Non-functioning paraganglioma is a rare neuroendocrine tumor arising from neural crest-derived paraganglion cells (most often in the para-aortic region at the level of renal hilia, organ of Zuckerkandl, thoracic paraspinal region, bladder, and carotid body) not associated with catecholamine secretion. These tumors are usually clinically silent and symptoms, if present, are nonspecific and depend on the location of the tumor. Association with certain hereditary cancer-predisposing syndromes, such as multiple endocrine neoplasia, neurofibromatosis type 1 or von Hippel Lindau syndrome, may be observed.
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Non-functioning paraganglioma is a rare neuroendocrine tumor arising from neural crest-derived paraganglion cells (most often in the para-aortic region at the level of renal hilia, organ of Zuckerkandl, thoracic paraspinal region, bladder, and carotid body) not associated with catecholamine secretion. These tumors are usually clinically silent and symptoms, if present, are nonspecific and depend on the location of the tumor. Association with certain hereditary cancer-predisposing syndromes, such as multiple endocrine neoplasia, neurofibromatosis type 1 or von Hippel Lindau syndrome, may be observed.
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Publicações mais relevantes
A Novel Case of Robotic Retroperitoneal Lymph Node Dissection for Metastatic Sertoli Cell Tumour: A Case Report.
Sertoli cell tumours of the testes (SCTT) are a rare subgroup of sex cord stromal tumours. This report describes a novel use of robotic retroperitoneal lymph node dissection (RPLND) in a rare case of metastatic disease that posed significant diagnostic challenges. A 60-year-old male presented with a left testicular mass; post-orchidectomy histological review confirmed high-risk Sertoli cell tumour. A subsequent PET-CT identified metastatic para-aortic lymph nodes; an incidental finding of a non-functioning paraganglioma was also noted. The patient underwent a successful robotic RPLND, performed using the DaVinci Xi robotic platform. Postoperative recovery was uneventful, and the patient was discharged on postoperative day two. Histology confirmed metastasis in two out of 10 lymph nodes. This report highlights the potential of robotic RPLND as a viable treatment course, with its success emphasising the critical role of multidisciplinary team collaboration. While we describe a novel approach, further studies and long-term oncological outcomes in larger patient cohorts are necessary to establish definitive treatment guidelines.
Nonfunctioning paraganglioma of the bladder mimicking nested variant of urothelial cancer: A case report and clinical management.
Non-functioning paraganglioma of the bladder is extremely rare disease. It is important to know the presence of paraganglioma of the bladder as the early and correct diagnosis affects the management and prognosis of the patient. Pathologists may misinterpret it as part of cystitis cystica/glandularis or nested variant urothelial carcinoma, or secondary bladder involvement by prostatic adenocarcinoma. This report will contribute to the existing literature and provide valuable insights into the clinical characteristics and treatment options for this rare tumor. 35 years old female patient presented after incidental finding of bladder mass on abdominal ultrasound during routine antenatal follow up. Cystoscope showed 2 × 2 cm single solid bladder mass. Microscopic examination was suggestive of neuroendocrine tumor with paragangliom as a differential diagnosis. Imminohistochemical studies showed positive for synaptophysin, S100, GATA-3 and negative for panCK. The patient was managed with partial cystectomy. Non-functioning paragnaglioma of the urinary bladder is a rare extra-adrenal pheochromocystoma. The diagnosis is usually challenging and it may mimic urothelial carcinoma. Histopathology and immunohistochemistry are almost always confirmatory. Urinary bladder paraganglioma may mimic nested variant of the bladder tumor. High clinical suspicion is important. Confirmation is with histopathology and immunohistochemistry. Partial cystectomy is treatment of choice.
Non Functioning Paraganglioma in the Urinary Bladder: a Case Report.
Paragangliomas are tumors that arise from autonomic nervous system. Non-functioning bladder paraganglioma is rare and usually misdiagnosed. Here we describe a case of a 45-year-old man with primary urinary bladder paraganglioma. The patient had no active signs and symptoms, and histological and immunohistological examinations of a transurethral resection specimen confirmed correct diagnosis. After successful transurethral resection of the tumors, the patient showed no signs of recurrence at one-year follow-up.
