Paraganglioma and adrenal pheochromocytoma presenting simultaneously in an elderly female. Case report and review of the literature

Paragangliomas are chromaffin tissue tumors arising in an extra-adrenal location. It is quite rare to find a paraganglioma concurrently with a pheochromocytoma. We report a patient who underwent resection of a retroperitoneal mass that was characterized pathologically as a malignant paraganglioma. An incidental finding was a microscopic pheochromocytoma in the ipsilateral adrenal gland.     

Hereditary paragangliomas and pheochromocytomas

Paraganglioma are rare tumours, which develop in the paraganglionic sites of the head and the neck, and around the medulla of the adrenal gland and the sympathetic ganglions of the thoracoabdominal and pelvic regions. Their morbidity-mortality is due to the local and regional spread to the adjacent nervous and vascular structures, their hypervascularisation, the major cardiovascular complications when they are secreting tumours and the risk of recurrences and metastases. Paraganglioma can secrete catecholamines. The only curative treatment is surgical exeresis. The hereditary paraganglioma are more often multifocal, recurrent, sometimes malignant, and with an earlier occurrence than the sporadic forms. The genes responsible for the familial syndromes are the coding genes for the three sub-units of succinate dehydrogenase (SDHD, SDHB and SDHC) or mitochondrial complex II. Recent discoveries show that these genes are truly implicated in the sporadic forms of the illness and that the research for a germline mutation of a SDH gene is essential for managing patients with paraganglioma and/or pheochromocytoma and their families.     

Incidental posterior mediastinal paraganglioma: The safe approach to management,case report

IntroductionParagangliomas are neuroendocrine tumors arising from chromaffin cells located in sympathetic paraganglia. Mediastinal paragangliomas are extremely rare and can be classified as functional or non-functional according to their ability for secreting catecholamines. Patients can be asymptomatic and the diagnosis is usually incidental. Complete surgical resection remains the standard of care for paragangliomas.Presentation of caseWe present a 44-year-old woman with a functional mediastinal paraganglioma incidentally found during the perioperative imaging workup for a diagnosed breast carcinoma. Chest radiograph and computed tomography (CT) showed a well-defined lesion in the posterior mediastinum suspicious for an esophageal malignancy. Endoscopic and CT-guided biopsies were performed confirming the diagnosis of a neuroendocrine tumor. Laboratory studies showed elevated catecholamines and chromogranin A levels, consistent with a paraganglioma. Appropriate pre-operative management was done and successful surgical resection without catecholamine related complications was achieved.DiscussionThe workup and treatment of incidentally discovered adrenal and extra-adrenal lesions are controversial. Because of the absence of symptoms and the wider differential diagnosis of extra-adrenal lesions, an attempt for biopsying and surgically remove these lesions prior to biochemical testing is not an uncommon scenario, although this could be potentially harmful. Surgeons should have an index of suspicion for catecholamine-secreting tumors and hormonal levels should be assessed prior to biopsy or surgical resection.ConclusionSurgeons should consider paragangliomas as a differential diagnosis for extra-adrenal lesions. Biochemical testing with catecholamines and chromogranin A levels should be performed prior to biopsy or surgical removal in order to avoid catastrophic complications.     

Malignant pheochromocytoma. A case report and comments on a rare pathology

Pheochromocytomas and functioning paragangliomas are rare tumors arising from the primitive neural crest, and found in the adrenal medulla or elsewhere within the sympathetic paraganglion axis. Clinical symptoms are related to catecholamine production or less frequently to dopamine or other neuropeptides secretion. Malignant pheochromocytomas are very rare tumors comprising between 5-35%, but this value is uncertain because the usual criteria for malignancy, such as mitotic activity, nuclear pleomorphism, are not suitable to discern benign from malignant pheochromocytomas. A specific diagnosis of malignancy requires evidence of invasion of the adjacent organs and the occurrence of metastases. Personal experience is presented with 92 patients affected by: adrenal pheochromocytomas (51 cases), cervical paragangliomas (32 cases), and extra-adrenal paragangliomas (9 cases). Malignant forms were observed in a 23-year-old young woman affected by malignant pheochromocytoma with lymphatic para-aortic metastases (1.9%), and in 2 patients affected by cervical paragangliomas (1 CBT, 1 VBT) with lymph nodal metastases. Careful follow-up of all patients with measurement of the urinary catecholamine is necessary to detect metachronous neoplasm and later metastases, identified with RMN and 131I-MIBG scintiscan.     

