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Grant Closeout. Cancer Training at NCI. Resources for Trainees. Funding for Cancer Training. Click here to sign up. Download Free PDF. Pheochromocytoma crisis due to glucocorticoid administration: a case report and review of the literature Archives of Iranian medicine, Farin Rashid-farokhi. A short summary of this paper. Pheochromocytoma crisis due to glucocorticoid administration: a case report and review of the literature.
The first attack was associated with pulmonary edema, while the second attack was associated with high anion gap metabolic acidosis, renal failure, hyperglycemia, and hypokalemia. The attacks D led to the diagnosis of pheochromocytoma, which was confirmed by appropriate diagnostic tests.
The tumor was excised successfully and the patient is presently asymptomatic. We believe that these episodes were initiated by glucocorticoid injection, an event reported in a few cases. We briefly review potential mechanisms resulted in hypertensive crisis in such patients.
The patient was visited one day before admission in an P heochromocytoma is a rare neuro- outpatient clinic because of persistent dry cough endocrine tumor derived from chromaffin after an upper respiratory tract infection. He had ive cells of the sympathetic nervous system. However, there are many reports of headache, tremor, anxiety, abdominal pain, and other unusual presentations.
Echocardiography was normal E-mail: farinrf yahoo. Rashid-Farokhi, A. Cheraghvandi, M. Masjedi , et al. The patient became more dyspneic requiring intubation and mechanical ventilation.
A brain computed tomography CT yielded normal findings. The patient was treated with intravenous antibiotics with the presumed diagnosis of pneumonia and adult respiratory distress syndrome ARDS. Five hours after initiation of mechanical ventilation, he became agitated and three hours later he was completely conscious. He was therefore extubated and oral nutrition was begun. High resolution CT showed diffuse ill-defined opacities filling the Figure 1.
Magnetic resonance imaging shows an air spaces. Multiple blood cultures were negative. Pheochromocytoma as a catecholamine-producing tumor: implications for clinical practice. Hypertension in pheochromocytoma: characteristics and treatment. Catecholamines and adrenergic receptors. Pheochromocytoma diagnosis, localization, and treatment. Blackwell Publishing; Clinical application of arterial stiffness; definitions and reference values.
Am J Hypertens. Manger W, Gifford RW. The clinical and experimental pheochromocytoma. Malden: Blackwell Science; Pheochromocytoma with adrenergic biochemical phenotype shows decreased GLP-1 secretion and impaired glucose tolerance.
Pheochromocytoma: recommendations for clinical practice from the first international symposium. Nat Clin Pract Rev. Uptake of meta-iodobenzylguanidine in neuroendocrine tumors is mediated by vesicular monoamine transporters. Br J Cancer. Endocrine hypertension: then and now. Endocr Pract. Endoc Relat Cancer. Point of controversy: perioperative care of patients undergoing pheochromocytoma removal—time for a reappraisal?
Eur J Endocrinol. Pheochromocytoma-update on disease management. Ther Adv Endocrinol Metab. Approach to the patient: perioperative management of the patient with pheochromocytoma or sympathetic paraganglioma.
Perioperative considerations in patients with adrenal tumors. J Surg Oncol. Cancers Basel. Published Oct 8. Bravo EL, Tagle R. Laparoscopic adrenalectomy can be performed transperitoneally or retroperitoneally depending on the surgeon's preference and expertise , Seeding and recurrence of tumors in the adrenal bed or throughout the abdominal cavity can occur if pheochromocytomas are fractured during dissection , mandating precise and gentle dissection.
Specimen bags used for tumor retrieval should not tear. The operation should be converted to open resection if the laparoscopic approach is difficult.
Hand assistance or robot assistance may be helpful in patients with large tumors that are difficult to resect and are used per the surgeon's discretion. Partial adrenalectomy is safe, with no increased surgical risks over complete adrenalectomy Use of energy devices such as ultrasonic shears and bipolar sealers lowers the risk of bleeding from the cut edges of the adrenal gland.
Selective removal of medullary tissue leaving only cortical tissue is attempted, but usually some medullary tissue remains, which can cause tumor recurrence. For patients with prior contralateral adrenalectomies, a successful partial adrenalectomy preserving sufficient adrenal cortex can prevent postoperative adrenal insufficiency and requirements for glucocorticoid and mineralocorticoid replacement — Larger tumors result in smaller remnants and a lower chance for steroid independence.
Partial adrenalectomy increases the risk for tumor recurrence from the remnant. The cumulative recurrence rate for MEN2 patients after adrenal-sparing surgery at 5 and 10 years is The risk of surgical complications when resecting a recurrent tumor in a previously dissected area may be higher than for primary resections; open adrenalectomy may be needed for reoperation.
Some surgeons advocate partial adrenalectomy even for initial pheochromocytoma in patients who are at high risk for subsequent contralateral adrenalectomy for pheochromocytoma.
The decision to perform partial adrenalectomy depends on the relative value placed on two competing problems. Complete bilateral adrenalectomy results in hypocortisolism, with lifelong steroid dependence and the need to adjust steroid doses during physiological and pathological stress. Partial adrenalectomy inevitably leaves some adrenal medullary tissues with risk for recurrent pheochromocytoma. The resulting potential for reoperations likely to be more difficult, with higher conversion and complication rates must be balanced against the risks associated with chronically treated adrenal cortical insufficiency.
