Figure - available from: Case Reports in Oncological Medicine
This content is subject to copyright. Terms and conditions apply.
(a) Clusters of cells surrounded by reticulin fibrosis known as Zellballen arrangement, typical of paraganglioma. (b) CD34 immunostain marking the numerous small capillaries in the tumor. (c) Chromogranin A immunohistochemical stain.
Source publication
Paragangliomas are very rare tumors derived from neuroendocrine cells of autonomic nervous system. Extra-adrenal paragangliomas account for only 10 to 15% of all paragangliomas and may present incidentally as a mass. Typical triad of fluctuating hypertension, headache, and sweating is not always present which makes the diagnosis difficult sometimes...
Similar publications
Post-acute sequelae of SARS-CoV-2 (PASC), or long COVID syndrome, is emerging as a major health issue in patients with previous SARS-CoV-2 infection. Symptoms commonly experienced by patients include fatigue, palpitations, chest pain, dyspnea, reduced exercise tolerance, and "brain fog". Additionally, symptoms of orthostatic intolerance and syncope...
Citations
... Except for the adrenal medulla, paragangliomas predominantly arise in the head and neck region, most commonly involving the carotid bodies, the jugular glomus, and the vagal bodies (1,2). According to the literature, extraadrenal paragangliomas account for only 10% to 15% of all paragangliomas (3); paragangliomas affecting the central nervous system are particularly rare, comprising approximately 0.6% of all head and neck neoplasms (4). Although paragangliomas are generally considered to be indolent entities corresponding histologically to World Health Organization (WHO) grade I (5), a high recurrence rate following surgical resection (10% for paragangliomas of carotid body and 50% to 60% for those occurring in other sites) and even metastasis (~10%) have been reported (6). ...
Background
Paraganglioma in the sellar region is an extremely rare entity, with a limited number of cases reported in the literature. Due to the paucity of clinical evidence, the diagnosis and treatment of paragangliomas in the sellar region remain challenging. Herein, we reported a case of sellar paraganglioma with parasellar and suprasellar extension. Particularly, the dynamic evolution of this benign tumor within a 7-year longitudinal observation was presented. Additionally, the relevant literature regarding sellar paraganglioma was comprehensively reviewed.
Case description
A 70-year-old woman presented with progressive visual deterioration and headache. Brain magnetic resonance imaging demonstrated a mass in the sellar region with parasellar and suprasellar extension. The patient refused surgical treatment. Seven years later, brain magnetic resonance imaging showed the lesion significantly progressed. Neurological examination revealed bilateral tubular contraction of visual fields. Laboratory examinations showed endocrine hormone levels were normal. Surgical decompression was performed via a subfrontal approach, and subtotal resection was achieved. Histopathological examination confirmed a diagnosis of paraganglioma. Postoperatively, she developed hydrocephalus, and ventriculoperitoneal shunting was performed. Eight months later, cranial CT showed no recurrence of the residual tumor, and the hydrocephalus had been relieved.
Conclusion
Paraganglioma occurring in the sellar region is rare, and the preoperative differential diagnosis is difficult. Owing to the infiltration to the cavernous sinus and internal carotid, complete surgical resection is usually impracticable. There has been no consensus regarding postoperative adjuvant radiochemotherapy for the tumor residue. In-situ recurrence and metastasis have been reported in the literature, and close follow-up is warranted.
... At least 30% of the patients have been shown to have certain hereditary cancer-predisposing disorders, such as multiple endocrine neoplasia, neurofibromatosis type 1, or von Hippel-Lindau syndrome [20]. Retroperitoneal extra-adrenal paragangliomas make up the majority of extra-adrenal paragangliomas, accounting for 85% of all extra-adrenal paragangliomas [21]. e renal pelvis (4.9%) is the third most common site of paragangliomas in the genitourinary tract, after the bladder (79.2%) and urethra (12.7%) [22]. ...
Spontaneous retroperitoneal bleeding is a rare occurrence that might have catastrophic implications. We present a 58-year-old male with a 4-day history of progressively worsening left-sided flank pain due to retroperitoneal hemorrhage from a nonfunctional renal paraganglioma. Abdominal contrast CT scan was helpful in locating the tumor, estimating tumor size and extent of bleeding, visualizing the interaction between the tumor and the surroundings, and ruling out any potential metastasis; however, it lacked specificity in identifying the origin of the mass, needing histologic investigation for a conclusive diagnosis. MRI was not available at our center. We report a rare case of spontaneous retroperitoneal bleeding as a complication of an unusual nonfunctional renal paraganglioma, which was initially misdiagnosed as renal cell carcinoma but later confirmed by postoperative histopathology.
