|
Congenital mesoblastic nephroma: a clinicopathologic study of five
cases.
Med
Wieku Rozwoj. 2006 Jul-Sep;10(3 Pt 1):677-86.
Congenital
mesoblastic nephroma (CMN) accounts for approximately 5% of paediatric
renal tumours with the highest peak of incidence during the first 3
postnatal months. CMN almost always has a favourable prognosis.
Therefore, CMN needs to be correctly diagnosed and differentiated,
principally histologically, from other pediatric neoplasms. We present
a clinicopathologic study of 5 cases of CMN.
Atypical
congenital mesoblastic nephroma presenting in the perinatal period.
Pediatr Surg Int. 2007
Mar;23(3):205-9. Epub 2006 Nov 9.
Congenital
mesoblastic nephroma (CMN) is a rare tumour of infancy having an
overall good prognosis. The less common, atypical CMNs have cellular
elements in them and tend to have an unpredictable course. Occurrence
in the perinatal period may further change the outcome. By reporting
three patients presenting in the perinatal period with atypical CMN,
an attempt is made in this paper to characterize the clinical
behaviour of these variant tumours. Though one of our patients had an
uneventful course, the other two had several complications including
polyhydramnios, prematurity, hypertension, haemodynamic instability
and tumour spillage. The course was complicated by recurrence in the
latter two and refractoriness to chemotherapy and death in one. That
the atypical subset of CMNs occurring in the perinatal period can have
a stormy course is well illustrated by this report. Possible
prognostic factors are evaluated and the sparse reports of similar
cases in the literature are reviewed and compared.
Congenital
mesoblastic nephroma presenting with massive hematuria and hemorrhagic
shock: report of one case.Acta
Paediatr Taiwan. 2006 May-Jun;47(3):135-8.
Congenital mesoblastic nephroma (CMN) is a rare benign tumor that
occurs during the neonatal period and early infancy. The vast majority
of these tumors present as asymptomatic palpable abdominal masses. We
describe an unusual presentation of a CMN in a 10-month-old male
infant who presented with massive hematuria and the development of
hemorrhagic shock. Abdominal ultrasound showed a heterogeneous solid
complex mass measuring 4.8 x 3.5 cm arising from the upper pole of the
left kidney. The patient was resuscitated using intravenous fluids and
blood transfusions because persistent massive bloody urine leading to
progressive shock occurred the night of the admission day.
Preoperative diagnosis was possible Wilms tumor of the left kidney.
The histopathological findings were consistent with the character of a
cellular variant of CMN. The patient was free of recurrence and
metastasis at the 2-year follow-up examination. Our case report
suggests that CMN is a rare benign renal tumor during infancy and may
present with unusual massive hematuria and shock.
Expression of
ETV6-NTRK in classical, cellular and mixed subtypes of congenital
mesoblastic nephroma.Histopathology.
2006 May;48(6):748-53.
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.
Prenatal diagnosis of congenital mesoblastic nephroma. A case report.
Arch Pediatr. 2005 May;12(5):561-3.
Antenatal
ultrasounds allow the detection of renal tumors, especially renal
mesoblastic nephromas, but only the pathological analysis of the
surgical specimen can confirm this diagnosis postnatally. OBSERVATION:
We report the prenatal discovery of a mesoblastic nephroma because of
premature labour. Postnatal early surgery was decided because of
possible complications in this premature infant. Histology revealed
mesoblastic nephroma. COMMENTS: We point out the diagnostic elements
of congenital mesoblastic nephroma, especially in what is related to
arterial hypertension and hypercalcemia, histology and cytogenetics.
Multicystic
congenital mesoblastic nephroma.Int
J Surg Pathol. 2002 Jan;10(1):59-63.
This report
describes an unusual example of congenital mesoblastic nephroma
cellular variant that presented in a 1-week-old neonate as a
multicystic tumor of the kidney. Extensive pseudocystic cavitation
resulted from progressive accumulation of ground substance in a
loosely myxoid tissue composed of stellate- and spindle-shaped cells
that compressed and infiltrated renal tissue. The cells of the tumor
were positive for vimentin and smooth muscle actin. The patient is
alive and well 16 years after surgery. Differential diagnosis from
segmental cystic dysplasia, cystic intralobar nephrogenic rest, cystic
nephroma, cystic partially differentiated nephroblastoma, cystic
nephroblastoma, and cystic clear cell sarcoma of the kidney, all of
which may present at this age, is discussed.
|
/K-14new/K-14A.jpg){kind=link}
/K-14new/K-14B.jpg){kind=link}
/K-14new/K-14C.jpg){kind=link}