PMC9300936_01
Axial (A) and coronal (B) contrast enhanced CT demonstrate a pancreatic tail mass with central necrosis ( ) positioned between the stomach, spleen, and kidney. MRI of the abdomen reveals a mass in the tail of the pancreas ( ) with intermediate T2 signal (C), diffusion restriction (D) and peripheral contrast enhancement (E). Additional enhancing lesions ( ) were seen in the liver consistent with metastatic disease (F).
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Her symptoms continued to persist, and a review of her prior abdominal computed tomography ( Figures 1A, B ) and magnetic resonance imaging (MRI) study showed a mass in the pancreatic tail with hepatic metastases ( Figures 1C-F ).
(A) Hemotoxylin and eosin (H&E) staining of primary tumor. Cells are arranged in nests with finely stippled chromatin. The tumor cells expressed synaptophysin, chromogranin, and pankeratin (not shown) supporting the diagnosis of well-differentiated neuroendocrine tumor. (B) H&E stained section of metastatic tumor focus in the liver with similar morphology to the primary tumor. (C) Ki-67 immunostained section of the metastatic tumor focus in the liver. The proliferation index was found to be 11% in a similar hot spot by digital image analysis. (D) Somatostatin receptor subtype 2A (SSTR2A) immunostained section was positive (3+, 90%). (E) Tumor molecular profiling showing a variant in VHL (c.500G>A, p.R167Q) at a frequency of 66.3%). (F) Tumor molecular profiling showing a variant in SDHA (c.1054C>T, p.R352*) at a frequency of 50%). (G) Germline molecular profiling showing an identical variant in VHL (c.500G>A, p.R167Q). (H) Germline molecular profiling showing an identical variant in SDHA (c.1054C>T, p.R352*).
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Histopathologic examination of the pancreatic tail mass revealed a well-differentiated neuroendocrine tumor ( Figure 2A ). Metastatic tumor deposits were identified in two of fifteen regional lymph nodes and multiple metastatic liver foci were identified ( Figure 2B ). A Ki-67 immunostain was performed on the primary tumor, local lymph node metastasis, and liver metastasis ( Figure 2C ) and the proliferation index was determined to be 14%, 8.5%, and 11%, respectively, using digital image analysis. Somatostatin receptor subtype 2A (SSTR2A) immunostaining was positive (3+, 90%) ( Figure 2D ) and ATRX immunostain was intact. Molecular test performed on the tumor sample showed pathogenic variants in the VHL gene (c.500G>A, p.R167Q) ( Figure 2E ) involving 66% of tumor cells, and SDHA gene (c.1054C>T, p.R352*) ( Figure 2F ) involving 50% of tumor cells. Figure 2G ) and SDHA (c.1054C>T, p.R352*) genes ( Figure 2H ). Familial testing could not be performed due to patient's adoptive status.