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Paragangliomas (PGs) are rare tumors that most commonly occur in the head and neck region and along the sympathetic chain. Fine-needle aspiration cytology is not commonly used for the diagnosis of PG due to the potential risk of hemorrhage and hypertensive crisis. As a result, limited studies describe the cytological features of PGs. In this case series, we will discuss the fine-needle aspiration features of three cases of extra-adrenal PGs. The cellular arrangement in smears was either singly scattered or loosely cohesive clusters. The cells were polygonal with pleomorphic nuclei, abundant granular cytoplasm, and bland chromatin. Cellblock showed two types of cells with focal acinar formation. Immunohistochemistry also confirmed the diagnosis. These results were also in keeping with radiological findings. Fine-needle aspiration cytology, along with clinicoradiological findings, can help in making an accurate preoperative diagnosis of PG.

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Paraganglioma is a rare neuroendocrine tumor that arises outside of the adrenal gland, typically originating from the chromaffin tissue of the sympathetic or parasympathetic ganglia. It can manifest at any age, with a peak incidence occurring between 40 and 50 years old. When the tumor secretes catecholamines, it is referred to as "functional." Currently, there is no standardized therapeutic approach. However, the management of metastatic forms is based on a systemic treatment with tri-chemotherapy. Herein, we present the case of a young male patient with heavily metastatic functional malignant paraganglioma, which represents the first case managed in our department. After seven months of Somatuline treatment, our patient experienced disease progression. Subsequently, he received tri-chemotherapy comprising cyclophosphamide, vincristine, and dacarbazine, which proved to be suboptimal due to poor hematological tolerance and a progression-free survival of less than three months. In the third line of treatment, Sunitinib was administered, but the therapeutic response was poor, with clinical progression observed within two months, ultimately leading to the patient's demise at home. The overall survival was two years.

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Paragangliomas are rare neuroendocrine tumors that arise from the paraganglia, which are clusters of neuroendocrine cells associated with the autonomic nervous system. These tumors are commonly found in the adrenal medulla but can also occur in other locations outside the adrenal gland. Here, we present a case report of a slow-growing paraganglioma in the left neck with spinal metastasis in a 60-year-old man. This case highlights the importance of considering paraganglion tumors in the differential diagnosis of neck masses and the need for early diagnosis and management to prevent potential complications. Importantly, both the clinical picture and anatomical location of these tumors is important when determining treatment plans.

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Autosomal dominant hereditary paraganglioma-pheochromocytoma syndrome (HPPS) is a rare genetic disorder characterized by neuroendocrine tumor development associated with pathogenic variants in succinate dehydrogenase (SDH) enzyme complex genes. Particularly, HPPS linked to SDHB mutation poses a significant clinical challenge due to its association with aggressive tumor features and a high risk of malignancy. Our report underscores the diversity in the presentation of patients with SDHB-mutated paraganglioma and the feasibility of managing it with a minimally invasive surgical approach. In the first case, a 17-year-old female was diagnosed with a metabolically active, poorly differentiated extra-adrenal retroperitoneal paraganglioma that required challenging robotic resection. Cascade genetic testing revealed an SDHB mutation not only in her but also in three family members, pointing to the inherited nature of the syndrome. Conversely, the second case involves a 37-year-old male with an asymptomatic well-differentiated left paraaortic paraganglioma incidentally found during an unrelated medical examination. Robotic converted-to-open resection allowed the successful removal of the mass. Subsequent germline testing confirmed a deleterious SDHB mutation, initiating a process of familial cascade testing. Both patients remained symptom- and recurrence-free at 12 and six months, respectively. Through these cases, and supported by a literature review, we highlight the variable clinical presentations of HPPS, arising from the same genetic alteration. The successful application of minimally invasive surgical techniques, combined with genetic evaluation, emphasizes the necessity for a comprehensive, tailored approach to treatment and surveillance. This strategy not only addresses the immediate clinical needs but also fosters proactive management of at-risk family members, ensuring a multidisciplinary approach to this complex hereditary condition.

