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What Richard Deals With

  • Symptoms appear suddenly -- paroxysmal
  • Pain -- head, chest, and abdomen
  • Pain may be sudden
  • Pressure in the head or Blurred Vision
  • Palpitations -- heart racing or pounding,elevated blood pressure
  • Increased sweating
  • Pallor -- pale, ghost color of skin
  • Anxiety
  • Nausea
  • Vomiting
  • Fever
  • Flushing is rare -- redness of the face
  • Weightloss

About Paraganglioma

Paragangliomas are neuroendocrine tumors that arise from sympathetic nerve ganglia. They can develop anywhere from the neck (chemodectomas) to the pelvis but are most commonly found in the abdomen, particularly at the aortic bifurcation or in the peri-aortic region. Paragangliomas are histologically identical to pheochromocytomas that arise from the adrenal medulla. Malignancy is more common in paragangliomas (30%–50%) than in pheochromocytomas (10%–15%), with local invasion, destruction of adjacent vertebrae, and distant metastases to lungs, lymph nodes, and bones.

Patients with metastatic paraganglioma have been reported to have a median survival of about 4.5 years; however, indolent disease does occur, and prolonged survival has been reported. Malignancy must be distinguished from peritoneal seeding of tumor that can occur spontaneously or during the resection of a retroperitoneal paraganglioma, yielding multiple intra-abdominal tumors (pheochromocytomatosis) that rarely metastasize beyond the abdomen.

Familial paraganglioma is caused by a germline mutation in one of the genes encoding succinate dehydrogenase (SDHB, SDHC, or SDHD). In this condition, multiple paragangliomas can arise in the same individual and must be distinguished from metastatic disease, which may coexist. Paragangliomas and pheochromocytomas occur at an early age in 20% of patients with von Hippel-Lindau (VHL) disease, and such tumors may be malignant.

Treatment

131I-MIBG
High-dose 131I-MIBG was first used to treat malignant pheochromocytomas in 1983 at the University of Michigan. Like sympathetic synapses, pheochromocytomas and paragangliomas are usually avid for norepinephrine. Benzylguanidine resembles norepinephrine, and iodine 131 is tagged to it, producing 131I-MIBG, a targeted radionuclide. As the iodine 131 decays, it releases local high-energy beta particles that damage or destroy the tumor cell; concomitant release of gamma rays allows post-therapy whole-body scanning. In order to deliver a therapeutic dose to the tumors, a large amount of 131I-MIBG must be administered to the patient.
Although 131I-MIBG is readily available in doses used for diagnostic scanning (eg, 5 mCi), it is not commercially available in the large amounts required for a treatment (500–800 mCi). Such high doses of 131I-MIBG must be synthesized on site at the treating medical center; this requires special licensing of the facility, an experienced nuclear pharmacist, and a "hot cell" for its preparation. High-dose 131I-MIBG therapy must be done with stringent radiation precautions, and the patient must remain quarantined in a lead-shielded room for about 5 days or until radiation emissions have declined to a level safe enough for the patient to be discharged. 131I-MIBG therapy is available at the UCSF Medical Center under a phase II study protocol with the approval of the UCSF Committee on Human Research, the UCSF Comprehensive Cancer Center, and the UCSF Pediatric Clinical Research Center.

High-dose 131I-MIBG therapy has been moderately successful in treating patients with malignant paraganglioma or pheochromocytoma whose tumors are avid for the isotope. Following 131I-MIBG therapy, the majority of such patients experience a partial remission and stabilization of disease. When treated with very high doses of 131I-MIBG, 3 of 12 such patients have experienced complete remissions.

Bone marrow suppression is the main side effect of high-dose 131I-MIBG therapy. The majority of patients require transfusions and marrow support for low blood counts, which occur about 3 weeks after therapy. Pancytopenia is usually transient, but stem-cell harvest has been obtained prior to therapy for all patients who are to receive 131I-MIBG doses over 12 mCi/kg or who have already received chemotherapy or external radiation therapy to bones.

External Radiation Therapy

Radiation therapy is useful for treating patients with painful bone metastases. However, irradiated tumors lose their avidity for 131I-MIBG. Therefore, external radiation therapy ideally should be administered after 131I-MIBG therapy to patients who remain symptomatic. External radiation therapy should not be administered to treat intra-abdominal paragangliomas, due to its side effects and lack of effectiveness.

Chemotherapy

Combined use of cyclophosphamide, vincristine, and dacarbazine (CVD) appears to be the most effective chemotherapeutic regimen for patients with metastatic paraganglioma or pheochromocytoma. However, complete long-term remissions have not occurred with any chemotherapy regimen, and multiple cycles of CVD chemotherapy must be administered chronically every 21 days.



PowerPort* Implanted Port
(That will be implanted in Richard in April 2008)

The PowerPort* device provides the benefit of power injection (when used with the PowerLoc* safety infusion set) with the ability to see through the port with minimal artifact. The PowerPort* device is radiopaque and allows patients to receive IV therapies and CECT scans without the need for repeated needlesticks.

It combines reliable venous access with the unique ability for power-injected Contrast-Enhanced Computed Tomography (CECT) scans. Power-injected CECT scans produce superior images to help better manage patient care. Such scans are often used to track tumor markers or perform pulmonary embolism studies.

The palpation points and unique triangular shape of PowerPort* implanted ports make them easily distinguishable from older non-power ports. PowerPort* devices offer easy, flexible placement and access.

Features & Benefits

Unique Benefits of the PowerPort* Device

 - Lightweight for patient comfort
 - Reduced artifact - better imaging capability

Enables Superior Imaging

 - MRI-safe to 3 tesla
 - Power injection and CECT scan capable when used with PowerLoc* safety infusion set
 - Withstands 5 ml/s power injection @ 300 psi pressure limit setting

Easily Identifiable

 - Soft palpation points on septum
 - Unique triangular port shape
 


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This site was created and is maintained by Corey Lasley in hopes that others will give Rich and Brianne an Helping Hand