Malignant rhabdoid tumor of the liver presented with initial tumor rupture

Kachanov, D. Yu.; Телешова, М. В.; Kim, Eduard; Dobrenkov, Konstantin; Моисеенко, Р. А.; Usychkina, Anastasya; Филин, А. В.; Semenkov, A.V.; Mitrofanova, Anna; Коновалов, Д. М.; Shamanskaya, T. V.; Novichkova, Galina; Варфоломеева, С. Р.

doi:10.1016/j.cancergen.2014.04.006

Cited by 9 publications

(5 citation statements)

References 15 publications

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“…Notably, tumor rupture, which is an uncommon finding in childhood tumors, was observed in 14 of 53 cases (26.4%) in our study (11 cases of MRTK and 3 cases of EERT). Tumor rupture had not been yet reported in patients with MRT except for some sporadic case reports . Otherwise, according to our center analysis, from 2008 to 2017, about 45 out of 565 cases (45/565, 7.96%) of Wilms tumor occurred tumor rupture.…”

Section: Discussionmentioning

confidence: 65%

Clinical and Prognostic Characteristics of 53 Cases of Extracranial Malignant Rhabdoid Tumor in Children. A Single-Institute Experience from 2007 to 2017

et al. 2019

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Background The aim of this study is to add to the current knowledge regarding extracranial malignant rhabdoid tumor (MRT), a rare and highly aggressive tumor that occurs most commonly in infants and young children. Patients and Methods A retrospective medical record review was conducted on 53 patients with pathologically confirmed MRT in Beijing Children's Hospital between January 2007 and October 2017. Results Fifty‐three patients were diagnosed with MRT at a median age of 16 months, including 32 cases of malignant rhabdoid tumor of the kidney (MRTK) and 21 cases of extrarenal extracranial rhabdoid tumor (EERT). Fourteen (14/32, 43.75%) patients with MRTK and five (5/21, 23.81%) patients with EERT had metastases at diagnosis, and quite a few number of cases occurred tumor rupture (26.42%). Among the 53 patients, 40 (75.47%) patients died, 10 (18.87%) patients survived, and 3 patients (5.66%) were lost to follow‐up. Among the 40 dead patients, 38 patients died from rapid progression of the disease or tumor recurrence, and 2 patients died of severe postoperative complications. Most of the recurrent or relapsed cases (94.11%) occurred within 8 months, with a median time of 76 days after diagnosis. The overall survival rates of 3 years and 5 years for the entire cohort were 23.71% and 18.44%, respectively. After survival analysis, it was clear that a younger age at diagnosis and distant stage patients had relatively poor outcomes. The effect of treatment was the most difficult to analyze because patients were not treated uniformly. Statistically significant differences in survival were noted among patients treated with standard chemotherapy, total resection, and radiotherapy. Conclusion Extracranial MRT is still a highly aggressive tumor in children. Younger patients and those suffering from metastatic disease were most likely to have a poor outcome because of rapid progression or recurrence of the tumor. Implications for Practice This is the largest single‐institutional report that investigates the clinical characteristics and outcomes of extracranial malignant rhabdoid tumor (MRT) in China. Our study showed that gross hematuria and tumor rupture were typical characteristics of malignant rhabdoid tumor of the kidney. After survival analysis, it was found that the advanced stage of the tumor and an age ≤12 months at diagnosis were significantly associated with poorer survival. Although extracranial MRT is still a highly aggressive tumor in children, multimodal treatment approach, including chemotherapy, surgery, and radiotherapy, should be employed for this disease.

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Section: Discussionmentioning

confidence: 65%

Clinical and Prognostic Characteristics of 53 Cases of Extracranial Malignant Rhabdoid Tumor in Children. A Single-Institute Experience from 2007 to 2017

et al. 2019

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“…The use of ultrasound shows a heterogeneous solid mass that may have cystic changes and calcification [76,77,79,82]. In cases of rupture, hematoma may be seen around the tumor.…”

Section: Imaging Featuresmentioning

confidence: 99%

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

Bhagat,

Vij,

Raju

et al. 2023

Diagnostics

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Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children.

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“…To evaluate the role of surgery in management of pediatric MRT of the liver, we collected 44 pediatric cases with detailed reports from the English literature, 9,[11][12][13][14][15][16][17][18] and added the present case and another case from our institute (Table S2, case 45). The details of 46 patients are shown in the Table S2.…”

Section: The Role Of Living-donor Liver Transplantation In Children W...mentioning

confidence: 99%

The role of living‐donor liver transplantation in children with an advanced malignant rhabdoid tumor of the liver in the era of transplant oncology: Case report and literature review

Yanagi

Sakamoto

Nakao

et al. 2023

Pediatric Blood & Cancer

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Malignant rhabdoid tumor of the liver presented with initial tumor rupture

Cited by 9 publications

References 15 publications

Clinical and Prognostic Characteristics of 53 Cases of Extracranial Malignant Rhabdoid Tumor in Children. A Single-Institute Experience from 2007 to 2017

Clinical and Prognostic Characteristics of 53 Cases of Extracranial Malignant Rhabdoid Tumor in Children. A Single-Institute Experience from 2007 to 2017

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

The role of living‐donor liver transplantation in children with an advanced malignant rhabdoid tumor of the liver in the era of transplant oncology: Case report and literature review

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