PBLOOD VESSELS

TUMOR and TUMOR-LIKE CONDITIONS of BLOOD and LYMPH VESSEL HEMANGIOMA Benign but can become very large and unsightly Can be fatal if they affect vital structures Almost never become malignant High percentage occur in children and many are already present at birth Over half of cases are in the head and neck area May also occur in the trunk and extremities Most are solitary When multiple (with or without associated lesions in then known as (multifocal) angiomatosis) Lesions with dilated, interconnecting thin walled channels with occasional pseudocapillary projections are designated as sinusoidal hemangiomas

LARGE VESSEL HEMANGIOMA Composed of vessels with structure of veins (venous hemangiomas) or combination of veins and arteries ( racemose, cirsoid, or arterio-venous hemangiomas) Structure of vessel wall is often abnormal and not easily identifiable as arterial or venous They occur in the back, gluteal region, thigh and sometimes entire extremity is involved Thrombosis and calcification are common

Classified according to their clinical appearance and caliber of the vessel involved CAPILLARY HEMANGIOMA Small vessels of capillary caliber Can occur in any organ Benign Hemangioendothelioma or Juvenile or Hyperplastic hemangioma is the most common and distinctive variant Most common location is the skin appearing as elevated nodule with intense crimson color Usually present at birth or appears during the first month and enlarges rapidly during the first few months of life but regresses Outer locations include salivary gland and breast MICROSCOPICALLY shows vaguely lobulated configuration with masses of closely packed spindle cells seen with neoformed spaces containing little blood o There is also component of pericytes & fibroblasts o Mitotic figures are usually present o At the periphery, tumor may be seen invading subcutaneous tissue or skeletal muscle o Mast cells may be numerous

SKELETAL MUSCLE (Intramuscular) HEMANGIOMA: Usually have a venous or cavernous microscopic appearance They are very cellular with plump nuclei, mitotic figures, intraluminal papillary projections and even infiltration of the perineurial spaces

GLOMUS TUMOR Also known as Glomangioma originates in the neuromyoarterial glomus, a normal arteriorvenous shunt abundantly supplied with nerve fibers and fulfilling a temperature-regulating function. Classical location is the subungual region, but it can also occur elsewhere in the skin, soft tissues (flexor surfaces of the arms and about the knee), nerves, stomach, nasal cavity and trachea Subungal regions are supplied by numerous nerve fibers and exquisitely painful It may erode the terminal phalanx or even present as an intraosseous lesion in this location Superficial lesions are well circumscribed In children, it tends to be multiple and of an infiltrative nature Microscopically consist of blood vessels lined by normal endothelial cells surrounded by a solid proliferation of round or cuboidal epithelioid cells with perfectly round nuclei and acidophilic cytoplasm 3 microscopic types recognized: solid, angiomatous and myxoid Behaves in aggressive fashion, with local recurrences and invasion of adjacent structures

CAVERNOUS HEMANGIOMA Composed of larger vessels with cystically dilated lumina and thin walls Those occurring in the skin are traditionally known as Portwine nevus or nevus flammeus Present at birth and grows very slowly It becomes nodular and soft It does not regress spontaneously Larger lesions may undergo thrombosis, ulceration and infection

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PBLOOD VESSELS
HEMANGIOPERICYTOMA Less organoid type of glomus tumor arising from Zimmerma;s pericytes Occur principally in adults and is often deep-seated Orbit is another common location Surgical excision can be difficult because of profuse bleeding Grossly always solitary and solid with smooth surf ace with areas of hemorrhage, necrosis, and cystic degeneration Tumor is well circumscribed or encapsulated Microscopically cells are oval to spindle, and have close relationship with blood vessels Separated from the normal appearing endothelial cells by layer of basement membrane mixed with collagen fibers Vessels are branching and exhibit characteristic staghorn configuration Secondary features include extensive areas of fibrosis and hyalinization and myxoid changes dermis and subcutaneous tissue of distal extremities and combines histologically features of cavernous hemangioma and Kaposi sarcoma Development of recurrences or new lesions is common but metastases have only been documented minimally following repeated recurrences and radiation therapy

MALIGNANT ENDOVASCULAR PAPILLARY ANGIOENDOTHELIOMA (Dabskas Tumor) Extremely rare but distinct tumor of childhood located in the skin or soft tissues Characterized by papillary tufts lined by plump endothelial cells located within dilated vascular lumina, some with glomeruloid configuration Many of the tumor cells have epithelioid or histiocytoid features including cytoplasmic eosinophilia and vacoulation Has a uniformly good prognosis though nodal metastases have occurred

HEMANGIOENDOTHELIOMA Vascular tumor of an endothelial nature that occupy an intermediate position between the benign hemangioma and a full-blown angiosarcoma

ANGIOSARCOMA Malignant neoplasm arising from the endothelial cells of blood vessels (malignant hemangioendothelioma) Usually seen in adults Most common sites are the skin, soft tissue, breast, bone, liver and spleen Some soft tissue angiosarcoma arise from major vessels (inferior vena cava, pulmonary or aorta) Tend to have a very undifferentiated appearance and a solid pattern of growth Have been reported in previously irradiated fields Grossly tend to be highly hemorrhoidal and invasive Microscopic appearance ranges from well differentiated as to simulate a benign hemangioma to undifferentiated and solid as carcinoma, malignant melanoma or other types of sarcoma Diagnostic areas are represented by freely anastomosing vascular channels lined by atypical endothelial cells Neoplastic cells range from very elongated to plump and epithelioid and size vary from small to giant with occasional development of multinucleated forms

EPITHELIOID (histiocytoid) HEMANGIOENDOTHELIOMA Tumor composed of distinctive type of endothelial cells having an epithelia-like or histiocyte-like appearance Cytoplasm is abundant and eosinophilic often vacuolated Nucleus is round, vesicular, and occasionally indented Vascular lumina are present, most are small Mitoses, pleomorphism and necrosis are variable but usually scanty or absent Inflammatory infiltrate is often present at the periphery Tumors with these set of features are found in large numbers of sites such as the skin, bone, lung, pleura, liver, peritoneum and lymph nodes Those located in soft tissues are found mostly in adults and often arise in the wall of vein in an extremity It may also occur in the head and neck area Most patients are cured by excision but metastases are noted in 1/5 of cases

SPINDLE-CELL HEMANGIOENDOTHELIOMA May present at any age with male predominance occurring preferentially in the

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