Vascular tumors

Published on 08/03/2015 by admin

Filed under Dermatology

Last modified 22/04/2025

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Chapter 23

Vascular tumors

Dirk M. Elston

Angiokeratoma

Key features

• Hyperkeratosis

• Acanthosis

• Ectatic thin-walled vessels in contact with the epidermis

• Resembles a “bloody seborrheic keratosis”

Fig 23-1 Angiokeratoma

Lymphangioma

Key features

• “Frog spawn” clinically

• D2-40+

Fig 23-2 Lymphangioma

Nevus flammeus

Key features

• Dilated capillary-sized vessels

Fig 23-3 Nevus flammeus

Angioma serpiginosum

Key features

• Dilated tortuous capillaries in dermal papillae and the upper dermis

• Vessels lack alkaline phosphatase activity

Fig 23-4 Angioma serpiginosum

Angioma serpiginosum presents as a progressive vascular lesion on a woman’s leg. The ectatic vessels begin as minute puncta in clusters, but merge to form a serpiginous array. They often bleed freely when traumatized.

Venous lake

Key features

• Irregular, thin-walled, ectatic vessel

• Usually collapses after biopsy

Fig 23-5 Venous lake

Venous lakes are common on the lips and ears of older patients. They may appear very dark, but blanch easily when compressed.

Glomus tumor

Key features

• Rows of round dark nuclei with little cytoplasm (“string of black pearls”)

• Glomus cells surround delicate vascular spaces

• Commonly tender

Fig 23-6 Glomus tumor

Pearl

• Normal glomus bodies are found on the sides of digits. A large physiologic glomus coccygeum is present in the sacral area

• Glomus cells are modified smooth muscle cells (SMA+/CD31−)

• In general, vascular smooth muscle stains reliably with vimentin and smooth muscle actin, but not with desmin.

Glomangioma

Key features

• Commonly multiple

• One to two layers of glomus cells around prominent vessels

Fig 23-7 Glomangioma

The vessels usually have thicker walls than the vessels in a glomus tumor. Glomangiomas have been described as vascular malformations with a few glomus cells, whereas glomus tumors have been described as tumors of glomus cells surrounding inconspicuous vessels.

Pyogenic granuloma

Key features

• Eruptive lobular capillary hemangioma

• Early lesions show solidly packed endothelial cells

• Later lesions show more ectatic vessels, erosion, and crusting

• Epidermal collarette common

• Commonly impetiginized on surface

Fig 23-8 Pyogenic granuloma

Bacillary angiomatosis

Key features

• Usually not distinctly lobular

• Clusters of neutrophils within lesion

• Amphophilic collections of organisms

Fig 23-9 Bacillary angiomatosis

Differential Diagnosis

Pyogenic granulomas are distinctly lobular. Although surface crusting is common in pyogenic granulomas, they lack the deep clusters of neutrophils that characterize bacillary angiomatosis.

Cherry angioma

Key features

• Capillary hemangioma

• Pink hyalinized vessel walls

Fig 23-10 Cherry angioma

Infantile hemangioma

Key features

• Early lesions show solidly packed endothelial cells

• Later lesions show more ectatic vessels

• Glucose transporter 1 (GLUT1)+

Fig 23-11 Infantile hemangioma

Differential Diagnosis

Rapidly involuting congenital hemangioma (RICH) and non-involuting congenital hemangioma (NICH) are fully grown at birth and are GLUT1 negative.

Angiolymphoid hyperplasia with eosinophilia

Key features

• Central thick-walled vessels with hobnail endothelium

• Peripheral proliferation of smaller vessels

• Nodular lymphoid aggregates with eosinophils

Fig 23-12 Angiolymphoid hyperplasia with eosinophilia

Subtypes include histiocytoid and epithelioid hemangioma. Histiocytoid endothelial cells are common.

Kimura’s disease

Fig 23-13 Kimura’s disease (Courtesy of James Fitzpatrick, MD)

Intravascular papillary endothelial hyperplasia of Masson (IPEH)

Key features

• Recanalizing thrombus within a vascular space

• Fibrin in thrombus

• Papillary projections with hyalinized cores

IPEH can occur in any vascular space. It is common in angiokeratomas.

Fig 23-14 Intravascular papillary endothelial hyperplasia

Arteriovenous malformation (arteriovenous hemangioma)

Key features

• Thick- and thin-walled vessels

• Thick-walled vessels tend to be central

Fig 23-15 Arteriovenous malformation

Targetoid hemosiderotic hemangioma (hobnail hemangioma)

Key features

• Central superficial dilated vessels with hobnail nuclei

• Peripheral proliferation of small vessels

• Peripheral vascular proliferation tends to surround pre-existing vessels and adnexae (like Kaposi’s sarcoma)

• Hemosiderin

Fig 23-16 Targetoid hemosiderotic hemangioma

Targetoid hemosiderotic hemangiomas probably arise as a result of trauma to a pre-existing hemangioma.

Eccrine angiomatous hamartoma

Key features

• Discrete lobules

• Each composed of capillaries, mature eccrine glands, and ducts

Fig 23-17 Eccrine angiomatous hamartoma

Usually a solitary bluish nodule. Often involves acral sites, although the example shown was on the back. May be tender.

