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Pheochromocytoma and Paraganglioma

Paragangliomas are rare tumors which form in neural crest tissue, a type of nerve tissue found in the adrenal glands and near certain blood vessels and nerves throughout the body. Paragangliomas that develop in the adrenal glands are called pheochromocytomas. Those that develop outside the adrenal glands ('extra-adrenal') are called paragangliomas. Most of these tumors are considered to be benign and about a quarter of cases are known to be related to inherited genetic conditions.

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Endocrine Cancers

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Lemelin A, Lapoirie M, Abeillon J, et al.
Pheochromocytoma, paragangliomas, and pituitary adenoma: An unusual association in a patient with an SDHD mutation. Case report.
Medicine (Baltimore). 2019; 98(30):e16594 [PubMed] Related Publications
RATIONALE: Pituitary adenomas and paragangliomas are both rare endocrine diseases. Paragangliomas (PGL)/pheochromocytomas (PHEO) are part of an inherited syndrome in about 30% to 40% of cases. Among familial cases, mutations of the succinate dehydrogenase (SDH) subunit genes (succinate dehydrogenase subunit [SDH]B, SDHC, SDHD, succinate dehydrogenase subunit AF2 [SDHAF2] , and SDHA) are the most common cause.
PATIENT CONCERNS: We here report a 31-year-old patient with a known SDHD mutation whose disease has been revealed by a left PHEO during childhood and who presented at age 29 years a large paraganglioma of the right jugular foramen, a concomitant PHEO of the left adrenal and 2 retroperitoneal paragangliomas. A pituitary incidentaloma was found during investigations on a fluorodeoxyglucose (FDG)-positron emission tomography (PET) (FDG-PET).
DIAGNOSIS: A pituitary magnetic resonance imaging (MRI) confirmed the presence of a 14 mm pituitary macroadenoma. The pituitary function was normal except for hypogonadotropic hypogonadism. On examination of the fundus, a diagnosis of Pseudo Foster-Kennedy syndrome was made due to a venous compression of the right jugular vein caused by the paraganglioma (PGL). The pituitary adenoma was not compressive to the optic chiasm.
INTERVENTIONS: A treatment with acetazolamide was started in order to improve intracranial hypertension. The patient couldn't benefit of a surgical approach for the paraganglioma of the right jugular foramen; the patient has been treated with stereotactic radiosurgery (Gamma Knife).
OUTCOMES: The most recent MRI revealed that the right jugular foramen PGL is stable and the latest visual assessment demonstrated stability despite a recent reduction in acetazolamide dosage. A surveillance by MRI of the pituitary adenoma has been planned.
LESSONS: The association of a pituitary adenoma to paragangliomas within a same patient is very uncommon and raises the question of related physiopathological mechanisms.

Wang J, Yuan D, Lu Y, et al.
A large pheochromocytoma requiring aortic and inferior vena caval reconstruction: A case report.
Medicine (Baltimore). 2019; 98(29):e16494 [PubMed] Related Publications
RATIONALE: It is difficult to discriminate malignant pheochromocytoma (PCC) from benign PCC. The requirement of abdominal aortic and inferior vena cava reconstruction is extremely rare.
PATIENT CONCERNS: We here report a case of a large pheochromocytoma in a 56-year-old woman who complained of only hand trembling and had no hypertension or other symptoms. The operation was difficult because of a tight adhesion to the circumference of great vessels. A replacement of the aortocaval vessels with grafts was necessary to remove the tumor completely.
DIAGNOSES: Ultrasonography, computed tomography (CT), and catecholamine assay revealed suspecting the retroperitoneal PCC.
INTERVENTIONS: Tumor excision and reconstruction of the abdominal aorta and inferior vena cava by externally supported polytetrafluoroethylene (ePTFE) vessels were successfully performed.
OUTCOMES: A follow-up CT angiography showed no recurrence and graft vessels presented good patency after 7years. Catecholamine in urine and serum assay was normal.
LESSONS: The complete resection of the tumor and infiltrated great vessels were necessary for the long-term survival of patients with a large PCC. The ePTFE graft is a good substitute for vessel reconstruction.

Yue Y, Xin H, Li FQ, et al.
Asymptomatic left posterior mediastinal functional paraganglioma: A case report.
Medicine (Baltimore). 2019; 98(28):e16383 [PubMed] Free Access to Full Article Related Publications
RATIONALE: Paraganglioma refers to a set of neuroendocrine tumors derived from the chromaffin cells of the adrenal diplomatic ganglion. Paragangliomas can be classified as functional or nonfunctional based on the ability to synthesize and release catecholamines.
PATIENT CONCERNS: We report a 47-year-old man with a functional paraganglioma in the left posterior mediastinum and highlight the key elements of management of mediastinal paragangliomas.
DIAGNOSES: A left posterior mediastinal mass was found by computed tomography (CT) scan and Chest-enhanced CT. Preoperative ultrasound-guided biopsy suggested the possibility of a paraganglioma. A diagnosis of paraganglioma was established by immunohistochemistry.
INTERVENTIONS: The patient underwent single-stage resection of the lesion via left thoracotomy after preoperative oral α-adrenoceptor (phenoxybenzamine) therapy and intravenous fluid resuscitation for two weeks.
OUTCOMES: The postoperative period was uneventful. The patient exhibited no abnormal blood pressure or recurrence during the 12-month follow-up period.
LESSONS SUBSECTIONS AS PER STYLE: Pathological examination alone cannot determine whether it was a benign or malignant paraganglioma, which can be determined by pathological examination combined with distant metastasis. Long-term follow-up is required to assess the treatment effect.

