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Angiosarcoma in Arteriovenous Fistula after Kidney Transplantation

Nutsiri Kittitirapong, MD, Artit Jinawath, MD, Suthas Horsirimanont, MD

PII: S2468-4287(21)00007-1
DOI: https://doi.org/10.1016/j.jvscit.2020.12.016
Reference: JVSCIT 681

To appear in: Journal of Vascular Surgery Cases and Innovative Techniques

Received Date: 15 August 2020

Accepted Date: 23 December 2020

Please cite this article as: N. Kittitirapong, A. Jinawath, S. Horsirimanont, Angiosarcoma in

Arteriovenous Fistula after Kidney Transplantation, Journal of Vascular Surgery Cases and Innovative
Techniques (2021), doi: https://doi.org/10.1016/j.jvscit.2020.12.016.

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© 2021 The Author(s). Published by Elsevier Inc. on behalf of Society for Vascular Surgery.
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1 Angiosarcoma in Arteriovenous Fistula after Kidney Transplantation

2
3 Nutsiri Kittitirapong MD1, Artit Jinawath MD2, Suthas Horsirimanont MD1,

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5 Division of Vascular Surgery, Department of Surgery, Faculty of Medicine Ramathibodi

6 Hospital, Mahidol University, Bangkok, Thailand

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Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University,

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Bangkok, Thailand

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10 Corresponding author:
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11 Nutsiri Kittitirapong MD
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12 Division of Vascular Surgery, Department of Surgery, Faculty of Medicine Ramathibodi

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13 Hospital, Mahidol University, Bangkok, Thailand

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15 Post-publication corresponding author:

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16 Nutsiri Kittitirapong MD

17 Division of Vascular Surgery, Department of Surgery, Faculty of Medicine Ramathibodi

18 Hospital, Mahidol University, Bangkok, Thailand

19
20 Telephone: +66855539293; Fax: +6622011316; email: nutsiri.kit@mahidol.ac.th

21
22 Previous Presentation: No

23
 2

1 Sources of Funding: No

2
3 Conflict of Interest Statement: No

4
5 Key words: angiosarcoma, arteriovenous fistula, renal transplant, epithelioid tumor, vascular

6 access

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1 Abstract:

2 Angiosarcoma is a rare complication of both functioning and nonfunctioning fistulae. It is

3 an aggressive soft tissue sarcoma arising from vascular or lymphatic endothelial cells. We report

4 a case of angiosarcoma from a nonfunctional fistula in a kidney transplantation patient receiving

5 immunosuppressive drugs. The patient presented with arm pain mimicking a thrombosed

6 arteriovenous fistula.

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1 Introduction

2 Angiosarcoma is a rare aggressive soft-tissue sarcoma1 characterized by rapidly

3 proliferating and extensively infiltrating anaplastic cells, which derived from blood vessels and

4 lining irregular blood-filled spaces. It is a rare complication of arteriovenous fistulae.2 In renal

5 transplant recipients, angiosarcoma is an extremely rare type of cancer, <1% of all cancers and

6 2% of localized soft tissue cancer.3 Because of its rarity, most clinicians are unaware of this

7 condition, which leads to delayed diagnosis and treatment. After Institutional Review

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8 Board/Ethics Committee approval, a retrospective chart analysis is conducted. Related literatures

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9 are also reviewed.
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10 Case report
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11 A 58-year-old man presented with right arm pain at a brachiocephalic arteriovenous

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12 fistula (BCAVF). He was in end-stage kidney disease and began hemodialysis via a right

13 BCAVF 5 years earlier. After 1 year of hemodialysis, he underwent living related kidney
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14 transplantation (LRKT). He had been receiving immunosuppression with prednisolone,

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15 mycophenolate mofetil and tacrolimus. His baseline creatinine was 0.6 mg/dL. Later, he

16 complained of right arm pain and was diagnosed with a partially thrombosed AVF. After 6 -

17 month of conservative treatment, his pain and weakness became severe. Also, paresthesia in his

18 right arm and the bleeding erosion from BCAVF were notified. The diagnosis was impending

19 rupture of an AVF aneurysm. He followingly underwent aneurysmal resection. Preoperative