Multifactorial aetiology for non-uremic calciphylaxis: a case report.
Calciphylaxis is commonly associated with end-stage renal disease patients on haemodialysis. We present a rare case of calciphylaxis in a non-uremic patient. The diagnosis was made clinically and confirmed with skin biopsy showing calcification of the dermal and subcutaneous tissues in the von Kossa stain. We believe that the combination of uncontrolled diabetes mellitus, a non-functioning paraganglioma and vitamin D deficiency in a susceptible female patient was responsible for causing calciphylaxis in our patient. An index of suspicion should be maintained by clinicians for calciphylaxis even in patients without uremia.
Soft tissue metastasis of mediastinal non-functioning paraganglioma with unusual TTF-1 expression: a potential diagnostic pitfall.
Metastatic extra-adrenal paragangliomas are very rare and can represent diagnostic challenges. We report a case of 69-year-old man with a tumor of the right shoulder. Histologic and immunohistochemical examinations confirmed the diagnosis of paraganglioma. Surprisingly, tumor cells were diffusely thyroid transcription factor 1 (TTF-1) positive. Succinate dehydrogenase complex subunit B (SDHB) deficiency has not been detected. Inherited syndromes associated with paragangliomas were ruled out. The primary tumor was identified in the mediastinum. This is the first report of TTF-1 expression in paraganglioma. To avoid misdiagnosis, careful clinical and pathological examination of any tumor with organoid growths pattern is necessary.
Publicações recentes
A Novel Case of Robotic Retroperitoneal Lymph Node Dissection for Metastatic Sertoli Cell Tumour: A Case Report.
🥈 Ensaio clínicoNonfunctioning paraganglioma of the bladder mimicking nested variant of urothelial cancer: A case report and clinical management.
Non Functioning Paraganglioma in the Urinary Bladder: a Case Report.
Multifactorial aetiology for non-uremic calciphylaxis: a case report.
Soft tissue metastasis of mediastinal non-functioning paraganglioma with unusual TTF-1 expression: a potential diagnostic pitfall.
📚 EuropePMC10 artigos no totalmostrando 7
A Novel Case of Robotic Retroperitoneal Lymph Node Dissection for Metastatic Sertoli Cell Tumour: A Case Report.
CureusNonfunctioning paraganglioma of the bladder mimicking nested variant of urothelial cancer: A case report and clinical management.
International journal of surgery case reportsNon Functioning Paraganglioma in the Urinary Bladder: a Case Report.
Urology journalMultifactorial aetiology for non-uremic calciphylaxis: a case report.
Journal of community hospital internal medicine perspectivesSoft tissue metastasis of mediastinal non-functioning paraganglioma with unusual TTF-1 expression: a potential diagnostic pitfall.
Polish journal of pathology : official journal of the Polish Society of PathologistsRetroperitoneal non-functioning paraganglioma: A difficult tumour to diagnose and treat.
International journal of surgery case reportsNon-functioning paraganglioma occurring in the urinary bladder: A case report and review of the literature.
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Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- A Novel Case of Robotic Retroperitoneal Lymph Node Dissection for Metastatic Sertoli Cell Tumour: A Case Report.
- Nonfunctioning paraganglioma of the bladder mimicking nested variant of urothelial cancer: A case report and clinical management.
- Non Functioning Paraganglioma in the Urinary Bladder: a Case Report.
- Multifactorial aetiology for non-uremic calciphylaxis: a case report.
- Soft tissue metastasis of mediastinal non-functioning paraganglioma with unusual TTF-1 expression: a potential diagnostic pitfall.Polish journal of pathology : official journal of the Polish Society of Pathologists· 2017· PMID 29517207mais citado
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:94080(Orphanet)
- MONDO:0019788(MONDO)
- GARD:19248(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788878(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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