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.

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.

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.

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.

UNASSIGNED: To assess outcomes of carotid body tumors (CBTs) managed with active surveillance.
UNASSIGNED: Retrospective chart review of CBTs managed with active surveillance from 2001 to 2019.
UNASSIGNED: 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%).
UNASSIGNED: Active surveillance may be a reasonable approach for a subset of CBTs.

BACKGROUND: 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.
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%.
CONCLUSIONS: 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.

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.

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.