral nephrectomy was the most appropriate treatment.
Mesoblastic nephroma is a rare tumor of kidney characterized by interlacing bundles of spindle mesenchymal cells. The tumor usually occurs in the newborn, and scarcely in the old child. In this article, the authors report a case of an unusual age, a 12-year-old boy, with mesoblastic nephroma which was found by routine physical examination.
Mesoblastic nephroma is a rare, benign congenital disease with a clinical presentation similar to that of Wilms tumour. It was described for the first time by BOLANDE in 1967. The authors report the case of a 6-month-old infant admitted for investigation of an abdominal mass. The radiological assessment (ultrasonography. CT) concluded on a right intrarenal retroperitoneal tumour occupying almost all of the abdominal cavity, predominantly cystic suggesting a diagnosis of non-metastatic Wilms tumour. Radical ureteronephrectomy was performed. The outcome was favourable with a follow-up of 2 years. Histological examination corrected the diagnosis to mesoblastic nephroma by showing a fibrous tumour composed of spindle cells resembling muscle cells, associated with intracystic haemorrhage. The authors emphasize the clinical and radiological features of this tumour, its treatment (exclusively surgical) and its good prognosis (98% survival).
Thirty eight mesoblastic nephromas were studied. The age range of the patients was between the neonatal period and 18 months. The presence of cartilage is consistent with a mesoblastic origin, but squamous epithelium was a feature in three tumours. Particular attention was given to the adjacent renal tissue in which various histological features were noted: vacuolated and dysplastic tubules; cysts; and subcapsular epithelial tumourlets. The findings had aspects in common with both dysplastic kidneys and nephroblastoma. Classification of the tumours as normocellular and hypercellular was attempted, but there was considerable overlap. The behaviour of the tumour was good in all cases, although follow up was relatively short on some patients, and deaths from non-neoplastic causes occurred.
Congenital mesoblastic nephroma (CMN) is usually cured by surgery. The sensitivity of this tumor to chemotherapy is unknown. The recent description of a t(12;15)(p13;q25) chromosomal translocation in both cellular CMN and congenital infantile fibrosarcoma suggests that these entities have a common pathogenesis, and that cellular CMN might respond to chemotherapy like congenital infantile fibrosarcoma does. The authors describe three patients with recurrent cellular CMN who showed a complete response to chemotherapy. Based on these patients and a review of the literature, the authors suggest that chemotherapy be considered as a part of the therapy for recurrent or unresectable cellular CMN.