Stolfo GD, Mastroianno S, Maggio A, et al.
Electrical Remodeling of Ventricular Repolarization Abnormality after Treatment in Pheochromocytoma: U Wave Finding in a Retrospective Analysis.
Biomed Res Int. 2019; 2019:2605323 [PubMed] Free Access to Full Article Related Publications
Background: Pheochromocytoma is a rare neuroendocrine tumor, clinically characterized by high blood pressure, palpitations, and headache. It is often associated with abnormalities of the ventricular repolarization phase; the dispersion of ventricular repolarization is the basis for ventricular arrhythmias (torsion de point, ventricular tachycardia or ventricular fibrillation).
Objectives: Analysis of abnormal ventricular repolarization focused on the presence and amount of U wave in patients affected by pheochromocytoma and its modification after surgery.
Materials and Methods: We reviewed pathology records of 722 patients admitted for adrenal nodule or suspected chromaffin-cell tumor and identified 39 patients affected by pheochromocytoma. Metanephrine, normetanephrine, and 3-methoxytyramine have been assessed by determining concentrations in 24-hour urine collection. Standard 12-lead electrocardiogram records have been reviewed with analysis of heart rate, P wave, PR interval, QRS duration, QTc, and U wave. Then we selected and compared 22 patients of 39 affected by pheochromocytoma, with both clinical and electrocardiographic data before and after surgery.
Results: In our cohort of 39 patients affected by pheochromocytoma, we found U wave in ECG, before treatment, in 82.8 percent of patients, while only 37.0 percent after treatment (p<0.001) and we observed a statistically significant correlation between this wave and the urinary metanephrine. After surgery, in the selected 22 patients, we observed a clear significant reduction in systemic blood pressure, fasting glucose, metanephrine, normetanephrine, and 3-methoxytyramine. We found a significant reduction of U wave presence and leads involved in these patients after surgery (90.9% versus 9%). We observed a linear correlation between the amount of U waves in 12-lead electrocardiogram and metanephrine (r
Conclusions: In our retrospective analysis, patients affected by pheochromocytoma presented U wave in electrocardiogram. The presence and amount of U wave were associated with the metanephrine levels and the tumor size with significant reduction after surgical removal.

Inan HC, Yener HM, Karaman E, et al.
Role of Preoperative Embolization in Surgical Treatment of the Carotid Body Paragangliomas.
J Craniofac Surg. 2019 May/Jun; 30(3):e267-e270 [PubMed] Related Publications
In this study, we aimed to show the benefits of preoperative embolization on surgery of carotid body tumors.This is a retrospective study of the medical records of 20 patients who were operated for carotid body paragangliomas during 2011-2017. Computed tomography angiography (BTA) and/or magnetic resonance angiography (MRA) were performed for the entire patients. Patients were assessed according to the Shamblin classification. All patients were subjected to direct cerebral angiography by the femoral route 24 to 48 hours before the surgery and tumor embolization was performed in the appropriate cases (19 patients). The amount of hemorrhage and transfusion need during surgery has been reported. The ages of the patients ranged from 21 to 66, with an average of 45.65. In 20 patients ascendan pharyngeal artery was found as the main artery of tumor. No complications were seen after the embolization procedure. Tumor sizes ranged from 12 mm to 95 mm diameter (mean: 45, 5 mm). 10 patients (50%) were Shamblin type 1, 6 patients (30%) were Shamblin type 2, 4 patients (20%) were Shamblin type 3. In 5 patients (25%), the nerve (N. vagus or hypoglossus) was sacrificed due to cranial nerve involvement. Vagal and hypoglossal nerve sacrificed together in 4 patients.These patients' tumor sizes were bigger than 70 mm. Only in 1 patient, internal carotid artery injured and reconstructed with saphenous vein graft. Neurological deficit did not develop postoperatively. Bleeding was mean 275 mL (125 mL-700 mL). In conclusion, we believe that preoperative embolization of carotid paraganglioma is an effective procedure that helps surgeon during dissection with minimal blood loss. The embolization is relatively easy procedure without no additional complications.

Xue X, Chen H, Xia Q, et al.
Detection of Plasma Catecholamines in Human Pheochromocytoma and Primary Hypertension Based on Liquid Chromatography Tandem Mass Spectrometry.
Ann Clin Lab Sci. 2019; 49(2):204-211 [PubMed] Related Publications
BACKGROUND: The measurement of plasma catecholamines (CAs) including dopamine (DA), epinephrine (E), and norepinephrine (NE) and their derivatives including metanephrine (MN), normetanephrine (NMN), vanillylmandelic acid (VMA), and homovanillic acid (HVA) has been used in the diagnosis of pheochromocytoma and paraganglioma (PPGL) and primary hypertension (PH) but are typically detected individually when clinical testing. In this study, pre-column derivatization with dansyl chloride (DNS-Cl) combined with an ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) method was developed to simultaneously quantify HVA, VMA, MN, NMN, DA, E, and NE in the plasma from patients with PPGL and PH.
METHODS: Plasma samples were extracted by acetonitrile and derivatized with DNS-Cl, followed by reverse phase separation and triple quadruple detection. Quantification of the CAs and their derivatives in 10 PPGL, 10 PH, and 100 healthy subjects was performed by UPLC-MS/MS analysis.
RESULTS: All the values of detected CAs/derivatives were in the linearity ranges of the fitted curves. The expression levels of the seven CAs in the PPGL and PH patients were significantly higher than the healthy controls, suggesting increased CA production in the former. There were significant differences in plasma NE, NMN, and VMA levels between the PPGL and PH patients, but there was no significant difference in plasma E, MN, DA, and HVA. A discriminant analysis showed that 90% of the final cases were classified correctly based on the detected CAs/derivatives.
CONCLUSIONS: Our results show that the combined detection of the seven CAs/derivatives could be used for the clinical diagnosis of PPGL and PH.

