Angiosarcoma

Angiosarcoma
	Diagnosis in short
	; Epithelioid angiosarcoma. H&E stain.
LM	atypical cells - usu. spindle cells, occasionally epithelioid; vascular differentiation (abundant capillaries - "red" at low power, +/-cytoplasmic vacuolization, +/-hobnail endothelial cells)
LM DDx	Kaposi sarcoma, anastomosing hemangioma, other vascular tumours
IHC	CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve
Molecular	+/-MYC amplification
Gross	red lesion[citation needed]
Site	skin, head and neck, elsewhere
Syndromes	Stewart–Treves syndrome
Clinical history	+/-chronic lymphedema, vinyl chloride exposure (liver angiosarcoma)
Prevalence	uncommon
Prognosis	poor

Angiosarcoma is an uncommon malignant vascular tumour.

General

Malignant tumour - general has a poor prognosis.^[1]

Epidemiology:

May arise secondary to chronic lymphedema related to breast carcinoma.
- Known as Stewart–Treves syndrome.^[2]
Association with radiation - breast,^[3] urinary bladder.^[4]
Liver angiosarcomas are associated with vinyl chloride exposure.^[5]
Cutaneous angiosarcomas are classically seen on the head and neck of whites over 60 years old.^[6]

Gross

Red/dark tan lesion.
Typically poorly circumscribed.

Angiosarcoma. (WC/Yale Rosen)

Microscopic

Features:

Spindle cell lesion.
- Occasionally an epithelioid lesion.
Very many small capillaries of irregular shape lined with:
- Pleomorphic nuclei - important.
  - May have hobnail morphology.
- Usually "red" at low power - due to many RBCs - important.
Mitoses.
Cytoplasmic vacuoles.
- Cells trying to form lumina - embryologic.

Notes:

Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble melanoma or a carcinoma (such as hepatocellular carcinoma or urothelial carcinoma^[7]).

DDx:

Atypical vascular lesion.
Kaposi sarcoma.
Anastomosing hemangioma - especiallly in the kidney.
Poorly differentiated carcinoma, e.g. urothelial carcinoma.
Other vascular tumours.

Images

Case 1

Epithelioid angiosarcoma - very low mag. (WC/Nephron)
Epithelioid angiosarcoma - intermed mag. (WC/Nephron)
Epithelioid angiosarcoma - very high mag. (WC/Nephron)
Epithelioid angiosarcoma - CD31 - intermed. mag. (WC/Nephron)

Case 2

Angiosarcoma (kidney) - low mag.
Angiosarcoma (kidney) - low mag.
Angiosarcoma (kidney) - high mag.
Angiosarcoma (kidney) - very high mag.
Angiosarcoma (kidney) - low mag.
Angiosarcoma (kidney) - low mag.
Angiosarcoma (kidney) - high mag.

Additional

Breast - Epithelioid angiosarcoma - high power (SKB)
Breast - Epithelioid angiosarcoma - high power (SKB)
Breast - Epithelioid angiosarcoma - medium power (SKB)
Breast - Angiosarcoma - high grade - high power (SKB)
Breast - Angiosarcoma - high grade - high power (SKB)
Breast - Angiosarcoma - high grade - high power (SKB)
Breast - Angiosarcoma - high power (SKB)
Breast - Angiosarcoma - low grade area - encircling fat - high power (SKB)
Breast - Angiosarcoma - high power (SKB)
Breast - Angiosarcoma - high grade area - high power (SKB)
Breast - Angiosarcoma - high power (SKB)
Breast - Angiosarcoma - high power (SKB)
Breast - Angiosarcoma - low power (SKB)
Breast - Angiosarcoma - Medium power (SKB)
Breast - Angiosarcoma - low power (SKB)

IHC

CD34 +ve.
CD31 +ve.
ERG +ve ^[8]
FLI-1 +ve.^[9]
D2-40 +ve/-ve.^[10]
HHV-8 -ve.

Molecular

Amplification of MYC^[11] - especially in secondary angiosarcoma.^[12]

References

↑ Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (May 2010). "Angiosarcoma". Lancet Oncol. doi:10.1016/S1470-2045(10)70023-1. PMID 20537949.
↑ Pincus, LB.; Fox, LP. (Aug 2008). "Images in clinical medicine. The Stewart-Treves syndrome.". N Engl J Med 359 (9): 950. doi:10.1056/NEJMicm071344. PMID 18753651. http://www.nejm.org/doi/full/10.1056/NEJMicm071344.
↑ Chikarmane, SA.; Gombos, EC.; Jagadeesan, J.; Raut, C.; Jagannathan, JP. (Dec 2014). "MRI findings of radiation-associated angiosarcoma of the breast (RAS).". J Magn Reson Imaging. doi:10.1002/jmri.24822. PMID 25504856.
↑ Bahouth, Z.; Masarwa, I.; Halachmi, S.; Nativ, O. (2015). "Primary angiosarcoma of urinary bladder: 13th reported patient.". Case Rep Oncol Med 2015: 652870. doi:10.1155/2015/652870. PMID 25688314.
↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 212. ISBN 978-1416054542.
↑ Albores-Saavedra, J.; Schwartz, AM.; Henson, DE.; Kostun, L.; Hart, A.; Angeles-Albores, D.; Chablé-Montero, F. (Apr 2011). "Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007.". Ann Diagn Pathol 15 (2): 93-7. doi:10.1016/j.anndiagpath.2010.07.012. PMID 21190880.
↑ Matoso A, Epstein JI (October 2015). "Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases". Am J Surg Pathol 39 (10): 1377–82. doi:10.1097/PAS.0000000000000444. PMID 25929352.
↑ Miettinen, M.; Wang, ZF.; Paetau, A.; Tan, SH.; Dobi, A.; Srivastava, S.; Sesterhenn, I. (Mar 2011). "ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma.". Am J Surg Pathol 35 (3): 432-41. doi:10.1097/PAS.0b013e318206b67b. PMID 21317715.
↑ Rossi, S.; Orvieto, E.; Furlanetto, A.; Laurino, L.; Ninfo, V.; Dei Tos, AP. (May 2004). "Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody.". Mod Pathol 17 (5): 547-52. doi:10.1038/modpathol.3800065. PMID 15001993.
↑ Kahn, HJ.; Bailey, D.; Marks, A. (Apr 2002). "Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas.". Mod Pathol 15 (4): 434-40. doi:10.1038/modpathol.3880543. PMID 11950918.
↑ Kurisetty, V.; Bryan, BA. (Apr 2013). "Aberrations in Angiogenic Signaling and MYC Amplifications are Distinguishing Features of Angiosarcoma.". Angiol Open Access 1. doi:10.4172/2329-9495.1000102. PMID 25374893.
↑ Styring, E.; Seinen, J.; Dominguez-Valentin, M.; Domanski, HA.; Jönsson, M.; von Steyern, FV.; Hoekstra, HJ.; Suurmeijer, AJ. et al. (Jul 2014). "Key roles for MYC, KIT and RET signaling in secondary angiosarcomas.". Br J Cancer 111 (2): 407-12. doi:10.1038/bjc.2014.359. PMID 24983371.