Primary paraganglioma of seminal vesicle

INTRODUCTIONParagangliomas are rare tumors arising from neural crest tissue located outside the adrenal gland. Primary seminal vesicle paraganglioma is extremely rare entity.PRESENTATION OF CASEA 26-year-old male patient presented with symptoms and signs of acute appendicitis where a CT of abdomen and pelvis showed an inflamed appendix and incidental finding of left seminal vesicle mass. The patient underwent uneventful laparoscopic appendectomy followed by transrectal ultrasound (TRUS) guided seminal vesicle biopsies. Histopathology revealed a neuroendocrine neoplasm consistent with paraganglioma. Surgical excision of the left seminal vesicle was carried out.DISCUSSIONParaganglioma of genitourinary tract is rare. The urinary bladder is the most common site, followed by the urethra, pelvis and ureter. Seminal vesicle paragangliomas were reported in association with other genitourinary organ involvement such as bladder and prostate. Isolated seminal vesicle paraganglioma is extremely rare and surgical excision remains the standard treatment for localized paraganglioma.CONCLUSIONPrimary tumors of seminal vesicle are rare and represent a diagnostic challenge. Differential diagnosis includes a list of benign and malignant tumors. Primary seminal vesicle paraganglioma is a rare but important diagnosis to be included in the differential diagnosis.     

Paraganglioma (pheochromocytoma) of the posterior mediastinum: a case report and review of the literature

Pheochromocytoma is an uncommon tumor in the pediatric population. Paraganglioma, or extra-adrenal pheochromocytoma, accounts for only a small fraction of pediatric pheochromocytomas, with most of these tumors presenting within the abdominal cavity. Herein the authors report the extremely rare case of a biochemically active paraganglioma presenting as a posterior mediastinal mass in an 8-year-old boy.     

Malignant catecholamine-secreting carotid body paraganglioma

The second known case of a malignant catecholamine-secreting (DA)-secreting carotid body paraganglioma is presented. Dopamine synthesis and secretion can be increased in malignant tumors derived from neural crest cells. Whether this is true, in addition, for extra-adrenal paragangliomas is not yet clear. Malignant paragangliomas of the carotid body and larynx, although rare, frequently have been accompanied by increased catecholamine secretion. Malignant catecholamine-secreting carotid body paragangliomas are best treated by composite resection (internal carotid artery and neck dissection), with special attention being given to measures preventing severe hypertension and arrhythmias in the perioperative period.     

New perspectives in pheochromocytoma

Pheochromocytomas are tumors that arise from chromaffin cells. Although the majority occur in the adrenal gland, these tumors can be found anywhere from the neck to the base of the pelvis. This condition, which is the cause of hypertension in only a small percentage of patients, can be cured in approximately 90 per cent of cases but can be lethal if left untreated. Pheochromocytomas may be familial in 10 per cent of patients, and in such cases, they may be associated with a variety of other conditions. The authors review the biochemical and radiologic diagnosis of adrenal and extra-adrenal tumors and discuss the treatment, including the special problems of pheochromocytoma during pregnancy.     

Sympathetic paraganglioma presenting with Horner's syndrome in a child.

Paragangliomas of the head and neck arise from collections of cells from the neural crest associated with cranial or sympathetic nerves. Such lesions are rare in children. The majority of paragangliomas in the paediatric age groups have been familial, occurring in association with an adrenal pheochromocytoma. Children typically present with a neck mass and systemic evidence of catecholamine hypersecretion. We present the case of a 12-year-old boy with a history of increasing headache, drowsiness and Horner's syndrome for 8 months. The patient underwent exploration of the neck mass under general anaesthesia. The lesion was found to be in continuity with the left sympathetic chain. It was highly vascular but was removed en mass and sent for histological analysis. The histology showed that the mass was a well-circumscribed encapsulated tumour and that complete excision was achieved. The post-operative period was uneventful and the patient recovered well after the operation. On review at 6 months following surgery, his scars were maturing satisfactorily but the Horner's syndrome was persistent. Sympathetic chain paragangliomas are very rare tumours in the head and neck but should be considered in the differential diagnosis when clinical and radiographic evidence suggest a paraganglioma. The presentation is typically of a slow-growing neck mass with the presence of an ipsilateral Horner's syndrome. To our knowledge, this is the first reported case of a sympathetic chain paraganglioma in a child.     