Tyagi S, Singh SK, Narain TA, et al.
Synchronous Paraganglioma Masquerading as 68Ga-PSMA PET/CT-Avid Metastasis in Carcinoma Prostate-How Specific Is 68Ga-PSMA PET/CT?
Clin Nucl Med. 2019; 44(6):e420-e422 [PubMed] Related Publications
We present a case of biopsy-proven prostatic adenocarcinoma with a tracer-avid left paravertebral location at D4 to D5 vertebrae, suggestive of metastasis. However, in view of uncommon location of single-site metastasis at paravertebral region with no tracer-avid regional lymphadenopathy, image-guided biopsy was done, which was suggestive of paraganglioma. Ga-PSMA is known to have variable tracer avidity in multiple nonprostatic benign as well as malignant lesions, and our case adds to the list of nonspecific uptake by this once considered "specific" to prostate PET radiotracer.

Tripathy S, Tripathi M, Dattagupta S, et al.
In Vivo PSMA Expression in Head and Neck Paragangliomas on 68Ga PSMA 11 PET/CT.
Clin Nucl Med. 2019; 44(6):e398-e400 [PubMed] Related Publications
Head and neck paragangliomas are rare and often asymptomatic tumors and mostly present as painless masses. We describe Ga PSMA 11 PET/CT and Ga DOTANOC PET/CT findings of a 40 year old man with triple head and neck paragangliomas with emphasis on exploring the possible theranostic options.

Chowdhary P, Makar GS, Holdbrook T, Spalla TC
Giant Cell Tumor of the Carotid Body: A Rare Tumor in a Novel Location.
Ear Nose Throat J. 2019; 98(3):165-168 [PubMed] Related Publications
Primary giant cell tumor of soft tissue (GCT-ST) is a rare entity that is considered the soft tissue equivalent of giant cell tumor of bone. It most commonly arises in soft tissues of the trunk and extremities, with occurrence in the head and neck being extremely rare. We report a case of GCT-ST of the carotid body, the first report of a tumor of this kind arising from this site in the neck. Giant cell tumor of soft tissue is generally considered a benign tumor with low malignant potential; thus, surgical excision is usually curative. However, due to the location and invasive nature of this patient's tumor, complete excision was not possible. We discuss the implication of this for long-term management of this patient, as well as similarities and differences in clinical presentation, histology, and biological behavior between this case and previously reported cases of GCT-ST.

Jaiswal SK, Sarathi V, Memon SS, et al.
Endocr Pract. 2019; 25(3):211-219 [PubMed] Related Publications
OBJECTIVE: Most of the Indian studies on pheochromocytoma/paraganglioma (PCC/PGL) have focused on PCC, and there is a paucity of information regarding sympathetic paraganglioma (sPGL). Here, we describe the clinical, biochemical, and imaging features of sPGL compared with PCC.
METHODS: This retrospective study included 75 patients with sPGL and 150 patients with PCC. Diagnosis of PCC/PGL was based on surgical histopathology, and if histopathology was not available, on biochemistry and/or radiology.
RESULTS: sPGL was more frequently detected incidentally ( P = .03), normetanephrine-secreting ( P<.01), and metastatic compared with PCC ( P≤.01). sPGL was most commonly located in the organ of Zuckerkandl (OOZ) (49%) and infradiaphragmatic area above the OOZ (27%). Patients with mediastinal sPGL were significantly older than those with sPGL in the OOZ ( P = .03). Primary tumors of metastatic sPGL were significantly larger than those without metastasis (7.8 ± 4 cm vs. 5.6 ± 3.2 cm; P = .004). Percentage arterial enhancement (PAE) >100% was seen in 98% of sPGLs.
CONCLUSION: Incidental presentation, normetanephrine-secreting phenotype, and metastatic disease were more frequent in patients with sPGL than those with PCC. sPGL arose most commonly in the OOZ. Tumor size is an independent predictor of malignancy among sPGL patients. PAE >100% is almost a universal finding in sPGL, and its absence is a sensitive parameter to differentiate sPGL from other abdominal masses.
ABBREVIATIONS: AP = arterial phase; CECT = contrast-enhanced computed tomography; CT = computed tomography; DP = delayed phase; EVP = early venous phase; FDG = fluorodeoxyglucose; fPFMN = fractionated plasma free metanephrine; HU = Hounsfield units; MIBG = metaiodobenzylguanidine; MRI = magnetic resonance imaging; OOZ = organ of Zuckerkandl; PAE = percentage arterial enhancement; PCC = pheochromocytoma; PET = positron emission tomography; PFNMN = plasma free normetanephrine; PGL = paraganglioma; PRRT = peptide receptor radionuclide therapy; PVE = percentage venous enhancement; sPGL = sympathetic paraganglioma; UP = unenhanced phase; VMA = vanillyl mandelic acid.

