Vascular tumors Notes

NOTES NOTES VASCULAR TUMORS GENERALLY, WHAT ARE THEY? PATHOLOGY & CAUSES ▪ Abnormal growths of blood/lymph vessels ▪ Can be benign/malignant, can occur anywhere in body ▪ Vascular tumors are rare, but most commonly found in neonates (e.g. hemangiomas), HIV-positive individuals (e.g. Kaposi’s sarcoma) DIAGNOSIS DIAGNOSTIC IMAGING ▪ Visual identification, imaging studies (MRI, CT scan, ultrasound with Doppler, biopsy) ▫ Determine location, tumor size, extent of spread LAB RESULTS ▪ Biopsy for definitive diagnosis COMPLICATIONS ▪ Metastasis ▪ Complications from chemo/radiation therapy SIGNS & SYMPTOMS TREATMENT ▪ Depends on type, location, severity, malignancy; see individual disorders ▪ See individual disorders ANGIOSARCOMA osms.it/angiosarcoma PATHOLOGY & CAUSES ▪ Rare blood vessel malignancy involving blood vessel endothelial lining ▪ Aggressive, rapidly proliferating → poor prognosis ▪ Can occur anywhere; usually occurs in sunexposed areas (head, neck, breast) ▫ Cutaneous angiosarcomas (occur beneath skin’s surface) most common ▪ Can affect liver blood vessels CAUSES ▪ Most likely due to lymphedema (fluid buildup causing sweeling), radiation exposure, carcinogens RISK FACTORS ▪ Biologically male (twice as likely), elderly, sun-exposure, radiation therapy, chronic post-mastectomy lymphedema, frequent exposure to vinyl chloride monomer gas in PVC manufacturing/arsenic insecticides ▫ High-grade: aggressive, fast-growing OSMOSIS.ORG 157

▫ Low-grade: less aggressive, slowgrowing COMPLICATIONS ▪ High chance of metastasis, poor prognosis. Better prognosis for individuals with smaller tumors with clearly delineated margins. Low grade breast angiosarcoma has better prognosis than tumors with poorly-defined borders SIGNS & SYMPTOMS ▪ Lesion resembling non-healing bruise/ wound ▪ Violet color ▪ Soft, visible, tactile lump/swelling ▪ Can form irregular vascular channels that disrupt tissue planes ▪ Fatigue ▪ Bone pain ▪ Anemia DIAGNOSIS LAB RESULTS ▪ Biopsy, usually diagnosed late after the disease has spread TREATMENT MEDICATIONS ▪ Chemotherapy SURGERY ▪ Difficult to resect due to delay in diagnosis OTHER INTERVENTIONS ▪ Radiation Figure 24.1 A surgically excised angiosarcoma. Figure 24.2 Histological appearance of an angiosarcoma composed of malignant endothelial cells with vascular spaces containing red blood cells. 158 OSMOSIS.ORG

Chapter 24 Vascular Tumors GLOMUS TUMOR osms.it/glomus-tumor TREATMENT PATHOLOGY & CAUSES ▪ Benign tumor arising from modified smooth muscle cells of skin’s thermoregulatory glomus bodies ▪ Derives from small vessels/arteriovenous anastomoses in glomus bodies ▪ Malignancy, metastasis rare ▪ Etiology includes loss-of-function mutation of protein glomulin in familial glomangiomas SURGERY ▪ Resection RISK FACTORS ▪ Adults: 20–40 years old ▪ Most lesions solitary, localized ▪ Autosomal dominant inheritance pattern COMPLICATIONS ▪ Good prognosis, low recurrence rate after resection ▪ Malignant glomus tumors rare, have good prognosis when treated with wide excision ▪ Metastasis associated with poor prognosis Figure 24.3 Homogenous enhancement of a glomus tumor of the nail bed at the ulnar aspect of the left index finger. SIGNS & SYMPTOMS ▪ Painful, small, red-blue growths ▫ Pain associated with solitary lesions ▫ Younger individuals: multiple tumors, usually asymptomatic ▪ Usually found on distal extremities ▪ Paroxysmal pain depending on temperature, pressure changes ▫ Cold, pressure worsens pain DIAGNOSIS Figure 24.4 Histological appearance of a glomus tumor. OTHER DIAGNOSTICS ▪ Visual inspection ▪ Occasional imaging OSMOSIS.ORG 159

