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Tumor Embolization/Epistaxis
Carlos E. Baccin, MD; James D. Rabinov, MD
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KEY FACTS

  • Terminology

    • Pre-Procedure

      • Procedure

        • Outcomes

          TERMINOLOGY

          • Definitions

            • Capillary hemangioma:Vascular neoplasm of endothelial cell origin
              • Tumor growth from proliferation of these cells
            • Kasabach-Merritt syndrome: Also known as hemangioma with thrombocytopenia
              • Large hemangioma/hemangioendothelioma
                • Occurs in infants; often present at birth
                • Lesions can be in trunk, upper/lower extremities, retroperitoneum, cervical/facial areas
                • Characterized by platelet consumption
              • Consumptive coagulopathy depletes clotting factors
                • Coagulopathy due to blood stasis within abnormal vasculature
                  • Activates clotting cascade
                • Can progress to disseminated intravascular coagulation (DIC) and death
                • Risk of intracranial hemorrhage
                • 30% mortality rate
            • Hereditary hemorrhagic telangiectasia (HHT)
              • Also known as Osler-Weber-Rendu syndrome
              • Autosomal dominant genetic abnormality, affecting either gene ENG or ALK1; causes development of arteriovenous (AV) shunts
                • May affect nasal mucosa, skin, gastrointestinal (GI) tract, lung, central nervous system (CNS)
                • 4 major diagnostic criteria for HHT
                  • Epistaxis
                  • Telangiectasias
                  • Visceral lesions
                  • Family history of 1st-degree relative with HHT
                • HHT likelihood by number of criteria present
                  • 3 criteria present: "Definite"
                  • 2 criteria present: "Possible"
                  • 1 criterion present: "Unlikely"
            • Juvenile angiofibroma (JAF):Nonencapsulated benign vascular neoplasm; often grows aggressively
              • Local invasion of nasal fossa, anterior skull base
                • Involves upper medial pterygoid lamina early
                • Intracranial spread in 10-20%
              • Found almost exclusively in adolescent males
            • Meningioma: Nonglial primary intracranial tumor
            • Paraganglioma:Usually benign vascular tumor derived from primitive neural crest tissue
              • General: Also known as chemodectoma
                • May be hereditary and part of genetic syndrome (e.g., von Hippel-Lindau, neurofibromatosis)
              • Carotid body paraganglioma:Accounts for 40%
                • Origin: Carotid body paraganglia (glomus bodies)
                • Location: Common carotid artery (CCA) bifurcation; vascular mass splaying external/internal carotid arteries (ECA/ICA)
                  • Possible invasion/stenosis of branch vessels
                  • Malignancy in 6-12.5%, most frequently malignant head and neck paraganglioma
              • Glomus vagale paraganglioma: Accounts for 3%
                • Origin: Arises from nests of paraganglionic tissue within perineurium of vagus nerve
                • Location: Below jugular foramen near mandibular angle between skull base/hyoid
              • Jugulotympanic paraganglioma
                • Location: Jugular fossa and tympanic cavity
                  • Tympanic paragangliomas: Originate along tympanic branch of glossopharyngeal nerve; auricular branch of vagus nerve
                  • Jugular paragangliomas: Originate within jugular bulb adventitia
                • Jugulotympanic paragangliomas cause symptoms (e.g., pulsatile tinnitus) by invading/compressing adjacent structures such as accompanying nerves
              • Laryngeal paraganglioma
                • Uncommon/rare paraganglioma thought to arise from superior/inferior laryngeal paraganglia
                  • Location: > 90% occur in supraglottic larynx

          PRE-PROCEDURE

          • Indications

            • Contraindications

              • Pre-Procedure Imaging

                • Getting Started

                  PROCEDURE

                  • Procedure Steps

                    • Alternative Procedures/Therapies

                      POST-PROCEDURE

                      • Expected Outcome

                        • Things to Do

                          OUTCOMES

                          • Complications

                            Selected References

                            1. Alexiou GA et al: Management of meningiomas. Clin Neurol Neurosurg. 112(3):177-82, 2010
                            2. Kelly M: Kasabach-Merritt phenomenon. Pediatr Clin North Am. 57(5):1085-9, 2010
                            3. Shen W et al: Kasabach-Merritt syndrome: case reports of successful treatment with partial tumor resection and vincristine chemotherapy. Ann Plast Surg. 65(3):361-3, 2010
                            4. Harrigan MR et al: Handbook of Cerebrovascular Disease and Neurointerventional Technique. Totowa, NJ: Humana Press, 2009
                            5. Unlü Y et al: Management of carotid body tumors and familial paragangliomas: review of 30 years' experience. Ann Vasc Surg. 23(5):616-20, 2009
                            6. Wieneke JA et al: Paraganglioma: carotid body tumor. Head Neck Pathol. 3(4):303-6, 2009
                            7. Berenstein A et al: Surgical Neuroangiography Vol. 2. 2nd ed. Heidelberg, Berlin: Springer Verlag, 2004
                            8. Connors JJ III et al: Interventional Neuroradiology: Strategies and Practical Techniques. Philadelphia: WB Saunders Company, 1999
                            9. Segal DH et al: Predictive value of balloon test occlusion of the internal carotid artery. Skull Base Surg. 5(2):97-107, 1995
                            Related Anatomy
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                            Related Differential Diagnoses
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                            References
                            Tables

