The inferior performance of [68Ga]Ga-FAPI-04 PET/CT as a diagnostic and theranostic biomarker in [177Lu]Lu-DOTATATE refractory well-differentiated neuroendocrine tumors
ConclusionOur preliminary results demonstrated that [68Ga]Ga-FAPI-04 PET/CT mainly failed in well-differentiated  NETs refractory to [177Lu]Lu-DOTATATE therapy and had a limited role as an alternative diagnostic or theranostic agent. Further investigations with a larger patient population are required to determine the impact of [68Ga]Ga-FAPI-04 PET/CT on NETs. (Source: European Journal of Nuclear Medicine and Molecular Imaging)
Source: European Journal of Nuclear Medicine and Molecular Imaging - January 22, 2024 Category: Nuclear Medicine Source Type: research

Imaging of Pheochromocytomas and Paragangliomas
Endocr Rev. 2024 Jan 11:bnae001. doi: 10.1210/endrev/bnae001. Online ahead of print.ABSTRACTPheochromocytomas/paragangliomas are unique in their highly variable molecular landscape driven by genetic alterations, either germline or somatic. These mutations translate into different clusters with distinct tumor locations, biochemical/metabolomic features, tumor cell characteristics (e.g. receptors, transporters) and disease course. Such tumor heterogeneity calls for different imaging strategies in order to provide proper diagnosis and follow-up. This also warrants selection of the most appropriate and locally available imagin...
Source: Endocrine Reviews - January 11, 2024 Category: Endocrinology Authors: Henri J L M Timmers David Ta ïeb Karel Pacak Jacques W M Lenders Source Type: research

Imaging of Pheochromocytomas and Paragangliomas
Endocr Rev. 2024 Jan 11:bnae001. doi: 10.1210/endrev/bnae001. Online ahead of print.ABSTRACTPheochromocytomas/paragangliomas are unique in their highly variable molecular landscape driven by genetic alterations, either germline or somatic. These mutations translate into different clusters with distinct tumor locations, biochemical/metabolomic features, tumor cell characteristics (e.g. receptors, transporters) and disease course. Such tumor heterogeneity calls for different imaging strategies in order to provide proper diagnosis and follow-up. This also warrants selection of the most appropriate and locally available imagin...
Source: ENDOCR REV - January 11, 2024 Category: Endocrinology Authors: Henri J L M Timmers David Ta ïeb Karel Pacak Jacques W M Lenders Source Type: research

The combination of doxazosin and metyrosine as a preoperative treatment for pheochromocytomas and paragangliomas
ConclusionThe combination of metyrosine and doxazosin as a preoperative treatment for PPGLs affects intraoperative circulatory hemodynamics, such as a reduced occurrence of blood pressure elevation during surgery. Further research is necessary to identify patients who will benefit most from this combination treatment. (Source: Endocrine)
Source: Endocrine - January 11, 2024 Category: Endocrinology Source Type: research

Novel alternative tools for metastatic pheochromocytomas/paragangliomas prediction
ConclusionsModels established in this study provided alternative and reliable tools for clinicians to predict PPGLs patients ’ metastasis and MFS. More importantly, this study revealed for the first time that IHC of ATRX could act as an independent predictor of metastasis in PPGLs. (Source: Journal of Endocrinological Investigation)
Source: Journal of Endocrinological Investigation - January 11, 2024 Category: Endocrinology Source Type: research

The combination of doxazosin and metyrosine as a preoperative treatment for pheochromocytomas and paragangliomas
ConclusionThe combination of metyrosine and doxazosin as a preoperative treatment for PPGLs affects intraoperative circulatory hemodynamics, such as a reduced occurrence of blood pressure elevation during surgery. Further research is necessary to identify patients who will benefit most from this combination treatment. (Source: Endocrine)
Source: Endocrine - January 11, 2024 Category: Endocrinology Source Type: research

Novel alternative tools for metastatic pheochromocytomas/paragangliomas prediction
ConclusionsModels established in this study provided alternative and reliable tools for clinicians to predict PPGLs patients ’ metastasis and MFS. More importantly, this study revealed for the first time that IHC of ATRX could act as an independent predictor of metastasis in PPGLs. (Source: Journal of Endocrinological Investigation)
Source: Journal of Endocrinological Investigation - January 11, 2024 Category: Endocrinology Source Type: research

Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan
ConclusionWhen complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases. (Source: European Archives of Oto-Rhino-Laryngology)
Source: European Archives of Oto-Rhino-Laryngology - January 6, 2024 Category: ENT & OMF Source Type: research

Animal and Cell Culture Models of PPGLs - Achievements and Limitations
Horm Metab Res. 2024 Jan;56(1):51-64. doi: 10.1055/a-2204-4549. Epub 2024 Jan 3.ABSTRACTResearch on rare tumors heavily relies on suitable models for basic and translational research. Paragangliomas (PPGL) are rare neuroendocrine tumors (NET), developing from adrenal (pheochromocytoma, PCC) or extra-adrenal (PGL) chromaffin cells, with an annual incidence of 2-8 cases per million. While most PPGL cases exhibit slow growth and are primarily treated with surgery, limited systemic treatment options are available for unresectable or metastatic tumors. Scarcity of appropriate models has hindered PPGL research, preventing the tr...
Source: Cell Research - January 3, 2024 Category: Cytology Authors: Bhargavi Karna Natalia Simona Pellegata Hermine Mohr Source Type: research