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Paragangliomas (PGLs) are rare neuroendocrine tumors. Sometimes, these tumors secrete excessive catecholamines, which results in the manifestations of various signs and symptoms, usually with a triad of hypertension, tachycardia, and headache. We report the case of a 42-year-old woman presenting with uncontrolled hypertension, right facial palsy, vomiting, and disturbed gait. Diagnosis for PGL was confirmed on postoperative histological examination of the excised mass and correlated with preoperative clinical and radiological findings. Tumor excision was done via a suboccipital craniotomy approach. Our case presents the typically severe features of a jugulotympanic PGL, but most importantly, it highlights the necessity of biochemical diagnosing, thorough probing of the causes of hypertension, and a multi-disciplinary approach in dealing with these tumors. Moreover, the case emphasizes necessitating the use of preoperative embolization in vascular tumors of the head and neck to avoid a hemorrhagic crisis during surgery. Unfortunately, due to a lack of adequate hospital funds, the surgeon had to proceed without preoperative embolization. Despite such a risk, the excision was a success.

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We report a rare case of a solitary paraganglioma arising from the small bowel mesentery, found in a 70-year-old female who presented with abdominal pain. Paragangliomas are rare neuroendocrine, neural crest-derived tumors, most commonly found in the adrenal medulla. While extra-adrenal paragangliomas arise from diverse locations, mesenteric origins are extremely rare. Our comprehensive review shows 35 previously documented cases and updates the epidemiology, clinical features, and outcomes of mesenteric paragangliomas.

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Retroperitoneal paraganglioma remains an extremely rare type of tumor that arises from either sympathetic or parasympathetic neural crest cells. It could be functional or non-functional. Non-functional paraganglioma may present as a diagnostic challenge since patients are usually asymptomatic and tend to present to the hospital with complications from the invasion of the tumor. Malignancy is usually determined by the degree of metastasis. The gold standard of diagnosis is biopsy and obtaining a sample for histological examination. This author presents a case of asymptomatic, non-functional retroperitoneal paraganglioma with distant metastasis.

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Gallbladder paragangliomas are extremely rare with only a handful of cases reported so far. There are no definitive guidelines for the management of gallbladder paragangliomas due to their rarity. We present a case of a 53-year-old male who was found to have gallbladder paraganglioma post-laparoscopic cholecystectomy, performed for right upper abdominal pain. On review of the literature, all previously reported cases had been nonsecretory and benign. For patients who have no symptoms of secretory paragangliomas and no family history of endocrine syndromes, cholecystectomy and clinical follow-up may be a sufficient initial management following an incidental finding of gallbladder paraganglioma.

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OBJECTIVES: To assess outcomes of carotid body tumors (CBTs) managed with active surveillance. METHODS: Retrospective chart review of CBTs managed with active surveillance from 2001 to 2019. RESULTS: A total of 115 cases were identified during chart review. Sixty-five of these patients were managed with active surveillance, and 11 patients had bilateral tumors for a total of 76 tumors. Follow-up records with symptomatic outcomes were available for 51 patients, and 47 tumors had follow-up imaging. Thirty-one (66%) actively surveilled CBTs remained stable or decreased in size while 16 (34%) increased in size. Patients undergoing active surveillance developed symptoms in 12 cases, 6 of these patients underwent surgical intervention. Nine CBTs managed with active surveillance (18%) were ultimately resected. The majority of patients who did not undergo surgical intervention never developed symptoms (36/42, 86%). CONCLUSIONS: Active surveillance may be a reasonable approach for a subset of CBTs.

