Undifferentiated Renal Cell Carcinoma in Infancy: Report of a Case and Review of Literature

A case of rapidly progressed undifferentiated renal cell carcinoma in a 2 2/12-month-old boy is reported. The histology is characterized by sarcomalike spindle and pleomorphic cells and bizarre giant cells, thus creating many difficulties in the differential diagnosis. The diagnosis of renal cell carcinoma was established on the basis of tubular formation by clear and granular cells observed in a few discrete areas.

The literature on renal cell carcinoma occurring under 10 years of age was reviewed, with special attention given to histological typing. It is general & believed that renal cell carcinoma in children is well differentiated, but 2 of 39 cases reviewed were undifferentiated. We conclude that renal cell carcinoma in infants or young children may have an undifferentiated, atypical histological appearance and can progress rapidly. The differential diagnosis from anaplastic Wilms' tumor and sarcomatous tumors of the kidney in children is discussed.     

Anaplastic renal cell carcinoma following neuroblastoma

Renal cell carcinoma is unusual in children. We report a case of anaplastic renal cell carcinoma arising in a 7-year-old girl following treatment for Stage III neuroblastoma. The renal cell carcinoma has unusual histologic and ultrastructural features, which are discussed. The case is further unusual in that few children with advanced stage neuroblastoma survive long enough to develop second malignant neoplasms. © 1995 Wiley-Liss, Inc.     

Multicentric Bilateral Renal Cell Carcinomas and Avascular Leiomyoma in a Child

We report a unique case of multicentric bilateral renal cell carcinomas and a simultaneous large renal vascular leiomyoma in an 11 -year-old child with sickle cell anemia. The patient presented with several episodes of massive hematuria. Abdominal sonography and computed tomography demonstrated bilateral renal neoplasms and the patient was clinically thought to have bilateral Wilms' tumor. An initial biopsy of the lower pole of right kidney revealed a renal cell carcinoma. Accordingly, bilateral renal angiography followed by right total nephrectomy and left upper pole partial nephrectomy were performed. Pathologic studies showed multicentric, bilateral renal cell carcinomas (two in the right kidney and one in the left kidney), of clear, granular, and oncocytic cell types. A simultaneous large vascular leiomyoma was also present in the right kidney. The smooth muscle nature of the leiomyoma was determined by light microscopy, immunohistology, and electron microscropy. The diagnostic difficulties in distinguishing them from other renal tumors are discussed.     

Metastatic renal cell carcinoma in a child: 11-Year disease-Free survival following surgery

A child with metastatic renal cell carcinoma (RCC) is presented. This case is unusual in that the patient has remained disease free for 11 years following surgery and only one course of chemotherapy prior to thoracotomy. The management of metastatic RCC is reviewed and the genetic mechanisms leading to its development briefly discussed. Med. Pediatr. Oncol. 28:201–204 © 1997 Wiley-Liss, Inc.     

Hereditary Renal Adysplasia: New Observations and Hypotheses

Renal agenesis and dysplasia are frequently regarded by pathologists, even pediatric pathologists, as sporadic malformations. We report six fetal autopsy cases of hereditary renal adysplasia (HRA): two pairs of siblings, one case with paternal unilateral renal agenesis, and one case with an autosomal balanced 6p/19q translocation. The main purpose of this paper is to emphasize that nonsyndromal renal agenesis and dysplasia are pathogenetically related and often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. A subsidiary purpose is to present a case of bilateral multicystic dysplasia with a balanced 6p/19q translocation. This observation further supports the assignment of one of the loci for HRA to chromosome 6p.     

Renal cell carcinoma harboring somatic TSC2 mutations in a child with methylmalonic acidemia

Pediatric renal cell carcinoma (RCC) is a rare cancer that can be associated with inherited diseases including tuberous sclerosis complex (TSC) caused by germline mutations in TSC1 or TSC2. Somatic mutations in TSC1 and TSC2 have also been reported in adult RCC, which predict response to mTOR inhibitors. Here, we present the first case of RCC in a child with methylmalonic acidemia (MMA). Clinical whole exome sequencing of blood and tumor samples confirmed the diagnosis of MMA and revealed two somatic inactivating mutations in TSC2, suggesting the potential consideration of an mTOR inhibitor in the event of tumor recurrence.     

Undifferentiated renal cell carcinoma in infancy: report of a case and review of literature

A case of rapidly progressed undifferentiated renal cell carcinoma in a 2 2/12-month-old boy is reported. The histology is characterized by sarcomalike spindle and pleomorphic cells and bizarre giant cells, thus creating many difficulties in the differential diagnosis. The diagnosis of renal cell carcinoma was established on the basis of tubular formation by clear and granular cells observed in a few discrete areas. The literature on renal cell carcinoma occurring under 10 years of age was reviewed, with special attention given to histological typing. It is generally believed that renal cell carcinoma in children is well differentiated, but 2 of 39 cases reviewed were undifferentiated. We conclude that renal cell carcinoma in infants or young children may have an undifferentiated, atypical histological appearance and can progress rapidly. The differential diagnosis from anaplastic Wilms' tumor and sarcomatous tumors of the kidney in children is discussed.     

