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Frontiers in Veterinary Science 2020Over the last 20 years, substantial knowledge has been developed in Veterinary oncology, and tumors previously reported only in humans have been identified in animals....
Over the last 20 years, substantial knowledge has been developed in Veterinary oncology, and tumors previously reported only in humans have been identified in animals. Primary paragangliomas of the tongue are extremely rare tumors in human beings and have never been reported in animals. A Chow Chow dog showed an ulcerated nodule at the lingual body, deeply infiltrated, which extended to the base of the tongue. A full clinical and pathological investigation was conducted, and a post-surgical follow-up of 6 months did not detect recurrence. Cytological, histological, and immunohistochemical features are presented and support the diagnosis of lingual paraganglioma. The paraganglioma of the tongue reported in this Chow Chow dog shares many similarities with the human counterpart.
PubMed: 32851012
DOI: 10.3389/fvets.2020.00422 -
Cureus Jul 2023Paragangliomas are mostly benign, slow-growing, hypervascular tumors originating from neural crest derivatives. Head and neck (H&N) paragangliomas represent <1% of all...
Paragangliomas are mostly benign, slow-growing, hypervascular tumors originating from neural crest derivatives. Head and neck (H&N) paragangliomas represent <1% of all H&N tumors and <5% are malignant. They are mostly non-secreting tumors that originate from autonomous parasympathetic paraganglia. We present a case of right middle ear jugulotympanic paraganglioma, a subtype of H&N paragangliomas, which had been misdiagnosed as otosclerosis for about 10 years. The patient was suffering from worsening tinnitus along with hearing impairment. High clinical suspicion of jugular paraganglioma prevented us from taking a biopsy. Complete surgical excision after preoperative embolization was decided. Embolization resulted in facial nerve paralysis, however, facial nerve rerouting was performed during the complete surgical excision of the tumor. The patient remains disease-free three years postoperatively, with House-Brackmann III facial nerve paralysis.
PubMed: 37593266
DOI: 10.7759/cureus.41997 -
Vascular Apr 2021Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The most...
OBJECTIVES
Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The most common location of these tumors is within the adrenal medulla. Extra-adrenal pheochromocytomas (EAPs) may occur in any portion of the paraganglion system. The most common location of EAPs is at the organ of Zuckerkandl, which is a collection of chromaffin cells near the origin of the inferior mesenteric artery.
METHODS
We present a case of an EAP of the organ of Zuckerkandl incidentally discovered and resected during urgent open repair of a symptomatic 6.7-cm juxtarenal abdominal aortic aneurysm (AAA).
RESULTS
The patient underwent successful open surgical repair of a juxtarenal AAA and resection of the pheochromocytoma.
CONCLUSIONS
Concomitant pheochromocytomas and abdominal aortic aneurysms are rare, with a small number described in the literature. We describe the case of a simultaneous EAP of the organ of Zuckerkandl and AAA repair. This case demonstrates that these lesions can be safely resected in the same setting as AAA repair.
Topics: Adrenal Gland Neoplasms; Aged; Aortic Aneurysm, Abdominal; Blood Vessel Prosthesis Implantation; Humans; Incidental Findings; Male; Para-Aortic Bodies; Pheochromocytoma; Treatment Outcome
PubMed: 32838691
DOI: 10.1177/1708538120950868 -
Clinical Endocrinology Dec 2019Nuclear imaging findings by virtue of phenotyping disease heavily depend on genetic background, metabolites, cell membrane specific targets and signalling pathways. PPGL...
OBJECTIVES
Nuclear imaging findings by virtue of phenotyping disease heavily depend on genetic background, metabolites, cell membrane specific targets and signalling pathways. PPGL related to succinate dehydrogenase subunits mutations (SDHx mutations) are less differentiated than other subgroups and therefore may lack to concentrate F-FDOPA, a precursor of catecholamines biosynthesis. However, this F-FDOPA negative phenotype has been reported mostly in SDHx-PPGL of sympathetic origin, suggesting that both genotype status and location (from sympathetic vs parasympathetic paraganglia; adrenal vs extra-adrenal) could influence F-FDOPA uptake. The aim of this study was to test if SDHx drives F-FDOPA uptake in presence of normal epinephrine/norepinephrine concentrations.
DESIGN
Retrospective study PATIENTS: A cohort of 86 head and neck PPGL patients (including three metastatic) with normal metanephrines underwent F-FDOPA PET/CT. The relationships between F-FDOPA uptake and tumour genotype were evaluated.
RESULTS
In nonmetastatic HNPGL (50 non-SDHx/33 SDHx), no significant difference was observed between these two groups for SUVmax (P = .256), SUVmean (P = .188), MTV 42% (P = .596) and total lesion uptake (P = .144). Metastatic HNPGL also had high elevated uptake values.
CONCLUSIONS
Our results suggest that SDH deficiency or metastatic behaviour have no influence on F-FDOPA uptake in HNPGL probably due to their very-well differentiation status, even at metastatic stage. The potential prognosticator value of F-FDOPA uptake would need to be further explored in the setting of metastatic PPGL of sympathetic origin.