20 chest X-ray showed a right lung mass. During intraoperative period, no mass in intraluminal

21 cephalic vein could be identified as showed in Figure 1. Unfortunately, his severe arm pain

22 persisted postoperatively and the X-ray showed multiple osteolytic lesions in the right humeral
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1 shaft, proximal ulna and radius as showed in figure 2. Chest computed tomography (CT)

2 revealed a 2 cm. lobulated pulmonary nodule in the right upper lung. Therefore, we suspected

3 primary lung cancer with bone metastasis. Fifteen days later, he came back with acute limb

4 ischemia on right arm. CT angiography showed no contrast filled in proximal radial and ulnar

5 arteries at distal brachial to brachial bifurcation. There was an eccentric filling defect at right

6 brachial artery as showed in figure 3. The intraoperative finding revealed soft tan brown tissue

7 from brachial arterial embolectomy. The cytology from blood clot showed atypical cell. As well,

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8 tissue pathology reported epithelioid angiosarcoma. Bone biopsy of the right humerus revealed

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9 angiosarcoma. Three months after aneurysm resection, the patient underwent right shoulder

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disarticulation. Histopathology of right shoulder disarticulation revealed angiosarcoma
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11 involving the AVF, soft tissue of the cubital fossa and proximal humerus as figure 4 and 5.
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12 Eventually, the final diagnosis was angiosarcoma at the AVF with bone and pulmonary
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13 metastasis. Therefore, we planned palliative chemotherapy with Taxol weekly. Unfortunately,

14 the patient developed hemoptysis. He died from massive hemothorax 10 months after symptom
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15 onset and 1 month after diagnosis. Since the patient died and we were unable to contact his
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16 relatives, the consent was not obtained.

17 Discussion

18 According to the literature review showed in Table I, 23 cases in 20 English language

19 published case reports described that angiosarcoma occurred from vascular access. Most patients

20 were men (83.3%), and the mean age was 52.7 ± 15.2 years. The most vascular access was AVF

21 (95.6%). Thirteen (65%) patients had undergone deceased donor kidney transplantation. Five
 6

1 (25%) patients had undergone LRKT, two (10%) patients were non-KT patients. Twenty (93%)

2 kidney grafts were functional. Only five patients had received a second KT.

3 The pathophysiology of angiosarcoma at an AVF after KT remains unclear. The possible

4 mechanisms are11: 1. impairment of local immune response caused by the increased lymphatic

5 workload and the decreased lymphatic drainage from the increased venous pressure at the AVF

6 site24; 2. turbulent blood flow in AVF associates with the increased production of reactive

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7 oxygen species and matrix metalloproteinase-9 which results in a proliferative response leading

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8 to vascular remodeling25; and 3. large hypoxic area of tumors caused by rapid cell proliferation

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exceeds the capacity of the oxygen supply. These mechanisms induce T cell suppression, which
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10 lead to a diminished immune response.26 Furthermore, renal transplant patients have a three- to

11 five-fold increased risk of overall cancer incidence.27 Also, prolonged immunosuppression

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12 associates with an increased cancer risk.28

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13 According to the literature review, the most common clinical presentations were non-
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14 specific pain (58.3%), swelling (29.2%) and mass (29.2%). The initial diagnosis for pain at AVF
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15 were thrombosed AVF (44%), aneurysm (25%), infection (25%) and cancer (6.3%). Therefore,

16 conservative treatments including antibiotics and ligation without biopsy were commonly

17 implemented. These treatments usually led to delayed diagnosis. The median time from

18 presentation to diagnosis was 10.8 weeks (range, 4–12 weeks). A high index of suspicion for

19 angiosarcoma is required.

20 In our brachial arterial embolectomy case, we sent clot and tissue for histopathology. The

21 cytology from blood clot showed atypical cell and tissue pathology showed epithelioid

22 angiosarcoma. Shoulder disarticulation pathology showed extensive tumor invasion; however,

 7

1 the origin of tumor could not be identified. It might be anticipated that the origin of

2 angiosarcoma came from arterial side. In my opinion, we should test both blood clot in aneurysm

3 of AVF for cytology and tissue of AVF for pathology in any kidney transplantation case that

4 presents such pain or aneurysm change.