Unusual presentations of BK virus infections in pediatric renal transplant recipients

BKV has emerged as a significant pathogen in the field of transplantation, predominantly causing BKV nephropathy in renal transplant recipients and hemorrhagic cystitis in HSCT recipients. However, case reports describe more diverse complications, and we too present three unusual cases of BKV infections in pediatric renal transplant recipients. First, we describe a case of biopsy‐proven renal damage secondary to BKV prior to the onset of viremia, demonstrating that BKV nephropathy can occur without preceding viremia. We also present two renal transplant recipients with persistent BK viruria, one with BKV‐associated hemorrhagic cystitis and the other with microscopic hematuria. Therefore, we conclude that BKV manifestations may be more diverse than previously thought and suggest clinical utility in urine BKV qPCR testing in specific transplant recipients.     

Recurrent pancreatitis after renal transplantation in a child

Abstract:  A five-yr-old girl, who was a renal transplant recipient, presented with nausea, vomiting, epigastric discomfort, papules, and vesicles on her body. She was diagnosed with acute pancreatitis and varicella zoster infection because her serum amylase and lipase levels were positive. Fourteen months later, she was readmitted with nausea, vomiting, and epigastric pain similar to the previous symptoms and was diagnosed with acute pancreatitis. This case report indicates that acute pancreatitis can be one of a number of complications following pediatric renal transplantation and can recur because of various causes.     

Vascular endothelial growth factor (VEGF) receptor antibody bevacizumab (avastin) induces regression of renal cell carcinoma in an adolescent resulting in residual tumorectomy

Renal cell carcinoma is a chemo- and radiation-resistant malignancy that is very rare in childhood. In advanced cases, survival is extremely poor, but cure can be achieved with complete tumorectomy. We report a 14-year-old female with left renal cell carcinoma, with regional lymphadenopathy extending up to the diaphragm. Complete tumor resection was impossible. The tumor progressed after 10 months of treatment with high-dose interleukin-2. After 7 months of outpatient vascular endothelial growth factor receptor antibody bevacizumab and no significant adverse effects, dramatic tumor shrinkage enabled complete resection.     

A RARE TYPE OF RENAL CELL CARCINOMA IN A GIRL: Hybrid Renal Cell Carcinoma

Hybrid renal cell carcinoma (HRCN) is a rare type of renal tumor with characteristic pathologic features, including oncocytic and chromophobe cellular content, and shows more favorable prognosis than renal cell carcinoma. The early-stage tumors show favorable outcome, and postoperative regular clinical and radiological follow-up is adequate in most cases. However, close follow-up is mandatory for tumors with histopathologically coexistence of squamous, papillary, and/or eosinophilic RCC component. This report describes a 12-year-old girl with a stage I HRCN who was closely followed after nephrectomy with no further treatment.     

Papillary renal cell carcinoma in a child.

AIM: To report a case of papillary renal cell carcinoma in a pediatric patient. METHOD: A seven-year-old boy complained of hematuria for one year. CT scan showed a mass in the middle zone of the right kidney. Radical nephrectomy was performed without incident. RESULT: Histology of the specimen showed papillary renal cell carcinoma. As the tumor was stage one, no chemotherapy was offered. At follow-up after 6 months the child was without recurrence or metastases. CONCLUSIONS: Papillary renal cell carcinoma is an uncommon kidney tumor in children and in its early stages it can be cured by nephrectomy alone.     

Primary renal lymphoma presenting with hypertension

Primary renal lymphoma (PRL) is a rare lymphoma which usually presents with hematuria, flank pain, abdominal mass, and weight loss. PRL is more diagnosed in adults than children. We describe an asymptomatic child who presented with hypertension and was subsequently diagnosed with primary renal lymphoma. This case represents an atypical presentation for PRL.     

Acute renal failure after exercise in a child with renal hypouricemia

We describe a 15 year old boy with renal hypouricemia who developed acute renal failure after a school athletics meeting, accompanied by appendicitis. During acute renal failure, the highest level of uric acid was 5.0 mg/dL, creatinine 7.9 mg/dL and urea nitrogen 58.6 mg/dL. After recovery, the serum uric acid fell to 0.9 mg/dL and the fractional excretion of uric acid (FEu_A) exceeded the normal range. The probenecid and pyrazinamide tests showed that the patient had a total defect of uric acid reabsorption. This case suggested that strenuous exercise could be responsible for acute renal failure in patients with renal hypouricemia.     

Angioplasty for recurrent renal artery stenosis in flare‐up of Hashimoto's thyroiditis

Takayasu arteritis is the most common disease seen in children presenting with renovascular hypertension (RVH) in Asia, and can manifest anatomically as renal artery stenosis (RAS). We report the case of a 16‐year‐old girl presenting with RVH due to recurrent and novel RAS in Hashimoto's thyroiditis (HT) flare‐up. After treatment with thyroxin and percutaneous transluminal renal angioplasty (PTRA), she was free of hypothyroidism and systemic hypertension. RVH due to recurrent and novel RAS in HT flare‐up has not previously been reported in the English‐language literature. PTRA is the procedure of choice, providing there is no renal artery dissection or aneurysm.     