Glomeruloid hemangioma

Key features

• Capillary loops within a dilated vascular space, resembling a glomerulus

• Sequestered degenerating erythrocytes

Fig 23-18 Glomeruloid hemangioma

Glomeruloid hemangioma is associated with POEMS syndrome (Crow–Fukase syndrome, polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) and Castleman’s disease. Two types of endothelial cell have been noted: cells with large vesicular nuclei, an open chromatin pattern, and large amount of cytoplasm, and a second population with small basal nuclei, a dense chromatin pattern, and scant cytoplasm. Lesions not associated with POEMS syndrome have been referred to as papillary hemangioma.

Microvenular hemangioma

Key features

• Monomorphous, elongated blood vessels with small lumens

• Surrounding pericytes

Fig 23-19 Microvenular hemangioma

Sometimes occurs in POEMS syndrome.

Tufted angioma (angioblastoma)

Key features

• “Cannonball” tufts of capillary-sized vessels in the dermis

• Slowly expanding plaque clinically, often on the shoulder of a child

Fig 23-20 Tufted angioma

Differential Diagnosis

• Glomeruloid hemangioma has sequestered degenerating erythrocytes and tufts protruding into a crescent-like space

• Dermal pyogenic granuloma has fibrous septae with ropey collagen between lobules

Kaposiform hemangioendothelioma

Key features

• Large lobules

• Less well circumscribed than tufted angioma

• Involves deeper tissues

• Spindled endothelial cells

• Slit-like, crack-like and staghorn vascular spaces

• Deep extension to soft tissue and bone

Fig 23-21 Kaposiform hemangioendothelioma

Kasabach–Merritt coagulopathy is usually associated with kaposiform hemangioendothelioma or tufted angioma, and hybrid tumors have been described.

Pearl

Kaposiform hemangioendothelioma shows CD34 staining restricted to luminal endothelial cells, tufted angiomas show a proliferation of CD34-positive endothelial cells with few actin-positive cells, and infantile hemangiomas show actin-positive cells outnumbering CD34-positive cells.

Hemangiopericytoma

Key features

• Endothelial-lined vessels surrounded by a proliferation of pericytes

• Concentric or curlicue pattern of spindle cells

• Stag-horn ectatic vascular spaces

• Most represent examples of solitary fibrous tumor with stag-horn vessels

Fig 23-22 Hemangiopericytoma

Hemangiopericytomas occur in the skin and soft tissues. It may be difficult to distinguish between benign and malignant hemangiopericytoma histologically. Large size and higher mitotic rate suggest a malignant potential. Infantile tumors are typically cutaneous, with a good prognosis.

Spindle cell hemangioma (spindle cell hemangioendothelioma)

Key features

• At scanning power, resembles “hemorrhagic lung” with alveolar spaces

• Solid areas of spindle cells

• Phleboliths

Fig 23-23 Spindle cell hemangioendothelioma. (a) Arrow points to Phlebolith

Epithelioid hemangioendothelioma

Key features

• Dilated vascular channels with solid epithelioid and spindle cell areas

• Intracytoplasmic lumens

• Variable pleomorphism and mitotic activity

Fig 23-24 Epithelioid hemangioendothelioma

Epithelioid hemangioendotheliomas tend to occur on the extremities of young people. They are best regarded as low-grade malignancies.

Retiform hemangioendothelioma

Key features

• Arborizing blood vessels reminiscent of rete testis

Fig 23-25 Retiform hemangioendothelioma

Retiform hemangioendothelioma is a low-grade angiosarcoma that occurs mostly on the extremities of young adults.

Angiosarcoma

Key features

• Crack-like spaces between collagen bundles

• Spaces lined by hyperchromatic endothelial cells

• Nodular areas commonly epithelioid with more pronounced atypia

Fig 23-26 Angiosarcoma

Fig 23-27 Diffuse dermal angiomatosis

Angiosarcomas typically appear as bruise-like lesions on the forehead or scalp of an older patient. Epithelioid variants may be nodular. Stewart–Treves syndrome is angiosarcoma in the setting of a lymphedematous limb. Often, there is a history of radiation therapy.

Pearl

Markers of angiosarcoma:

• Factor VIII (unreliable)

• CD34 (clean stain, little background, not specific)

• CD31 (very specific, but background staining common)

• Ulex europeus lectin (clean staining, marks endothelium and epithelium)

• D2-40+

• MYC is amplified in post-radiation angiosarcoma, but not in atypical vascular lesion (AVL) occurring after radiation therapy.

Differential Diagnosis

Diffuse dermal angiomatosis is an acquired benign vascular proliferation in response to stasis or ischemia. It occurs in association with arteriovenous fistulae or in large pendulous breasts. The patients are often heavy smokers. It is characterized by poorly circumscribed, violaceous plaques with frequent ulceration. New vessels dissect between collagen bundles, but atypia and mitoses are absent.

Kaposi’s sarcoma

Key features

• HHV-8 associated

• Patch, plaque, and tumor stages

Early-patch-stage Kaposi’s sarcoma

Key features

• Bizarre stag-horn ectatic lymphatic-like vessels

• Plasma cells

Fig 23-28 Early-patch Kaposi’s sarcoma

Later-patch/plaque Kaposi’s sarcoma

Key features

• Busy dermis surrounding adnexal structures and pre-existing vessels

• Pale appearance of busy area (appears understained)

• Promontory sign

Fig 23-29 Plaque Kaposi’s sarcoma

Plaque lesions of Kaposi’s sarcoma are characterized by “vascular wrapping.” New vessels wrap and surround pre-existing vascular and adnexal structures. The pre-existing structure commonly protrudes into a lake-like ectatic space (promontory sign).