Paraganglionic Cells Research Articles

A Case of Jugular Paraganglioma Presented as Temporomandibular Joint Disorder

Background:Jugular paraganlioma is a slow-growing tumor originating from the paraganglion cells. The diagnosis of a jugular paraganglioma may be delayed due to its atypical symptoms. We report a case of jugular paraganglioma presented as temporomandibular joint (TMJ) disorder. Clinical presentation: 29-years old woman with preliminary diagnosis probable atypical face pain. She experienced her pain for the first time 2 months prior to reference. Pain intensity was seven to eight out of ten points on Visual Analogue Scale, simple and combined analgetics and NSAIDs were ineffective. Pain was dull, aching, localized in the right temporal area with irradiation to the ipsilateral maxillar and periauricular areas. Pain was more intensive in the nighttime. Temporomandibular joint movement (maximum unassisted and assisted opening, right to left movements) also increased pain. Significant dilatation of right foramen jugularis with its right side destruction was detected by computed tomography with contrast. The radiological findings were consistent with those of a jugular paraganglioma. Conclusion:This case confirms that in refractory headache and TMJ pain other reasons should be considered with rigorous investigation including neuroimaging

Neuroendocrine Cells of the Prostate Derive from the Neural Crest

The histogenesis of prostatic neuroendocrine cells is controversial: a stem cell hypothesis with a urogenital sinus-derived progeny of all prostatic epithelial cells is opposed by a dual origin hypothesis, favoring the derivation of neuroendocrine cells from the neural crest, with the secretory and basal cells being of urogenital sinus origin. A computer-assisted 3D reconstruction was used to analyze the distribution of chromogranin A immunoreactive cells in serial sections of human fetal prostate specimens (gestation weeks 18 and 25). Immunohistochemical double labeling studies with YFP and serotonin antisera combined with electron microscopy were carried out on double-transgenic Wnt1-Cre/ROSA26-YFP mice showing stable YFP expression in all neural crest-derived cell populations despite loss of Wnt1 expression. 3D reconstruction of the distribution pattern of neuroendocrine cells in the human fetal prostate indicates a migration of paraganglionic cells passing the stroma and reaching the prostate ducts. Double-transgenic mice showed 55% double labeling of periurethral neuroendocrine cells expressing both serotonin and YFP, whereas single serotonin labeling was observed in 36% and exclusive YFP labeling in 9%. The results favor the assumption of a major fraction of neural crest-derived neuroendocrine cells in both the human and murine prostates.

Paragangliomas of head and neck - A surgical challenge.

Paragangliomas of the head and neck region are rare and predominantly asymptomatic tumors. These neoplasms arise from widely distributed paraganglionic cells, which originate from both mesodermal elements of third branchial arch and the neural crest residues. Despite the rare occurrence of paragangliomas, problems encountered in their diagnosis, unclear malignancy and treatment ensure that they still remain in the focus of head and neck surgeons. This is a retrospective study of the medical records of patients treated in the ENT Department of the 5th Military Hospital in Krakow during the period 2010-2014. All the preoperative, intraoperative and postoperative data were carefully analyzed for each patient. Thirteen patients (16 tumors) were treated during the study period. All the patients with a pre-operative suspicion of paraganglioma underwent computed tomography angiography. The whole cohort of patients was treated surgically. Paraganglioma should be always considered in the differential diagnosis for painless neck masses. These tumors require thorough radiological pre-operative evaluation and skilled operative technique. Surgical treatment occurs to provide good cure rates with minimal recurrence and morbidity rates.

A Series of 2 Rare Cases of Filum Terminale Paraganglioma – A Case Report

Paraganglioma is a neuroendocrine tumour arising from non chromaffin paraganglion cells anywhere in the body. Most common site is adrenal gland. In central nervous system, they are found at petrous ridge, pineal gland, sella turcicia and spine. In the spine, cauda equine localization of paraganglioma is common however it is much rarer in filum terminale site. We are presenting two cases of middle age gentlemen with complaints of radicular pain of lower limb. MRI lumbar spine was suggestive of intradural tumor arising from filum terminale at lumbar site. The tumor was completely excised and histopathology revealed to be paraganglioma. We hope this case will be documented in the literature and help in understanding this entity more clearly.

Carotid Body Tumors: Surgical Management and Review of Patients Over 10 Years

Background: Carotid body tumours (CBTs) are rare but highly vascular neoplasms originating in the paraganglionic cells of the carotid bifurcation. Exact etiology of these tumors is not known. Male and female distributions are equal except at high altitude where females appear to predominate. Diagnosis of a chemodectoma usually begins with a color flow duplex scan. Magnetic resonance angiography are also useful, especially to evaluate bilateral disease. Conventional Arteriography with CT Angiography are valuable, especially in larger tumours, and are regarded as the best tools for diagnosis. The treatment of choice for carotid body tumours is surgical removal. Shamblin's classification system is used to categorize carotid body tumours based on their size and the difficulty of surgical resection. Results: It was observed that majority of the patients in this study were females numbering 35(79.45%) whereas there were 9 (20.43%) males. commonest age group involved was of patients 50 to 59 years of age. Surgery was done in all 44 (100%) patients with complete resection in 41 (93.07%) and incomplete in 3 (6.81%) patients. 10 (23%) were Shamblin grade 1, 29 (66%) grade 2 and 5 (11%) grade 3. ECA repair was done in 4 (9.08%) of patients, ICA repair in 2 (4.54%), ECA ligation in 2 (4.54%) and vascular graft was used in 1 (2.27%) of patients. 7 (15.89%) had transient cranial nerve palsy most commonly involving hypoglossal nerve. 3 (6.81%) had permanent cranial nerve palsy. In 6 (13.62%) patients there was local wound infection .There was post op stroke in 2 (4.54%) of patients. No operative mortality was seen.43 (97.61%) patients were proved to be paraganglionomas on HPE while 1 (2.27%) patients had inconclusive biopsy on Histopathological examination. Conclusion: Although rare, Carotid body tumor is still a pathology that we encounter in our experience and it should be kept in mind as a differential diagnosis for painless lateral neck masses.