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INTRODUCTION: Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the presenting symptoms, secretory status, genetics, imaging features, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution. MATERIAL AND METHODS: We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020. RESULTS: Seventy-three patients were included in the study, encompassing 89 head and neck paragangliomas. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign paragangliomas and five with disseminated malignant disease). Regarding location, our series encompassed 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), two laryngeal paragangliomas (2.2%) and one sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Sixty-four patients (87.7%) underwent genetic testing. Of those, 24 (37.5%) exhibited pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), encompassing 55 tumors, underwent surgery as primary treatment modality, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and three patients (4.4%, encompassing three solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%. CONCLUSION: Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.

Introdução: Os paragangliomas apresentam frequentemente um comportamento benigno e um crescimento indolente. Apesar disso, são localmente invasivos, podendo causar morbilidade significativa. O objetivo deste trabalho foi descrever as manifestações clínicas, atividade secretora, estudos genéticos e imagiológicos, modalidades terapêuticas, complicações e sobrevivência dos doentes com paragangliomas da cabeça e pescoço. Material e Métodos: Estudo retrospetivo dos doentes com paragangliomas da cabeça e pescoço observados num centro hospitalar terciário entre 1997 e 2020. Resultados: Foram incluídos no estudo 73 doentes, englobando 89 paragangliomas. Quarenta e oito doentes (65,8%) eram do sexo feminino e 15 (20,5%) apresentavam múltiplos focos tumorais (10 por multicentricidade e cinco por doença maligna disseminada). Foram incluídos 40 paragangliomas do osso temporal (44,9%), 24 tumores do corpo carotídeo (27%), 22 vagais (24,7%), dois laríngeos (2,2%) e um nasossinusal (1,1%). A secreção excessiva de catecolaminas foi detetada em 11 doentes (15,1%). Sessenta e quatro doentes (87,7%) foram alvo de teste genético. Desses, 24 (37,5%) exibiram mutações patogénicas do complexo succinato desidrogenase. Dos doentes com doença primária, 45 (66,2%; 55 tumores) foram submetidos a tratamento cirúrgico, 20 (29,4%; 23 tumores) a radioterapia e três (4,4%) ficaram sob vigilância. Aos cinco anos, a sobrevida global foi de 94,9% e a sobrevida livre de doença foi de 31,9%. Conclusão: Os paragangliomas da cabeça e pescoço são tumores raros, de crescimento lento, mas localmente agressivos que resultam em elevadas taxas de morbilidade, mas baixas taxas de mortalidade.

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Paraganglioma is a rare tumor arising from paraganglia. Few reports have described paragangliomas in the superior mediastinum. We report a case of superior mediastinal paraganglioma treated in our department. A 28-year-old woman visited our department because of suspected mediastinal tumor during a medical checkup. Contrast-enhanced CT showed a 39 × 35 × 65-mm tumor with a well-defined border extending from the lower pole of the left thyroid gland to the superior mediastinum. Laboratory tests showed no evidence of catecholamine overproduction. Mediastinal thyroid goiter was the most suspected preoperative diagnosis. We decided to perform a transcervical excision for both diagnosis and treatment. The tumor was easily detached from the lower pole of the left thyroid gland and was not continuous. The tumor capsule was brittle and bled easily. The operating time was 3 h and 11 min, and the amount of bleeding was 571 mL. The pathological diagnosis was paraganglioma. Paragangliomas are characterized by abundant blood flow and are likely to result in a high intraoperative bleeding volume. In addition, if the tumor is functional, circulatory abnormalities can occur during the perioperative period. Accurate preoperative diagnoses are important, and the possibility that paragangliomas can develop in the superior mediastinum should be considered.

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Pheochromocytoma is a tumor arising from chromaffin cells of the medulla of adrenal gland and secretes excessive amounts of catecholamines: epinephrine and norepinephrine. It can also arise from sympathetic ganglia when it is referred to as catecholamine-secreting paragangliomas or extra-adrenal pheochromocytoma. Pheochromocytoma has been referred to as "the masquerader" for its numerous atypical presentations, which makes its diagnosis medically challenging. Here, we present a case of a 66-year-old female, presenting with high-grade fever for two weeks associated with generalized body aches. She had an extensive infectious, rheumatological and hematological workup. Ultimately, she was diagnosed with pheochromocytoma. After adrenalectomy, her fever and body ache resolved.

