Teratoid Hepatoblastoma With Abundant Neuroendocrine and Squamous Differentiation With Extensive Parenchymal Metastasis

Abstract

Teratoid hepatoblastoma is a rare histologic subtype accounting for 4% to 10% of all hepatoblastomas and is characterized by the presence of divergent differentiation, including squamous, mucinous, melanocytic, cartilaginous, osseous, skeletal muscle, and neural elements. It is postulated that the teratoid elements might derive from multipotential less-differentiated stem cells. Teratoid hepatoblastoma responds poorly to chemotherapy and therefore recognition of this variant has prognostic implications. We describe a 1½-year-old child with teratoid hepatoblastoma characterized by unusual clinical and morphologic patterns including prominent neuroendocrine and squamous differentiation. (Arch Pathol Lab Med. 2012;136:911-914; doi: 10.5858/ arpa.2012-0212-CR) REPORT OF A CASE A 1½-year-old girl presented to another institution with decreased oral intake, lethargy, and increased abdominal girth. On physical examination she was found to have an abdominal mass. A computed tomography scan of her abdomen demonstrated numerous nodules replacing most of the left lobe of her liver in addition to many nodules throughout the right lobe of her liver and both lungs. Serum alpha fetoprotein level was 105 000 ng/mL (reference range, ,8.0 ng/mL). She underwent open liver biopsy and was treated according to Children's Oncology Group protocol for stage IV hepatoblastoma. She had significant reduction in the liver tumor burden; lung biopsies showed no viable tumor. She underwent liver transplantation at our institution and is alive with no evidence of disease 1 year later. PATHOLOGIC FINDINGSReview of the pretreatment liver biopsy revealed an epithelial neoplasm with prominent desmoplastic stroma. Tumor cells were arranged in irregular angulated nests and nodules with central comedo-type necrosis. Epithelial tumor cells varied from basaloid at the periphery of the nodules to larger cells centrally with squamous differentiation and keratin pearl formation. The nuclei were hyperchromatic and nucleoli were inconspicuous. Variable amounts of eosinophilic to clear cytoplasm were seen with distinct cytoplasmic borders. Mitotic figures were easily seen. Tumor cells stained focally with immunohistochemical stains directed against epithelial membrane antigen, keratin 5, 6, and 19, and P63. An immunohistochemical stain for afetoprotein was equivocal and stains for both hepatocyte antigen and glypican 3 were negative. A stain for b-catenin showed nuclear and cytoplasmic staining.The explanted liver weighed 550 g. The capsular surface was extensively nodular. The cut section showed many wellcircumscribed whitish tumor nodules in both lobes; the largest, in the posterior portion of the left lobe, measured 2.5 cm and showed necrosis and hemorrhage (Figure 1). The right lobe nodules clustered in the anterior portion without obvious treatment effect.Extensive sampling of the whitish nodules from both lobes revealed tumor with exuberant desmoplasia and squamous differentiation similar to the biopsy without hepatocytic or any other line of differ...

Teratoid Hepatoblastoma with Abundant Cholangioblastic Component in a Child with Full Trisomy 13

“…Retrospective review of the hepatectomy showed that malignant mucinous morphology was foeally present in the original hepatoblastoma. Rabah [5] described a teratoid hepatoblastoma with abundant neuroendocrine and squamous differentiation. In figures 3 and 6 from that manuscript, foci of similar primitive glandular structures were present.…”

Teratoid Hepatoblastoma With Yolk Sac-Like and Neuroendocrine Elements

“…9 Teratoid HB is a mixed HB with a mixture of heterologous components represented by neural/neuroectodermal differentiation. 6,8,[10][11][12][13][14][15][16] We present a unique case of teratoid HB with neuroendocrine and yolk sac-like elements. In addition, this case could represent difficulty as it presented with yolk sac-like elements in a metastatic site.…”

Hepatoblastoma and the Hepatoblastoma Family of Tumors

“…39, 40, and 41). These lesions are termed teratoid hepatoblastomas (Misugi and Reiner 1965;Shabanov et al 1981;Manivel et al,1986;Lackner et al 1990;Stocker 1994;Kim et al 2001;Zhang et al 2007;Buccoliero et al 2008;Rabah 2012). Teratoid hepatoblastoma also occurs in adult patients (Zhang et al 2007).…”

“…Teratoid tumors can contain rhabdomyoblastic elements (Shabanov et al 1981), which may in some tumors even dominate the histologic pattern, similar to what is seen in some pediatric renal neoplasms. Teratoid hepatoblastomas sometimes show squamous differentiation (Rabah 2012), otherwise often seen following chemotherapy. Thyroid tumors may contain mucinous epithelia of bronchial or intestinal type, sometimes with formation of complex patterns involving mucin-producing and non-mucinous cells and formation of glandular structures.…”