5 Tumor histology in angiosarcoma varies widely from well-differentiated to poor-

6 differentiated. Abnormal, pleomorphic and malignant endothelium are the hallmark of

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7 angiosarcoma. The cell appearance can be rounded, polygonal, fusiform and possibly

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8 epithelioid.2 Epithelioid angiosarcoma is a variant of angiosarcoma composed of neoplastic cells

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with an epithelioid appearance. The most sensitive and specific marker for endothelial
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10 differentiation is CD-31, which indicates platelet–endothelial cell adhesion molecule

11 expression.29 From our review, all patients were positive for cluster of differentiation (CD)-31.
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12 According to our case, the pathological findings showed that the tumor was high grade
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13 pleomorphic epithelioid cells with positivity to ERG immunohistochemistry and other vascular
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14 markers such as FLI1, CD31 and CD34.

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15 Angiosarcoma behaves aggressively, recurs locally and spreads widely. Moreover, it has

16 a high rate of lymph node and systemic metastases. Based on our review, nearly half of the

17 patients had metastasis at the time of diagnosis. 85% of patients experienced recurrence, with

18 7.75% in bone and 76.95% in the lungs. The mean survival was 24.2 ± 14.4 weeks. Therefore,

19 the tumor-related death rate is considerably high.

20 The ideal primary treatment is radical surgery with complete resection; however,

21 problems with wide excision are tumor size and location, which result in functional impairment

22 and difficulty in wound reconstruction. Due to high local recurrence rate, adjuvant radiotherapy
 8

1 is recommended. Angiosarcoma spreads mainly through hematogenous and the most common

2 metastatic site is lungs. Chemotherapy is the primary treatment option for metastatic

3 angiosarcoma. Biological therapies, particularly antiangiogenic therapies, are considered to be

4 interesting options for angiosarcoma-specific treatment.

5 Conclusion:

6 Angiosarcoma at vascular access sites can occur in both KT and non-KT patients. Since

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7 the presentation is not specific, a high index of suspicion is required to prevent delayed

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8 diagnosis. Angiosarcoma has aggressive behavior as well as high local recurrence and metastasis

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rate. The primary treatment of angiosarcoma is radical surgery with complete resection.
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10 Chemotherapy is the primary treatment for metastatic angiosarcoma whereas local recurrence
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11 can be prevented by radiation. However, despite of multimodal treatment, patients’ prognosis

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12 remains poor.
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13 Acknowledgement: The authors gratefully acknowledge Nipapan Choonu for the statistical
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14 analysis.
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3 patterns in the U.S. : an analysis of 12,114 cases. Cancer 2008;113:616-627.

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9 angiosarcoma arising in an arteriovenous fistula. Histopathology 1992;21:87-89.

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13 6. Wehrli BM, Janzen DL, Shokeir O, Masri BA, Byrne SK, O'Connell JX. Epithelioid

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16 1998;22:1154-1159.

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20 8. Keane MM, Carney DN. Angiosarcoma arising from a defunctionalized arteriovenous

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1 9. Bessis D, Sotto A, Roubert P, Chabrier PE, Mourad G, Guilhou JJ. Endothelin-secreting

2 angiosarcoma occurring at the site of an arteriovenous fistula for haemodialysis in a renal

3 transplant recipient. Br J Dermatol 1998;138:361-363.

4 10. Farag R, Schulak JA, Abdul-Karim FW, Wasman JK. Angiosarcoma arising in an

5 arteriovenous fistula site in a renal transplant patient: a case report and literature review.

6 Clin Nephrol 2005;63:408-412.

7 11. Webster P, Wujanto L, Fisher C, Walker M, Ramakrishnan R, Naresh K, et al.

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9 important differential diagnosis. Am J Nephrol 2011;34:42-48.

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12. Demey K, Reyns LM, Schepers S. Angiosarcoma arising in an arteriovenous fistula in a
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11 patient without kidney transplant. Acta Chir Belg 2014;114:75-78.
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12 13. Roy SF, Ghazawi FM, Alsarheed A, Lach KD, Watters K, O'Brien E. Angiosarcoma
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13 arising within a nonfunctioning arteriovenous fistula. Int J Dermatol 2018;57:1513-1515.

