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Case Reports
. 2020 Mar 26;8(6):1108-1115.
doi: 10.12998/wjcc.v8.i6.1108.

Abnormal serum carbohydrate antigen 19-9 levels in a patient with splenic retiform haemangioendothelioma concomitant with hepatic amyloidosis: A case report

Kai-Di Sun  1 , Yu-Jie Zhang  2 , Lan-Ping Zhu  1 , Bo Yang  1 , Sai-Yu Wang  1 , Zi-Han Yu  1 , Hai-Cheng Zhang  1 , Xin Chen  3
Affiliations
Case Reports

Abnormal serum carbohydrate antigen 19-9 levels in a patient with splenic retiform haemangioendothelioma concomitant with hepatic amyloidosis: A case report

Kai-Di Sun et al. World J Clin Cases. .

Abstract

Background: Carbohydrate antigen 19-9 (CA 19-9) is a glycoprotein that is used as a reliable tool for monitoring pancreatic cancer. Serum CA 19-9 levels are increased in patients suffering from liver, lung, and other non-malignant diseases. Haemangioendothelioma is a vascular neoplasm with a borderline biological behaviour. However, no case of haemangioendothelioma has yet been reported to be associated with CA 19-9.

Case summary: A 54-year-old Chinese man was referred to our hospital for discontinuous fatigue and unintentional weight loss for over one year. Laboratory investigations revealed an elevated serum CA 19-9 concentration of 39 IU/mL (reference interval, 0-37 IU/mL) over one year before admission. Afterwards, coagulopathy appeared, and the patient's serum CA 19-9 concentration increased continuously. At the time of admission, abdominal pain and haemorrhagic shock burst occurred, and emergency medical operation was performed. Laboratory investigations conducted upon admission showed a serum CA19-9 concentration of 392.56 IU/mL. Surgical resection of the spleen was undertaken, and pathological examination showed retiform haemangioendothelioma. The patient developed jaundice ten days after surgical excision of the spleen. Pathological examination of needle biopsy samples of the liver yielded a diagnosis of hepatic amyloidosis.

Conclusion: We describe a rare case of splenic retiform haemangioenthelioma concomitant with hepatic amyloidosis. Physicians should note abnormal serum CA 19-9 levels with early symptoms of fatigue and unintentional weight loss.

Keywords: Amyloidosis; Carbohydrate antigen 19-9; Case report; Haemangioendothelioma; Liver; Spleen.

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Conflict of interest statement

Conflict-of-interest statement: The authors declare no conflict of interest regarding this manuscript.

Figures

Figure 1
Figure 1
Computed tomography images. A: Computed tomography (CT) scan performed 4 mo before admission showed haepatomegaly, diffuse hypodense liver lesions, and low-density plaques in the spleen; B: CT scan after surgery showed haepatomegaly and diffuse hypodense liver lesions; C: CT scan after jaundice appeared showed haepatomegaly and diffuse hypodense liver lesions.
Figure 2
Figure 2
Microscopic appearance and immunophenotypes of the spleen. A: Haematoxylin and eosin staining of the biopsy specimen displayed elongated arborising vessels arranged in an anastomosing pattern (Haematoxylin and eosin staining, × 200); B: Platelet endothelial cell adhesion molecule-1 [cluster of differentiation (CD) 31] (× 200); C: CD34 antigen (CD34) (× 200); D: Cytokeratin (× 200); E: Friend leukemia integration 1 (× 200); F: Ki-67 antigen (× 200).
Figure 3
Figure 3
Microscopic appearance and immunophenotypes of liver biopsy. A: Haematoxylin and eosin staining of the biopsy specimen revealed deposits of acellular amyloid matrix (Haematoxylin and eosin staining, × 200); B: Positive staining for Congo red could be observed; C: Positive staining for methyl violet could be observed; D: Positive staining for haepatocytes could be observed; E: Platelet endothelial cell adhesion molecule-1 [cluster of differentiation (CD) 31] (× 200); F: CD34 antigen (CD34) (× 200).
Figure 4
Figure 4
Magnetic resonance imaging showing diffuse haepatomegaly and even signals.
See this image and copyright information in PMC

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