Congenital mesoblastic nephroma cmn is a common solid renal tumor in the neonate. Sonograms show mesoblastic nephroma as a complex mass that may c. These cells have the features of secondary mesenchyme, which, in contrast with those of primary mesenchyme mesoblast, lack the capacity to form epithelial structures. Mesoblastic nephroma is the most common congenital renal neoplasm. How is mesoblastic nephroma differentiated from wilms tumor on. The monarch initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.
Chemotherapy risk, morbidity, and mortality should be considered at an early age. Ucsf congenital mesoblastic nephroma clinical trials san. Mesoblastic nephroma is a benign hamartoma of mesenchymal connective tissue. Specific computed tomography imaging characteristics of. Two top images are coronal reconstructions from a contrastenhanced ct urogram demonstrating a cystic mass in the upper pole white arrows of the left kidney that is septated and displacing the normal collecting system. Congenital mesoblastic nephroma cmn is the most frequent renal neoplasm of newborns and young infants, accounting for 5% of all renal tumors. Congenital mesoblastic nephroma with distant metastasis in a. This study aimed to assess surgical complications and. Renal tumors are infrequently diagnosed in infants younger than 6 months of age 1,2, and neonatal renal tumors account for only 7% of all neonatal tumors 1,3,4. While cmn is more common than wilms tumor, it is difficult to distinguish cmn from wilms tumor using ultrasonography usg 1,6,7,8. Congenital mesoblastic nephroma classic congenital renal tumors comprise 2. Mesoblastic nephroma hector gonzalo quiroga pacheco, md. This tumor was first described as a separate entity by bolande et al in 1967. Jul 21, 2009 there are a variety of imaging findings for congenital mesoblastic nephroma cmn and two main pathological variants.
In our case, the imaging features could not help with the differentiating between the wilms tumor and mesoblastic nephro. Together, us and ct may be the studies of choice because they enable the evaluation of cystic lesions, stromal tissue, and the perfusion of this stroma. How is clear cell sarcoma differentiated from wilms tumor on imaging. This program was designed to help urology residents and fellows familiarize. Renal function within two typical congenital mesoblastic nephromas was demonstrated by computed tomography in one case and renal scintigraphy in the other. Congenital mesoblastic nephroma definition of congenital. Multilocular cystic nephroma mlcn is a benign mixed mesenchymal and epithelial neoplasm of the kidney. Prenatal diagnosis of congenital mesoblastic nephroma. Large 5 10 cm, unilateral and solitary multiloculated tumor composed of multiple noncommunicating thin walled cysts of varying size that are filled with clear fluid. Mesoblastic nephroma an overview sciencedirect topics. Scintigraphic and sonographic diagnosis of neonatal. Infantile fibrosarcoma and congenital mesoblastic nephroma are tumors of infancy traditionally associated with the etv6ntrk3 gene fusion. There are a variety of imaging findings for congenital mesoblastic nephroma cmn and two main pathological variants. Sharply demarcated from adjacent kidney by thick fibrous capsule.
May be more cellular than the classical form of mesoblastic nephroma image c. Congenital mesoblastic nephroma can be divided into classic, cellular, and mixed types. Jun 27, 2019 however, imaging results cannot be used to definitively differentiate a mesoblastic nephroma from a congenital wilms tumor. Unilateral extensive sampling of margin should be performed classic. It contains fibroblastic cells connective tissue cells, and may spread to the other kidney or to nearby tissue. It is the most common solid renal mass in the neonate wilms tumor is unusual in the neonate.
Sep 01, 2014 congenital mesoblastic nephroma cmn is a mesenchymal renal tumor. Nephroma, mesoblastic nephroma, mesoblastic is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings. Mesoblastic nephroma, also sometimes known as a congenital mesoblastic nephroma cmn or fetal renal hamartoma, is, in general, a benign renal tumor that typically occurs in utero or in infancy. Congenital mesoblastic nephroma cmn is a renal stromal neoplasm of infancy. There are several kinds of nephroma, which fit into two main types. The renal cortex is the outer portion of the kidney that filters the blood and removes waste inside the body. Mesoblastic nephroma and wilms tumor look very similar on imaging as both present as large solid renal masses. We report a case of cmn in a 30 week old premature female neonate seen at autopsy who was born to a 26yearold woman by emergency cesarean section on account of polyhydramnios. Congenital mesoblastic nephroma is the only primary renal neoplasm with radiographically demonstrable function.
Ultrasonography is widely available and routinely performed antenatally. Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first three months of life and accounts for 35% of all childhood renal neoplasms. Aug 20, 2019 congenital mesoblastic nephroma clinical trials at ucsf. Imaging findings correlated with gross and histological appearance. Ultrasound demonstrates a large mixed echogenic intrarenal mass.