14 14. Aldaabil RA, Alkhunaizi AM, Al-Dawsari NA, Dawamneh MF, Rabah R. Angiosarcoma
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15 at the site of nonfunctioning arteriovenous fistula in a kidney transplant recipient. J Vasc

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16 Surg Cases Innov Tech 2016;2:53-55.

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20 16. Costa BNL, Rivera CF, Rodriguez MC, Romero TH, Muniz AL, Hermida TF, et al.

21 Angiosarcoma developing in an arteriovenous fistula after kidney transplantation. Case

22 Rep Transplant 2017;2017:2426859.

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1 17. Chanyaputhipong J, Hock DL, Sebastian MG. Disseminated angiosarcoma of the dialysis

2 fistula in 2 patients without kidney transplants. Am J Kidney Dis 2011;57:917-920.

3 18. Gale AL MR, Liberman SR, Zavlin D, Echo A. Case report: angiosarcoma in the upper

4 extremity related to a nonfunctioning arteriovenous fistula. Hand (N Y) 2017;12:NP132-

5 NP135.

6 19. Kakisis JD, Antonopoulos C, Moulakakis K, Taliadoros A, Rontogianni D, Brountzos E,

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9 20. Jansen R, McHargue C. Angiosarcoma in a condensed sporotrichoid distribution arising

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11 21. Mark Kleman M, Bermudez C, Gray J. Angiosarcoma in an unused arteriovenous fistula
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13 22. Andre J, Parsikia A, Minimo C, Khanmoradi K, Campos S, Zaki R, et al. Soft tissue

14 sarcoma at a dialysis access site in a transplant recipient. Exp Clin Transplant

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15 2012;10:410-415.
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19 24. Bordea C, Cortina-Borja M, Wojnarowska F, Morris PJ. Distribution of upper limb skin

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22 25. Castier Y, Ramkhelawon B, Riou S, Tedgui A, Lehoux S. Role of NF-kappaB in flow-

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1 26. Doedens AL, Stockmann C, Rubinstein MP, Liao D, Zhang N, DeNardo DG, et al.

2 Macrophage expression of hypoxia-inducible factor-1 alpha suppresses T-cell function

3 and promotes tumor progression. Cancer Res 2010;70:7465-7475.

4 27. Grulich AE, van Leeuwen MT, Falster MO, Vajdic CM. Incidence of cancers in people

5 with HIV/AIDS compared with immunosuppressed transplant recipients: a meta-analysis.

6 Lancet 2007;370:59-67.

7 28. Wong G, Chapman JR. Cancers after renal transplantation. Transplant Rev (Orlando)

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8 2008;22:141-149.

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9 29. Ohsawa M, Naka N, Tomita Y, Kawamori D, Kanno H, Aozasa K. Use of

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immunohistochemical procedures in diagnosing angiosarcoma. Evaluation of 98 cases.
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11 Cancer 1995;75:2867-2874.
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 Table I Summary of published case reports of angiosarcoma at an arteriovenous fistula site

Study/ S Age Vasc- AVF Trans- Trans- Immuno- Present- Initial Time Time Time Metas- Treat- Re- Sur-

Refer- e (y) ular status plant- plant suppres- ation Dx to Dx from from tasis ment currence vival

ence # x acc- ation status sive drug VA to KT before

ess type AS to AS Tx

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1. Byers, M 36 RC- Func- DDKT Func- Azathio- Swelling Throm- 20 w 12 y 8y No Above- Post- 1y

pr
19924 AVF tion tional prine, botic elbow nasal

e-
Pr
2. Parrott, Cyclo- VA, ampu- space,

19935 sporin, infec- tation lung and

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Pred- tion axillary

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nisolone lymph

node

metas-

tasis

2.Wehrli, M 64 RC- Throm- DDKT Func- Aza- Swelling, Throm- 4w 10 y 7y No Radia- Local Alive

