Tuesday, October 4, 2011
From the Penn GI News
The Penn NET Treatment Program is a major regional source of referrals for patients with pheochromocytomas. The elevated catecholamines induced by these tumors can cause a variety of diverse (and often confusing) symptoms, including (but not limited to) the classic triad of headache, palpitations and sweating. Many patients will have hypertension, which can be labile.
Patients with pheochromocytoma entering the Penn NET Treatment Program are diagnosed and treated by an interdisciplinary team of clinicians within the Renal-Electrolyte and Hypertension Division and the Division of Endocrine and Oncologic Surgery. Typically, initial therapy focuses upon relief of the symptoms caused by hormone over-secretion.
Coordinating Treatment for GEP-NETs and PETs
A nurse coordinator at the Abramson Cancer Center provides an important element of both patient care and confidence, overseeing diagnostics and treatment plans, coordinating visits and follow-up and administering central scheduling for all patients. The nurse coordinator also serves as the liaison for research should appropriate clinical trials become available to patients. According to Dr. Metz, the dedicated nurse coordinator is at the core of the process, ensuring cohesion in the treatment plan, involving the patient at every step and providing updates to the treatment team about important developments. Octreotide, MIBG and other necessary medications are administered through the divisions of Gastroenterology, Hematology-Oncology, Renal Electrolyte and Hypertension, Interventional Radiology and Nuclear Medicine and Medical Genetics.
Case Study 1
Mrs. G, a 49-year-old female, was referred to the Penn Neuroendocrine Tumor Treatment Program for carcinoid tumor surgery. Several months before presenting at Penn, she had developed lower leg edema, flushing and diarrhea.
These symptoms led her to visit her ob/gyn, who ordered an abdominal CT scan that found widely dispersed tumors in her liver. At Penn, a 24-hour urine test for 5-hydroxyindolacetic acid (5-HIAA), the main urinary metabolite of serotonin,measured >150 mg/day (normal=<6 mg/day); an assessment of chromogranin A (CgA), a NET marker, found levels >100 u/L (normal range = 2-18 u/L). An octreoscan identified a primary tumor in the terminal ileum and an extensive tumor burden in the right lobes of her liver but no metastases beyond the liver.
Mrs. G was diagnosed with widely metastatic carcinoid tumors in her liver and carcinoid syndrome and began octreotide LAR, 20 mg/month, which improved, but did not resolve her symptoms. Her dose was increased to 30mg/month and following an interdisciplinary review of her tests and scans, it was recommended that Mrs. G have chemoembolization of the tumors in her right liver followed by debulking surgery.
Following two visits to interventional radiology for chemoembolization, she had liver resection surgery in the division of gastroenterological surgery. She recovered from these procedures without incident. At this time, her 5-HIAA and CgA levels were within normal levels. Six months post-surgery, a CT scan revealed no new hepatic lesions and no new metastases. At one year, Mrs. G’s status remains stable on octreotide maintenance therapy.
Case Study 2
Mr. R presented at age 12 with headaches and diarrhea; he was diagnosed with a right adrenal pheochromocytoma and underwent right adrenalectomy.
At age 37, Mr. R was seen at the Penn Center for Complex Hypertension with recurrence of diarrhea and headaches. His BP was 132/80 mm Hg and he was not on any antihypertensive medications. He was found to have a left adrenal mass consistent with pheochromocytoma and was scheduled for a second adrenalectomy.
He was treated with dibenzylene for preoperative alpha blockade and alpha methyl-tyrosine. A left-sided adrenalectomy was performed; now unable to produce endogenous steroids, Mr. R began a regimen of hydrocortisone and fludrocortisone. Because certain genetic mutations are associated with bilateral adrenal pheochromocytoma, Mr. R was referred for genetic testing.
Genotyping studies were positive for Von Hippel Lindau V84L mutation, an autosomal dominant trait with a 50 percent risk of inheritance. Mr. R’s 18-year-old son, JW, was also found to be carrying the vHL mutation. JW had no symptoms; supine and sitting BP were 120/78 mm Hg and 118/82 mm Hg. Standing blood pressure was 96/74 mm Hg with a heart rate of 120 beats/minute.
Urine studies show elevated normetanephrine levels. MRI of the abdomen showed a left adrenal mass and laparoscopic adrenocortical sparing surgery was performed. While his BP remained normal with home BP monitoring, JW’s plasma and urine metanephrines never “normalized.”
A repeat MRI performed a year later revealed a new tumor in the right adrenal gland and a second adrenal cortex sparing surgery for his second pheochromocytoma was performed. Both Mr. R and JW remain disease-free several years later with yearly surveillance with blood tests and imaging for recurrent pheochromocytoma.
Treating Pheochromocytomas at Penn Medicine
Debbie Cohen, MD
Associate Professor of Medicine
David Metz, MD
Professor of Medicine
To refer a patient to Penn Medicine, please contact Penn PhysicianLinkTM here or at 877-937-7366.
Download a pdf of the Winter 2011 Penn GI News.