                            Tables

                            KEY FACTS

                            • Terminology

                              • Pre-Procedure

                                • Procedure

                                  • Outcomes

                                    TERMINOLOGY

                                    • Definitions

                                      • Capillary hemangioma:Vascular neoplasm of endothelial cell origin
                                        • Tumor growth from proliferation of these cells
                                      • Kasabach-Merritt syndrome: Also known as hemangioma with thrombocytopenia
                                        • Large hemangioma/hemangioendothelioma
                                          • Occurs in infants; often present at birth
                                          • Lesions can be in trunk, upper/lower extremities, retroperitoneum, cervical/facial areas
                                          • Characterized by platelet consumption
                                        • Consumptive coagulopathy depletes clotting factors
                                          • Coagulopathy due to blood stasis within abnormal vasculature
                                            • Activates clotting cascade
                                          • Can progress to disseminated intravascular coagulation (DIC) and death
                                          • Risk of intracranial hemorrhage
                                          • 30% mortality rate
                                      • Hereditary hemorrhagic telangiectasia (HHT)
                                        • Also known as Osler-Weber-Rendu syndrome
                                        • Autosomal dominant genetic abnormality, affecting either gene ENG or ALK1; causes development of arteriovenous (AV) shunts
                                          • May affect nasal mucosa, skin, gastrointestinal (GI) tract, lung, central nervous system (CNS)
                                          • 4 major diagnostic criteria for HHT
                                            • Epistaxis
                                            • Telangiectasias
                                            • Visceral lesions
                                            • Family history of 1st-degree relative with HHT
                                          • HHT likelihood by number of criteria present
                                            • 3 criteria present: "Definite"
                                            • 2 criteria present: "Possible"
                                            • 1 criterion present: "Unlikely"
                                      • Juvenile angiofibroma (JAF):Nonencapsulated benign vascular neoplasm; often grows aggressively
                                        • Local invasion of nasal fossa, anterior skull base
                                          • Involves upper medial pterygoid lamina early
                                          • Intracranial spread in 10-20%
                                        • Found almost exclusively in adolescent males
                                      • Meningioma: Nonglial primary intracranial tumor
                                      • Paraganglioma:Usually benign vascular tumor derived from primitive neural crest tissue
                                        • General: Also known as chemodectoma
                                          • May be hereditary and part of genetic syndrome (e.g., von Hippel-Lindau, neurofibromatosis)
                                        • Carotid body paraganglioma:Accounts for 40%
                                          • Origin: Carotid body paraganglia (glomus bodies)
                                          • Location: Common carotid artery (CCA) bifurcation; vascular mass splaying external/internal carotid arteries (ECA/ICA)
                                            • Possible invasion/stenosis of branch vessels
                                            • Malignancy in 6-12.5%, most frequently malignant head and neck paraganglioma
                                        • Glomus vagale paraganglioma: Accounts for 3%
                                          • Origin: Arises from nests of paraganglionic tissue within perineurium of vagus nerve
                                          • Location: Below jugular foramen near mandibular angle between skull base/hyoid
                                        • Jugulotympanic paraganglioma
                                          • Location: Jugular fossa and tympanic cavity
                                            • Tympanic paragangliomas: Originate along tympanic branch of glossopharyngeal nerve; auricular branch of vagus nerve
                                            • Jugular paragangliomas: Originate within jugular bulb adventitia
                                          • Jugulotympanic paragangliomas cause symptoms (e.g., pulsatile tinnitus) by invading/compressing adjacent structures such as accompanying nerves
                                        • Laryngeal paraganglioma
                                          • Uncommon/rare paraganglioma thought to arise from superior/inferior laryngeal paraganglia
                                            • Location: > 90% occur in supraglottic larynx

                                    PRE-PROCEDURE

                                    • Indications

                                      • Contraindications

                                        • Pre-Procedure Imaging

                                          • Getting Started

                                            PROCEDURE

                                            • Procedure Steps

                                              • Alternative Procedures/Therapies

                                                POST-PROCEDURE

                                                • Expected Outcome

                                                  • Things to Do

                                                    OUTCOMES

                                                    • Complications

                                                      Selected References

                                                      1. Alexiou GA et al: Management of meningiomas. Clin Neurol Neurosurg. 112(3):177-82, 2010
                                                      2. Kelly M: Kasabach-Merritt phenomenon. Pediatr Clin North Am. 57(5):1085-9, 2010
                                                      3. Shen W et al: Kasabach-Merritt syndrome: case reports of successful treatment with partial tumor resection and vincristine chemotherapy. Ann Plast Surg. 65(3):361-3, 2010
                                                      4. Harrigan MR et al: Handbook of Cerebrovascular Disease and Neurointerventional Technique. Totowa, NJ: Humana Press, 2009
                                                      5. Unlü Y et al: Management of carotid body tumors and familial paragangliomas: review of 30 years' experience. Ann Vasc Surg. 23(5):616-20, 2009
                                                      6. Wieneke JA et al: Paraganglioma: carotid body tumor. Head Neck Pathol. 3(4):303-6, 2009
                                                      7. Berenstein A et al: Surgical Neuroangiography Vol. 2. 2nd ed. Heidelberg, Berlin: Springer Verlag, 2004
                                                      8. Connors JJ III et al: Interventional Neuroradiology: Strategies and Practical Techniques. Philadelphia: WB Saunders Company, 1999
                                                      9. Segal DH et al: Predictive value of balloon test occlusion of the internal carotid artery. Skull Base Surg. 5(2):97-107, 1995