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Paragangliomas arise from neural cells and are found in different anatomical locations in the body. Paragangliomas in the adrenal glands are called pheochromocytoma, while the others are known as extra-adrenal paragangliomas. They are usually benign and are extremely rare in children. We present a case of a 13-year-old female patient who presented with complaints of hematuria for one year and left lower lumbar pain. Imaging investigations depicted a urinary bladder mass that was causing a mass effect at the left ureteric opening and backpressure changes in the left kidney. The patient underwent transurethral resection of bladder mass, and the histopathology confirmed the presence of paraganglioma. Though the paragangliomas of the urinary bladder are extremely rare in the pediatric age group, we suggest keeping paragangliomas on differentials when investigating a patient with bladder mass.

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RESUMEN Los paragangliomas son tumores originados en las células neuroendocrinas que forman el sistema nervioso autónomo. Se consideran benignos aunque pueden desarrollar malignidad, por lo que su tra tamiento es quirúrgico. La presentación de paraganglioma de ubicación mesentérica es muy inusual.

ABSTRACT Paragangliomas are rare neuroendocrine tumors that arise in the autonomic nervous system. Although these tumors are considered benign, they must be removed by surgery due to their potential malig nant transformation. Mesenteric paragangliomas are extremely rare.

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Non-functional, extra-adrenal, retroperitoneal paraganglioma is a rare, neuroendocrine, and potentially malignant tumor. Its diagnosis and treatment may be challenging. A 69-year-old female patient was admitted because of a left para-aortic, solid, 4.4-cm mass, incidentally discovered during abdominal ultrasonography for screening purposes. Her clinical examination was unremarkable. Preoperative differential diagnosis based on cross-sectional imaging included tumor of neuroendocrine or mesenchymal origin. Hormonal investigation with 24-hour urinary catecholamines and metanephrines and plasma-fractionated metanephrines was in the normal range. Following consultation with the endocrinologist and anesthesiologist, the tumor was removed by using the three-dimensional (3D) laparoscopic transperitoneal surgical approach. The perioperative course was uneventful and the patient was discharged on the third postoperative day. Histopathologic findings were consistent with the diagnosis of retroperitoneal extra-adrenal paraganglioma of 5 cm in maximum diameter.

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Paraganglioma is an extra-adrenal neuroendocrine neoplasia derived from neural crest precursors that arise in association with autonomic ganglia. Also called extraadrenal pheochromocytomas, these tumors are defined by their anatomical site and whether they are hormonally functional. Paragangliomas are rare neoplasms of the female genital tract and may be located in the ovaries, uterus or cervix. Those that appear in the vulvovaginal region are extremely rare. Clinical manifestations depend on unregulated catecholamine secretion and location. Diagnosis is based on morphological and immunohistochemical findings. Surgical resection is the primary treatment for this tumor as it does not respond to chemotherapy and radiotherapy. Due to its low frequency and nonspecific symptomatology, diagnosis may be difficult. We present a case of primary vulvovaginal paraganglioma.

El paraganglioma es una neoplasia neuroendocrina extraadrenal derivada de precursores de la cresta neural, que surgen en asociación con los ganglios autónomos y, en ocasiones, también son denominados feocromocitomas extraadrenales. Se definen por su sitio anatómico y si son o no hormonalmente funcionales. Los paragangliomas son neoplasias poco frecuentes del tracto genital femenino y pueden aparecer en ovarios, útero o cuello uterino. Los que aparecen en la región vulvovaginal son extremadamente raros. Las manifestaciones clínicas dependen de la secreción no regulada de catecolaminas y la localización. El diagnóstico se basa en hallazgos morfológicos e inmunohistoquímicos. La resección quirúrgica es un tratamiento primario para este tumor, ya que no responde bien a la quimio-radioterapia. Debido a su baja frecuencia y sintomatología inespecífica, puede mostrar dificultades para realizar el diagnóstico. Se presenta un caso de paraganglioma vulvovaginal primario.