Malignant abdominal paraganglioma presenting as a giant intra-peritoneal mass

INTRODUCTIONParaganglioma is a malignant tumor that arises from the extra adrenal paraganglionic cells of the sympathetic or parasympathetic systems. Herein, we present a case of abdominal mass of unknown histology that underwent a very difficult surgical mass resection. Its final histologic diagnosis was non-functional paraganglioma that was presented as a huge intra-peritoneal mass.PRESENTATION OF CASEThe patient was a 55-year-old Iranian lady who referred to our center with a giant abdominal mass. Fine needle aspiration (FNA) biopsy showed undifferentiated carcinoma. After laparotomy for mass resection pathology evaluation revealed; malignant paraganglioma, non-metastatic type. Further post-operative patients evaluations showed that the tumor was sporadic in nature and the subsequent patient's natural history was uneventful.DISCUSSIONPathologic evaluations have key roles in the exact diagnosis of abdominal masses with unidentified sources. For the paragangliomas, all of them should be regarded malignant until proved otherwise. However, combined use of biochemical markers, immunohistochemical techniques (IHC), and genetic analysis have key roles in the diagnosis and treatment of paragangliomas. Additionally, surgical removal is the proved curative way of paraganglioma treatment.CONCLUSIONAsymptomatic intra-peritoneal paraganglioma is very rare, especially its malignant variety. This case emphasizes that full pathologic investigation would reveal the exact nature of idiopathic abdominal masses, especially in the state of absence of typical clinical and para-clinical symptoms.     

Cervical paraganglioma with subsequent intracranial and intraspinal metastases. Case report

Paragangliomas, tumors derived from the extra-adrenal paraganglion system, have commonly been found in the retroperitoneum, abdomen, mediastinum, skull base, and neck. Rare intraspinal cases have included involvement of the cauda equina and filum terminale, and a recent case has been reported of thoracic cord paraganglioma with metastasis to the cauda equina. The authors present the case of a patient with a cervical paraganglioma who underwent subtotal resection followed by postoperative irradiation; she subsequently developed multiple intracranial and intraspinal metastases 7 months following the first decompression procedure.     

Unusual cause of early preeclampsia: bladder paraganglioma

Ten to twenty percent of paragangliomas occur at extra-adrenal locations and less than 1% are at the urinary bladder. The most common presenting symptom of bladder paraganglioma is hypertensive attacks precipitated by micturition and hematuria. Paraganglioma of the urinary bladder occurring at pregnancy is extremely rare. We present a case of bladder paraganglioma as an unusual cause of early preeclampsia. After termination of the pregnancy, surgical resection was performed and the histopathologic diagnosis of paraganglioma confirmed. At 24 months of follow-up the patient felt well and was normotensive without any foci of paraganglioma. Although rare, paraganglioma must be considered in the differential diagnosis of early preeclampsia.     

A case of cervico-mediastinal paraganglioma mimicking an ectopic goiter

Introduction and importanceMediastinal paragangliomas are rare neuroendocrine tumors that originate from extra-adrenal paraganglia, occasionally secreting catecholamines. Nonfunctional mediastinal paragangliomas present nonspecific clinical and radiological features and represent a diagnostic challenge.Case presentationA 53-year old woman presented with cough and dyspnea increasing over time. CT-scan and ultrasonography showed a large vascularized cervico-mediastinal mass, consistent with an intrathoracic ectopic goiter. Preoperative angiography showed a blood supply from neck vessels. The lesion was completely removed through a cervical approach. The diagnosis of paraganglioma was a histological surprise. The patient is alive without recurrence 30 months after surgery.Clinical discussionWhen preoperatively diagnosed, the treatment of choice of a mediastinal paraganglioma is surgical excision. However, a preoperative diagnosis of mediastinal paraganglioma is difficult to obtain, especially in cases of nonfunctional lesions. Distinction between an intrathoracic goiter and a nonfunctional paraganglioma can be extremely difficult and, given the rarity of the latter, an ectopic goiter is suspected in first instance. CT-scan and ultrasonography are of little use in the differential diagnosis. However, scintigraphy with ¹²³I-metaiodobenzylguanidine can be an useful diagnostic tool when a paraganglioma is suspected. In case of vascularized cervico-mediastinal mass, such as paragangliomas or intrathoracic goiter, preoperative angiography should be performed to study the blood supply and orient the surgical approach.ConclusionAlthough uncommon, paragangliomas should be considered in the differential diagnosis of mediastinal masses, especially when an ectopic goiter is suspected.     

Catecholamine-Secreting Paragangliomas at the Skull Base

Paragangliomas (glomus tumors) comprise 15% of all neoplasms at the skull base. Despite extensive growth, these tumors usually do not secrete active biogenic substances into the circulation in sufficient quantities to produce symptoms. When they do secrete large amounts of catecholamines, they will cause symptoms that mimic a pheochromocytoma. The still confusing nomenclature of paragangliomas is reviewed, and the clinical work-up, surgical treatment, and follow-up of five patients with catecholamine-secreting paragangliomas of temporal bone (3), infratemporal fossa (1), and nasopharynx (1) are presented and discussed.     