Peitzsch M, Mangelis A, Eisenhofer G, Huebner A
Age-specific pediatric reference intervals for plasma free normetanephrine, metanephrine, 3-methoxytyramine and 3-O-methyldopa: Particular importance for early infancy.
Clin Chim Acta. 2019; 494:100-105 [PubMed] Related Publications
BACKGROUND: Availability of appropriately established reference intervals for biochemical tests can be troublesome in pediatrics. Here we establish age-specific continuous reference intervals for catecholamine O-methylated metabolites in children evaluated for catecholamine producing tumors, particularly younger children with suspected neuroblastoma.
METHODS: Plasma concentrations of 3-methoxytyramine, normetanephrine, metanephrine, and 3-O-methyldopa were analyzed by liquid chromatography tandem mass spectrometry in 533 children aged 2 days to 18 years.
RESULTS: Concentrations of plasma free normetanephrine, 3-methoxytyramine and 3-O-methyldopa were higher in neonates up until six months of age, but thereafter declined steeply to levels after one year that were <38% those of neonatal concentrations and to further lower concentrations in teenagers that were <23% those in neonates. In contrast, concentrations of plasma free metanephrine showed a reciprocal pattern with 50% lower concentrations in infants below one year compared to later in childhood.
CONCLUSION: The dynamic reciprocal changes in plasma concentrations of normetanephrine, 3-methoxytyramine and 3-O-methyldopa compared to metanephrine during early childhood suggest underlying developmental changes in extra-adrenal and adrenal chromaffin tissue that must be considered for pediatric reference intervals, particularly in infants. With such reference intervals at hand, biochemical testing for catecholamine producing tumors in young children is substantially improved.

Robertson V, Poli F, Hobson B, et al.
A Systematic Review and Meta-Analysis of the Presentation and Surgical Management of Patients With Carotid Body Tumours.
Eur J Vasc Endovasc Surg. 2019; 57(4):477-486 [PubMed] Related Publications
OBJECTIVES: The aim was to determine the mode of presentation and 30 day procedural risks in 4418 patients with 4743 carotid body tumours (CBTs) undergoing surgical excision.
METHODS: This is a systematic review and meta-analysis of 104 observational studies.
RESULTS: Overall, 4418 patients with 4743 CBTs were identified. The mean age was 47 years, with the majority being female (65%). The commonest presentation was a neck mass (75%), of which 85% were painless. Dysphagia, cranial nerve injury (CNI), and headache were present in 3%, while virtually no one presented with a transient ischaemic attack (0.26%) or stroke (0.09%). The majority (97%) underwent excision, but only 21% underwent pre-operative embolisation. Overall, 27% were Shamblin I CBTs; 44% were Shamblin II; and 29% were Shamblin III. The mean 30 day mortality was 2.29% (95% CI 1.79-2.93). The mean 30 day stroke rate was 3.53% (95% CI 2.91-4.29), while the mean 30 day CNI rate was 25.4% (95% CI 24.5-31.22). The prevalence of persisting CNI at 30 days was 11.15% (95% CI 8.42-14.64). Twelve series (544 patients) correlated 30 day stroke with Shamblin status. Shamblin I CBTs were associated with a 1.89% stroke rate (95% CI 0.92-3.82), increasing to 2.71% (95% CI 1.43-5.07) for Shamblin II CBTs and 3.99% (95% CI 2.34-6.74) for Shamblin III tumours. Twenty-six series (1075 patients) correlated CNI rates with Shamblin status: 3.76% (95% CI 2.62-5.35) for Shamblin I CBTs, 14.14% (95% CI 11.94-16.68) for Shamblin II, and 17.10% (95% CI 14.82-19.65) for Shamblin III tumours. The prevalence of neck haematoma requiring re-exploration was 5.24% (95% CI 3.45-7.91). The proportion of patients with a neck haematoma requiring re-exploration was not reduced by pre-operative embolisation (5.92%; 95% CI 2.56-13.08) vs. no embolisation (5.82%; 95% CI 2.76-11.88). Pre-operative embolisation did not reduce drainage losses (639 mL vs. 653 mL).
CONCLUSIONS: This is the largest meta-analysis of outcomes after CBT excision. Procedural risks associated with tumour excision were considerable, especially with Shamblin III tumours where 4% suffered a peri-operative stroke and 17% suffered a CNI.

Thuillier P, Risson JR, Eusen Y, et al.
Retroperitoneal Pelvic Solitary Fibrous Tumor With High Tracer Uptake in 68Ga-DOTATOC PET/CT: A Rare Differential Diagnosis of Paraganglioma.
Clin Nucl Med. 2019; 44(5):e370-e371 [PubMed] Related Publications
We report the case of a 27-year-old woman referred to our department for the characterization of a 7 × 6-cm left ischio-anal fossa mass by Ga-DOTATOC PET/CT. Several diagnoses were previously mentioned, in particular, myxoid sarcoma but also pelvic paraganglioma. Urinary methoxylated derivates and blood chromogranin A tests were negative. PET/CT showed a high Ga-DOTATOC uptake of the mass that was strongly consistent with paraganglioma diagnosis. Histopathological analysis surprisingly revealed a solitary fibrous tumor without aggressive criteria.

