Diagnosis Of Paraganglioma Research Articles

Endoscopic Ultrasound-Guided Fine Needle Biopsy for the Diagnosis of Paraganglioma

Paragangliomas are rare neuroendocrine tumors that originate from the extra-adrenal autonomic ganglia. They should be included in the differential diagnosis of retroperitoneal tumors. Endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) has been increasingly used as an effective tool for obtaining diagnostic tissue for histology and molecular studies. We are reporting a patient who was found to have a retroperitoneal mass as an incidental finding and was successfully diagnosed as a non-functional paraganglioma using EUS-FNB without any complications. 50-years-old man who presented to the emergency department after a high-speed motorcycle accident; Computed tomography showed large retroperitoneal mass measuring 9.6 x 12.5 cm. It was a peripherally enhancing, centrally necrotic mass that abuts the Inferior vena cava and aorta. EUS-FNB showed an epitheliod lesion composed of medium sized cells with moderate eosinophilic to clear cytoplasm. Immunostains showed the tumor cells to be positive for CD56 and synaptophysin but negative for pancytokeratins AE1/3 and CAM5.2, Pax-8, CK7, inhibin, S-100 and CD117. This indicates neuroendocrine differentiation, consistent with paraganglioma. He tolerated the procedure well without any complications. Biochemical studies were consistent with non-functioning extra adrenal paraganglioma. The following month, he underwent successful surgical resection of the mass. Paragangliomas can be found in different sites, with the majority being found in the abdomen. They could be either functioning or non-functioning. The functional ones secrete norepinephrine and normetanephrine and represent 36% to 60% of the lesions. Less than 10% could turn malignant. EUS-FNB allows preoperative diagnosis of such lesions. In patients with clinical signs of pheochromocytoma, it is advisable to do 24-hour urine collection of catecholamines and metanephrines before performing EUS-FNB as it is contraindicated in functioning paragangliomas as needle insertion could trigger an adrenergic crisis. In conclusion, EUS-FNB is a safe technique for evaluation of retroperitoneal masses. Physicians should consider biochemical testing to exclude functioning paraganglioma in patients with retroperitoneal masses who have clinical signs of pheochromocytoma prior to performing EUS-FNB to avoid intra-procedural complications.Figure: Abdominal CT (upper left) shows large retroperitoneal peripherally enhancing centrally necrotic mass that measures 9.6 x 12.5 X 10.7 cm. EUS (upper right) shows the mass with the needle going through for fine needle aspiration. FNA cytology slide (bottom left) stained with H&E stain shows small epithelioid cells with moderate pleomorphism, and fine salt and pepper chromatin similar to that seen in neuroendocrine tumors. The paraganglioma mass (bottom right) is shown after surgical resection.

Extradural Dorsal Spinal Paraganglioma: A Rare Case Report and Literature Review

AbstractParagangliomas are neuroendocrine tumors originating from specialized cells of neural crest derivative. They are mostly found in the carotid and jugulotympanic region. Paragangliomas are extremely rare in the spine, and in literature, most cases are described as intradural extramedullary lesion in the cauda equine or filum terminale region. Extradural dorsal spine paragangliomas are even rarer, and till date, only 11 cases are reported in available literature. We describe a case of an adult woman who presented with mid dorsal region pain and sensory-motor spastic paraplegia. Imaging features were suggestive of D5 Pott's spine with epidural granulation tissue. Perioperatively, the tumor was moderately vascular and complete excision done. On HPE and IHC, diagnosis of paraganglioma was made. There was no evidence of clinical recurrence of the lesion at 6 months follow-up.

Diagnosis and treatment experience of transurethral resection for paraganglioma of urinary bladder in 8 cases

Objective: To discuss the experience of diagnosis and treatment of transurethral resection of paraganglioma in urinary bladder. Methods: This retrospective study included 8 patients who underwent transurethral resection of paraganglioma in bladder from October 2009 to April 2015. Four males and 4 females were enrolled. The age ranged from 33 to 77 years (mean: 50.4 years), with a clinical course from 1 month to 8 years. Five cases presented with hypertension and 4 had high level of noradrenaline in blood. Preoperative ultrasound, CT and MRI were performed on every patient and the results showed all tumors were solitary and limited in bladder wall. Six patients received conventional transurethral electroresection and the other 2 patients received transurethral resection with thulium laser.Postoperative follow-up of each case was recorded. Results: All tumors were excised successfully without converting to open surgery. The maximum diameter of tumors ranged from 1.0 to 4.5 cm (mean: 2.3 cm), operative time varied from 15 to 35 min (mean: 28.1 min) and intraoperative blood loss ranged from 20 to 50 ml (mean: 31.9 ml). The variation of blood pressure during operation was 10 to 160 mmHg (mean: 66.3 mmHg). The loss of follow-up occurred in 1 case, tumor recurrence occurred in 1 case at 3 months after surgery and the follow-up data of other patients was normal. Conclusions: The diagnosis of paraganglioma in bladder should be combined with clinical symptoms, biochemical and imaging examination for some cases. Transurethral resection of tumors is a safe and reliable surgical manner.