19986 AVF bosed tional thioprine, Pain botic tion and lung 6m
 Cyclo- VA fol- metas- after

sporine, lowed tasis amp-

Pred- by uta-

nisone below- tion

elbow

f
oo
ampu-

pr
tation

e-
Pr
3. F 47 AVF Throm- DDKT Func- Cyclo- Nail-bed Throm- 12 w 14 y 8y Bone, CMT − 2m

Alvarez, bosed tional sporine, splinter botic or lung

al
rn
20133 MMF lesions, aneur-

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pain, ysmal

hand VA

retraction

4. Conlon, M 40 BC- Throm- 1st Reject Treated Pain and NA 4w 40 y 16 y No Skeletal- Local 5m

19937 AVF bosed DDKT -ion for swelling ization recur-

(ligated rejection of the rence

 aneur- 2nd Func- Pred- 7y anterior and lung

ysm) DDKT tional nisolone, compart metas-

Azathio- -ment of tasis

prine, the arm/

pred- RT

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nisone

pr
5. Keane, M 11 AVF Throm- 1st Reject Treated Mass, NA 8w NS NS No Wide Local 8m

e-
19938

Pr
bosed DDKT -ion for indur- excision, recur-
7y
(ligated rejection ation RT rence,

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rn
aneur- 2nd Func- Cortico- arising lung and

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ysm) DDKT tional steroids, from brain

Aza- AVF metas-

thioprine tasis

6. Bessis, M 61 BC- Throm- DDKT Func- Azathio- Pain, Throm- NS 7y 5y No Above- Lung 7m

19989 AVF bosed tional prine, swelling, botic or (>6 elbow metas-

Cyclo- tender aneur- w) ampu- tasis

 sporin, ysmal tation

Pred- VA

nisolone

7. Farag, M 26 AVF Throm- 1st Non _ Mass Throm- NS 13 y NS Lung CMT Local 11 m

200510 bosed DDKT func- botic or recur-

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(ligated com- tional aneur- rence

pr
aneur- bined ysmal 11 y

e-
Pr
ysm) with VA

PT

al
rn
Func-
2nd
u Azathio-
Jo
tional
DDKT prine,

Cyclo-

sporin,

Pred-

nisolone
8.Webster, M 59 BC- Throm- LRKT Func- Ritu- Pain, Throm- NS 3y 2y No Above- No Sur-

201111 AVF bosed tional ximab swelling botic elbow vived

Dac- and ampu-

lizumab, infec- tation

Tacro- tion

f
oo
limus VA

pr
F 41 BC- Throm- LRKT Func- Alemtu- Pain Throm- 4w NS NS NS NS Lung 4m

e-
Pr
AVF bosed tional zumab, botic metas-

Pred- VA tasis

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rn
nisolone,

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Tacro-

limus

M 44 BC- NS DDKT Func- Pred- Pain, NS 12 w 13 y 8y NS NS Lung 6m

AVF tional nisolone, mass metas-

Tacro- tasis

limus,
 MMF

9. Demey, M 80 AVF Func- Non- Non- Non- KT Pain, Aneur- 36 w 4.5 y Non- Bone Sup- − 1m

201412 tional KT KT swelling ysmal KT portive

VA care

10. Roy, M 63 AVF Throm- KT Func- MMF, Mass Mass NS NS 6y Lung _ _ 10 d

f
oo
201813 bosed tional Tacro-

pr
(ligated limus

e-
Pr
aneur-

ysm)

al
rn
11. M 46 BC- Throm- LRKT Func- MMF, Pain, NS NS NS 3y Lung, CMT, − 6m

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Aldaabil, AVF bosed tional Tacro- swelling bone above-

201614 limus, elbow

Pred- ampu-

nisone tation
12. M 48 AVF Func- 1st KT Non- - Pain Infec- NS 3y NS No ‘high’ No 9m

tional func- tion above- recur- sur-

Qureshi, 2y
tional elbow rence or vived
15
2010
2nd Func- Pred- ampu- metas-

LRKT tional nisolone, tation tasis

f
oo
MMF,

pr
Ritux-

e-
Pr
imab,

IVIG,

al
rn
Tacro-

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limus

13. Costa, F 70 BC- Throm- KT Func- Tacro- Dyspnea Pneu- NS 11 y 9y Lung NS NS NS

201716 AVF bosed tional limus, monia

(ligated MMF,

aneur- Pred-

ysm) nisolone
14. M 57 RC- Throm- DDKT Reject No Pain, Granu- 8w 17 y NS NS Wide Local 11 m