Unfortunately, the group of patients who would benefit from partial adrenalectomy is exactly the same group who are at higher risk for recurrent pheochromocytoma from the remnant. Partial adrenalectomy for smaller tumors, those in the periphery, and those away from the main adrenal vein is more likely to result in sufficient functioning of adrenal cortex.
Accumulating evidence shows that hereditary PPGLs are characterized by distinct clinical presentations and differences in biological behavior and mode of transmission that reflect underlying mutations , , — Mutations of RET and NF1 genes are almost always associated with adrenal tumors that produce normetanephrine and metanephrine 28 , Biochemical screening and interpretation of test results in these hereditary conditions can therefore benefit from a personalized approach that considers genotype-biochemical phenotype relationships.
Whereas VHL-associated tumors occur principally at adrenal locations, tumors due to mutations of SDHx genes occur mainly at extra-adrenal locations and include skull base and neck paragangliomas with some differences depending on the particular SDH subunit affected , , , Patients with mutations of the SDHB gene deserve special attention because they have a high risk of malignant disease that reflects both the typically large sizes and extra-adrenal location of associated tumors , , , Large tumor size and extra-adrenal location are both independent risk factors for malignant PPGLs that should be considered as part of the personalized management of any patient with PPGL , An increase in plasma methoxytyramine is also a common feature of patients with metastatic PPGLs and is a promising new biomarker to identify such patients , In addition to a personalized approach to biochemical testing and test interpretation, the above observations dictate a need for personalized approaches to tumor localization.
This need is further strengthened by additional findings that the underlying mutation and associated biological behavior impact the choice of functional imaging modality , , Figure 2. Decisional algorithm for functional imaging in patients with proven PPGL. Although localization in patients with RET and NF1 mutations or any patient with increased plasma or urine concentrations of metanephrine should primarily focus on the adrenals, localization in patients with mutations of SDHx genes should involve appropriate strategies for localizing extra-adrenal tumors.
Recent studies support the existence of a genotype-specific imaging approach in the localization of PPGLs The surgical approach should also be personalized according not only to tumor size and location but also to any underlying mutation; adrenal cortical-sparing surgery is a consideration for bilateral adrenal disease, whereas patients at risk for malignancy due to SDHB mutations should be considered for approaches that minimize the possibility of recurrent or metastatic disease , Finally, all mutation carriers should receive consideration for annual biochemical surveillance for PPGLs.
The nature of this surveillance should, however, take into account the particular gene affected according to the genotype-phenotype relationships described above, as well as considerations of penetrance and potential severity of disease.
For example, because the penetrance of PPGLs in NF1 is low, screening for these tumors need not be considered unless indicated by signs or symptoms.
At the other end of the spectrum, the high morbidity associated with undiagnosed PPGLs in patients with SDHB mutations mandates closer attention; in addition to biochemical testing, periodic imaging with MRI should be considered to detect biochemically silent tumors. To avoid ionizing radiation, CT and nuclear medicine imaging modalities should be reserved to further characterize detected tumors. The committee recognizes that currently there are no studies firmly establishing that a personalized approach provides improved outcome.
Nevertheless, it seems highly likely that such approaches will benefit patients, but they must also be considered according to cost; as covered below, a related point to any cost-benefit analysis is that such personalized approaches can only be feasible via specialist referral centers with appropriate multidisciplinary expertise. In particular, patients should be referred to such centers should there be pregnancy, metastatic disease, or issues concerning the complexity or difficulty in biochemical diagnosis; localization; performance, and interpretation of genetic testing; preoperative preparation; surgical treatment; and follow-up.
However, several cross-sectional studies showed that high-volume centers had lower postsurgical morbidity with shorter hospital stay than low-volume centers — Some studies found higher rates of complications and conversions to laparotomy in low-volume nonreferral centers than in high-volume centers These studies, however, did not focus on patients with pheochromocytoma. The above differences are not unexpected because they have also been shown for other complex interventions such as vascular surgery The strong inverse relation between case volume and postsurgical mortality for esophageal cancer surgery was based on the hospital setting in which these complex interventions were performed PPGL is a very rare disorder with fewer than five patients presenting per year, even in some larger medical centers.
The clinical presentation and course of PPGL are widely variable and can be part of a multisystem syndrome with many different organs affected. Most physicians are therefore unlikely to build sufficient specific experience to deal with this disorder. For a correct diagnosis, clinicians need to have appropriate experience in interpreting clinical and laboratory results, including results of genetic testing.
Other physicians such as radiologists and nuclear medicine specialists also play a crucial role for a reliable and accurate interpretation of imaging test results. Specialists such as cardiologists, anesthesiologists, and intensive care physicians must be involved in proper patient-tailored treatment.
Therefore, the committee believes that a multidisciplinary team, experienced in dealing with these patients, offers the best outcomes. Stefan K. E-mail: govt-prof endocrine. Telephone: Address all reprint requests for orders for or fewer to Society Services, Telephone: E-mail: societyservices endocrine. Grading quality of evidence and strength of recommendations. Google Scholar. A case for clarity, consistency, and helpfulness: state-of-the-art clinical practice guidelines in endocrinology using the grading of recommendations, assessment, development, and evaluation system.
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