[pmid20537949-1] Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (May 2010). "Angiosarcoma". Lancet Oncol. doi:10.1016/S1470-2045(10)70023-1. PMID 20537949.

[pmid18753651-2] Pincus, LB.; Fox, LP. (Aug 2008). "Images in clinical medicine. The Stewart-Treves syndrome.". N Engl J Med 359 (9): 950. doi:10.1056/NEJMicm071344. PMID 18753651. http://www.nejm.org/doi/full/10.1056/NEJMicm071344.

[pmid25504856-3] Chikarmane, SA.; Gombos, EC.; Jagadeesan, J.; Raut, C.; Jagannathan, JP. (Dec 2014). "MRI findings of radiation-associated angiosarcoma of the breast (RAS).". J Magn Reson Imaging. doi:10.1002/jmri.24822. PMID 25504856.

[pmid25688314-4] Bahouth, Z.; Masarwa, I.; Halachmi, S.; Nativ, O. (2015). "Primary angiosarcoma of urinary bladder: 13th reported patient.". Case Rep Oncol Med 2015: 652870. doi:10.1155/2015/652870. PMID 25688314.

[5] Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 212. ISBN 978-1416054542.

[pmid-6] Albores-Saavedra, J.; Schwartz, AM.; Henson, DE.; Kostun, L.; Hart, A.; Angeles-Albores, D.; Chablé-Montero, F. (Apr 2011). "Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007.". Ann Diagn Pathol 15 (2): 93-7. doi:10.1016/j.anndiagpath.2010.07.012. PMID 21190880.

[pmid25929352-7] Matoso A, Epstein JI (October 2015). "Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases". Am J Surg Pathol 39 (10): 1377–82. doi:10.1097/PAS.0000000000000444. PMID 25929352.

[8] Miettinen, M.; Wang, ZF.; Paetau, A.; Tan, SH.; Dobi, A.; Srivastava, S.; Sesterhenn, I. (Mar 2011). "ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma.". Am J Surg Pathol 35 (3): 432-41. doi:10.1097/PAS.0b013e318206b67b. PMID 21317715.

[pmid15001993-9] Rossi, S.; Orvieto, E.; Furlanetto, A.; Laurino, L.; Ninfo, V.; Dei Tos, AP. (May 2004). "Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody.". Mod Pathol 17 (5): 547-52. doi:10.1038/modpathol.3800065. PMID 15001993.

[pmid11950918-10] Kahn, HJ.; Bailey, D.; Marks, A. (Apr 2002). "Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas.". Mod Pathol 15 (4): 434-40. doi:10.1038/modpathol.3880543. PMID 11950918.

[pmid25374893-11] Kurisetty, V.; Bryan, BA. (Apr 2013). "Aberrations in Angiogenic Signaling and MYC Amplifications are Distinguishing Features of Angiosarcoma.". Angiol Open Access 1. doi:10.4172/2329-9495.1000102. PMID 25374893.

[pmid24983371-12] Styring, E.; Seinen, J.; Dominguez-Valentin, M.; Domanski, HA.; Jönsson, M.; von Steyern, FV.; Hoekstra, HJ.; Suurmeijer, AJ. et al. (Jul 2014). "Key roles for MYC, KIT and RET signaling in secondary angiosarcomas.". Br J Cancer 111 (2): 407-12. doi:10.1038/bjc.2014.359. PMID 24983371.

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Angiosarcoma

Contents

General

Gross

Microscopic

Images

Case 1

Case 2

Additional

IHC

Molecular

See also

References

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Angiosarcoma
Diagnosis in short
Epithelioid angiosarcoma. H&E stain.

LM	atypical cells - usu. spindle cells, occasionally epithelioid; vascular differentiation (abundant capillaries - "red" at low power, +/-cytoplasmic vacuolization, +/-hobnail endothelial cells)
LM DDx	Kaposi sarcoma, anastomosing hemangioma, other vascular tumours
IHC	CD31 +ve, FLI-1 +ve, CD34 +ve, HHV-8 -ve
Molecular	+/-MYC amplification
Gross	red lesion^{[citation needed]}
Site	skin, head and neck, elsewhere

Syndromes	Stewart–Treves syndrome

Clinical history	+/-chronic lymphedema, vinyl chloride exposure (liver angiosarcoma)
Prevalence	uncommon
Prognosis	poor