Congenital mesoblastic nephroma cmn is a rare renal tumor of infancy. Oct 29, 2015 us can provide the imaging results necessary for diagnosing multilocular cystic nephroma. Congenital mesoblastic nephroma cmn is an infantile spindle cell tumor of the kidney that is subdivided into classical and cellular forms based on the degree of cellularity and mitotic activity. The icd10cm alphabetical index is designed to allow medical coders to look up various medical terms and connect them with the appropriate icd codes. Nephroma, mesoblastic harvard catalyst profiles harvard. Histologic examination is the only definitive test. Prior to this, it was erroneously confused with congenital wilms tumor singh sp. Mesoblastic nephroma radiology reference article radiopaedia. Imaging of congenital mesoblastic nephroma with pathological. To determine whether imaging findings in children can predict the likely pathological variant. Mesoblastic nephroma also called fetal renal hamartoma is the most common renal tumor identified in the neonatal period and the most. However, imaging results cannot be used to definitively differentiate a mesoblastic nephroma from a congenital wilms tumor.
Mesoblastic nephroma definition of mesoblastic nephroma by. Mesoblastic nephroma, also sometimes known as a congenital mesoblastic nephroma cmn or fetal renal hamartoma, is, in general, a benign renal tumour that typically occurs in utero or in infancy. Congenital mesoblastic nephroma american urological association. Us can provide the imaging results necessary for diagnosing multilocular cystic. Mesoblastic nephroma is classified into classic and cellular types on basis of microscopic appearance. Mesoblastic nephroma jose luis duque, md mesoblastic nephroma andreas rempen, md,thomas kirchner, md,georg frauendienstegger, md,burkhard hocht, md. Medscape reference provides information on this topic. The mean age of presentation is 3 months with a palpable abdominal mass.
The classic type consists of fascicles and whorls of spindly cells fig. There are 1 terms under the parent term nephroma in the icd10cm alphabetical index. This case first appeared as performance improvement program in surgical pathology pip. Affiliation from the childrens tumour registry, department of epidemiology and social research, christie hospital and holt radium institute, manchester. Congenital mesoblastic nephrom or simply mesoblastic nephroma represents 310% of all pediatric renal tumors. Pathology outlines congenital mesoblastic nephroma. A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour mest and renal epithelial stromal tumour rest, is a type of rare benign kidney tumour contents 1 symptoms. Congenital mesoblastic nephroma cmn is a mesenchymal renal tumor. But cmn can easily be misdiagnosed as the other malignant renal tumors by radiology. You may need to register to view the medical textbook, but registration is free.
In our case, the imaging features could not help with the differentiating between the wilms tumor and mesoblastic nephroma, but because of the patient age wilms tumor was less likely. Excretion of contrast medium and radionuclide was due to functioning nephrons trapped within the tumor. Abstractmesoblastic nephroma mn presenting in an adult is extremely rare. Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking. Regression of a congenital mesoblastic nephroma request pdf. Hector gonzalo quiroga pacheco, md centro medico profesional rosancar. Diagnosis is usually made in the antenatal period or immediately after birth. Congenital mesoblastic nephroma cmn is a rare renal tumor that is frequently associated with polyhydramnios, premature birth, and neonatal hypertension.
Both are treated with surgical excision and the final distinction is made with pathology. Mesoblastic nephroma also called fetal renal hamartoma is the most common renal tumor identified in the neonatal period and the most frequent benign renal tumor in childhood. Mesoblastic nephroma occurs in the neonate whereas wilms tumor is very rare in the newborn. Ultrasonography us is the first radiologic examination performed for the evaluation of any abdominal mass. Conclusion cellular mesoblastic nephroma is a rare but aggressive congenital renal tumor. Learningradiology cystic, nephroma, nephroblastoma, renal. In 1967, bolande and colleagues were the first to describe the tumor as a separate entity from wt. Mesoblastic nephroma mn is a rare tumour which occurs mainly in early infancy and for which primary nephrectomy is the treatment of choice. Congenital mesoblastic nephroma genetic and rare diseases. An infant with cellular congenital mesoblastic nephroma, regardless of age, is cured with surgery alone given clear. Mesoblastic nephroma information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. The diagnosis may be confirmed by using either ct or mri.
This is a case of a pathologyconfirmed congenital mesoblastic nephroma of mixed subtype. Congenital mesoblastic nephroma in a premature neonate. Computed tomography image of large right renal mass prior to surgical resection. Most of the fetal kidney diseases are cystic and obstructive lesions 1, 2. Mlcn is classically described as a benign slowgrowing renal tumor, and there are more than 200 cases reported in the literature. Right kidney doppler image slight increase in vascular formation. Congenital mesoblastic nephroma typically 76% of cases presents as an abdominal mass which is detected prenatally 16% of cases by ultrasound or by clinical inspection 84% of cases either at birth or by 3.