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OBJECTIVE: 18F-Fluoro-L-dihydroxyphenylalanine (18F-DOPA) PET offers high sensitivity and specificity in the imaging of non-malignant extra-adrenal paraganglioma (PGL) and pheochromocytoma (PHEO) but lower sensitivity in metastatic disease. These tumours are of neuroendocrine origin and can be detected by 68Ga-DOTA-Tyr3-octreotide (68Ga-DOTA-TOC) PET. Therefore, we compared 68Ga-DOTA-TOC and 18F-DOPA as radiolabels for PET/CT imaging for the diagnosis of metastatic extra-adrenal PGL and PHEO. Combined cross-sectional imaging was the reference standard. METHODS: A total of 6 men and 4 women (age range 22-72 years) with anatomical and/or histologically proven metastatic PGL and PHEO were included in this study. Of these patients, 2 male patients suffered from PHEO, while the remaining 8 patients were diagnosed as metastatic extra-adrenal PGL disease. Comparative evaluation included morphological imaging with CT and functional imaging with 68Ga-DOTA-TOC and 18F-DOPA PET. The imaging results were analyzed on a per-lesion basis. The maximum standardized uptake value (SUVmax) of each functional imaging modality in concordant tumour lesions was measured. RESULTS: Compared with anatomical imaging, the per-lesion detection rate of 68Ga-DOTA-TOC was 100% (McNemar, P<0.01), and that of 18F-DOPA PET was 82.3% (McNemar, P<0.8) in metastatic extra-adrenal PGL and PHEO. Overall, 68Ga-DOTA-TOC PET identified 67 lesions; anatomical imaging identified 62 lesions, and 18F-DOPA PET identified 56 lesions. The SUVmax (mean±SD) of all concordant lesions was 29.3±19.9 for 68Ga-DOTA-TOC PET and 12.3±9.1 for 18F-DOPA PET (Mann-Whitney U test, P<0.0001). CONCLUSION: 68Ga-DOTA-TOC PET offers the highest detection rate in metastatic extra-adrenal PGL and PHEO compared to 18F-DOPA PET and even to diagnostic CT, particularly in bone lesions. Combined functional/anatomical imaging (68Ga-DOTA-TOC PET/CT) enables exact tumour extension to be detected in these rare tumour entities, especially in the case of unclear anatomical correlation.

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We present a case of unusual cardiac paraganglioma (PG) initially misdiagnosed as atypical carcinoid tumor of the lung and discuss key clinical and pathologic characteristics that guide surgical management of these rare chromaffin cell tumors. A 64-year-old female with persistent cough and back pain was found to have a 4 cm × 3 cm mass abutting multiple cardiopulmonary structures. A biopsy was performed at an outside institution and pathology reported "atypical neuroendocrine carcinoma, consistent with carcinoid". The patient was transferred to our institution and pericardial resection with right pneumonectomy was performed to excise the tumor. Histology of the mass was that of PG with multiple ethanol embolizations. Immunohistochemical examination revealed that type I (chief) cells were positive for neuroendocrine markers (chromogranin A and synaptophysin), while type II (sustentacular) cells were positive for S100. There was no evidence of atypical carcinoid tumor in the lung. PG is an entity of chromaffin cell tumors that often affects the adrenal glands and carotid body. PG rarely occurs in the thoracic region, accounting for just 1-2% of all PG. Proper diagnosis of cardiac PG is challenging owing to its rare prevalence, subtle symptoms of presentation, and the neuroendocrine histopathological features it shares with atypical carcinoids. These tumors are typically benign and are best treated by surgical resection. Our report examines the approach to appropriate diagnosis of cardiac PG vs. atypical carcinoid, preoperative management, and surgical treatment by describing successful resection through thoracotomy without the use of cardiopulmonary bypass.