... Among those extra-adrenal sites, retroperitoneum stands out as the most involved location but there are reported cases for unusual locations including the carotid body, orbit, lungs, stomach, and urinary tract. 2 Biliary system is an extremely uncommon site for extraadrenal paraganglioma. Up till now only four cases of gall bladder paragangliomas have been reported, while extrahepatic biliary tract paragangliomas have been documented five times. ...
Paragangliomas are rare tumours derived from the neural crest cells. Most common site for these tumours is the adrenal medulla, where they are called as pheochromocytomas. Biliary system is an extremely uncommon site for extra-adrenal paraganglioma. We report the case of a 55 year old female who presented with one-month history of jaundice and right upper quadrant discomfort. Imaging showed a well-defined rounded mass at porta hepatis with arterial enhancement causing compression over the proximal common bile duct, resulting in moderate intrahepatic biliary dilatation. The patient underwent radical excision of common bile duct and roux-en-y hepaticojejunostomy. Final histopathology confirmed paraganglioma. Approximately 10% of the patients may present with extra-adrenal disease, but biliary paraganglioma are extremely rare. Only few cases have been reported in literature and the management usually involves the surgical excision.
---Continue
... Because of malignant or metastatic potential and high recurrence rate, pre and postoperative imaging study and lifelong follow-up is recommended. [8] www.pacificejournals.com/apalm eISSN: 2349-6983; pISSN: 2394-6466 ...
Extraadrenal paraganglioma is rarely found in the mesentery. It accounts only for 5-10% of cases and may present incidentally as abdominal mass. We report a case of 30 year old male presented as mass per abdomen. A differential diagnosis of neurogenic tumor or cystic mesothelioma was considered based on CT-abdomen. Histopathological examination showed features of mesenteric paraganglioma and confirmed on immunohistochemistry. This case report highlighted several important key issues regarding diagnosis of paraganglioma, including malignant or metastatic potential, recurrence rate, pre and postoperative imaging study and lifelong follow-up of the patient.
... 5 There are less than 150 cases of NRS reported in the English literature. [6][7][8][9][10][11][12][13] It is essential to perform a pretherapeutic biopsy, even in cases of apparent respectability, and a preoperative biopsy was undertaken in this case, after confirming biochemical inactivity. A preoperative biopsy is contraindicated, if a pheochromocytoma is suspected. ...
There are less than 150 cases of non-functioning retroperitoneal abdominal schwannoma (NRS) reported. Hormonal assay is crucial in confirming the diagnosis of NRS, as manipulation of a functional retroperitoneal paraganglioma will instigate an abrupt liberation of catecholamines, resulting in devastating consequences. We report the case of 42-year-old woman who presented with headache, night sweats and abdominal discomfort. Cross-sectional imaging demonstrated a retroperitoneal mass adherent to the aorta and inferior vena cava but biochemical testing of blood and urine was negative for metanephrines and normetanephrines. She underwent successful tumour resection via laparotomy, as location increased the complexity and risk of laparoscopic resection.
... Up to 15% of retroperitoneal paragangliomas are non-functional, and up to 10% are functional without clinical manifestation, although they resemble functional ones histologically and immunologically [4][5][6]. Their clinical presentation varies depending on their location, size, and secretion status, while their diagnosis and treatment may be challenging due to their close proximity to major vessels and surrounding organs, demanding multidisciplinary medical approach and cooperation [2,7,8]. Our study aims to underline the importance of a multidisciplinary medical approach and the benefit of a minimally invasive surgical method for treating retroperitoneal paraganglioma. ...
... Retroperitoneal extra-adrenal paragangliomas are rare tumors originating from chromaffin cells of the primitive neural crest during their migration to form the paraspinal sympathetic paraganglia. They have an estimated incidence of 2-8 per million people in the general population [2,4,[7][8][9]. There is a great variation regarding their location, but 85% of them are retroperitoneal and are usually located between the origin of inferior mesenteric artery and the aortic bifurcation [7,8]. ...
... They have an estimated incidence of 2-8 per million people in the general population [2,4,[7][8][9]. There is a great variation regarding their location, but 85% of them are retroperitoneal and are usually located between the origin of inferior mesenteric artery and the aortic bifurcation [7,8]. ...
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.
... Paragangliomas are rare tumors which arise from the neural crest tissue that develops into sympathetic and parasympathetic paraganglia throughout the body [20] . Paraganglioma of adrenal medulla is known as pheochromocytoma, while paragangliomas located outside of the adrenal gland are called as extra adrenal paragangliomas. ...
Objective: In this study, we evaluated the pathological results
and clinical presentation of patients with retroperitoneal
tumors
Design: Retrospective study
Setting: Department of Urology at Hitit University, Çorum
Erol Olçok Training and Research Hospital, and Department
of Urology at Haydarpaşa Numune Training and Research
Hospital
Subjects: Fourteen patients with retroperitoneal tumors
Intervention: Retroperitoneal mass and clinical suspicion of
tumor
Main outcome measures: The symptoms at presentation
and pathologic reports of the tumors were recorded in these
patients.
