V. FOLLOW UP MANAGEMENT

V. FOLLOW UP MANAGEMENT

Synthesized by: Maria Lourdes Galvez and Zacchari Andrei Ochona


The key in a successful treatment of any disease is the follow up. For a long lasting survival of patients with pheochromocytoma after surgical treatment, except for those with malignant disease, A long-term follow up is inevitable because of a difficult pathologic-anatomical verification of the malignant nature of the disease as well as risk of relapse assessment (Balazovjech 2004). Therefore, Postoperatively, patients should be monitored closely for 24 hours in an intensive or immediate care unit. Hypotension and hypoglycemia are the two most common major complications seen at this time (Lenders JW, et al. (2005). Rarely, patients experience pulmonary edema or cardiomyopathy following surgery (Gordon RY, et al, 2004/ Brouwers F,et al., 2006).


In a study done by Castilho, (2009). The follow-up of patients was done based on data review from medical records and telephone contacts. During the first year of follow-up, each patient was examined in at least four different occasions. Their blood pressure was measured and registered in their records. In the sixth and tenth months following the surgery, each patient was submitted to dosage of cathecholamines in urine and serum. They were submitted to MIBG at the end of the first year after surgery. After the first year, each patient was examined at least once a year and submitted to laboratory exams and MIBG for at least five years. Therefore, there should be at least a minimum of 18 months in follow up.

A general follow-up protocol includes annual catecholamine analysis and an 131I-MIBG scan whenever these levels exceed the normal range. An arranged close follow-up of catecholamine levels, with cystoscopy every 3 to 6 months must be conducted. Local recurrence and metastasis have been reported over 20 years after primary resection. In this study by Tsai (2003), It maybe non conclusive since there were only a limited number of patients. In fact in a similar study by Huang (2007), who also involved around 10 patients as his respondents, metastases were present in postoperative pathology in seven patients. And on the other three patients, metastases were discovered six months to ten years after surgery. Here, he suggested to have a close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma. The possibility of malignancy should be kept in mind even though the initial pathology is benign. (Huang K, 2007)

If you examine further what each journal suggests, biochemical tests and imaging must be done to check for further metastasis. In a cohort study by Timmers (2009), he mentioned that life expectancy after surgery of benign sympathetic paraganglioma(sPGL), may either be normal or mildly decreased (Khorram-Manesh et al. 2005) as compared to the general population. For obvious reasons, long- term results after surgery for (succinate dehydrogenase) SDHx-related sPGL in particular are lacking. Considering the high risk of developing additional tumors (SDHB, SDHD) and/or metastatic lesions (SDHB), an indefinite post-operative follow-up with bi-annual measurement of blood pressure and annual plasma or urine metanephrines, as well as annual MRI of the neck, thorax, abdomen, and pelvis must be performed. Clinical follow-up must also be performed for SDHB mutation-associated disease, which includes, a later age of onset, extraadrenal (abdominal or thoracic) tumors, and a higher rate of malignancy. In contrast, SDHD mutation carriers, in addition to head and neck paragangliomas, should be observed for multifocal tumors, infrequent malignancy, and the possibility of extraadrenal pheochromocytoma. (Benn, D et al., 2006).


For management of undiagnosed pheochromocytoma, patient with grossly exaggerated and unexplained hypertension and tachycardia during anaesthesia needs to be followed up and investigated for pheochromocytoma. Drugs must be available in all the anaesthetic sites and all the anaesthetists must be familiar of their uses. (Bensghir M, et al,2009)


In conclusion, very close communication between the endocrine, surgical, medical, cardiology, pediatric, oncology, radiology, and anesthesia teams is essential. Such teamwork should be directed to meet the three most essential goals: treat hypertension and tachyarrhythmias, restore the vascular volume, and treat any tumor or catecholamine excess-associated medical problems. With all these current recommendations in place, the Hippocrates phrase “To do good or to do no harm” reflects a physician’s best effort toward a pheochromocytoma patient before surgery. (Pacak, Karel 2007). Together with the physicians, the nurses must work hand in hand in the wellness of patients with pheochromocytoma.

References:

1.) Balazovech L, et al., (2004). Retrospective analysis of patients clinical manifestations before and after pheochromocytoma surgery, Bratisl lek listy 2004; 105 (2): 72-77

2.) Bensghir M, et al (2009) Management of undiagnosed pheochromocytoma with acute appendicitis. World Journal of Emergency Surgery 2009, 4:35doi:10.1186/1749-7922-4-35

3.) Benn, D et al., (2006). Clinical Presentation and Penetrance of Pheochromocytoma/ Paraganglioma Syndromes. The Journal of Clinical Endocrinology & Metabolism 91(3):827– 836

4.) Brouwers F,et al ( 2006). Emergencies Caused by Pheochromocytoma, Neuroblastoma, or Ganglioneuroma. Endocrinology and Metabolis Clinical North American 35 (2006) 699–724

5.) Castilho, LN et al., (2009). Pheochromocytoma: A Long-Term Follow-Up of 24 Patients Undergoing Laparoscopic Adrenalectomy. International Brazil Journal of Urology. 2009; 35: 24-35

6.) Gordon RY, et al(2004). Case report: a 32-year-old woman with familial para-
gangliomas and acute cardiomyopathy. Transplant Proc 2004;36:2819–22.

7.) Huang K, et al., (2007). clinical and pathological data of 10 malignant pheochromocytoma: long term follow up in a single institute, international journal of urology, 2007, vol 12, pp. 181-185

8.) Khorram-Manesh A, et al., (2005) Long-term outcome of a large series of patients surgically treated for pheochromocytoma. Journal of Internal Medicine 258 55–66.

9.) Lenders JW, et al. (2005). Phaeochromocytoma. Lancet 2005;366: 665–75

10.) Pacak, Karel (2007). Approach to the patient: Preoperative Management of the Pheochromocytoma Patient. Journal of Clinical Endocrinology and Metabolism, November 2007, 92(11):4069 – 4079

11.) Timmers HJ, et al (2009). Clinical aspects of SDHx-related pheochromocytoma and paraganglioma. Endocrine-Related Cancer (2009) 16 391–400

12.) Tsai W, Yang S, (2004). bladder pheochromocytoma: a case report, Department of Urology, Mackay Memorial Hospital, No. 92 Sec. 2 (JTUA 15:19-23, 2004)