Anaesthetic Management for Laparascopic Adrenalectomy for Pheochromocytoma as Part of Vonn Hippel-Lindau Syndrome in Child

Pheochromocytoma is a tumor that originates from either chromaffincells in adrenal medulla or in other paraganglia tissues (paragangliomas) of the sympathetic nervous system. It might be familial and associated with hereditary tumors such as Von Hippel-Lindau syndrome and multiple endocrine neoplasia type II. The symptoms are variable related to the level of secreted catecholamine. The most common are hypertension, tachycardia, headache and abdominal pain. The most effective treatment is surgical resection as in recent years, laparoscopic surgery has been more preferred. The perioperative management is quite challenging especially in view of hemodynamic fluctuations. Although there is a considerable amount of information on the management of the adult with phaeochromocytoma, much less has been written concerning children with the disease. We present a case of 9-year-old hypertensive boy with left sided pheochromocytoma, scheduled for excision of tumour. He had presented with complaints of pain in abdomen, excessive sweating, headache and raised blood pressure, as well as family anamnesis for von Hippel-Lindau syndrome (father with confirmed diagnosis). Diagnosis was confirmed by CT scan (abdomen), raised 24-hour urinary catecholamine levels and genetics tests. Preoperative blood pressure was controlled with prazosin (ά-adrenergic blocker). The anaesthetic technique used was general anaesthesia. Child was later discharged on oral antihypertensive.

An Undiagnosed Non Functioning Retroperitoneum Paraganglioma, Complicating A Gynaecological Surgery; A Case Report

Paragangliomas are uncommon tumors arising from the neuroendocrine elements (chief cells) of the paraganglia and symmetrically distributed along the aorta in close association with the sympathetic chain. Although functional tumors are easier to diagnose due to the clinical findings caused by excess secretion of catecholamines and an elevation of the urinary catecholamine levels, however the diagnosis of nonfunctioning paraganglioma of the retroperitoneum is difficult and only few cases have been made before exploratory laparotomy. Although our patient was initially planned and operated by our gynaecological team, the incidental findings had to involve the general surgeons. She is fortunate that the tumour did not show any features of malignancy nor metastasis, however a long time follow up is needed in case of any possible recurrence.

Metabolic impact of pheochromocytoma/paraganglioma: targeted metabolomics in patients before and after tumor removal

Objective Excess catecholamine release by pheochromocytomas and paragangliomas (PPGL) leads to characteristic clinical features and increased morbidity and mortality. The influence of PPGLs on metabolism is ill described but may impact diagnosis and management. The objective of this study was to systematically and quantitatively study PPGL-induced metabolic changes at a systems level. Design Targeted metabolomics by liquid chromatography-tandem mass spectrometry of plasma specimens in a clinically well-characterized prospective cohort study. Methods Analyses of metabolic profiles of plasma specimens from 56 prospectively enrolled and clinically well-characterized patients (23 males, 33 females) with catecholamine-producing PPGL before and after surgery, as well as measurement of 24-h urinary catecholamine using LC-MS/MS. Results From 127 analyzed metabolites, 15 were identified with significant changes before and after surgery: five amino acids/biogenic amines (creatinine, histidine, ornithine, sarcosine, tyrosine) and one glycerophospholipid (PCaeC34:2) with increased concentrations and six glycerophospholipids (PCaaC38:1, PCaaC42:0, PCaeC40:2, PCaeC42:5, PCaeC44:5, PCaeC44:6), two sphingomyelins (SMC24:1, SMC26:1) and hexose with decreased levels after surgery. Patients with a noradrenergic tumor phenotype had more pronounced alterations compared to those with an adrenergic tumor phenotype. Weak, but significant correlations for 8 of these 15 metabolites with total urine catecholamine levels were identified. Conclusions This first large prospective metabolomics analysis of PPGL patients demonstrates broad metabolic consequences of catecholamine excess. Robust impact on lipid and amino acid metabolism may contribute to increased morbidity of PPGL patients.

