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Mesenchymal Hamartoma of the Liver in an Infant With Beckwith-Wiedemann Syndrome: A Rare Condition Mimicking Hepatoblastoma.

Abrahao-Machado LF, de Macedo FC, Dalence C, Stambo G, Abrahao-Machado EF, Abrahao-Machado EC, Bahrami A, Nascimento AG - ACG Case Rep J (2015)

Bottom Line: Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma.We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-fetoprotein levels.Histologic examination of the surgically excised mass revealed mesenchymal hamartoma of the liver (MHL), a benign hepatic neoplasm.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Barretos Cancer Hospital, Barretos, São Paulo, Brazil ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

ABSTRACT
Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma. We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-fetoprotein levels. The clinical impression was strongly suggestive of hepatoblastoma. Histologic examination of the surgically excised mass revealed mesenchymal hamartoma of the liver (MHL), a benign hepatic neoplasm.

No MeSH data available.


Related in: MedlinePlus

(A) The mesenchymal component composed of bland spindled to stellate cells at high magnification. (B) Foci of extramedullary hematopoiesis.
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Figure 4: (A) The mesenchymal component composed of bland spindled to stellate cells at high magnification. (B) Foci of extramedullary hematopoiesis.

Mentions: Exploratory laparotomy and excision of the abdominal mass found a 268-g, 9.5 x 7.0 x 5.0-cm well-circumscribed cystic mass with a smooth, pink-tan and glistening external surface (Figure 2). On sectioning, the mass was predominantly cystic with a variegated red to whitetan gelatinous cut surface containing multiloculated cystic and lobulated solid areas (Figure 2). The tumor was pre-dominantly composed of a mesenchymal component with a vaguely lobulated and myxoid appearance, containing stellate fibroblasts and thin-walled blood vessels. Within the loose mesenchymal tissue, particularly at the periphery of the lesion, there were entrapped cords of hepatocytes (Figure 3). Atypical epithelial elements were not present, and no heterologous differentiation was seen in the mesenchymal component. Small foci of extramedullary hematopoiesis were present within the tumor (Figure 4).


Mesenchymal Hamartoma of the Liver in an Infant With Beckwith-Wiedemann Syndrome: A Rare Condition Mimicking Hepatoblastoma.

Abrahao-Machado LF, de Macedo FC, Dalence C, Stambo G, Abrahao-Machado EF, Abrahao-Machado EC, Bahrami A, Nascimento AG - ACG Case Rep J (2015)

(A) The mesenchymal component composed of bland spindled to stellate cells at high magnification. (B) Foci of extramedullary hematopoiesis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508960&req=5

Figure 4: (A) The mesenchymal component composed of bland spindled to stellate cells at high magnification. (B) Foci of extramedullary hematopoiesis.
Mentions: Exploratory laparotomy and excision of the abdominal mass found a 268-g, 9.5 x 7.0 x 5.0-cm well-circumscribed cystic mass with a smooth, pink-tan and glistening external surface (Figure 2). On sectioning, the mass was predominantly cystic with a variegated red to whitetan gelatinous cut surface containing multiloculated cystic and lobulated solid areas (Figure 2). The tumor was pre-dominantly composed of a mesenchymal component with a vaguely lobulated and myxoid appearance, containing stellate fibroblasts and thin-walled blood vessels. Within the loose mesenchymal tissue, particularly at the periphery of the lesion, there were entrapped cords of hepatocytes (Figure 3). Atypical epithelial elements were not present, and no heterologous differentiation was seen in the mesenchymal component. Small foci of extramedullary hematopoiesis were present within the tumor (Figure 4).

Bottom Line: Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma.We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-fetoprotein levels.Histologic examination of the surgically excised mass revealed mesenchymal hamartoma of the liver (MHL), a benign hepatic neoplasm.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Barretos Cancer Hospital, Barretos, São Paulo, Brazil ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

ABSTRACT
Patients with Beckwith-Wiedemann syndrome (BWS) are known to be at an increased risk for childhood malignancies, particularly Wilms tumor and hepatoblastoma. We report a case of genetically confirmed BWS in a 5-month-old girl who presented with a 9.5-cm abdominal mass associated with elevated α-fetoprotein levels. The clinical impression was strongly suggestive of hepatoblastoma. Histologic examination of the surgically excised mass revealed mesenchymal hamartoma of the liver (MHL), a benign hepatic neoplasm.

No MeSH data available.


Related in: MedlinePlus