Cardiac and vascular tumors

1. Cardiac tumors — overview

• Metastatic tumors are the most common cardiac tumors (~5% of cancer patients). Most frequent primaries (descending): lung carcinoma, lymphoma, breast cancer, leukemia, melanoma, hepatocellular, colon.
• Primary cardiac tumors are uncommon and the five most common are benign (no malignant potential).
• Adult frequency order: myxoma > fibroma > lipoma > papillary fibroelastoma > rhabdomyoma (angiosarcoma = the malignant exception).

2. Primary cardiac tumors

Tumor	Key features
Myxoma	Most common adult cardiac tumor; left atrium at the fossa ovalis; single, 1–10 cm, globular/gelatinous/glassy; pedunculated forms “swing” into the mitral valve in systole → ball-valve obstruction (“wrecking-ball”); embolization, fever, malaise
Fibroma	2nd most common in children; ventricular myocardium (LV free wall / septum); mimics HCM; well-circumscribed; causes ventricular arrhythmias
Lipoma	Poorly encapsulated adipose mass; LV, LA or atrial septum; ball-valve obstruction, arrhythmia, or asymptomatic
Papillary fibroelastoma	On valves as hair-like projections; can embolize; may be organized thrombi
Rhabdomyoma	Most common in infants/children; defective apoptosis → myocyte overgrowth (assoc. tuberous sclerosis); valve/outflow obstruction; often regress spontaneously; histology: large round cells with glycogen vacuoles (“spider cells”)
Angiosarcoma	The only malignant primary cardiac tumor

3. Vascular tumors — framework

Divided into benign, intermediate, malignant. Arise from endothelial cells or perivascular support cells. Primary tumors of large vessels (aorta, pulmonary artery, vena cava) are rare CT sarcomas. Distinguishing rule:

• Benign: well-formed vascular channels filled with blood/lymph, lined by normal endothelium.
• Malignant: poorly organized vessels, more cellular with cytological atypia.

4. Benign vascular tumors

• Hemangioma: common in infancy/childhood; ↑ numbers of (ab)normal blood-filled vessels; mostly congenital, may regress (⅓ occur in liver).
  • Capillary: closely packed capillaries; skin, mucosa, liver/spleen/kidney; bright red-blue.
  • Cavernous: large dilated blood-filled channels; deep, unencapsulated, infiltrative; thrombosis + dystrophic calcification; component of von Hippel-Lindau (brain → hemorrhage risk).
  • Pyogenic granuloma: rapidly growing red, ulcerated nodule (skin/gingiva/oral mucosa) after trauma; can regress.
• Glomangioma (glomus tumor): painful, arises from glomus-body SMCs (thermoregulation); distal digits, under fingernails; glomus cells + branching capillaries.
• Vascular ectasia (NOT a true neoplasm): local dilation of existing vessels.
  • Telangiectasia — nevus flammeus (birthmark, head/neck; port-wine stain variant persists/thickens); spider telangiectasia (central core + radial arterioles; hyperestrogenic states: pregnancy, cirrhosis).

5. Intermediate-grade

Kaposi sarcoma: caused by HHV-8; spindle cells + RBCs + hemosiderin + inflammatory cells; purplish painless plaques/nodules; common in immunocompromised patients. Four forms (same pathogenesis): classic/European (elderly Mediterranean, HIV−, indolent), lymphadenopathic/African (young, aggressive, HIV−), transplant-associated (immunosuppression, aggressive/visceral), AIDS-associated (HIV+, early wide dissemination).

Hemangioendothelioma: spectrum between hemangioma and angiosarcoma; spindle endothelial cells with channels; skin/spleen/liver; ~40% recur, ~20% metastasize.

6. Malignant vascular tumors

Angiosarcoma: malignant endothelial tumor; elderly; skin/soft tissue/breast/liver.

• Hepatic angiosarcoma: chemical carcinogens — arsenic, polyvinyl chloride (PVC), Thorotrast.
• Lymphangiosarcoma: years after mastectomy/radiation (chronic lymphedema).
• Morphology: red nodules → large fleshy red-tan/grey-white masses with infiltrative margins, necrosis & hemorrhage; aggressive local invasion + metastasis. (Hemangiopericytoma is also malignant.)

💡 High-yield: Most common cardiac tumor overall = metastasis; most common primary adult = myxoma (LA, fossa ovalis, ball-valve obstruction); most common in children = rhabdomyoma (tuberous sclerosis, glycogen “spider cells”). Vascular: hemangioma (benign, capillary/cavernous; cavernous ↔ von Hippel-Lindau), glomus tumor (painful, subungual), Kaposi sarcoma (HHV-8, immunocompromised), angiosarcoma (malignant; hepatic ↔ arsenic/PVC/Thorotrast; lymphangiosarcoma ↔ post-mastectomy lymphedema).