Primary angiosarcoma of the Pancreas - A case Report and review of the literature

Abstract

Background

Angiosarcomas are malignant neoplasms that originate from endothelial cells. The symptoms exhibit a non-specific nature, and achieving a preoperative diagnosis is frequently challenging. They are seldom encountered in the abdomen, and their occurrence in the pancreas is even rarer.

Methods

Here we document a 67-year-old man with pancreatic angiosarcoma and analyse the literature to outline the clinicopathologic characteristics of this rare phenomenon.

Results

This patient with family history of pancreas cancer presented with abdominal pain, and the CT-scan revealed a 4 cm mass at the neck of the pancreas but CA19-9 was normal. Radiologic findings were unusual for ordinary pancreas cancer. Fine-needle aspiration biopsy through endoscopic ultrasound revealed “undifferentiated malignant cells for which the diagnosis of “carcinoma” was favoured. Total pancreatectomy, splenectomy and portal vein reconstruction were performed and epithelioid angiosarcoma were diagnosed. Despite an uneventful postoperative period, discharge on postoperative day 8 without any complications, as well as diligent post-discharge clinical care, the patient died 65 days postoperatively, attributed to the presence of extensive metastasis. A comprehensive literature search has identified a limited number of documented cases of primary pancreatic angiosarcoma, with only ten cases reported to date.

Conclusions

Pancreatic angiosarcomas are very rare and prone to misdiagnosis. The formation of a more demarcated but high-grade tumour with necrosis is a feature that distinguishes angiosarcomas from ordinary carcinomas of this organ. Pathologic diagnosis is also highly challenging closely resembling undifferentiated carcinomas. Angiosarcomas are highly aggressive when they occur in the pancreas. Prompt diagnosis at an early stage is crucial as surgery with curative intent serves as the primary treatment approach.

KEY POINTS

• Surgery with curative intent is the mainstay treatment for pancreatic angiosarcoma when diagnosed at an early stage.

• Oncological treatment options should be taken into consideration according to the follow-up data.

• Why does this paper matter?

• This article is important in that it is the most comprehensive review of the literature on pancreatic angiosarcoma, which is a very rare pathology, from the perspective of radiology, pathology and surgery.

Keywords:

• Angiosarcoma
• Mesenchymal malignancy
• Pancreas
• Rare tumours

Conflicts of interest/competing interests

Not applicable.

Availability of data and material

All data generated or analysed during this study are included in this published article.

Congress presentation

This study was presented in part at the 15th Biennial Congress of the European-African Hepato-Pancreato-Biliary Association (E-AHPBA) as a poster presentation. Lyon/France, 6-9 June 2023.

Author contributions

Emre Bozkurt, Asst. Prof: Substantial contributions to conception and design, and drafting the article. Samet Yigman, MD: Substantial contributions to conception and design, data collection and drafting the article. Volkan Adsay, Prof: Substantial contributions to conception and design and drafting the article. Bengi Gurses, Prof: Substantial contributions to conception and design and drafting the article. Gurkan Tellioglu, Prof: Substantial contributions to conception and design, and drafting the article. Orhan Bilge, Prof: Substantial contributions to conception and design, and drafting the article. All authors critically revised the manuscript and gave final approval of this version to be published.

The authors certify that this work is novel as it reviews diagnostic, therapeutic and follow-up options of pancreatic angiosarcoma which is an extremely rare disease.