Precarious encounter with Paraganglioma during cystoscopic excisional biopsy of urinary bladder tumour

Tumours secreting catecholamine are a known perioperative anaesthetic challenge. However, extra adrenal location of such tumors can cause sudden catastrophe in unprepared patients. A 45 year old, 50 kg female patient scheduled for transurethral cystoscopic resection of bladder tumour of suspected neoplastic aetiology, developed sudden, severe tachycardia, hypertension, and arrhythmias followed by hypotension under general anaesthesia. Tumour biopsy obtained during first surgery confirmed diagnosis of Paraganglioma (PGL). This was supported with normal vanillyl mandelic acid (VMA), 6.5mg/24hrs (normal 2-8mg/24hrs) and a large polypoidal mass on CT in a patient with episodic symptoms of headache and blackout associated with micturition; a classical triad reported about this tumour category. Findings of MIBG (Meta iodo benzyl guanidine) 131I scan that reported no MIBG avid disease, were equivocal. Accurate diagnosis, good perioperative control of blood pressure and volume status using invasive monitoring and cascade of drugs to manipulate circulation when required, formed the essence of successful management of the patient during her second surgery.

Retroperitoneal Paraganglioma Resected Through the Posterior Retroperitoneoscopic Approach

Background: Paragangliomas are rare neuroendocrine tumors arising from neural crest cells. Open exploration and resection are the standard surgical management of retroperitoneal paragangliomas. However, at select centers, both laparoscopic transabdominal and posterior retroperitoneoscopic resection of retroperitoneal paragangliomas have been found to be feasible and safe.1–6 The posterior retroperitoneoscopic approach may have shorter operative time, more direct exposure, less pain, and faster patient recovery than both the open and laparoscopic transabdominal approaches. Methods: The aim of this video is to provide detailed instruction of endoscopic paraganglioma resection through the retroperitoneoscopic approach. Results: The diagnosis of paraganglioma is established by biochemical evaluation and preoperative imaging. The surgical approach uses three incisions in the back, using two 5 mm ports and one central 12 mm port. The retroperitoneal space is developed by blunt dissection and enhanced by CO2 insufflation to 25–30 mmHg. The tumor is carefully dissected and surrounding retroperitoneal structures are preserved. In the current case, the retroperitoneoscopic approach avoided a hostile abdomen because of multiple previous intraabdominal procedures, such as cholecystectomy, appendectomy, hysterectomy, and salpingo-oophorectomy. Conclusions: The posterior retroperitoneoscopic resections of retroperitoneal paraganglioma are feasible and safe. In our experience, the operative time is shorter, with reduced postoperative pain and hospital stay versus the open and laparoscopic transabdominal approaches. It is particularly useful in patients with previous abdominal surgery with the potential of significant adhesions. The cosmetic outcome is excellent. T. S. and T. C. declare that there is no conflict of interests regarding the publication of this article. Runtime of video: 8 mins 26 secs

Multiple Retroperitoneal Paragangliomas: An Uncommon Entity

ABSTRACT Introduction Paragangliomas are neuroendocrine tumors and occur commonly in head and neck region and less frequently in the retroperitoneum. Multifocal paragangliomas are even rarer and highly suggestive of familial disease. To the best of our search, there are only two case reports of multiple retroperitoneal paragangliomas with no known familial association. This is the third report of this kind in the English literature. Case report A young adult with no significant past or family history presented with abdominal pain and anorexia. Abdominal examination revealed a soft nontender mass in the right hypochondrium. Contrast-enhanced computed tomography showed multiple retroperitoneal mass lesions. A clinicoradiological diagnosis of multicentric Castleman's disease/Lymphoma was made. Guided fine needle aspiration of the mass was suggestive of a neuroendocrine neoplasm. Tru-cut biopsy showed features of paraganglioma. Following this, the masses were excised and the diagnosis of paraganglioma was confirmed. Conclusion Multicentric retroperitoneal paragangliomas without any familial association are very rare with only two case reports in the English literature. Lack of symptoms makes the diagnosis difficult and also makes our case unique. Biopsy from paragangliomas and surgical intervention are known to cause life-threatening complications, such as profuse bleeding and abrupt changes in blood pressure. Hence, paragangliomas should be considered as a possibility, even if a remote one, in case of multicentric retroperitoneal tumors. This case also highlights the importance of cytology in the early diagnosis of retroperitoneal masses. How to cite this article Singh A, Shikha D, Agarwal S, Khurana N, Jain SL, Hadke NS. Multiple Retroperitoneal Paragangliomas: An Uncommon Entity. World J Endoc Surg 2017;9(1):20-23.