Clear cell sarcoma of the kidney in a 4-month-old infant: A case report

A case of clear cell sarcoma of the kidney is described in a 4-month-old male infant. The tumor had a typical histological pattern, consisting of sheets of tumor cells possessing “empty” nuclei set within a delicate capillary network. There was extensive necrosis and tumor cell cytoplasm contained vimentin. This tumor occurs only rarely in infants less than six months of age. The different renal neoplasms of childhood have diverse prognoses and demand specific therapy so that accurate diagnosis is mandatory. The present case illustrates that clear cell sarcoma, a highly malignant neoplasm, must be considered in the differential diagnosis of a renal mass occurring in this age group. © 1993 Wiley-Liss, Inc.     

Second primary neoplasms in a population-based series of patients diagnosed with renal tumours in childhood

Eight second malignant tumours developed in a population-based series of 218 patients diagnosed with renal tumours in childhood: renal cell carcinoma of the contralateral kidney, hepatocellular carcinoma, Hodgkin's disease, and 4 basal cell and 1 squamous cell carcinomas of skin. Excess risk of developing a second malignancy (excluding skin carcinomas but including a registrable spinal neurofibroma) was 14.7 (95% Cl 4.0-37.7, P = 0.0003) for Wilms' tumour patients. Cumulative incidence of second malignant neoplasms (excluding skin carcinoma) was zero at 10 years, 5.0% at 20 years, and 10.2% at 30 years. The most common second neoplasms seen were benign osseous/chondromatous tumours and 4 of the 7 Wilms' tumour patients with malignant tumours had previous or synchronous tumours of this kind. Development of bony exostoses may be a marker for those patients at particularly high risk of subsequent malignancy. © 1994 wiley-Liss, Inc.     

Hypercalciuria in renal glucosuria

Primary renal glucosuria is a benign condition in which serum glucose level is normal. Idiopathic hypercalciuria is defined as increased urinary calcium excretion of more than 4 mg/kg/day in normocalcemic individuals in whom all known causes of hypercalciuria have been excluded. In this paper, we report on a case who has both renal glucosuria and hypercalciuria.     

RENAL MEDULLARY CARCINOMA: A Potential Sickle Cell Nephropathy of Children and Adolescents

An extremely aggressive malignant epithelial neoplasm of the kidney has recently been described and named renal medullary carcinoma. The finding of this tumor is highly predictive of drepanocytes (sickle cells) in tissue sections and thus the presence of sickle hemoglobin, specifically sickle cell trait, in the patient. We present a case report of this rare tumor in a 10-year-old male. The tumor displayed a variable histologic architecture including gland-like areas with intra- and extracytoplasmic material resembling mucin with hematoxylin and eosin stain. This material was negative with periodic acid-Schiff and mucicarmine stains, stained only weakly with Alcian Blue, and was positive using antibodies against peanut agglutinin. Tumor cells stained positively with antibodies to epithelial membrane antigen, cytokeratin, vimentin, and Ulex europaeus lectin. The luminal face of tumor cells stained with peanut agglutinin. Stains using antibodies against carcinoembryonic antigen and alpha-fetoprotein were negative. Ultrastructurally, the tumor cells were characterized by short microvilli lining the luminal surface and lateral complex infoldings of adjacent plasma membranes. We discuss the relationship of this neoplasm to another renal pelvic neoplasm, collecting duct carcinoma, which may rarely occur in children. Renal medullary carcinoma should be included in the differential diagnosis of gross hematuria, which is most commonly benign self-limited hematuria, in young patients with sickle cell trait.     

Lupus nephritis in children in Malaysia

OBJECTIVES: To determine the pattern of renal histology, clinical outcome of children with lupus nephritis and to identify any associated risk factors predicting renal failure in these children. METHODS: Retrospectively, 27 children under 16 years of age with lupus nephritis who had renal biopsies done at Sultanah Aminah Hospital Johor, Malaysia from 1994 to 2002 were studied. The renal histology was graded according to WHO classification system (1982). The medical records, laboratory data and the clinical outcome of the patients were studied. RESULTS: There were 24 cases of WHO Class IV, two cases of WHO Class II and a case of WHO Class V. Twenty children were in the good renal outcome group while six children progressed into the poor renal outcome group and required renal replacement. One child was lost to follow-up. All six children in the poor renal outcome group had WHO Class IV histology. The 5-year patient and renal survival rates were 84% and 75%, respectively. Age, sex, activity and chronicity indices in the renal histology, anaemia, elevated serum creatinine, depressed levels of C3 and C4, heavy proteinuria or presence of urinary active sediments were not associated with progression to renal failure. CONCLUSIONS: Presently, children with lupus nephritis appeared to have better patient and renal survival rates. Assessment of renal histology in these children was important for diagnosis, treatment and probably prognosis. In this study, there was a 25% incidence of loss of renal function over 5 years in children with WHO Class IV renal histology.