TUMORS OF BLOOD VESSELS – Medium Robbins

BENIGN Tumors:
1) Vascular Ectasis:
Ectasia  Local Dilation
Telengactasia Permanent Dilation
“ Vessels forming discrete Red Lesions”

 Nevus Flammeus : Birthmark

Pink – Purple Flat Lesion on Head/ neck composed of dilated vessels
Port Wine Stain: A form of Nevus Flammeus
Grow During Childhood, Thicken Skin Surface , doesn’t regress, associated with mental
retardation, seizures, hemiplegia

 Spider telengectasia: Dilated Subcut arteries on face, neck , upper chest

Associated with hyperestrogenic state
 Hereditary hemorrhagic telengectasia: Autosomal Dominant
Rupture  Epistaxis, GIT bleed, hematuria

2)Hemangiomas:
Tumors Composed of Endothelium lined Blood Filled Vessels
 Capillary Hemangioma
Skin, Sub cut tissues, Mucous membrane oral cavity, liver, spleen,kidneys
Thin walled Capillaries with scant stroma
 Juvenile hemangioma
 Strawberry Hemangioma
 Grow in first months, fade 1-3 yrs of age, regress at 7
 Pyogenic granuloma
 Red Pedunculated lesion on skin, gingiva and oral mucosa
 Granulation Tissue
 Form after trauma, can bleed and ulcerate
 Cavernous Hemangioma
 Large Dilated Vascular Channels
 Infiltrative, do not regress
 Unencapsulated sharply defined mass
 3) Lymphangioma:
 Capillary Lymphangioma
 1-2 cm, slightly elevated lesions on head neck or axilla
 Endothelium lined spaces, No blood cells
 Cavernous Lymphangioma (Cystic Hygroma)
 Lymphatic Spaces lined by ECs
 CT stroma containing Lymphoid Aggregates

 Glomus Tumor:
 Glomus Bodies: Arteriovenous Structures in Thermoregulation
 Tumors arise from SMCs of Glomus Bodies
 Distal part of digits
  Bacillary Angiomatosis:
 Immunocompromised individuals  Invaded by Gram Negative Bacilli  Bacillary
Angiomatosis

 Proliferation of Capillaries lined by epithelioid ECs

INTERMEDIATE Tumors:
1) Kaposi Sarcoma Vascular Sarcoma due to KSHV i.e Kaposi Sarcoma Herpes Virus /HHV 8

Kaposi Sarcoma

Endemic African Transplantation

Classic KS AIDS Associated
KS associated

Red/Purple Lymph nodes,

Skin Plaques Mucosa, Viscera

Pathogenesis:
Kaposi Sarcoma

KSHV Protein
G Protein

VEGF Production 1) Disrupt Normal Cell Proliferation 2) Inhibit p53

↓ ↓
↓
EC Growth Inhibit Apoptosis
Latent Infection
↓ ↓
↑ Cell Proliferation Latent Infection
Morphology:

Patch: Pink/ Red Macules

--> Dilated Irregular Blood Vessels lined by ECs
-->Chronic Inflammatory Cells

Plaques: Violaceous Raised

--> Dilated Vascular Channels surrounded by Spindle Cells
--> Hemosiderin laden Macrophages, Red Cells
Extravasation

Nodules: Composed of Spindle Cells

in Dermis
Hemorrhage and Hemosiderin Common

2) Hemangioedothelioma : Epithelial hemangioendothelioma  Venous Tumor

MALIGNANT Tumors:
1) Angiosarcoma:
*Ipsilateral Upper extremity after radical mastectomy
 Occurs after lymphedema

MORPHOLOGY:
Small Sharply Demarcated Red Nodules
↓
Large Fleshy Red to gray masses

 ECs Forming Vascular Channels to Spindle Cell Tumors without Blood Vessels