Pierre C, Agopiantz M, Brunaud L, et al.
COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas.
Virchows Arch. 2019; 474(6):721-734 [PubMed] Related Publications
Current histoprognostic parameters and prognostic scores used in paragangliomas and pheochromocytomas do not adequately predict the risk of metastastic progression and survival. Here, using a series of 147 cases of paraganglioma and pheochromocytoma, we designed and evaluated the potential of a new score, the COPPS (COmposite Pheochromocytoma/paraganglioma Prognostic Score), by taking into consideration three clinico-pathological features (including tumor size, necrosis, and vascular invasion), and the losses of PS100 and SDHB immunostain to predict the risk of metastasis. We compared also the performance of the COPPS with several presently used histoprognostic parameters in risk assessment of these tumors. A PASS score (Pheochromocytoma of the Adrenal gland Scaled Score) ≥ 6 was significantly associated with the occurrence of metastases (P < 0.0001) and shorter PFS (P = 0.013). In addition, both MCM6 and Ki-67 LI correlated with worse PFS (P = 0.004 and P < 0.0001, respectively), and MCM6, but not Ki-67, was significantly higher in metastatic group (P = 0.0004). Loss of PS100 staining correlated with the occurrence of metastasis (P < 0.0001) and shorter PFS (P < 0.0001). At a value of greater or equal to 3, the COPPS correlated with shorter PFS (P < 0.0001), and predicted reproducibly (weighted Kappa coefficient, 0.863) the occurrence of metastases with a sensitivity of 100.0% and specificity of 94.7%. It thus surpassed those found for either PASS, SDHB, MCM6, or Ki-67 alone. In conclusion, while validation is still necessary in independent confirmatory cohorts, COPPS could be of great potential for the risk assessment of metastasis and progression in paragangliomas and pheochromocytomas.

Kumar M, Dabas A, Manchanda V, et al.
Hyponatremic-Hypertensive Syndrome in Ovarian Paraganglioma.
Indian Pediatr. 2019; 56(1):69-71 [PubMed] Related Publications
BACKGROUND: Hyponatremic-hypertensive syndrome (HHS) is characterized by combination of polyuria, polydipsia, hypertension, hyponatremia and hypokalemia in association with unilateral renal artery stenosis.
CASE CHARACTERISTICS: A 10-year- old girl presented with polyuria, polydipsia, hypertension, hyponatremia, hypokalemia and proteinuria. Ultrasonography with doppler study revealed bilateral normal renal arteries. Completed tomography of abdomen detected a left adnexal mass, which was later confirmed as ovarian paraganglioma on histopathology.
OUTCOME: After tumor excision, polyuria subsided and blood pressure normalized.
MESSAGE: Hyponatremic-Hypertensive Syndrome does not always result from unilateral renal artery stenosis. High index of clinical suspicion with appropriate imaging technique may clinch rare endocrine causes of hypertension, like paraganglioma.

Godoy-Reyes TM, Costero AM, Gaviña P, et al.
Colorimetric detection of normetanephrine, a pheochromocytoma biomarker, using bifunctionalised gold nanoparticles.
Anal Chim Acta. 2019; 1056:146-152 [PubMed] Related Publications
A simple and effective colorimetric method for the detection of normetanephrine (NMN), an O-methylated metabolite of norepinephrine, using functionalised gold nanoparticles is described. This metabolite is an important biomarker in the diagnosis of adrenal tumours such as pheocromocytoma or paraganglioma. The colorimetric probe consists of spherical gold nanoparticles (AuNPs) functionalised with two different ligands, which specifically recognize different functional groups in normetanephrine. Thus, a benzaldehyde-terminated ligand was used for the recognition of the amino alcohol moiety in NMN, by forming the corresponding oxazolidine. On the other hand, N-acetyl-cysteine was chosen for the recognition of the phenolic hydroxyl group through the formation of hydrogen bonds. The selective double molecular recognition between the probe and the hydroxyl and the amino-alcohol moieties of normetanephrine led to interparticle-crosslinking aggregation resulting in a change in the color of the solution, from red to blue, which could be observed by naked eye. The probe was highly selective towards normetanephrine and no color changes were observed in the presence of other neurotransmitter metabolites such as homovanillic acid (HVA) (dopamine metabolite), 5-hydroxyindoleacetic acid (5-HIAA) (serotonin metabolite), or other biomolecules present in urine such as glucose (Glc), uric acid (U.A), and urea. Finally, the probe was evaluated in synthetic urine with constituents that mimic human urine, where a limit of detection of 0.5 μM was achieved.

Mravec B, Dubravicky J, Tibensky M, Horvathova L
Effect of the nervous system on cancer: Analysis of clinical studies.
Bratisl Lek Listy. 2019; 120(2):119-123 [PubMed] Related Publications
Preclinical data have shown that neurotransmitters released in peripheral tissues from nerve endings may influence carcinogenesis, affect the tumor microenvironment, and directly potentiate both proliferation and migration of cancer cells. This stimulatory role of the nervous system in cancer initiation and progression has also been documented by clinical studies investigating the effect of attenuated signaling from nerves innervating cancer tissue. However, compared to preclinical studies, clinical studies are rarer and some of them have ambiguous results. In this retrospective analysis, to assess the effect of the nervous system on cancer, we analyzed published clinical studies investigating the incidence of cancer in patients with spinal cord injury or pheochromocytoma. Our findings support a concept of the neurobiology of cancer based on the assumption that the nervous system affects cancer initiation and progression (Ref. 60). Keywords: cancer, neurobiology of cancer, norepinephrine, sympathetic nervous system, spinal cord injury, pheochromocytoma.

