Malignant Rhabdoid Tumor Mimicking Hepatoblastoma: A Case Report and Literature Review

Wagner, Lars M.; Garrett, Jennifer K.; Ballard, Edgar T.; Hill, D. Ashley; Perry, Arie; Biegel, Jaclyn A.; Collins, Margaret H.

doi:10.2350/06-08-0155.1

Cited by 35 publications

(12 citation statements)

References 35 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The most extensive published series about congenital hepatic tumors describes a poor prognosis for hepatoblastomas, with common early metastases and high mortality rates [13], but this series probably includes some cases of congenital rhabdoid tumors, an aggressive lesion previously classified as hepatoblastoma with negative α-fetoprotein. As mentioned elsewhere in this article, immunochemistry tests for INI1/BAF47 protein, which is abnormally low in all rhabdoid tumors, have recently allowed a correct differentiation between the entities [36, 37, 53, 54], which present similar findings on imaging studies.…”

Section: Hepatic Tumorsmentioning

confidence: 82%

Congenital tumors: imaging when life just begins

et al. 2011

View full text Add to dashboard Cite

BackgroundThe technical developments of imaging methods over the last 2 decades are changing our knowledge of perinatal oncology. Fetal ultrasound is usually the first imaging method used and thus constitutes the reference prenatal study, but MRI seems to be an excellent complementary method for evaluating the fetus. The widespread use of both techniques has increased the diagnosis rates of congenital tumors. During pregnancy and after birth, an accurate knowledge of the possibilities and limits of the different imaging techniques available would improve the information obtainable, thus helping the medical team to make the most appropriate decisions about therapy and to inform the family about the prognosis.ConclusionIn this review article, we describe the main congenital neoplasms, their prognosis and their imaging characteristics with the different pre- and postnatal imaging methods available.

show abstract

Section: Hepatic Tumorsmentioning

confidence: 82%

Congenital tumors: imaging when life just begins

et al. 2011

View full text Add to dashboard Cite

show abstract

“…Surgical treatment of non-CNS MRTs is initially guided by the location. However, preoperative diagnosis is not always possible, as non-CNS MRTs are frequently mistaken for other more common tumors that arise in the location in which they are found [15]. For MRTs presenting in the kidney, the initial management strategy follows that of Wilms tumor.…”

Section: Discussionmentioning

confidence: 99%

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Farber

Shukla

Lim

et al. 2017

Journal of Pediatric Surgery

View full text Add to dashboard Cite

Introduction Non-central nervous system (non-CNS) rhabdoid tumors tend to present at a young age and have an extremely aggressive course, with dismal overall survival rates. Inactivation of the tumor suppressor gene SMARCB1 has been shown in rhabdoid tumors regardless of anatomic location, suggesting a common genetic basis. We retrospectively analyzed our institutional experience with non-CNS rhabdoid tumors to determine overall survival and prognostic variables. Methods We reviewed records of pediatric patients (age <22y) with non-CNS rhabdoid tumor at our institution between 1980 and 2014. Variables evaluated for correlation with survival included: age > or <1.5 years (median) at diagnosis, M1 status, and radiation therapy. The log-rank test was used to compare Kaplan-Meier probability distributions with P values adjusted for multiple testing using the false discovery rate approach. Results Nineteen consecutive patients (10 female) with histologically verified rhabdoid tumor were identified. Mean age at diagnosis was 3.2 years (median 1.5y, range 1.3mo–21.8y). Primary tumors were located in the kidney (n=10), head and neck (n=5), and in the liver, thigh, mediastinum and retroperitoneum (n=1 each). SMARCB1 expression was absent in all 10 patients tested. Eight patients had distant metastases at diagnosis. Median overall survival was 1.2 years. Age greater than the median and radiation therapy were associated with better outcome, with a median overall survival of 2.7 years (P=0.049 and P=0.003, respectively). Conclusion Survival rates for rhabdoid tumor remain poor, but prognosis is better in older children, regardless of primary tumor location. Because of its rarity, clinical trials with present agents are difficult to conduct. Further progress will require a focus on therapies targeted at tumor biology rather than anatomic location for non-CNS rhabdoid tumors.

show abstract

“…Rich glycogen within the cytoplasm is a notable and distinctive finding for ES/PNET, but is not specific [ 33 ]; MRT may show plentiful glycogen particles and no other specialized structures, mimicking ES/PNET ( Fig. 1B )[ 11 ]. MRT of soft tissue show large paranuclear whorls of intermediate filaments, with entrapped organelles and lipid droplets [ 34 ].…”

Section: Srct Of Unknown Histogenesismentioning

confidence: 99%

Utility of Transmission Electron Microscopy in Small Round Cell Tumors

Kim

Cho³

2015

J Pathol Transl Med

View full text Add to dashboard Cite

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin’s malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.

show abstract

Malignant Rhabdoid Tumor Mimicking Hepatoblastoma: A Case Report and Literature Review

Cited by 35 publications

References 35 publications

Congenital tumors: imaging when life just begins

Congenital tumors: imaging when life just begins

Prognostic factors and survival in non-central nervous system rhabdoid tumors

Utility of Transmission Electron Microscopy in Small Round Cell Tumors

Contact Info

Product

Resources

About