Chanyaput AVF bosed -ion immunes- mass loma, excision, recur-

-hipong, (ligated uprreesiv infec- RT, rence,

201117 aneur- e drug tion CMT lung and

ysm) bone

f
oo
metas-

pr
tasis

e-
Pr
M 63 BC- Throm- Non- Non- Non- KT Pain, AVF 3w 9y Non- No Wide Lung 9.5 m

AVF bosed KT KT mass malfor- KT excision, metas-

al
rn
(aneur- with mation RT, tasis

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ysm) bleeding or AS CMT

15. Gale, M 44 RC- Func- DDKT NS NS Necrotic Hand NS 6y NS No Fore- NS NS

201718 AVF tional fingers is- quarter

chemia ampu-

tation

16. M 60 RC- Throm- DDKT Func- Pred- Bleeding NS NS 11 y 6y No Above- Lung 6m

Kakisis, AVF bosed tional nisolone, from elbow metas-

201919 Cyclo- AVF ampu- tasis

sporine, tation

MMF

17. M 58 NS NS DDKT Func- Yes (NS) Nodule, NS NS NS NS NS NS NS NS

f
oo
Jansen, tional pain,

pr
201320 hand

e-
Pr
dysfunc-

tion

al
rn
18. M 71 AVF NS LRKT Func- Alemtu- Edema, Cancer NS NS NS No Resec- NS NS

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Kleman tional zumab, ery- tion of

201621 Tacro- thema, AVF,

limus, Non- RT,

MMF PTH CMT

hyper-

calcemia
19. Andre, M 62 AVF Func- 1st KT Non- NS Non - NS NS NS NS Lung RT, NS NS

201222 tional func- healed CMT

tional wound at NS

2nd KT Func- Pred- AVF

tional nisone, removal

f
oo
Tacro- site

pr
limus,

e-
Pr
MMF

20. F 55 AVG Throm- KT Func- Tacro- Mass NS 8w NS 15 y Lung Pallia- − 4m

al
rn
Figueiredo bosed tional limus, tive

201923 u
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(ligated Pred- care,

aneur- nisolone,
CMT
ysm) MMF

21. M 58 BC- Throm- LRKT Func- Pred- Pain Throm- 24 w 5y 4y Lung, Should- − 1m

Present AVF bosed tional nisolone, botic bone er

case, 2020 Tacro- VA disartic-

 limus, ulation,

MMF CMT

Abbreviations: M male, F female, RCAVF radiocephalic arteriovenous fistula, NS not stated, DDKT deceased donor kidney transplantation,
LRKT living related kidney transplantation, PT pancreatic transplant, PTH parathyroid hormone, MMF mycophenolate mofetil, VA vascular
access, Dx diagnosis, AS angiosarcoma, CMT chemotherapy, RT radiation therapy, y year, w week, d day.

f
oo
pr
e-
Pr
al
u rn
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 Figure:

Figure 1
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Figure Legends:
Figure 1:
During intraoperative period found thrombosed of cephalic vein. After removing the clot showed
no mass and no abnormality in cephalic vein.

Figure 2:
X-ray of right elbow showed osteolytic lesion at ulna (white arrow)

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Figure 3:
CTA showed total thrombosis of cephalic vein of brachiocephalic AVF. No contrast filled in

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proximal radial and ulnar arteries at distal brachial to brachial bifurcation. There was an
eccentric filling defect at right brachial artery (white arrow).
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Figure 4:
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High grade pleomorphic epithelioid cells with amphophilic cytoplasm and vasoformative growth
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Figure 5:
Positivity to ERG immunohistochemistry as well as other vascular markers such as FLI1, CD31
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and CD34 (figure not shown), confirmed the diagnosis of angiosarcoma.

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