-
Medicina (Kaunas, Lithuania) Apr 2024Paragangliomas (PGLs) are rare neuroendocrine extra-adrenal tumors that could be secreting mass. The symptoms are the typical triad of paroxysmal headache, hypertension...
Paragangliomas (PGLs) are rare neuroendocrine extra-adrenal tumors that could be secreting mass. The symptoms are the typical triad of paroxysmal headache, hypertension and sweating, but could also be accompanied by symptoms involving multiple organs. Surgery is the gold standard treatment for both PGLs and pheochromocytomas (PHEOs). We used a computerized endocrine surgery registry to record the demographic and clinical data of 153 patients who underwent surgery for PPGL between 2010 and 2023 at our hospital. Thirteen patients (8.43%) with paragangliomas underwent surgery at our institute. Five patients presented symptomatic syndrome. Preoperative investigations included enhanced abdominal CT (nine patients) and enhanced MRI (seven patients). In cases of suspicious mass, we performed 131I-MIBG scans (two patients) or 68GA-DOTATOC PET-CT scans (11 patients). Laparoscopic approach was used in four cases (30.7%) and abdominal laparotomy in the other nine (69.3%). Biochemical tests were performed on all patients. In this retrospective study, we discuss the multidisciplinary management in our institute of this rare disease, from its challenging diagnosis to the surgical strategy for PGLs. Laparoscopic surgery is the gold standard, but a tailored approach should be adopted for each patient.
Topics: Humans; Female; Male; Middle Aged; Adult; Retrospective Studies; Paraganglioma; Aged; Positron Emission Tomography Computed Tomography; Abdominal Neoplasms; Tomography, X-Ray Computed
PubMed: 38674250
DOI: 10.3390/medicina60040604 -
Cancers Apr 2024Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed...
Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with 69.0% female and 26.5% patients with multiple PGLs. Among 119 patients undergoing genetic testing, 70 (58.8%) patients had mutations, with SDHB (30) and SDHD (26) being the most common. The rates of metastasis and recurrence were higher among patients with SDHB mutations or SDHD mutations associated with multiple PGLs. Metabolic evaluation showed elevated plasma dopamine levels were the most common derangements in HNPGL. MRI and CT were the most common anatomic imaging modalities and DOTATATE was the most common functional scan used in this cohort. Most patients (81.5%) received surgery as the primary definitive treatment, while 22.5% patients received radiation treatment, mostly as an adjuvant therapy or for surgically challenging or inoperable cases. Systemic treatment was rarely used in our cohort. Our single-center experience highlights the need for referral for genetic testing and metabolic evaluation and for a team-based approach to improve the clinical outcomes of patients with HNPGLs.
PubMed: 38672605
DOI: 10.3390/cancers16081523 -
Cancers Apr 2024To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients.
OBJECTIVE
To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients.
STUDY DESIGN
Retrospective study.
METHOD
The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated.
RESULT
Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor ( < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold ( < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%.
CONCLUSIONS
Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.
PubMed: 38672548
DOI: 10.3390/cancers16081466 -
Medicine Apr 2024Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors with diverse clinical presentations. Alterations in energy expenditure state are commonly... (Review)
Review
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors with diverse clinical presentations. Alterations in energy expenditure state are commonly observed in patients with PPGL. However, the reported prevalence of hypermetabolism varies significantly and the underlying mechanisms and implications of this presentation have not been well elucidated. This review discusses and analyzes the factors that contribute to energy consumption. Elevated catecholamine levels in patients can significantly affect substance and energy metabolism. Additionally, changes in the activation of brown adipose tissue (BAT), inflammation, and the inherent energy demands of the tumor can contribute to increased resting energy expenditure (REE) and other energy metabolism indicators. The PPGL biomarker, chromogranin A (CgA), and its fragments also influence energy metabolism. Chronic hypermetabolic states may be detrimental to these patients, with surgical tumor removal remaining the primary therapeutic intervention. The high energy expenditure of PPGL has not received the attention it deserves, and an accurate assessment of energy metabolism is the cornerstone for an adequate understanding and treatment of the disease.
Topics: Humans; Energy Metabolism; Pheochromocytoma; Paraganglioma; Adrenal Gland Neoplasms; Catecholamines; Adipose Tissue, Brown; Chromogranin A
PubMed: 38669419
DOI: 10.1097/MD.0000000000037916 -
Diagnostics (Basel, Switzerland) Apr 2024A challenging task in routine practice is finding the distinction between benign and malignant paragangliomas and pheochromocytomas. The aim of this study is to conduct...
