Cystic atypical mesoblastic nephroma

 Cystic variants of atypical mesoblastic nephromas are very rare. The present communication deals with two such cases encountered in 3- and 6-month-old patients. The literature is briefly reviewed. The need for proper diagnosis of this tumor to distinguish it from cystic nephroma and Wilms' tumor is highlighted. Accepted: 27 November 2000     

Cystic nephroma: a rare benign renal tumor

We report two boys aged 1 year and 2 years 2 months, respectively, with cystic nephromas. Both presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the lower pole of the kidney in both cases with thin septa without solid parts. Macroscopically, the surface of the tumor was smooth. Both patients underwent a renal-sparing procedure; histology confirmed the diagnosis of cystic nephroma. Accepted: 2 April 1997     

Mesoblastic nephroma of infancy

This report presents features of mesoblastic nephroma, a rare benign tumor of kidney observed in perinatal period of life and highlights the role of imaging investigations in the proper management of this tumor. Awareness of this tumor may facilitate prevention and management of severe obstetric and neonatal complications and inadvertent and vigorous therapy compatible with that of Wilms’ tumor can be avoided.     

Unusual morphology in mesoblastic nephroma

Congenital mesoblastic nephroma (CMN) is a rare paediatric renal neoplasm. It is a diagnostic challenge to the pathologists due to its close differentials having ominous prognosis. We present three cases of CMN with unusual morphology including evidence of renal dysplasia in one of them.     

Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma Registry

OBJECTIVE: To characterize the association of pleuropulmonary blastoma (PPB) with cystic nephroma (CN) and other renal tumors. STUDY DESIGN: Complete clinicopathologic review of cases from the International PPB Registry and literature. RESULTS: We identified 18 patients with PPB associated with 20 renal tumors (15 CN), either in themselves or family members. All patients with PPB were <5 years of age. All but one of the renal diagnoses were made before 4 years of age. Eleven children had both PPB and renal tumor, one of whom also had a sibling with CN. Six children with PPB alone had one or more family members with CN. The mother of one child with PPB had Wilms' tumor. Pulmonary disease was bilateral in four patients. Renal disease was bilateral in three patients. Two children with PPB and bilateral renal cystic tumors also had intussusceptions because of small bowel juvenile polyps. In six families, dysplasia/neoplasia affected organs other than lung and kidney. CONCLUSIONS: CN or related tumors were found in 9.2% of 152 Registry-reviewed PPB cases. The occurrence of rare pulmonary and renal tumors together in patients and/or family members, the early age of onset, and the multiplicity of tumors is compatible with a constitutional genetic predisposition.     

Congenital mesoblastic nephroma: treatment options

Congenital mesoblastic nephroma (CMN) is an uncommon tumour in the neonatal period. Many CMNs are of typical histology and require no therapy other than nephrectomy. However, some are of atypical histology and are known to recur and metastasize. The use of chemotherapy in the atypical variant and in case of recurrence has been a matter of controversy. The presence of tumour at the excision margin seems to be the only important predictor of recurrence. The role of heminephrectomy for localised lesions has not been defined. Five cases of CMN seen at the Royal Children's Hospital, Melbourne, are discussed. Two of them were atypical histologically. One child had a heminephrectomy and is among the first to have had this procedure for CMN. Current treatment options are reviewed in relation to existing knowledge of the pathology and natural history of CMN. Correspondence to: J. M. Hutson     

The multilocular cystic nephroma in childhood. A case report]

A recently observed case of multilocular cyst of the kidney is reported. The differential diagnosis between Wilm's tumor and multilocular cyst of the kidney is pointed out.     

A mesoblastic nephroma with hypercalcaemia

Abstract A 14 week old female infant who presented with a mesoblastic nephroma was found to be hypercalcaemic. This was corrected prior to removal of the tumour and serum calcium concentrations remained within the normal range postoperatively. Hypercalcaemia is a life threatening complication of mesoblastic nephromas and should be investigated in all cases.     

Preoperative chemotherapy for mesoblastic nephroma

A neonate with extensive stage III mesoblastic nephroma was confirmed at operation to have a tumor that was too infiltrative and too advanced for primary excision. The abdomen was closed after multiple biopsies. Vincristine (1.1 mg/m²) was given intravenously once a week for a total of eight doses. Repeat CT scan confirmed shrinkage of the tumor and a nephrectomy could be performed safely and the tumor removed at the second laparotomy. CT scan repeated 1 year later showed no recurrence. This case illustrates the effective use of a single drug pre-excision chemotherapy which allowed a nephrectomy for extensive stage III mesoblastic nephroma to be carried out safely. © 1995 Wi1ey-Liss Inc.     

Regression of a congenital mesoblastic nephroma

Histologically, the cellular variant of congenital mesoblastic nephroma (CMN) is very similar to another rare tumor of infancy, infantile fibrosarcoma (IFS). In addition to the histologic similarities, these tumor types share cytogenetic abnormalities including translocation t(12;15)(p13;q25). We describe herein the case of a child who did not have immediate surgical resection of a CMN and whose tumor was untreated for 8 months. During that time, the tumor demonstrated a significant degree of regression. The shared translocation with IFS, a tumor with well‐documented potential for spontaneous regression, suggests that this genetic abnormality may have contributed to the favorable clinical course. Pediatr Blood Cancer. 2010;55:364–368. © 2010 Wiley–Liss, Inc.     

