Case Report: An Unusual Course of Angiosarcoma After Lung Transplantation

Abstract

A 35-year-old woman underwent bilateral lung transplantation for primary ciliary dyskinesia and developed vascular tumors over a slow time course. Initial presentation of non-specific vascular tumors in the lungs and liver for up to 6 years after transplantation evolved toward bilateral ovarian angiosarcoma. Tumor analysis by haplotyping and human leukocyte antigen typing showed mixed donor chimerism, proving donor origin of the tumoral lesions. In retrospect, the donor became brain dead following neurosurgical complications for a previously biopsy-proven cerebral hemangioma, which is believed to have been a precursor lesion of the vascular malignancy in the recipient. Donor-transmitted tumors should always be suspected in solid organ transplant recipients in case of uncommon disease course or histology, and proper tissue-based diagnosis using sensitive techniques should be pursued.

Keywords: angiosarcoma; case report; donor-related; lung transplantation; malignancy.

Figure 1

Time course of events after lung transplantation. ¹⁸F-FDG PET-CT, ¹⁸F-fluorodeoxyglucose PET-CT; PTNB, percutaneous transthoracic needle lung biopsies. Radiography images: 1) chest CT of March 2013, axial reconstruction pulmonary window: normal lung parenchyma. 2) Chest CT of March and September 2016, axial reconstruction pulmonary window: nodule in the upper right lobe. Axial reconstruction soft tissue window: normal liver parenchyma. 3) Chest CT of April 2017, axial reconstruction pulmonary window: two nodules at the left costodiaphragmatic sinus. Axial reconstruction soft tissue window: normal liver parenchyma. 4) Chest CT of April 2018, axial reconstruction pulmonary window: two nodules at the left costodiaphragmatic sinus; axial reconstruction soft tissue window: largest of two hypodense liver lesions with a diameter of 38 mm. 5) Chest CT of August 2018, axial reconstruction pulmonary window: two lung nodules have merged into one large lesion. Hepatic MRI, T2-weighted axial reconstruction: partly real increase in volume and partly due to intralesional bleeding. 6) Chest and abdominal CT of May 2019, axial reconstruction pulmonary and soft tissue window: significant decrease in volume of the lung and liver lesions after conversion to sirolimus. 7) MRI abdomen of November 2019, T2-weighted axial reconstruction: enlarged ovaries, especially the left one, with mixed solid tissue lesions. 8) CT abdomen of January 2020, axial reconstruction pulmonary window: volume increase of the lung lesion at the left costodiaphragmatic sinus; axial reconstruction soft tissue window: massive tumor progression of the liver lesions. 9) Chest and abdominal CT of October 2019, axial reconstruction pulmonary and soft tissue window: massive tumor progression of the lung and liver lesions. Large figures of the radiography can be found in the Supplementary Materials .

Figure 2

Histology images of donor and recipient lesions. (A) Histology image of donor brain lesion: irregular thin-walled vascular spaces, delineated by a flattened endothelium (blue asterisk). The vessels are embedded in gliotic tissue (yellow asterisk). (B–D) Lung lesion (recipient, September 2016). (B) Low power view of the lung lesion, showing the transition from the lung tissue (left, black arrow) to the vascular lesion (right, red arrow). (C) Dilated blood-filled spaces (yellow arrows), delineated by a normal flat endothelium (blue arrows). (D) CD31 stain (brown) confirms that the spaces are lined by endothelium. (E–G) Ovarian lesion (recipient, January 2020). (E) Transition from ovary cortical tissue (left, black arrow) to vascular lesion (right, red arrow). (F) Irregular vascular spaces delineated by hyperchromatic, atypical endothelial cells with numerous mitoses (circles). (G) CD31 stain (brown) shows the lesion’s chaotic architecture.