Renal tumors in infants less than 6 months of age

Glick, Richard D.; Hicks, M. John; Nuchtern, Jed G.; Wesson, David E.; Olutoye, Oluyinka O.; Cass, Darrell L.

doi:10.1016/j.jpedsurg.2003.12.007

Cited by 139 publications

(113 citation statements)

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“…In most cases, the diagnosis is made still in the neonatal period, as it can lead to polyhydramnios (71% of the gestations associated with the tumor), hydropsy and premature birth, in addition to hypertension (as a result of the increase in renin levels by renal infiltration) and hypercalcemia (due to the tumor's secretion of a substance similar to parathormone). 1,3,5 The ultrasonographic assessment discloses, in general, a solid mass affecting the renal sinus, with cystic and hemorrhagic areas, as well as infiltration of local tissue. 5 The CMN probably originates from nephrogenic mesenchymal proliferation.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, during the first year after the nephrectomy, the patient must be submitted to a series of ultrasonographic assessments, in order to detect early signs of local recurrence. 1,3,9 The cellular variant is associated with a worse prognosis, with a survival rate of 85%, when compared to 100% for the classic variant. 9 Metastatic disease, mainly into the lungs, liver, brain and heart and an extensive area of local recurrence are rare events.…”

Section: Discussionmentioning

confidence: 99%

“…1 This fact makes this diagnosis the most probable one when an abdominal mass is detected in a child's kidney, very often leading to the treatment directed at WT, even without pathological confirmation. 2 Renal neoplasms in children younger than 6 months are less common.…”

Section: Introductionmentioning

confidence: 99%

“…2 Renal neoplasms in children younger than 6 months are less common. In this group, the congenital mesoblastic nephroma (CMN) is the most frequent one, 1 with 90% of the tumors being diagnosed within the first year of life and, virtually, never occurring after three years of age. 3 This tumor generally has a favorable prognosis.…”

Section: Introductionmentioning

confidence: 99%

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Nefroma mesoblástico congênito subtipo celular: relato de caso

et al. 2011

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Introduction: Congenital Mesoblastic Nephroma (CMN) is a rare pediatric renal tumor. It comprises two histological subtypes, namely classic and cellular, with the second accounting for two thirds of all cases and being more often associated with poor prognosis. It remains a diagnostic challenge for pathologists due to its similarity with other more frequent pediatric kidney neoplasms. Case report: We describe the case of a 2-yearold girl who presented with a left renal mass. After nephrectomy, the specimen analysis showed, on gross examination, an extensive, granular and whitish tumor lesion occupying almost the entire kidney, invading the renal sinus, capsule and perirenal fat, with areas of hemorrhage and necrosis. Histologically, it was characterized by ovoid spindle cells, mitoses and no cell atypia, which led to a diagnosis of cellular mesoblastic nephroma. Adjuvant chemotherapy was carried out, but tumor recurrence occurred in the first year, presenting as an unresectable tumor that did not respond to adjuvant chemotherapy and the patient died at 4 years of age. Discussion: The cellular variant tends to be more aggressive, with a survival rate of 85% versus 100% for the classic variant. Recurrence generally occurs in the first year, particularly with the cellular variant.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Nefroma mesoblástico congênito subtipo celular: relato de caso

et al. 2011

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“…Mesoblastic nephromas are the most frequent solid tumors of the first three month of life. Symptoms like the palpable mass per se, or rarely haematuria or paraneoplastic syndromes like hypercalcaemia and hypertension can be indicating [20,21]. Compared to the newborn child, the incidence of malignant tumors increases with accelerating age.…”

Section: Typical Features Of Specific Malignanciesmentioning

confidence: 99%

Timely identification of children with cancer

et al. 2013

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AbstractBackground. Cancer in childhood is rare, but nevertheless one of the most frequent causes of disease related death. Initial symptoms are often unspecific, frequently leading to a delay of cancer diagnosis. As a timely diagnosis can be crucial for the clinical outcome, our aim is to point out when unspecific symptoms should be considered suspect of being associated with specific cancer entities. Data sources. A systematic literature research in PubMed and current biliographies, as well as an evaluation of published epidemiologic data was performed. Results. This article reviews the typical presenting features and epidemiologic characteristics of the more common childhood malignancies, elucidates when specific and virtually unspecific symptoms require further evaluation, and gives advice how to start a rational diagnostic workup. Furthermore, genetic syndromes requiring increased watchfulness for cancer in childhood are demonstrated. Conclusion. Patients showing suspect symptoms should early be referred to specialized centres to assure optimal diagnostic and therapeutic capabilities.

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Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)

Furtwaengler¹,

Reinhard²,

Leuschner³

et al. 2006

Cancer

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BACKGROUNDSurgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.METHODSIn total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.RESULTSForty‐five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty‐nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event‐free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.CONCLUSIONSRadical nephrectomy with accurate surgical‐pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy. Cancer 2006. © 2006 American Cancer Society.

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Renal tumors in infants less than 6 months of age

Cited by 139 publications

References 18 publications

Nefroma mesoblástico congênito subtipo celular: relato de caso

Nefroma mesoblástico congênito subtipo celular: relato de caso

Timely identification of children with cancer

Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)

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