Vascular tumours

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This article covers soft tissue vascular tumours. Vascular malformations are covered in the vascular malformations article.

Normal histology

Normal blood vessel histology is dealt with in the vascular disease article.

Mimics

Distinct entities

Hemangioma

Hemangioma
External resources
EHVSC 10172

General

  • May be found in the liver.
    • Classically subcapsular.
      • May rupture and be life-threatening.[1]

Hemangiomas to remember - if you're only going remember a few:

  • Glomeruloid, infantile, caverous, capillary, arteriovenous, venous and intramuscular.

Childhood

Common childhood hemangiomas:[2]

Soft tissue

Several types are seen in soft tissue:[7]

  • Capillary.
  • Cavernous.
  • Arteriovenous.
  • Venous.
  • Intramuscular.
  • Synovial.

Microscopic

Features:

  • Channels lined by benign endothelium containing RBCs.

DDx:

Images

www:

IHC

  • CD31 +ve.
  • D2-40 -ve.[8]

Juvenile hemangioma:[5]

  • GLUT-1 +ve.

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SUBCUTANEOUS NECK LESION, LEFT, EXCISION:
- CAVERNOUS HEMANGIOMA.
- NEGATIVE FOR MALIGNANCY.
LESION, LEFT SIDE OF FACE, EXCISION:
- CAPILLARY HEMANGIOMA.
- NEGATIVE FOR MALIGNANCY.

Micro

The sections show hair-bearing skin with abundant small superficial vascular channels containing red blood cells. The endothelial cells of the vascular channels do not have atypia. No mitotic activity is appreciated. The overlying epidermis is unremarkable. Extensive solar elastosis is present. No nevus is identified.

Lymphangioma

General

Treatment:

  • Surgical excision.

Microscopic

Features:[10][9]

  • Thin-walled channels lined by endothelium.
  • +/-Eosinophilic intraluminal material.
  • +/-Clusters of intraluminal lymphocytes.
  • +/-Occasional RBCs.

DDx:

Images:

IHC

Features:[11][8]

  • D2-40 +ve.

Kaposi sarcoma

Masson hemangioma

  • AKA intravascular papillary endothelial hyperplasia.[12]
  • AKA Masson tumor.[13]

General

  • Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
  • Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.[12]

Microscopic

Features:

  • Well-circumscribed - key (low power) feature.
  • Abundant small vascular channels with benign endothelium.
  • +/-Papillary formation with a fibrotic core covered by benign endothelium.[14]

Notes:

Images:

Angiosarcoma

Kaposiform hemangioendothelioma

General

  • Locally aggressive.[15]
  • Childhood tumour.[16]
  • Approximately half have Kasabach–Merritt phenomenon[16] = vascular tumour --> coagulopathy.

Microscopic

Features:[17]

  • Spindle cells lesions in sheets or nodules.
  • +/-Round tumour nodules - "cannon ball" appearance.

DDx:

IHC

Features:[17]

  • Vimentin +ve.
  • C31 +ve.
  • CD34 +ve.
  • UEA-1 lectin +ve.

Epithelioid hemangioendothelioma

General

  • Malignant.[15]
  • Adults - wide age range.
  • Associated with oral contraceptives, vinyl chloride.[18]

Microscopic

Features:[18]

  • Large epithelioid perivascular cells with:
    • Abundant pale eosinophilic cytoplasm.
    • Cytoplasmic vacuolation (some cells) - AKA "blister cells" - key feature.
      • May form lumen and have RBC within.
    • Vesicular nucleus with prominent nucleolus in some cells.
  • Tuft-like projections into capillaries.
  • Tumour cells may be in well-circumscribed paucicellular nodules or more cellular poorly formed aggregates.

DDx:

Images

www:

IHC

Features:[18]

  • CD31 +ve.
  • CD34 +ve.
  • Factor VIII +ve.

See also

References

  1. Vokaer, B.; Kothonidis, K.; Delatte, P.; De Cooman, S.; Pector, JC.; Liberale, G.. "Should ruptured liver haemangioma be treated by surgery or by conservative means? A case report.". Acta Chir Belg 108 (6): 761-4. PMID 19241936.
  2. Prieto VG, Shea CR (July 1999). "Selected cutaneous vascular neoplasms. A review". Dermatol Clin 17 (3): 507–20, viii. PMID 10410855.
  3. Uthup S, Balachandran K, Ammal VA, et al. (August 2006). "Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma". Am. J. Kidney Dis. 48 (2): e17–24. doi:10.1053/j.ajkd.2006.04.089. PMID 16860182.
  4. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 618. ISBN 978-0781765275.
  5. 5.0 5.1 North, PE.; Waner, M.; Mizeracki, A.; Mihm, MC. (Jan 2000). "GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas.". Hum Pathol 31 (1): 11-22. PMID 10665907.
  6. Dadras, SS.; North, PE.; Bertoncini, J.; Mihm, MC.; Detmar, M. (Sep 2004). "Infantile hemangiomas are arrested in an early developmental vascular differentiation state.". Mod Pathol 17 (9): 1068-79. doi:10.1038/modpathol.3800153. PMID 15143338.
  7. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 602. ISBN 978-0781765275.
  8. 8.0 8.1 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.
  9. 9.0 9.1 9.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 12. ISBN 978-0781765275.
  10. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 489. ISBN 978-0781765275.
  11. Kalof, AN.; Cooper, K. (Jan 2009). "D2-40 immunohistochemistry--so far!". Adv Anat Pathol 16 (1): 62-4. doi:10.1097/PAP.0b013e3181915e94. PMID 19098468.
  12. 12.0 12.1 Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP (2005). "Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma". Anticancer Res. 25 (2B): 1409–12. PMID 15865098.
  13. URL: http://path.upmc.edu/cases/case544/dx.html. Accessed on: 25 January 2012.
  14. URL: http://path.upmc.edu/cases/case544.html. Accessed on: 25 January 2012.
  15. 15.0 15.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 603. ISBN 978-0781765275.
  16. 16.0 16.1 Lyons, LL.; North, PE.; Mac-Moune Lai, F.; Stoler, MH.; Folpe, AL.; Weiss, SW. (May 2004). "Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.". Am J Surg Pathol 28 (5): 559-68. PMID 15105642.
  17. 17.0 17.1 17.2 Miller, K. (Mar 1991). "Sister-chromatid exchange in human B- and T-lymphocytes exposed to bleomycin, cyclophosphamide, and ethyl methanesulfonate.". Mutat Res 247 (1): 175-82. PMID 1706068. http://www.nature.com/modpathol/journal/v14/n11/full/3880441a.html.
  18. 18.0 18.1 18.2 Gupta, R.; Mathur, SR.; Gupta, SD.; Durgapal, P.; Iyer, VK.; Das, CJ.; Shalimar, SK.; Acharya, . (2010). "Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology.". Cytojournal 6: 25. doi:10.4103/1742-6413.58951. PMID 20165548.