Shen G, Su M, Kuang A
Primary Pulmonary Paraganglioma Revealed by FDG PET/CT.
Clin Nucl Med. 2019; 44(4):e311-e312 [PubMed] Related Publications
Primary pulmonary paraganglioma is a rare type of extra-adrenal paragangliomas arising from neuroectodermal-derived paraganglionic tissue. We report herein the case of a 49-year-old woman with a round-like well-defined nodule in the right middle lobe detected by chest CT. FDG PET/CT also revealed this homogeneous nodule with increased FDG accumulation. Finally, the patient underwent right middle lobe resection, and the pathological findings demonstrated the diagnosis of pulmonary paraganglioma.

Bobadilla-Rosado LO, Garcia-Alva R, Anaya-Ayala JE, et al.
Surgical Management of Bilateral Carotid Body Tumors.
Ann Vasc Surg. 2019; 57:187-193 [PubMed] Related Publications
BACKGROUND: Carotid body tumors (CBTs) are rare neoplasms located in the carotid bifurcation. The majority of these tumors are unilateral; bilateral CBTs represent approximately 5% of all affected patients, and the recommended treatment is to surgically remove them in staged-planned surgeries. We describe the experience, outcomes, and the surgical management of bilateral CBTs in our institution.
METHODS: A retrospective review of CBTs patients was completed; patient demographics, comorbidities, lesion location, anatomic characteristics, surgical techniques, complications, reinterventions, and other factors that may influence outcomes were evaluated.
RESULTS: A total of 109 patients with CBTs were treated surgically; of these, 8 had bilateral CBTs (7%); the mean age was 56 years, and 7 (87%) were females. Thirteen surgical resections were performed, and in 2 of the cases, the pathology report was malignant (15%). Five were classified as Shamblin I (31%), 5 as Shamblin II (31%), and remaining 6 as Shamblin III (38%). The mean time between the first and second procedure was of 10.7 months. Complications included one case of neck hematoma requiring evacuation and postoperative neurologic complications occurred in three patients (one patient with facial and two with vocal cord palsies). None of the studied individuals had a family history of CBT, and all of them lived in altitude areas higher than 2000 meters above mean sea level (mamsl). The mean tumor size was 3.55 cm and 2.75 cm for right and left CBTs, respectively.
CONCLUSIONS: A better understanding of the clinical characteristics of patients with bilateral CBTs may lead to a more standardized and optimal management with fewer complications and a better quality of life afterward.

Abbas A, Braswell M, Bernieh A, Brodell RT
Glomuvenous malformations in a young man.
Dermatol Online J. 2018; 24(10) [PubMed] Related Publications
A young man presented with blue nodules on the trunk, face, and extremities that gradually increased in number and size. His mother had similar lesions. Initially, blue rubber bleb nevus syndrome was suspected, but histological findings confirmed the diagnosis of hereditary glomuvenous malformations. Making the correct diagnosis spares the patient unnecessary evaluation for the arteriovenous malformations of the gastrointestinal tract associated with the former diagnosis.

Heinze A, Nikomanis P, Petzold F, et al.
Undiagnosed paraganglioma; A challenge during laparoscopic retroperitoneal resection.
Arch Ital Urol Androl. 2019; 90(4):297-298 [PubMed] Related Publications
OBJECTIVE: Report our experience of the management of a patient with undiagnosed retroperitoneal paraganglioma and the intraoperative complications that the theatre team faced.
CASE REPORT: We present a case of a 36-year-old patient who during oncological follow-up for a previous diagnosis of parotid acinar cell carcinoma was incidentally identified as having an interaortocaval tumour. Following routine preoperative assessment the patient was arranged to undergo a laparoscopic retroperitoneal tumour resection. After minimal tumour manipulation the patient developed cardiac rhythm abnormalities and became hypertensive. The tumour was successfully removed laparoscopically after a cautious inter- aortocaval dissection. Abruptly, prior to extraction of the tumour containing endobag, the patient developed cardiac arrest. Following 35 minutes of life support measures there was a return of spontaneous circulation. The endobag was laparoscopically removed from the abdominal cavity 24 hours later using the initial operative port sites. The patient´s progression was satisfactory and he could be discharged six days postoperatively.
CONCLUSIONS: Asymptomatic undiagnosed paragangliomas represent a real challenge during laparoscopic operations. Haemodynamic changes and life-threatening events can arise acutely intraoperatively, where an immediate and coordinated response of the whole theatre team may be required to avoid fatal outcome.

Mendenhall WM, Strojan P, Beitler JJ, et al.
Radiotherapy for parapharyngeal space tumors.
Am J Otolaryngol. 2019 Mar - Apr; 40(2):289-291 [PubMed] Related Publications
A wide variety of tumors, both benign and malignant, occur in the parapharyngeal space. Depending on histology and extent, treatment may include surgery and/or radiotherapy (RT). Herein we discuss the role of RT in the management of some of the more commonly encountered neoplasms, including salivary gland tumors, paragangliomas, schwannomas, and soft-tissue sarcomas.