A challenging task in routine practice is finding the distinction between benign and malignant paragangliomas and pheochromocytomas. The aim of this study is to conduct a comparative analysis of angiogenesis by assessing intratumoral microvascular density (MVD) with immunohistochemical (IHC) markers (CD31, CD34, CD105, ERG), and S100 immunoreactivity, Ki67 proliferative index, succinate dehydrogenase B (SDHB) expressiveness, tumor size with one the most utilized score Pheochromocytoma of Adrenal Gland Scales Score (PASS), using tissue microarray (TMA) with 115 tumor samples, 61 benign (PASS < 4) and 54 potentially malignant (PASS ≥ 4). We found no notable difference between intratumoral MVD and potentially malignant behavior. The group of potentially malignant tumors is significantly larger in size, has lower intratumoral MVD, and a decreased number of S100 labeled sustentacular cells. Both groups have low proliferative activity (mean Ki67 is 1.02 and 1.22, respectively). Most tumors maintain SDHB expression, only 6 cases (5.2%) showed a loss of expression (4 of them in PASS < 4 group and 2 in PASS ≥ 4). PASS score is easily available for assessment and complemented with markers of biological behavior to complete the risk stratification algorithm. Size is directly related to PASS score and malignancy. Intratumoral MVD is extensively developed but it is not crucial in evaluating the malignant potential.
PubMed: 38667494
DOI: 10.3390/diagnostics14080849 -
World Journal of Surgical Oncology Apr 2024Carotid body paraganglioma (CBP) is a rare, highly vascularized, and slow-growing neuroendocrine tumor. Surgical resection is the definitive treatment for CBP, however,...
BACKGROUND
Carotid body paraganglioma (CBP) is a rare, highly vascularized, and slow-growing neuroendocrine tumor. Surgical resection is the definitive treatment for CBP, however, it remains challenging due to the tumor's proximity to critical blood vessels and cervical cranial nerves. This study aimed to document the characteristics of CBP and examine the clinical outcomes of patients following surgical extirpation of CBP.
METHODS
This is a single-center retrospective review analyzed patients who underwent CBP extirpation. We examined the patient demographics, preoperative clinical features, tumor characteristics, levels of catecholamines and their metabolites in the serum and urine. Surgeries were performed by one vascular surgeon with follow-ups at 1,3,6 months and yearly thereafter. Logistic regression analysis was conducted to identify risk factors associated with the occurrence of either permanent or temporary cervival cranial nerve palsy (CNP).
RESULTS
From September 2020 to February 2023, this study examined 21 cases of CBP removal surgeries that were carried out in 19 patients. The mean age of the patients was 38.9 ± 10.9 years and the percentage of males was 57.1% (n = 12). The most common preoperative clinical feature was painless neck mass (n = 12; 57.1%). Complete resection was achieved in 20 cases; excluding one case with pathologically proven sclerosing paraganglioma. Vascular procedures were performed in four cases (ECA resection, n = 2; primary repair of ICA tear without carotid shunting, n = 1; and ICA patch angioplasty with carotid shunting, n = 1). Temporary cranial neurologic complications, specifically aspiration and hoarseness occurred in four (19.0%), and three (14.3%) cases, respectively. Hoarseness associated with permanent CNP persisted for more than 6 months in two cases (9.5%). No recurrence or mortality was observed during the follow-up period.
CONCLUSIONS
Surgical resection is the primay treatment approach for CBP; however, it poses risks of vascular or cervical CNP. The intraoperative estimated blood loss was the only identified risk factor for CNP.
Topics: Humans; Male; Female; Carotid Body Tumor; Retrospective Studies; Adult; Middle Aged; Republic of Korea; Follow-Up Studies; Prognosis; Postoperative Complications; Treatment Outcome
PubMed: 38664798
DOI: 10.1186/s12957-024-03390-w -
Journal of the Endocrine Society Apr 2024
PubMed: 38660142
DOI: 10.1210/jendso/bvae070 -
Cureus Mar 2024I-metaiodobenzylguanidine (I-MIBG) scintigraphy is a highly sensitive and specific imaging test for the diagnosis of pheochromocytoma. Typical pheochromocytomas are...
A Case of Pheochromocytoma With Coagulation Necrosis Due to Hypertensive Crisis Aggravated by Contrast-Enhanced CT Scan and Negative 123I-Metaiodobenzylguanidine (MIBG) Scintigraphy.
