Manoop S Bhutani1, Ayush Arora1, Uma Kundu2, Dipen Maru2. 1. Department of Gastroenterology, Hepatology and Nutrition, UT MD Anderson Cancer Center, Houston, Texas, USA. 2. Department of Pathology, UT MD Anderson Cancer Center, Houston, Texas, USA.
A 55-year-old female, with primary splenic angiosarcoma with metastatic lesions in liver, T-8 vertebra and left femur, status postsplenectomy, right hepatectomy and cholecystectomy, and multiple cycles of chemotherapy, was found to have a new 0.5 cm ill-defined attenuation focus in the body of pancreas on routine surveillance computed tomography (CT) scan. Follow-up of CT 2 months later revealed increase in size of the same mass to 0.8 cm [Figure 1], worrisome of progressive metastatic lesion from angiosarcoma. The patient was referred for endoscopic ultrasound (EUS)-guided fine-needle aspiration and biopsy (FNAB). On EUS [Figure 2], an 8 mm × 6 mm isoechoic rounded mass was seen in the pancreatic body. Transgastric EUS-guided fine-needle aspiration (two passes) and fine-needle core biopsy (two passes) were done using a 25 G, ProCore EUS needle (Cook). On cytology, specimens along with immunostains performed on the cell block sections showed that the neoplastic cells were positive for ERG and CD31 and focally/weakly positive for pan-cytokeratin. Immunohistochemical staining of the core biopsy revealed that the tumor cells were strongly positive for ERG (nuclear pattern), focally positive for CD31 and CD34, and negative for synaptophysin and chromogranin. This was consistent with metastatic angiosarcoma [Figures 3–6].
Figure 1
Computed tomography scan showing (arrow) a small, 8 mm, subtle, ill-defined mass in the pancreatic body
Figure 2
Endoscopic ultrasound exam revealing the same lesion (arrows) that was subjected to endoscopic ultrasound-guided fine-needle aspiration and core biopsy
Figure 3
Papanicolaou-stained smear shows a group of spindled to epithelioid cells with coarse chromatin pattern consistent with the patient's history of angiosarcoma
Figure 6
Immunohistochemistry performed on the cell block preparation shows positive staining for ERG, consistent with angiosarcoma
Computed tomography scan showing (arrow) a small, 8 mm, subtle, ill-defined mass in the pancreatic bodyEndoscopic ultrasound exam revealing the same lesion (arrows) that was subjected to endoscopic ultrasound-guided fine-needle aspiration and core biopsyPapanicolaou-stained smear shows a group of spindled to epithelioid cells with coarse chromatin pattern consistent with the patient's history of angiosarcomaH and E stain of a cell block preparation demonstrates a small of groups of neoplastic spindle cellsImmunohistochemistry performed on the cell block preparation shows positive staining for CD31, consistent with angiosarcomaImmunohistochemistry performed on the cell block preparation shows positive staining for ERG, consistent with angiosarcomaAngiosarcomas are rare, malignant, and frequently fatal soft tissue tumors and may metastasize to almost all visceral organs including pancreas. Definitive diagnosis can be made only with immunochemistry, and hence, obtaining biopsy is of paramount importance. EUS-FNAB has previously been described for diagnosing angiosarcoma in suprarenal gland[1] and spleen[2] in two prior case reports. To our knowledge, we present the first case of EUS-FNAB use for diagnosis of angiosarcoma within the pancreas. Primary angiosarcoma in pancreas may present as acute pancreatitis and diagnosis may be missed if biopsy is not performed as described by Csiszkó et al.[3] and Seth et al.[4] separately in their case reports. We recommend keeping angiosarcoma as a possible rare differential while evaluating pancreatic masses and using EUS-guided FNAB with these well-evaluated core needles[56] to confirm the diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patients understand that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.
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