Tomoyasu M, Mori Y, Fukase A, et al.
Pheochromocytoma presenting with severe hyperglycemia and metabolic acidosis following intra-articular glucocorticoid administration: a case report.
J Med Case Rep. 2019; 13(1):3 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: There are several reports of pheochromocytoma crisis triggered by systemic glucocorticoid administration. However, pheochromocytoma crisis after intra-articular glucocorticoid administration has been rarely reported.
CASE PRESENTATION: A 45-year-old Japanese man presented to our hospital with a sudden, severe headache. He had no history of diabetes. He had received an intra-articular injection of betamethasone (2 mg) for joint pain, 2 days prior to his admission. On examination, his blood pressure was 240/126 mmHg and pulse was 120 beats/minute. The possibility of cerebrovascular events was ruled out by imaging studies and lumbar puncture. Blood tests revealed severe hyperglycemia (523 mg/dL) and metabolic acidosis (pH 7.21, anion gap 26.2 mEq/L, lactate 11.75 mmol/L) with a glycosylated hemoglobin level of 5.7%. Although a urine sample could not be obtained, fulminant type 1 diabetes mellitus and diabetic ketoacidosis were suspected based on these findings. However, after the initial treatment for diabetic ketoacidosis, his insulin secretion was found to be normal and the plasma levels of ketones were not elevated. This excluded the possibility of fulminant type 1 diabetes mellitus and diabetic ketoacidosis. Subsequently, a left adrenal gland tumor and elevated levels of serum catecholamine and urinary catecholamine metabolites were detected, while his other hormone levels were normal. Serum catecholamine levels did not decrease following the clonidine test, and a functional scintigraphy using iodine-131 metaiodobenzylguanidine showed strong uptake in the region of the left adrenal gland. Although no signs of pheochromocytoma crisis, such as paroxysmal hyperglycemia and hypertension, had been observed since admission, a pheochromocytoma was diagnosed based on the investigations. After controlling his blood pressure, a left adrenalectomy was performed.
CONCLUSIONS: This case illustrates that intra-articular glucocorticoid administration can induce a pheochromocytoma crisis and an increase in hyperglycemia, and that pheochromocytoma crisis can resemble the clinical picture of fulminant type 1 diabetes mellitus owing to severe hyperglycemia with metabolic acidosis and normal glycosylated hemoglobin levels, especially under the influence of glucocorticoid.

Gibson J, Coucher J, Coulter C, Eather G
Pleuropulmonary tuberculosis with spinal lesions due to metastatic malignancy differentiated definitively on imaging.
BMJ Case Rep. 2018; 11(1) [PubMed] Related Publications
A healthy 31-year-old man presenting with back pain was found to have multiple spinal enhancing lesions on MRI. An incidental asymptomatic large pleural effusion was identified on investigations for the back pain and pleural and pulmonary tuberculosis (TB) was subsequently diagnosed. The radiographical features on MRI spine were not typical of spinal TB and a Ga68 DOTATATE Positron Emission Tomography (PET)/CT confirmed metastatic paraganglioma with multiple bone metastases. Although metastatic paraganglioma is rare, this case highlights that even in young patients dual pathology needs to be considered. Most importantly, it is a reminder to physicians managing TB of the clues that help distinguish spinal TB from important alternative causes, including metastatic malignancy.

Tafreshi S, Naqvi SY, Thomas S
Extra-adrenal pheochromocytoma presenting as inverse takotsubo-pattern cardiomyopathy treated with surgical resection.
BMJ Case Rep. 2018; 11(1) [PubMed] Related Publications
Pheochromocytoma is a rare catecholamine-secreting tumour that is typically located in the adrenal medulla or along the sympathetic ganglia. The typical symptoms are episodic in nature and include tachycardia, sweating and headache. These tumours can present as transient, reversible cardiomyopathy similar to takotsubo cardiomyopathy (TCM). TCM is characterised by transient hypokinesis of the left ventricular apex and is typically induced by emotional stress. We describe the case of a 26-year-old woman with a medical history significant for headaches who presented initially to her family physician with nausea, vomiting, headache and hypertension. She was started on lisinopril 10 mg daily. One week later, she presented to the emergency department with substernal severe chest pressure. Her troponin level was elevated. Coronary angiogram showed normal coronary arteries and left ventriculogram showed inverse TCM pattern. Serum catecholamines were very elevated confirming pheochromocytoma. She was successfully treated with alpha-blockers followed by surgical resection.

Liang YC, Wu YP, Chen DN, et al.
Building a Competing Endogenous RNA Network to Find Potential Long Non-Coding RNA Biomarkers for Pheochromocytoma.
Cell Physiol Biochem. 2018; 51(6):2916-2924 [PubMed] Related Publications
BACKGROUND/AIMS: Accumulating evidence has shown that long non-coding RNAs (lncRNAs) in competing endogenous RNA (ceRNA) networks play crucial roles in tumor survival and patient prognosis; however, studies investigating ceRNA networks in pheochromocytoma (PCC) are lacking. In this study, we investigated the pathogenesis of PCC and whether lncRNAs acting through ceRNAs networks were associated with prognosis.
METHODS: A total of 183 PCC samples and 3 control samples from The Cancer Genome Atlas database were analyzed. The Empirical Analysis of Digital Gene Expression Data package in R (edgeR) was used to analyze differentially expressed RNAs. Biological processes and pathways functional enrichment analysis were performed based on the Database for Annotation, Visualization, and Integrated Discovery (DAVID) database. LncRNA/mRNA/miRNA ceRNA network was constructed by Cytoscape v3.0 software based on the differentially expressed RNAs Survival package in R was used to perform survival analysis.
RESULTS: In total, 554 differentially expressed lncRNAs, 1775 mRNAs and 40 miRNAs were selected for further analysis. Subsequently, 23 lncRNAs, 22 mRNAs, and 6 miRNAs were included in the constructed ceRNA network. Meanwhile, two of the 23 lncRNAs (C9orf147 and BSN-AS2) were identified as independent predictors of overall survival in PCC patients (P< 0.05).
CONCLUSION: This study improves the understanding of lncRNA-related ceRNA networks in PCC and suggests that the lncRNAs C9orf147 and BSN-AS2 could be independent prognostic biomarkers and potential therapeutic targets for PCC.