I-metaiodobenzylguanidine (I-MIBG) scintigraphy is a highly sensitive and specific imaging test for the diagnosis of pheochromocytoma. Typical pheochromocytomas are positive on I-MIBG scintigraphy; however, cases of paragangliomas eliciting negative results have been reported. We encountered a case of hypertensive crisis resulting in extensive coagulative necrosis of a pheochromocytoma and negative findings on I-MIBG scintigraphy. A 50-year-old Japanese female presented with an acute onset of vomiting, epigastralgia, and abdominal pain. Immediately after contrast-enhanced CT, the patient developed respiratory failure and was intubated. The CT scan revealed a 5-cm left adrenal mass, and a pheochromocytoma crisis was suspected. The patient's condition stabilized following phentolamine administration. Regarding the assessment for pheochromocytoma, plasma metanephrine levels were not markedly increased, and I-MIBG scintigraphy was negative. However, a histological examination of the left adrenal mass revealed extensive coagulative necrosis of the entire adrenal mass, comprising trabecular and alveolar growth of large polygonal cells that were immunopositive for chromogranin A/synaptophysin, thereby suggesting a diagnosis of pheochromocytoma. There have been three reported cases of I-MIBG scintigraphy-negative pheochromocytomas because of pure avascular necrosis without hemorrhage or rupture. To the best of our knowledge, this is the first reported case of massive tumor necrosis due to hypertensive crisis exacerbated after contrast-enhanced CT imaging. In conclusion, pheochromocytoma cannot be ruled out even with negative findings on I-MIBG scintigraphy. Accordingly, clinical judgment must be made based on a comprehensive assessment of the clinical course and pathological diagnosis, especially for cases involving a hypertensive crisis.
PubMed: 38659567
DOI: 10.7759/cureus.56878 -
Acta Otorhinolaryngologica Italica :... Apr 2024In carotid paraganglioma surgery, magnification is crucial to properly evaluate the anatomical relationships between mass, carotid wall, cranial nerves, tumour vascular...
OBJECTIVES
In carotid paraganglioma surgery, magnification is crucial to properly evaluate the anatomical relationships between mass, carotid wall, cranial nerves, tumour vascular supply and fascial envelope. The aims of this study are to describe the microsurgical technique, along with the underlying microsurgical anatomy, and to assess outcomes in terms of disease control, complications and functional results.
METHODS
Twenty-six patients, accounting for 29 carotid paragangliomas, treated with microsurgery by the same senior surgeon over a 35-year period, were included.
RESULTS
No carotid injury requiring repair, nor peri- or post-operative stroke occurred in this series. No surgical injury of the main trunk of VII to XII cranial nerves occurred. Complete excision was obtained in all cases and no recurrence was observed during follow-up.
CONCLUSIONS
The small study size and its retrospective nature suggests caution; however, our results show that microsurgery can allow a safe and precise dissection of the carotids and nerves.
Topics: Humans; Carotid Body Tumor; Microsurgery; Middle Aged; Male; Female; Retrospective Studies; Adult; Aged
PubMed: 38651551
DOI: 10.14639/0392-100X-N2761 -
Frontiers in Endocrinology 2024Surgery is the only way to cure pheochromocytoma; however, postoperative hemodynamic instability is one of the main causes of serious complications and even death. This...
PURPOSE
Surgery is the only way to cure pheochromocytoma; however, postoperative hemodynamic instability is one of the main causes of serious complications and even death. This study's findings provide some guidance for improved clinical management.
PATIENTS AND METHODS
This study was to investigate the factors leading to postoperative hemodynamic instability in the postoperative pathology indicated pheochromocytoma from May 2016 to May 2022. They were divided into two groups according to whether vasoactive drugs were used for a median number of days or more postoperatively. The factors affecting the postoperative hemodynamics in the perioperative period (preoperative, intraoperative, and postoperative) were then evaluated.
RESULTS
The median number of days requiring vasoactive drug support postoperatively was three in 234 patients, while 118 (50.4%) patients required vasoactive drug support for three days or more postoperatively. The results of the multivariate analysis indicated more preoperative colloid use (odds ratio [OR]=1.834, confidence interval [CI]:1.265-2.659, P=0.001), intraoperative use of vasoactive drug (OR=4.174, CI:1.882-9.258, P<0.001), and more postoperative crystalloid solution input per unit of body weight per day (ml/kg/d) (OR=1.087, CI:1.062-1.112, P<0.001) were risk factors for predicting postoperative hemodynamic instability. The optimal cutoff point of postoperative crystalloid use were 42.37 ml/kg/d.
CONCLUSION
Hemodynamic instability is a key issue for consideration in the perioperative period of pheochromocytoma. The amount of preoperative colloid use, the need for intraoperative vasoactive drugs, and postoperative crystalloid solution are risk factors for predicting postoperative hemodynamic instability (registration number: ChiCT2300071166).
Topics: Pheochromocytoma; Humans; Female; Male; Adrenal Gland Neoplasms; Hemodynamics; Middle Aged; Adult; Postoperative Complications; Cohort Studies; Adrenalectomy; Retrospective Studies; Risk Factors; Aged; Vasoconstrictor Agents; Crystalloid Solutions
PubMed: 38650714
DOI: 10.3389/fendo.2024.1336128