Cardiac Paraganglioma—A Rare Subset of a Rare Tumor

Background: Cardiac paraganglioma is a rare entity of an uncommon neuroendocrine tumor. Clinically, non-secreting tumors are often diagnosed because of their growth effects, secreting tumors present symptoms related to catecholamine. Correct diagnosis of a paraganglioma can be reached by biochemical investigations and imaging. Surgical resection is the treatment of choice and has to be planned carefully and interdisciplinarily. Aim: On the basis of a patient with a vague clinical presentation and an unclear situation after primary investigations, we highlight the diagnostic challenge of this rare subset of paragangliomas. Case presentation: We present the case of a 42-year-old woman whose unspecific symptoms and further investigations revealed a paracardiac mass with unknown local behavior and dignity. Surgical resection and histopathological examination led to the diagnosis of a cardiac paraganglioma. Conclusion: Cardiac paragangliomas are extremely rare, but may be treated curatively by resection after careful preoperative investigation and interdisciplinary planning.

Retroperitoneal Paraganglioma – a rare cause of arterial hypertension

Paragangliomas are rare neuroendocrine tumours, most commonly found in adults. These differ from pheochromocytomas in that their location is extra-adrenal, and they are responsible for about 1% of arterial hypertension aetiologies. We report the case of a 30-year-old female whose past medical history was unremarkable. However, her arterial hypertension led to further examination in search of secondary aetiologies, in which a retroperitoneal mass and an increase in levels of catecholamines were detected; findings that led to the final diagnosis of paraganglioma. A multidisciplinary team, whose approach was to use pharmacological alpha-adrenergic blocking agents and a surgical resection of the lesion, treated the patient. The patient is clinically well but will continue to be monitored as an outpatient, and genetic testing is being encouraged

Radiosurgery for Paragangliomas of the Head and Neck: Another Step for the Validation of a Treatment Paradigm

Paragangliomas are highly vascular and predominantly benign neoplasms that traditionally have been treated by surgery, embolization, and/or external beam radiotherapy. The aim of this study was to evaluate long-term local tumor control and the safety of radiosurgery for head and neck paragangliomas. Data were obtained from prospectively maintained databases of patients affected by brain tumors treated with radiosurgery at our institution. The inclusion criteria were histologically proven or radiologic-suspected diagnosis of paragangliomas; a follow-up period of at least 12 months, an magnetic resonance imaging-based tumor growth control analysis and a signed written consent. Twenty patients (21 paragangliomas) met the eligibility criteria and were included in the present study. All patients were clinically and radiologically evaluated before and after treatment. The mean follow-up at the time of the present analysis was 46 months. Seven patients had a follow-up longer than 60 months. Seven lesions underwent a single-session radiosurgery with a mean dose of 12.2 Gy (range 11-13 Gy). Fourteen lesions underwent multisession radiosurgery with a mean dose of 25.7 Gy (range 20-30 Gy) delivered in 3-5 fractions. The mean tumor volume for single-session radiosurgery was 4 cc (range 1.4-9.2). The mean volume for multisession radiosurgery was 18.9 cc (range 1.3-50.9). None of the lesions showed progression on radiology during the follow-up period. Neurologic conditions generally are maintained or improved. Both single and multisession radiosurgery were confirmed as a safe and effective treatment modality for paragangliomas. Multisession radiosurgery appears effective to treat large lesions.

More Common than You Would Think: A Case of Catecholamine-Secreting Paraganglioma in the Urinary Bladder

A 57-year-old woman presented with headache, palpitations and hypertension associated with micturition and was found to have a bladder tumour on imaging. During cystoscopy, with manipulation of the mass, the patient became symptomatic and hypertensive, and a catecholamine-secreting paraganglioma was suspected. Biochemical testing confirmed the diagnosis of paraganglioma of the urinary bladder and she underwent successful resection after preoperative treatment with alpha- and beta-adrenergic blockade and volume expansion. In this article, we outline the prevalence of extra-adrenal paragangliomas and their possible genetic basis. We also overview the diagnosis, perioperative management, follow-up testing and prognosis of these tumours.