Association Between Urinary Catecholamine Excretion and Urine Volume

Several confounders must be considered in the evaluation of urinary catecholamine excretion. However, literature is contradictory about potential confounders. The aim of the present study was to assess correlations between catecholamine excretion and anthropometric or clinical parameters with special attention to urine volume. A total of 967 24-h urinary catecholamine measurements were performed in 593 patients for diagnostic purposes. The indication for urine examination was suspicion of secondary hypertension, phaeochromocytoma, or paraganglioma. From the patients examined, 57% were females and 43% were males. The patients’ age ranged between 15 and 87 years with a median [Q1; Q3] of 51 [39; 62] years. Seventy-eight percent of the patients suffered from hypertension. Seventy percent of patients took one or more antihypertensive drugs. The most commonly used drugs were ACE inhibitors (43%), while α-blockers (15%) were the least used drugs. Urinary excretion was between 500 and 11 950 ml/24 h with a median of 2200 [1600; 2685] ml/24 h. The median body mass index (BMI) was 26.7 [24.0; 30.4] kg/m2. The excretion of all catecholamines was greater in men than in women (all p<0.0001). Epinephrine (p=0.0026), dopamine (p<0.0001), and metanephrine (p=0.0106) excretion decreased with age. BMI was associated with urinary excretion of dopamine (p<0.0001), norepinephrine (p=0.0026), normetanephrine (p<0.0001), and homovanillylmandelic acid (HVMA; p=0.0251). Urine volume correlated with urinary dopamine (p=0.0127), metanephrine (p<0.0001), normetanephrine (p=0.0070), and HVMA (p<0.0028) excretion. In addition to the established associations between urinary catecholamine excretion and age, gender, and BMI in the present study, urinary catecholamine excretion correlated also with urine volume.

Secretory cervical schwannoma: first of its kind

Schwannomas arising from cervical sympathetic chain are rare benign neoplasms which are slow growing, usually asymptomatic, biochemically non-secretory and functionally inactive tumours. We present a case of secretory schwannoma arising from the cervical sympathetic chain, causing hypertension and associated with raised urinary catecholamine degradation by-products. Transcervical excision of the tumour was followed by normalisation of blood pressure and urinary vanillylmandelic acid levels and pathologically the tumour was proved to be a schwannoma.

Anxiety and undiagnosed pheochromocitoma

IntroductionPheochromocytomas are rare catecholamine-secreting tumours from the adrenal medulla. The clinical presentations may mimic other disorders due to nonspecific symptoms produced by catecholamines in blood.ObjectivesWe report a case of undiagnosed pheochromocytoma in a 29-year-old woman with a two year history of anxiety. We undertook a literature review.MethodsPubMed search using “pheochromocytoma”, “anxiety” as key words. Follow by a manual review of identified publication. We report a case of a 29-year-old with a two-year history of anxiety pharmacologically treated without success. She presents episodes of acute anxiety accompanied, sometimes, by nausea or headache, without high blood pressure. She presented a previous medical history of thyroid carcinoma. The patient was referred to the endocrinology service for thyroid review, postulated as potential source for drug resistance. High catecholamine levels were found and pheochromocytoma diagnosis was confirmed histopathologically.ResultsThe releasing catecholamine in blood can lead to a number of symptoms that can include paroxysmal hypertension, headache, palpitations, anxiety, chest/abdominal pain, nausea. Hypertension may not be the main symptom. These symptoms can be misinterpreted as psychiatric disorders, in particularly anxiety disorders. Elevated urinary catecholamine suggested the diagnosis of pheochromocytoma.ConclusionsPheochromocytoma should be included in the differential diagnosis of anxiety disorders, especially if pharmacological treatment is not effective. The suspected cases should be investigated, even in the absence of hypertension, due to the potential morbidity of untreated pheochromocytoma.Disclosure of interestThe authors have not supplied their declaration of competing interest.