KAPOSI SARCOMA osms.it/kaposi PATHOLOGY & CAUSES STAGING TYPES AIDS-related Kaposi’s sarcoma ▪ Takes three factors into account ▪ Extent/severity of tumor ▫ T0: localized tumor ▫ T1: widespread, multiple lesions that spread to other organs ▪ CD4 cell count (immune status) ▫ I0: CD4 count above 150 cells/mm3 ▫ I1: CD4 count less than 150 cells/mm3 ▪ Presence/absence of systemic illness ▫ S0: no systemic illness/opportunistic infections, and/or B symptoms. B symptoms: systemic fever symptoms, night sweats, weight loss, diarrhea ▫ S1: presence of systemic illness, opportunistic infections, and/or B symptoms AIDS-related ▪ Most common malignancy in AIDS COMPLICATIONS ▪ Malignant vascular tumor/lesions of blood vessel endothelial cells ▪ Due to human herpesvirus 8 ▪ Virus penetrates cells, causing uncontrollable replication ▪ May involve visceral organs ▪ Progression, severity of tumor depends on underlying factor ▫ Genetic: usually seen in older Eastern European males; tumor localized to skin ▫ AIDs: tumor spreads (see staging below) ▫ Organ transplant recipients: tumor spreads Immunocompromised & iatrogenic-related Classic/sporadic Endemic (African) ▪ Burkitt’s lymphoma due to Epstein-Barr virus RISK FACTORS ▪ Immunocompromised individuals ▫ AIDS ▫ Kaposi’s sarcoma associated human herpesvirus-8 (HHV-8) ▫ Organ transplant ▪ Biologically male ▪ Eastern European ▪ Higher risk: biologically-male individuals engaging in same-sex sexual acts (“MSM“) 160 OSMOSIS.ORG ▪ ▪ ▪ ▪ ▪ Lymphedema Bleeding Infection Long term hyperpigmentation Prognosis depends on individual’s immune status, viral load (amount of HIV virus in blood) SIGNS & SYMPTOMS ▪ Most common symptoms affect skin, also affect mouth, GI tract, respiratory tract ▫ Progresses from flat lesion → plaque → ulcerating nodule ▫ Purple, red lesion similar to bruise that does not blanch ▫ Lesion starts off flat, may become raised, more painful

Chapter 24 Vascular Tumors ▫ Lesions in other tissues (e.g. mouth, nose, throat, lymph nodes, lungs, gastrointestinal tract); Commonly found in mucous membranes (esp. hard palate) ▪ Pulmonary symptoms: pulmonary Kaposi’s sarcoma ▫ Coughing (possibly bloody cough) ▫ Dyspnea DIAGNOSIS DIAGNOSTIC IMAGING ▪ Bronchoscopy/endoscopy LAB RESULTS ▪ Biopsy MEDICATIONS ▪ HIV/AIDS management with antivirals ▫ Control HIV/AIDS → lesions shrink ▪ Removal of drugs (e.g. corticosteroids) allows immune system to recover ▫ Treatment more difficult in immunocompromised individuals ▪ Chemotherapy SURGERY ▪ Surgically remove affected skin OTHER INTERVENTIONS ▪ Cryotherapy → freeze affected skin ▪ Radiation TREATMENT ▪ Depends on ▫ Severity of immunosuppression ▫ Number, location of tumors ▫ Symptoms Figure 24.5 Kaposi sarcoma of the gingiva in a HIV positive individual. The tumor has replaced the gingiva of the upper right side of the jaw. There is overlying oral candidiasis. Figure 24.6 A Kaposi sarcoma composed of spindle cells which form slits filled with erythrocytes. OSMOSIS.ORG 161