Paraganglioma of maxillary sinus associated with Addison’s disease mimicking a vascular tumour: A case report

Paraganglioma of the nasal and paranasal sinuses are quite rare neuroendocrine tumours, usually hormonally inactive, presentingas nasal polyps with or without epistaxis. We present a rare case of a 23 year old male, with Addison’s disease, who was referredfrom endocrinology department with a complaint of right nasal blockage associated with recurrent epistaxis. A provisionalclinical diagnosis of vascular tumour was made, Computed tomography and MRI suggested hemangioma /inverted papilloma.However, biopsy reported a highly vascular tissue mimicking angiofibroma, further immunostaining studies with Synaptophysinand S-100 confirmed the diagnosis of Paraganglioma. This rare tumour in the paranasal sinuses may mimic vascular tumour andcan only be confirmed with histopathological immunostains studies. According to our knowledge, this is first case reported to beassociated with Addison’s disease in the English literature.

Delayed Paraganglioma Diagnosis: A Case Report and Literature Update

Delayed Paraganglioma Diagnosis: A Case Report and Literature Update

Clinical characteristics and surgical outcomes of primary spinal paragangliomas.

Spinal paragangliomas are extremely rare tumors, most frequently involving the cauda equina and the filum terminale. We aimed to investigate the clinical manifestations, radiological features, management, and follow-up data of primary spinal paraganglioma. We present the clinical data and long-term outcomes from a consecutive surgical series of 19 patients with pathologically diagnosed spinal paragangliomas. All of the patients had undergone surgical resection. Pre- and postoperative magnetic resonance imaging was performed and follow-up data and neurological functional assessment are presented and discussed. The mean age at diagnosis was 47.7 years, with a significant male predominance. The primary clinical symptoms were low back pain and sciatica. Magnetic resonance images (MRI) showed characteristic signs that help differentiate paragangliomas from other spinal tumors, including a "salt & pepper" sign, serpiginous flow void, and a peripheral hypointense rim. Also, a well-encapsulated appearance can be found intraoperatively. During a mean follow-up period of 62.1 months, remnant tumor progression was noted on MRI in three patients with incomplete resection. Pain symptoms were relieved immediately after surgical intervention, while motor and sphincter dysfunction were much slower to improve. Differential diagnosis of paraganglioma based on MR images alone is challenging, but the presence of specific characteristic features provides suggestive clues; however, accurate diagnosis depends on pathological criteria. Despite the benign course, gross total resection is ideal, given an increased risk of recurrence in situ. Timely recognition and surgical treatment should be emphasized to avoid progressive neurological deficits.

Nonfunctioning paraganglioma of the urinary bladder: A rare entity

Paraganglioma of the urinary bladder is a rare tumor accounting for ˂0.06% of all bladder tumors and ˂1% of all pheochromocytomas. The distinction of paragangliomas from urothelial carcinoma is likely to be critical because of differences in therapeutic management. We report a case of 24-year-old female who presented with a complaint of intermittent hematuria and no other characteristic symptoms of paraganglioma. Ultrasonography revealed a lobulated mass arising from the anterior wall of the urinary bladder. Imaging studies and other relevant investigations were inconclusive. Transurethral resection was done and sent for histopathology. On histopathology, final diagnosis of paraganglioma was made. Patient is symptom-free postoperatively and is on regular follow-up 6 monthly.

A new approach to the treatment of metastatic paraganglioma: sorafenib.

Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. Patients with paragangliomas may develop metastatic diseases. There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. In this report, we presented a case of a 43-year-old woman who was admitted to the hospital with a history of episodic headaches, diaphoresis, and weakness. Elevated plasma catecholamine levels and a right paraaortic mass were observed on computed tomography. The mass was excised, and a diagnosis of paraganglioma was confirmed. After 20 months of follow-up, local recurrence and metastases were detected in the thorax, abdomen, and skeletal system. Plasma and urinary catecholamine levels were high. Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patient’s lesions had completely regressed.