The Great Imitator: Pulmonary Paraganglioma Mimicking Pulmonary Metastasis of Breast Cancer on 18F-FDG PET/CT
We report a case of 58-year-old woman recently diagnosed with multifocal right breast cancer and suspicious right axillary lymph nodes. 18F-FDG PET/CT showed a well-defined marginated lesion in the lower lobe of the right lung with intense FDG uptake. Findings were suspicious for pulmonary metastases. CT-guided biopsy results were consistent with a paraganglioma. Paragangliomas can mimic metastases, especially if they are large or have a high degree of FDG uptake, and careful evaluation of imaging features and clinical findings can help differentiate these entities. Most of the time biopsy is necessary to confirm the diagn...
Source: Clinical Lung Cancer - January 3, 2024 Category: Cancer & Oncology Authors: Sharjeel Usmani Khulood Al Riyami Anjali Jain Rajesh Joseph Selvakumar Vipin V Jayakrishan Source Type: research

The Great Imitator: Pulmonary Paraganglioma Mimicking Pulmonary Metastasis of Breast Cancer on 18F-FDG PET/CT
We report a case of 58-year-old woman recently diagnosed with multifocal right breast cancer and suspicious right axillary lymph nodes. 18F-FDG PET/CT showed a well-defined marginated lesion in the lower lobe of the right lung with intense FDG uptake. Findings were suspicious for pulmonary metastases. CT-guided biopsy results were consistent with a paraganglioma. Paragangliomas can mimic metastases, especially if they are large or have a high degree of FDG uptake, and careful evaluation of imaging features and clinical findings can help differentiate these entities. Most of the time biopsy is necessary to confirm the diagn...
Source: Clinical Breast Cancer - January 3, 2024 Category: Cancer & Oncology Authors: Sharjeel Usmani Khulood Al Riyami Anjali Jain Rajesh Joseph Selvakumar Vipin V Jayakrishan Source Type: research

The Great Imitator: Pulmonary Paraganglioma Mimicking Pulmonary Metastasis of Breast Cancer on 18F-FDG PET/CT
We report a case of 58-year-old woman recently diagnosed with multifocal right breast cancer and suspicious right axillary lymph nodes. 18F-FDG PET/CT showed a well-defined marginated lesion in the lower lobe of the right lung with intense FDG uptake. Findings were suspicious for pulmonary metastases. CT-guided biopsy results were consistent with a paraganglioma. Paragangliomas can mimic metastases, especially if they are large or have a high degree of FDG uptake, and careful evaluation of imaging features and clinical findings can help differentiate these entities. Most of the time biopsy is necessary to confirm the diagn...
Source: Clinical Genitourinary Cancer - January 3, 2024 Category: Cancer & Oncology Authors: Sharjeel Usmani Khulood Al Riyami Anjali Jain Rajesh Joseph Selvakumar Vipin V Jayakrishan Source Type: research

Animal and Cell Culture Models of PPGLs – Achievements and Limitations
Horm Metab Res 2024; 56: 51-64 DOI: 10.1055/a-2204-4549Research on rare tumors heavily relies on suitable models for basic and translational research. Paragangliomas (PPGL) are rare neuroendocrine tumors (NET), developing from adrenal (pheochromocytoma, PCC) or extra-adrenal (PGL) chromaffin cells, with an annual incidence of 2–8 cases per million. While most PPGL cases exhibit slow growth and are primarily treated with surgery, limited systemic treatment options are available for unresectable or metastatic tumors. Scarcity of appropriate models has hindered PPG...
Source: Hormone and Metabolic Research - January 3, 2024 Category: Endocrinology Authors: Karna, Bhargavi Pellegata, Natalia Simona Mohr, Hermine Tags: Review Source Type: research

Genetics of Cardiac Tumours: A Narrative Review
Cardiac tumours can occur in association with genetic syndromes. Rhabdomyomas have been reported in association with tuberous sclerosis, myxomas with Carney's complex, cardiac fibromas with Gorlin syndrome, and paragangliomas with multiple endocrine neoplasm syndrome. The presentation and prognosis of cardiac tumours associated with genetic syndromes differ compared with sporadic cases. Knowledge about the associated syndromes' genetic features and extracardiac manifestations is essential for the diagnosis, prognosis, and management of cardiac neoplasms. (Source: Heart, Lung and Circulation)
Source: Heart, Lung and Circulation - December 30, 2023 Category: Cardiology Authors: Maha M. AlRasheed Tags: Review Source Type: research

Cardiovascular Complications in Pheochromocytoma and Paraganglioma: Does Phenotype Matter?
CONCLUSIONS: We found a 28% incidence of cardiovascular complications in a consecutive group of patients with pheochromocytoma and paraganglioma. Patients presenting with a noradrenergic phenotype have a higher incidence of atherosclerotic complications, while the adrenergic phenotype is associated with a higher incidence of acute myocardial damage due to takotsubo-like cardiomyopathy.PMID:38152977 | DOI:10.1161/HYPERTENSIONAHA.123.21902 (Source: Atherosclerosis)
Source: Atherosclerosis - December 28, 2023 Category: Cardiology Authors: Ond řej Petrák Zuzana Kr átká Robert Holaj Mat ěj Zítek Thi Minh Phuong Nguyen Nikr ýnová Judita Kl ímová Barbora Kolo šová Petr Waldauf David Michalsk ý Kv ětoslav Novák Alice Markvartov á Luk áš Zlatohlávek Tom áš Grus Jaroslava Du Source Type: research