Catecholamine-secreting paragangliomas at the skull base

Paragangliomas (glomus tumors) comprise 15% of all neoplasms at the skull base. Despite extensive growth, these tumors usually do not secrete active biogenic substances into the circulation in sufficient quantities to produce symptoms. When they do secrete large amounts of catecholamines, they will cause symptoms that mimic a pheochromocytoma. The still confusing nomenclature of paragangliomas is reviewed, and the clinical work-up, surgical treatment, and follow-up of five patients with catecholamine-secreting paragangliomas of temporal bone (3), infratemporal fossa (1), and nasopharynx (1) are presented and discussed.     

Management of an anterior mediastinal pheochromocytoma causing tracheomalacia

Thoracic paragangliomas are rare tumors that arise from extra-adrenal chromaffin cells and have the capacity to secrete catecholamines. Surgical excision is the optimal treatment of these tumors as they are resistant to chemotherapy and radiation therapy. Although these tumors are most commonly found in the abdomen, 10% of paraganagliomas are located in the thorax, usually in the posterior mediastinum. Occasionally these tumors present in the anterior mediastinum, which can pose a significant surgical challenge due to the proximity of the great vessels and airway. In this report we describe the treatment of an anterior mediastinal pheochromocytoma that presented with tracheal obstruction and required pulmonary artery reconstruction and airway stenting.     

Primary symptomatic perivesical paraganglioma: a case report

Paraganglioma, extra-adrenal pheochromocytomas, are relatively rare in adults, with most arising from para-aortic sympathetic and visceral organs, such as the bladder. Paraganglioma localized at the extravesical retroperitoneal pelvic cavity is extremely rare. We report a case of symptomatic perivesical pheochromocytoma in a 34-year-old man treated by surgical excision. Symptoms related to cathecolamine secretion ceased after surgery, and the patient has remained disease-free for 24 months.     

Paraganglioma presenting as a vascular malformation: case report.

Paragangliomas are rare tumours that arise from extra-adrenal chromaffin cells or neuroectodermal chemoreceptor. They are high vascularised and can be functional, secreting catecholamines and other peptides. They occur anywhere from the skull base to the pelvic floor, within the paraganglion system. We present a case of paraganglioma with unusual location, atypical presentation and interesting vascular anatomy.     

Paragangliomas of the urinary bladder: A report of 6 cases and review of the literature

ObjectiveExtra-adrenal pheochromocytomas are known as paragangliomas. The majority of extra-adrenal tumors occur intra-abdominally along the sympathetic chain. However, they may also occur in the urinary tract, including the urinary bladder.Materials and methodsIn this study, we report a clinical series of six patients diagnosed with bladder paragangliomas in our hospital over the past two decades, and review the literature regarding this unusual disease.ResultsAmong the six cases, two were male and four were female. The most common presentation was painless gross hematuria, with five of the six patients (83.3%) having this chief complaint. Two patients presented with hypertension and one with palpitations. Two had malignant bladder paragangliomas, and one died within one year after the diagnosis. Four patients received transurethral resection of the bladder tumors, and one patient developed tumor recurrence within one year of follow-up.ConclusionBladder paraganglioma is an extremely rare tumor with a high recurrence rate, and the most common presenting symptom in our series was painless gross hematuria. Large, polypoid, and multiple tumors indicate malignancy.     

Pheochromocytoma: current status and changing trends

From 1971 through 1980, 106 patients underwent operations for pheochromocytoma. Twelve patients had a pheochromocytoma as a manifestation of the multiple endocrine neoplasia type 2 syndrome. Twenty patients were found to have extra-adrenal paragangliomas. Fifteen patients (14%) had malignant tumors; eight of these tumors were encountered in the group with extra-adrenal lesions. The cumulative survival probability at 5 years was 53%. Determination of urinary metanephrines and vanillylmandelic acid were the most sensitive diagnostic aids, with accuracy rates of 95% and 89%, respectively. In recent years, the measurement of fractionated urinary and plasma catecholamine levels has greatly increased the diagnostic accuracy. Preoperative localization of pheochromocytoma has improved during the last decade. Nephrotomography, selective angiography, and venous sampling have essentially been abandoned in favor of computed tomography. With an accuracy of more than 90%, computed tomography represents the major step forward in the overall management of pheochromocytoma during the past decade.