Topics: Adult; Female; Genotype; Head and Neck Neoplasms; Humans; Male; Middle Aged; Mutation; Paraganglioma; Positron Emission Tomography Computed Tomography; Radionuclide Imaging; Retrospective Studies; Succinate Dehydrogenase
PubMed: 31479526
DOI: 10.1111/cen.14086 -
Case Reports in Endocrinology 2020Takotsubo syndrome is a rare but emerging form of acute reversible myocardial injury characterized by transient systolic LV dysfunction, often related to emotional or...
Takotsubo syndrome is a rare but emerging form of acute reversible myocardial injury characterized by transient systolic LV dysfunction, often related to emotional or physical stress. Pheochromocytoma is increasingly recognised as another possible trigger. Pheochromocytoma is a rare catecholamine-secreting tumour arising from chromaffin cells within the adrenal medulla or extra-adrenal paraganglia. The pathognomonic quartet of paroxysmal hypertension, palpitations, headache, and diaphoresis is rarely present, and diagnosis is often delayed. We describe a 43-year-old formerly healthy patient with an adrenal pheochromocytoma, presenting as an "inverted" takotsubo syndrome complicated with acute heart failure and pulmonary oedema.
PubMed: 32257461
DOI: 10.1155/2020/2521046 -
Cureus Sep 2023Pheochromocytoma (PCC) is a rare neuroendocrine catecholamine-secreting tumour of the adrenal gland. It originates from the chromaffin cells found within the adrenal...
Pheochromocytoma (PCC) is a rare neuroendocrine catecholamine-secreting tumour of the adrenal gland. It originates from the chromaffin cells found within the adrenal medulla or the extra-adrenal paraganglia. We present a case report of a 24-year-old female who presented with hypertension, headache, palpitations, chest pain and blurry vision. On ultrasound evaluation, a right suprarenal mass was noted, which was further evaluated using contrast-enhanced computed tomography (CT). Based on our imaging findings, the patient was diagnosed with a case of right-sided pheochromocytoma. The patient was operated on, and our diagnosis was confirmed with histopathological examination.
PubMed: 37814748
DOI: 10.7759/cureus.44891 -
Annals of Vascular Surgery Jul 2020Paragangliomas (PGs) are rare neuroendocrine tumors arising from the extra-adrenal autonomic paraganglia that are tiny organs formed by bundles of neuroendocrine cells...
BACKGROUND
Paragangliomas (PGs) are rare neuroendocrine tumors arising from the extra-adrenal autonomic paraganglia that are tiny organs formed by bundles of neuroendocrine cells derived from the embryonic neural crest and capable of catecholamines secretion. Diagnosis and treatment of aortic PGs could be a challenging issue when they present as an emergency setup (sudden abdominal pain and radiological images resembling a vascular emergency).
CASE REPORT
We present a rare case of a 16-year-old man with a symptomatic and bleeding left para-aortic mass, treated in emergency with embolization, before a staged videolaparoscopic resection. Histology of the mass showed the presence of a large aortic PG.
CONCLUSIONS
In case of active bleeding, in emergency, vascular consultants are always involved. Sometimes, circumstances are very atypical; therefore, it is essential to keep in mind rare pathologies. In such settings, multidisciplinary approach is primary to obtain a prompt diagnosis and appropriate treatment.
Topics: Adolescent; Aorta; Embolization, Therapeutic; Hemorrhage; Humans; Laparoscopy; Male; Paraganglioma, Extra-Adrenal; Treatment Outcome; Vascular Neoplasms; Vascular Surgical Procedures; Video-Assisted Surgery
PubMed: 31904513
DOI: 10.1016/j.avsg.2019.12.016 -
SAGE Open Medical Case Reports 2024Paraganglioma is derived from the paraganglia tissue in the neck, along the sympathetic trunk, and in the pelvis. Paraganglioma has malignant potential and can...
Paraganglioma is derived from the paraganglia tissue in the neck, along the sympathetic trunk, and in the pelvis. Paraganglioma has malignant potential and can metastasize to remote organs such as the liver, lungs, and bones. Most metachronous metastases occur within several years after the initial diagnosis of paraganglioma. Here, we report the case of a 71-year-old male patient who developed bony metastasis 52 years after the resection of a large paraganglioma at the aortic bifurcation. The biopsy-proven paraganglioma metastasis to the lesser trochanter of left femur presented as an avulsion fracture. His normetanephrine level was elevated. DOTATATE PET (positron emission tomography) did not find any other metastatic lesions. The bony metastasis was treated with radiation therapy. We believe that the patient had one of the longest gaps ever reported, 52 years, between the initial diagnosis and metastasis of paraganglioma. This case highlights the importance of long-term surveillance of patients with paraganglioma for metastasis.
PubMed: 38333518
DOI: 10.1177/2050313X241229853 -
Pathology International Jul 2020
Topics: Adenocarcinoma; Aged; Colon, Sigmoid; Colonic Neoplasms; Diagnosis, Differential; Humans; Lymphatic Metastasis; Male; Mesocolon; Neuroendocrine Tumors; Paraganglioma
PubMed: 32342607
DOI: 10.1111/pin.12939