Abe I, Fujii H, Ohishi H, et al.
Differences in the actions of adrenaline and noradrenaline with regard to glucose intolerance in patients with pheochromocytoma.
Endocr J. 2019; 66(2):187-192 [PubMed] Related Publications
Glucose intolerance is often observed in patients with pheochromocytoma. However, it remains controversial issue that glucose intolerance on pheochromocytoma is caused by impaired insulin secretion and/or by increased insulin resistance. We aimed to reveal the mechanism of glucose intolerance on pheochromocytoma with regard to the type and amount of catecholamines released. We evaluated 12 individuals diagnosed with pheochromocytoma and who underwent surgery to remove it. We examined glycemic parameters before and after surgery and investigated the association between the change of parameters of insulin secretion (homeostasis model assessment of β-cell function (HOMA-β)), insulin resistance (homeostasis model assessment of insulin resistance (HOMA-IR)) and that of urinary levels of metanephrine/normetanephrine before and after surgery. Overall, fasting plasma glucose, glycated hemoglobin (HbA1c), HOMA-β, and HOMA-IR were improved significantly after surgery. Regression analysis showed that the improvement in HOMA-β from before to after surgery was significantly positively associated with an improvement in urinary levels of metanephrine from before to after surgery and showed a significantly negative association with improvement in urinary levels of normetanephrine from before to after surgery. The improvement in HOMA-IR from before to after surgery was significantly positively associated with an improvement in urinary levels of normetanephrine from before to after surgery. Our results showed that pheochromocytoma extirpation improved glycemic parameters. Furthermore, the different effects elicited by excess amounts of adrenaline and noradrenaline on glucose intolerance were demonstrated.

Noel JE, Sajjadi H
KTP-laser-assisted endoscopic management of glomus tympanicum tumors: A case series.
Ear Nose Throat J. 2018; 97(12):399-402 [PubMed] Related Publications
Endoscopic technology is widely used in rhinology and anterior skull base surgery, but it has been less quickly incorporated into otologic practice. The design of the instrumentation forces surgeons to work one-handed and limits depth perception. Nevertheless, endoscopy also offers wide fields of view and access to spaces that are typically difficult to visualize. Its advantages have broadened the type and extent of operations that can be performed via the ear canal. We describe a method of endoscopic resection of glomus tympanicum tumors in 5 adults who had undergone endoscopic or endoscopy- assisted resection. A successful resection was achieved in all patients-exclusively via the ear canal in 4 of them. A KTP laser was used to address the tumor's vascular supply. Attachment of a neonatal feeding tube to the endoscope for use as a suction catheter obviated the need to repeatedly switch instruments while using the laser. At a minimum of 12 months of follow-up, all patients were free of recurrence. Postoperative audiometry detected no significant adverse hearing outcomes in any patient. We conclude that the minimally invasive endoscopic transcanal approach is a feasible technique for addressing middle ear tumors. We have also developed a method that allows surgeons constant use of the KTP laser to resect a glomus tympanicum tumor.

Almeida MQ, Bezerra-Neto JE, Mendonça BB, et al.
Primary malignant tumors of the adrenal glands.
Clinics (Sao Paulo). 2018; 73(suppl 1):e756s [PubMed] Free Access to Full Article Related Publications
Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

Bai S, Yao Z, Zhu X, et al.
Risk factors for postoperative cardiovascular morbidity after pheochromocytoma surgery: a large single center retrospective analysis.
Endocr J. 2019; 66(2):165-173 [PubMed] Related Publications
Surgical resection is the primary treatment strategy for pheochromocytoma; however, it carries a high risk of morbidity and mortality, especially with respect to cardiovascular complications, which is the most common kinds of morbidity. The risk factors for morbidity remain unclear and require further exploration, moreover no studies focus on risk factors for cardiovascular morbidity. Herein we identified the risk factors for cardiovascular morbidity after pheochromocytoma surgery in Chinese patients. We retrospectively reviewed 262 patients who underwent unilateral surgical resection of pheochromocytoma at our center between 1 January 2007 and 31 December 2016. Patient demographics and extensive perioperative data were recorded and evaluated. Adjusted odds ratios and 95% confidence intervals were determined by multivariate logistic regression. Cut-off values and the area under the curve for continuous risk factors were calculated based on receiver operating characteristic curve analysis. A p-value <0.05 was considered statistically significant. Of the 262 patients, 63 (24.0%) had cardiovascular morbidity. The independent risk factors for cardiovascular morbidity were low body mass index, large radiographic tumor size, coronary heart disease, no preoperative crystal/colloid administration, and intraoperative hemodynamic instability; the corresponding odds ratio were 0.762 (p < 0.001), 1.208 (p = 0.010), 2.378 (p = 0.012), 2.720 (p = 0.011), and 4.764 (p = 0.001), respectively. The optimal cut-off values for body mass index and radiographic tumor size were 24.59 kg/m

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