Results: Of the patients evaluated for retroperitoneal tumor,
eight patients were male. The mean age of the study group was
55.7 ± 11.4 years with a range of 41 to 75 years. Liposarcoma
was the most common pathological diagnosis of the
retroperitoneal tumors in these patients (35.7%). Schwannoma
and gastrointestinal stromal tumor were reported in two
patients. The other retroperitoneal masses were diagnosed
as atypical lipomatous tumor, paraganglioma, myelolipoma,
lipomatous hemanjiopericytoma and Castleman disease.
Conclusion: Retroperitoneal tumors are rare neoplasms
in daily urological practice. With the development of
radiological imaging techniques in recent years, most of
the patients have been diagnosed incidentally at abdominal
imagings for other purposes. The management of these
patients is important because of the aggressive behavior of
sarcomas.
... Paragangliomas are rare neuroendocrine tumours arising from the paraganglia, which refer to chromaffin cells of the sympathetic paravertebral ganglia of the thorax, abdomen and pelvis, or of the parasympathetic ganglia located along the glossopharyngeal and vagal nerves in the neck and base of the skull [1,2]. The adrenal medulla hosts the largest proportion of paraganglia. ...
... The incidence of extra-adrenal paragangliomas is only 2-8 per million [5]. They occur either sporadically or as part of hereditary syndromes, such as MEN2, von Hippel Lindau, hereditary PGL/phaeochromocytoma syndromes and Carney syndrome (extra-adrenal paraganglioma, GIST, pulmonary chondroma) [1,5]. Extra-adrenal para-gangliomas are more aggressive than their adrenal counterparts, with metastases occurring in 20-70% of cases, compared to only 2-10% of adrenal phaeochromocytomas [4,5]. ...
... Functioning paragangliomas secrete norepinephrine and normetanephrine and account for 30-60% of tumours [3]. Catecholamine production may lead in clinical manifestations including palpitation, headache and hypertension [1,6]. On the contrary, non-functioning paragangliomas tend to be asymptomatic and can be found incidentally, like in our case, or as masses compressing the surrounding organs and causing abdominal pain [6]. ...
A 67-year-old female underwent left lumpectomy for an invasive mucinous breast carcinoma. She had an unre-markable medical history and physical examination revealed only the known palpable mass in the left breast. Laboratory findings were all within normal limits. Chest and abdomen computed tomography (CT) scan was performed for staging purposes (Fig. 1). Due to abnormal findings in the pancreatic region, the patient underwent a multiphasic pancreatic CT (Figs. 2, 3) and magnetic resonance imaging (MRI) (Figs. 4, 5).
... In cases with nonfunctional lesions, symptoms are rather related to the mass syndrome or even to the invasion of the surrounding viscera or blood vessels; the non-functional paragangliomas account for up to 15% of cases (9). In isolated cases, patients with non-functional intra-abdominal paragangliomas might have paralytic ileus or chest pain (10)(11). Due to the low specificity of these symptoms, non-functional lesions are usually difficult to be diagnosed pre-operatively (12). ...
Paragangliomas, as well as phaeochromocytomas, are vascular neuroendocrine lesions that might be encountered between neck and pelvis. In certain cases, a preoperative diagnosis is difficult to be established, so resection might be needed whenever a suspect mass is discovered at the imaging studies. Moreover, there are cases in which resection and reconstruction of an important vascular structure might be imposed. In this case report a 59-year-old patient was investigated for upper abdominal pain with postprandial features and was diagnosed with a retroperitoneal tumor of 4/6/7cm invading the celiac trunk. The tumor was successfully resected and the common hepatic artery was reinserted in the celiac stump.
... Non-functioning paragangliomas usually do not produce any symptom; however, when enlarged in size, pain may be the presenting symptoms due to compression of surrounding organ. 4 We report a rare case of retroperitoneal paraganglioma presented as abdominal mass with symptoms of headache, palpitation and sweating. ...
... About 10-15% of paragangliomas are extra-adrenal in nature. 4 Retroperitoneal paragangliomas originate from neural crest cells present along the aorta and sympathetic chain, i.e. parganglia. 5,6 The patient with functional paraganglioma may have symptoms of hypertension, headache, sweating and abdominal mass. ...
... 5,6 The patient with functional paraganglioma may have symptoms of hypertension, headache, sweating and abdominal mass. 4 Our patient had history of intermittent headache, palpitations and sweating. Though there was an abdominal mass, patient did not have any pain in abdomen. ...
