Hepatoblastoma with solid and multicystic aspect mimicking a mesenchymal hamartoma: imaging and anatomopathologic findings

Staziaki, Pedro V.; Teixeira, Bernardo Corrêa de Almeida; Pedrazzani, Bruno Maurício; Gugelmin, Elizabeth Schneider; Zapparolli, Mauricio

doi:10.1590/0100-3984.2015.0163

Cited by 7 publications

(7 citation statements)

References 8 publications

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“…To our knowledge, a predominantly cystic-appearing HB has been described only once in literature where the histology was mixed mesenchymal /epithelial and serum AFP levels were markedly raised. 6 Our case differs from that case in being an HB with a predominantly cystic appearance representing an epithelial subtype. Although a prior case series described the ultrasonographic features of "cystic hepatoblastomas", this seems to be an entirely different entity where the lesions were purely cystic on imaging, and the diagnosis was made on histopathology that described tumor cells lining the cyst.…”

Section: Histopathologycontrasting

confidence: 68%

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A Malignant Hepatoblastoma Mimicking a Benign Mesenchymal Hamartoma: Lessons Learned

Singh

Wong²,

Nathan

et al. 2023

Journal of Pediatric Hematology/Oncology

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Differentiating hepatoblastomas from other congenital benign hepatic tumors is key to surgical management. We, herein, present an unusual case of an antenatally diagnosed liver lesion assessed in the neonatal period. Because of its predominantly cystic ultrasound/MRI appearance and borderline alpha-fetoprotein serum levels the diagnosis of mesenchymal hamartoma was favored and protocol-based tumor resection was performed. Due to the intraoperative diagnosis of a fetal subtype of hepatoblastoma with positive resection margins the child had to undergo a second laparotomy. This report raises awareness to an unusual appearance of hepatoblastoma and discusses noninvasive imaging clues to consider atypical appearances of hepatoblastoma preoperatively as they can have profound implications in patient management.

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Section: Histopathologycontrasting

confidence: 68%

Section: Discussioncontrasting

confidence: 48%

A Malignant Hepatoblastoma Mimicking a Benign Mesenchymal Hamartoma: Lessons Learned

Singh

Wong²,

Nathan

et al. 2023

Journal of Pediatric Hematology/Oncology

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“… 5 Ultrasound is clinically easier to perform in the pediatric population since anaesthesia is not required; a tumor mass and its internal components can be mapped using ultrasound, however, this modality lacks the refined image quality of MRI. 17 To monitor recurrence post-treatment, Fluorodeoxyglucose positron emission tomography (FDG-PET) can be used, particularly to identify early recurrence. 1 , 5 Unfortunately, due to the low FDG avidity in cells of pure fetal type, these tumors may be challenging to distinguish from normal background liver, and recurrence may not be readily identified.…”

Section: Radiologic Assessmentmentioning

confidence: 99%

“…19 , 21 FNA has been described in the diagnostic workup of mesenchymal hamartoma: HB is a major differential for the slightly less common mesenchymal hamartoma and has its own distinctive cytological features, such as clusters of tumor cells showing acinar and trabecular patterns, small round tumor cells with a high nuclear to cytoplasmic ratio, hyperchromatic nuclei with prominent nucleoli, and the presence of extramedullary haemopoiesis. 17 , 22 Due to the heterologous nature of both HB and mesenchymal hamartoma, and the narrow sampling potential of FNA, these two lesions with vastly different clinical trajectories can be mistaken for one another. FNA may miss the spindle cell mesenchymal element classically associated with mesenchymal hamartoma.…”

Section: Diagnostic Considerationsmentioning

confidence: 99%

Surgical Pathology Diagnostic Pitfalls of Hepatoblastoma

Auld

Sergi

2022

Int J Surg Pathol

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Hepatoblastoma (HB) is the most common malignancy within the rare cohort of pediatric primary liver tumors. It may arise sporadically or in association with germline mutations in specific genetic syndromes. Histogenesis recapitulates fetal hepatic development, however, this tumor can exhibit a markedly heterogeneous appearance both macroscopically and under the microscope. Histologic subtypes are classified based on morphologic appearance, with additional discrimination based on emerging molecular and immunohistochemical features. Numerous diagnostic pitfalls exist from clinical presentation through to ancillary testing; at all stages, the surgical pathologist must be discerning and open to collaboration with colleagues of different specialties. Problematic areas include the adequacy of tissue sampling, correlation of histology with radiologic appearance and alpha feto-protein (AFP) serology, forming a diagnostic consensus within the pediatric pathology community and choosing a shrewd immunohistochemical panel. This review discusses the sequence of events leading up to histologic assessment, and the nuances of microscopic evaluation. Along the way, pitfalls are highlighted, providing a tool for the surgical pathologists to support their individual approach.

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“…Imaging exams have played an important role in the study of liver diseases ( 1 - 5 ) . Hereditary hemorrhagic telangiectasia is a dominant autosomal disease with a prevalence of 10-20 cases per 100,000 population ( 6 ) .…”

mentioning

confidence: 99%