Adult-Onset Pancreatoblastoma

Abstract

Pancreatoblastoma (PB), an extremely rare malignancy of the pancreatic tissue, comprises less than 1% of all pancreatic tumors. Owing to the rarity of the malignancy and the potential for multiple differential diagnoses, adult-onset PB can be challenging to diagnose. The hallmark characteristic in diagnosing adult PB is found histologically. In approximately 90% of cases, squamous corpuscles are observed. We present the case of a patient diagnosed with adult-onset PB, who presented with key clinical and pathological features, as well as rarer characteristics, associated with the malignancy.

Keywords: adult pancreatoblastoma; pancreatoblastoma; squamoid nests.

Figure 1.

Computed tomography of the abdomen/pelvis with contrast revealing a large heterogeneously enhancing mass at the head/uncinate region of the pancreas measuring approximately 9.0 × 7.1 × 8.2 cm.

Figure 2.

Image (20× magnified) with immunohistochemical stain for lymphoid enhancer-binding factor 1 highlights the intervening squamoid nests. Imaging courtesy of Dr. Aatur Singhi.

Figure 3.

Hematoxylin and eosin-stained image (4× magnified) detailing a multilobular neoplasm separated by fibrous bands. Image courtesy of Dr. Aatur Singhi.

Figure 4.

Hematoxylin and eosin-stained image (20× magnified) detailing neoplastic cells with an organoid arrangement of acinar, solid, and trabecular formation. Occasionally, nuclear clearing is identified as consistent with the intranuclear accumulation of biotin. Image courtesy of Dr. Aatur Singhi.

Figure 5.

Image (20× magnified) with immunohistochemical stain demonstrating that most neoplastic cells are Bcl-10 positive. Image courtesy of Dr. Aatur Singhi.