Biliary Adenofibroma with Invasive Carcinoma: Case Report and Review of the Literature

Abstract

We report a case of biliary adenofibroma with an invasive carcinoma in a 71-year-old female who presented with bilateral upper abdominal pain. Imaging revealed a 6.3 cm heterogeneously enhancing mass in the left lateral segment of the liver. Histologically, the adenofibroma showed the characteristic components as previously described of biliary adenofibromata, namely, cystic and tubular structures lined by cuboidal to low columnar biliary type epithelium and a dense fibrous stroma composed of spindled cells. Intimately admixed with the adenofibroma was a distinct tumor composed of malignant clear cells which demonstrated stromal and vascular invasion. Although mitotic figures were inconspicuous, Ki67 was brisk and p53 demonstrated 25–50% positivity. Sections also showed a von Meyenberg complex located adjacent to the tumor. This case expands the understanding of this rare tumor and proves two important assertions from previous case reports. First, the presence of an associated von Meyenberg complex with similar morphology and immunohistochemical staining pattern suggests that biliary adenofibromata and von Meyenberg complexes may share related histogenesis. Second, biliary adenofibromata harbor malignant potential and may show malignant transformation. Furthermore, this case highlights the need for these rare tumors to be followed aggressively, as their biological behavior is poorly understood.

Biliary adenofibroma: A rare hepatic lesion with malignant features

“…BAF is an extremely rare tumor affecting both sexes, with only 10 BAFs and 2 cases of unclassified tumor with BAF features reported in the literature (Table ). Although it has been reported as a benign biliary neoplasm, 3 cases in the literature showed features of malignant transformation . Other than frank invasion and distant metastasis, Tsui et al suggested that dysplastic and papillary epithelial changes might be precursors to malignancy .…”

Proliferative index facilitates distinction between benign biliary lesions and intrahepatic cholangiocarcinoma

“…Accordingly, anything less than strong nuclear staining should not be used to differentiate benign from malignant lesions due to the poor specificity of weaker staining for malignancy. In addition, given the great variability in p53 staining in benign tissue, it is possible that other benign processes, such as a rare biliary adenofibroma [21,22], will have strong nuclear p53 staining.…”

Malignant transformation of biliary adenofibroma: a rare biliary cystic tumor

“…However, imaging alone cannot reliably differentiate biliary cystadenoma from cystadenocarcinoma (13). Moreover, the reports that include imaging findings of malignant BAF are similar to other biliary cystic tumors (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)13). As such, a tissue diagnosis is needed.…”

“…Biliary adenofibromas (BAFs) are rare, benign biliary cystic tumors with uncommon potential for malignant transformation (1,2). To our knowledge, only 11 cases of BAF have been reported in the literature (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

“…To our knowledge, only 11 cases of BAF have been reported in the literature (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). We performed a database search to find cases of BAF diagnosed at Mayo Clinic from years 1994 to 2016.…”