Benign body-surface tumors in children

We reviewed 138 benign body-surface tumors in children under the age of 16 years that were surgically resected at the Medical Center for Sick Children, Fukuoka City, from 1980 to 1987. The male-to-female ratio was 0.81, and 60% of the patients were less than 3 years old. Hemangioma was the commonest tumor and was followed by lymphangioma, dermoid and epidermoid tumors, pilomatrixoma, and lipoma. A total of 46% of the tumors were found on the scalp, face, and neck. Lipomas were distributed evenly over the body and had the largest mean diameter. This study describes some of the characteristics of benign body-surface tumors in children.Department of Pediatric Surger, Faculty of Medicine, Kyushu University, Maedashi 3-1-1, Higashiku, Fukuoka 812, Japan Offprint requests to: M. Kubota's present address     

Atypical congenital mesoblastic nephroma presenting in the perinatal period

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 50 years after its recognition: A narrative review

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an up‐to‐date overview of the literature. Complete surgical removal is curative in most cases. The risk of treatment‐related mortality (both surgery‐ and chemotherapy‐related) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment.     

先天性中胚层肾瘤5例临床特点并文献复习

目的 回顾性分析先天性中胚层肾瘤的临床特点、治疗方案及临床结局,为中胚层肾瘤的临床诊疗提供思路和依据。方法 回顾性分析2013年1月至2020年12月新华医院儿血液肿瘤科初诊为先天性中胚层肾瘤患儿的发病年龄、肿瘤大小、病理分型、ETV6-NTRK融合基因、治疗方案及临床结局。结果 7年内共收治5例新发中胚层肾瘤患儿,4例男婴,1例女婴;中位确诊年龄4.5个月(0.3～11个月)。3例产检时发现肾脏肿块且出生后早期确诊的患儿ETV6-NTRK融合基因均阴性,2例确诊年龄>6个月患儿ETV6-NTRK融合基因均阳性。肿瘤分期：Ⅰ期1例,Ⅱ期1例,Ⅲ期3例;病理分型：经典型1例,细胞型2例(Ⅱ期患儿为具有上皮样形态的细胞型),混合型2例。所有患儿均接受手术治疗,其中1例行2周期新辅助化疗后手术切除;Ⅲ期患儿术后予9周期VAC方案化疗。中位随访时间19个月(11～34个月),1例Ⅱ期患儿在手术+化疗综合治疗结束后1年复发,其余均无病生存。结论 先天性中胚层肾瘤患儿在手术联合辅助化疗后,大部分预后良好。患儿确诊年龄一般<1岁,部分患儿可因产检时肾脏肿块而早期确诊。手术仍是主要治疗手段...     

Benign cystic nephroblastoma

A benign cystic nephroblastoma in a 6-month-old boy is presented. Erroneous interpretation as malignant nephroblastoma led to unnecessarily aggressive therapy. There is no evidence of recurrence or metastatic disease 12 years after the initial resection. As shown in our case and in the others previously reported in the literature, benign cystic nephroblastoma represents a distinct clinicopathological entity which does not show malignant behavior.     

Cytogenetic abnormalities in mesoblastic nephroma: A link to Wilms' tumour?

Cytogenetic analysis of tumour material from a congenital mesoblastic nephroma is reported. Two cell lines were found, one with a normal 46,XY karyotype and the other with a hyperdiploid 51,XY karyotype, including a rearrangement of chromosome 11 at 11p15. This finding is of interest since loss of allelic heterozygosity at polymorphic 11p15 loci has been described in sporadic Wilms' tumour [1], and both cytogenetic [2] and molecular [3] changes of 11p15 are found in the Wiedemann-Beckwith syndrome, a condition with a predisposition to embryonal tumours, particularly Wilms' tumour. Our results lead us to speculate on the implications relating to the pathogenesis of this relatively benign tumour variant with respect to the current understanding of the genetics of Wilms' tumour. © 1993 Wiley-Liss, Inc.     

Prenatally diagnosed cystic neuroblastoma

An exceedingly rare case of prenatally diagnosed cystic neuroblastoma (NB) is described. Prenatal ultrasonography at 28 weeks' gestation revealed a cystic right suprarenal mass that measured 15 × 17 mm in diameter. The mass increased parallel to fetal size until it was 41 × 44 mm in diameter at 39 weeks' gestation. Magnetic resonance imaging performed prenatally and after delivery showed two different intracystic intensities with fluid levels, suggesting intracystic hemorrhage. The patient underwent a right adrenalectomy at 20 days of age, and the resected tumor was diagnosed as a poorly-differentiated ganglioneuroblastoma. Eight months after surgery, the patient is generally healthy with no evidence of recurrence. We reviewed 25 additional cases of prenatally diagnosed adrenal cystic NB and examined the clinical features of this rare entity. Accepted: 25 November 1997