Paraganglioma van het ganglion celiacum bij een hond

Een tienjarige Franse buldog werd aangeboden voor acuut braken. Echografisch en computertomografisch onderzoek van de buik gaf een duidelijk beeld van een duidelijk afgelijnde massa rond de arteria coeliaca, dicht bij de plaats waar zij uit de aorta ontspringt. Via chirurgie kon de massa volledig weggenomen worden. Histopathologisch onderzoek in combinatie met immunohistochemisch onderzoek resulteerde in de diagnose van paraganglioma.

Characterization and plasma measurement of the WE-14 peptide in patients with pheochromocytoma.

Granins and their derived peptides are valuable circulating biological markers of neuroendocrine tumors. The aim of the present study was to investigate the tumoral chromogranin A (CgA)-derived peptide WE-14 and the potential advantage to combine plasma WE-14 detection with the EM66 assay and the existing current CgA assay for the diagnosis of pheochromocytoma. Compared to healthy volunteers, plasma WE-14 levels were 5.4-fold higher in patients with pheochromocytoma, but returned to normal values after surgical resection of the tumor. Determination of plasma CgA and EM66 concentrations in the same group of patients revealed that the test assays for these markers had an overall 84% diagnostic sensitivity, which is identical to that determined for WE-14. However, we found that WE-14 measurement improved the diagnostic sensitivity when combined with the results of CgA or EM66 assays. By combining the results of the three assays, the sensitivity for the diagnosis of pheochromocytoma was increased to 95%. In fact, the combination of WE-14 with either CgA or EM66 test assays achieved 100% sensitivity for the diagnosis of paragangliomas and sporadic or malignant pheochromocytomas if taken separately to account for the heterogeneity of the tumor. These data indicate that WE-14 is produced in pheochromocytoma and secreted into the general circulation, and that elevated plasma WE-14 levels are correlated with the occurrence of this chromaffin cell tumor. In addition, in association with other biological markers, such as CgA and/or EM66, WE-14 measurement systematically improves the diagnostic sensitivity for pheochromocytoma. These findings support the notion that granin-processing products may represent complementary tools for the diagnosis of neuroendocrine tumors.

Laparoscopic Resection of Retrocaval Non-functioning Paraganglioma

Retroperitoneal paraganglioma is located primarily at the para-aortic area and functioning tumor, however, non-functioning and retrocaval located tumors are rare. Laparoscopic retrocaval paraganglioma resection is technically challenging due to vena cava close relation. A 49-year-old male was referred for a retroperitoneal tumor, detected by FDG-PET scan. He had undergone right thyroidectomy for papillary carcinoma one year ago. Abdominal computed tomography (CT) scan showed a mass measuring 2 cm in size located behind the inferior vena cava (IVC), which deviate IVC anteriorly. I123-metaiodobenzylguanidine (MIBG) scan also demonstrated abnormal focal activity. Result of preoperative adrenal function test was normal. A laparoscopic retrocaval tumor excision was performed successfully. The operative time was 160 minutes. The patient’s postoperative recovery was uneventful and he was discharged on the fourth postoperative day. Pathologic examination was consistent with the diagnosis of paraganglioma. To the best of our knowledge, this is the first report on laparoscopic resection of a retrocaval non-functioning paraganglioma. In conclusion, laparoscopic retrocaval paraganglioma resection is safe and feasible.

Nonfunctional Pelvic Retroperitoneal Paraganglioma Mimicking Postmenopausal Adnexal Mass

Paraganglioma is a rare tumor originates from paraganglial tissue which is composed of extraadrenal chromaffin cells. The pelvic and retroperitoneal location of this tumor is extremely rare and it can mimic adnexal mass. The diagnosis of paraganglioma in this region is difficult. The exact diagnosis may only be reached after the total excision and histopathologic examination of the tumor. In this report, for the first time, we presented a postmenopausal woman who had nonfunctional retroperitoneal paraganglioma mimicking postmenopausal adnexal mass. This case emphasizes that extraadrenal paraganglioma should be kept in mind in the differential diagnosis in all patients including postmenopausal woman with retroperitoneal mass even in the presence of nonspecific symptoms like abdominal pain.

Familial paraganglioma of head and neck

Paragangliomas are tumors originated from non-chromaffin paranganglia cells derived from the neural crest. These highly vascular tumors are located in the region which extends from skull base to arcus aorta. Head and neck paragangliomas have rare occurence constituting 0.012% of all the body tumors and 0.6% of head and neck tumors. Head and neck paragangliomas occur in sporadic and familial forms. The ratio of familial head and neck paragangliomas is about 10%. Familial form has a characteristic of autosomal dominant inheritance. In this article, four cases having first and second degree of affinity that we followed for paraganglioma diagnosis are presented.