Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand?

Jehangir, Susan; Kurian, Jujju Jacob; Selvarajah, Dharshini; Thomas, Raji; Holland, A.J.A.

doi:10.1007/s00383-017-4149-5

Cited by 31 publications

(36 citation statements)

References 35 publications

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“…As suggested by the aggressive pathological features, the cellular variant is more aggressive with local recurrence, and distant metastases 17 . Since recurrence and metastasis in cellular CMN are much more common and carry a high mortality, it is suggested that children with cellular and mixed CMN are closely followed‐up clinically and radiologically for a minimum of 12 months after primary surgery 18 …”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis and postnatal management of congenital mesoblastic nephroma: Experience at a single center in China

Chen

Huang

et al. 2021

Prenatal Diagnosis

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Objective To review the prenatal and postnatal clinical characteristics and pathological subtypes, as well as the surgical outcome for congenital mesoblastic nephroma (CMN) cases. Method A retrospective review was performed in 11 cases with CMN prenatally diagnosed at a single center between 2015 and 2019. The clinical characteristics, surgical outcome, histopathology, and follow‐up were retrospectively obtained and reviewed. Results The median gestational age at which the sonographic diagnosis was made was 35 weeks. Polyhydramnios was found in four (36.4%) cases, and all resulted in a preterm birth. Nine infants had hypertension. Ten cases underwent radical nephrectomy, and one underwent radical nephrectomy and partial adrenalectomy. The pathological results showed that six tumors were classical variants, four mixed variants, and one was a cellular variant. Three cases presented as a stage I, eight as stage II, and no stage III or IV cases were diagnosed. All patients are alive so far. At a median follow‐up of 14 months, no local recurrence, or remote metastases were found. Conclusion The prognosis of prenatal CMN cases is excellent after early surgery.

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Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis and postnatal management of congenital mesoblastic nephroma: Experience at a single center in China

Chen

Huang

et al. 2021

Prenatal Diagnosis

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“…Referencias bibliográficas local, invasión al retro peritoneo y metástasis pulmonares y cerebrales en la variante celular 20,21 . En la histología, el NMC presenta haces entrelazados de células fusiformes con aspecto fasciculado, rodeando a túbulos y glomérulos [17][18] como en nuestro caso.…”

Section: Discussionunclassified

“…El NMC se considera benigno y con pronóstico favorable luego de la nefrectomía radical temprana abierta o laparoscópica y con amplios márgenes de seguridad 19 . Hay casos dependiendo de la variedad histológica y del estadio, con tendencia a la recidiva o con metástasis ganglionares, pulmonares o cerebrales que pueden eventualmente responder a la quimioterapia 20,21 .…”

Section: Congenital Mesoblastic Nephroma: Case Reportunclassified

Nefroma mesoblastico congénito: reporte de un caso

et al. 2021

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El nefroma mesoblastico congénito es un tumor renal infrecuente, pero común en el periodo neonatal. De comportamiento benigno y pronóstico excelente en su variedad clásica pero en el subtipo celular puede presentar recurrencia local, invasión retroperitoneal y metástasis pulmonares y cerebrales. El tratamiento es la nefrectomía radical. Los factores pronósticos son la edad menor a tres meses y la resección quirúrgica completa. Paciente de 17 días nacida a las 35 semanas por polihidramnios, con diagnóstico prenatal de masa abdominal. Al examen físico masa en flanco derecho e hipertensión arterial (121/79 mm Hg, TAM 77mm Hg). Ecografía abdominal con riñón derecho aumentado de tamaño y voluminosa formación sólida, de 4,4 x 4,3 x 4,8 cm, heterogénea y vascularizada al Doppler en polo inferior. Tomografía de abdomen con formación nodular heterogénea de 5,0 x 4,1 x 5,1 cm en polo inferior del riñón derecho. Se realizó nefrectomía radical derecha sin complicaciones. Evolución en los últimos 20 meses satisfactoria y libre de enfermedad.

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“…Recurrences are rare and have been described in approximately 4% of the cases . Relapses more commonly occur locally, but distant metastasis in the lung and liver have been reported; bone metastases are extremely rare and account for 5.5% of reported cases with metastasis …”

Section: Discussionmentioning

confidence: 99%

“…12 Relapses more commonly occur locally, but distant metastasis in the lung and liver have been reported; bone metastases are extremely rare and account for 5.5% of reported cases with metastasis. 9,13,14 Subsets of patients who are at high-risk for recurrence include those who are older than 3 months of age at diagnosis, those with a cellular type that contains an NTRK gene-fusion, and those with an incomplete surgical resection of the mass. 9,12,13 Gooskens et al estimated that recurrence with cellular CMN was as high as 10% with a mortality of 57% in the cohort reviewed.…”

Section: Discussionmentioning

confidence: 99%

Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma

Halalsheh

McCarville

Neel

et al. 2018

Pediatric Blood & Cancer

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Mesoblastic nephroma is the most frequent renal tumor in newborns and young infants, and the cellular type is characterized by an ETV6-NTRK fusion, which constitutively activates the tropomyosin-related kinase (TRK) signaling pathway. Larotrectinib is a highly selective TRK inhibitor with activity in adult and pediatric patients who have TRK fusions. We present a rare case of a patient with mesoblastic nephroma metastatic to bone who had a dramatic response to larotrectinib.

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Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand?

Cited by 31 publications

References 35 publications

Prenatal diagnosis and postnatal management of congenital mesoblastic nephroma: Experience at a single center in China

Prenatal diagnosis and postnatal management of congenital mesoblastic nephroma: Experience at a single center in China

Nefroma mesoblastico congénito: reporte de un caso

Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma

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