Anatomy, physiology, and genetics of paragangliomas

Paragangliomas arise from an extra-adrenal paraganglionic cells, derived from the neural crest of the autonomic nervous system and make up the most common class of benign vascular neoplasms of the neck. PGLs of the head and neck originate most commonly from the paraganglia within the carotid body, the jugular foramen, the middle ear and the vagus nerve. Knowledge of the embryology, anatomy and genetics of these rare tumors is paramount to successful diagnosis and treatment of head and neck paragangliomas.

Transcranial approach to jugular paragangliomas

Glomus jugulare paragangliomas are a group of rare benign tumors originating from paraganglion cells of the parasympathetic nervous system and are usually hypervascular and slow growing, invading the temporal bone as they grow and may invade the intracranial space. Complete surgical removal is the preferred treatment especially in young, healthy individual and has been reported to be achieved in up to 80% of patients. However, radiosurgery has become a valuable treatment option in small tumor, in elderly patient, and as a complimentary treatment for surgery in large tumor invading the intracranial space. Complex transcranial skull base approaches are required for large tumors invading the middle and upper part of petrous bone, extending into the petroclival region and compressing the brainstem.

“Carotid body tumour: A case report”

Carotid body tumours are rare, slowly growing benign neoplasm arising from paraganglionic cells at the region of carotid bifurcation. They are the rare tumours of head and neck region derived from neural crest. We report here a case of carotid body tumour which was successfully treated surgically. Key Word: Carotid body tumour ; Paraganglioma ; Chemodectoma

Diagnosis of carotid body paragangliomas by various imaging techniques

Introduction: Carotid body paragangliomas are rare hypervascular lesions arising from neural crest paraganglia cells, with a tendency of slow, but progressive growth, giving rise to external compression, or/and involvement of the carotid arteries, craniofacial nerves and other neighbouring structures.

Paraganglioma in supraglottic area: A rare case report

Paragangliomaof the larynx is a rare tumourthat arises from paraganglion cells. These tumors are generallyconsidered benign and have to be differentiated from other neuroendocrine tumors. Diagnosis relies mostly on histopathological examination followed by immunohistochemistry. Surgical excision generally confers complete cure of the tumor. We hereby present a case of laryngeal paragangliomain a 50yearoldfemale, who presented with breathlessness, underwent total laryngectomy following a diagnostic microlaryngeal biopsy, which was supported by immunohistochemistry. KeywordsImmunohistochemistry; Larynx; Neuroendocrine; Paraganglioma

Carotid body tumor with thyroid involvement

Introduction: the carotid body tumor (paraganglioma) is a tumor that originates from paraganglion cells. the tumor is usually begnin and non-functional, and can expand early but rarely metastasis. these tumors are rare and make up 65% of all head and neck tumors. case report: Herein we discuss a case of a 66-yearold female patient who presented with a right side neck mass measuring 2x3 cm, soft and nontender. the mass was gradually increasing in size. blood pressure 142/182 mmHg, pulse 120 beats/min. blood workup: Hemoglobin 10.5 g/dL (11.0–15,0 g/dL), platelets count 479x10 3 / µL (150–400x10 3 /µL), thyroid stimulating hormone 0.26 mIU/L (0.34–5.6 mIU/L), thyroxin 13.5 g/dL, retro viral disease exposed, and liver function test was normal. conclusion: carotid body tumors are rare entity; they account for 65% of all head and neck tumors and should be considered in evaluating all lateral neck masses. they are diagnosed in the 4–5th decade of life. treatment of choice is surgical excision, but their differential diagnosis is important for all neck masses and the definite diagnosis is needed before surgery.

A Rare Surgical Tumor of Mesenteric Paraganglioma Presenting As A Fibroid Uterus

Paragangliomas are rare tumors originating from the parenchymal cells of paraganglia. In adults the adrenal medulla is the largest paraganglion and paragangliomas most frequently occur in the adrenal medulla. We report a rare case of a solitary primary paraganglioma arising in the mesentery, found in a 38 yr old woman who presented with complaints of infertility dysmenorrhoea, mass per abdomen, abdominal distension since 6 months. Clinical examination and ultrasound abdomen suggested multiple fibroids. Exploratory laparotomy revealed a large mesenteric cyst The histopathology and immunohistochemistry confirmed the diagnosis of mesenteric paraganglioma. Key words :Paraganglioma, Fibroid

Composite paraganglioma and ganglioneuroma in the retroperitoneum: a case report

Reports of a composite paraganglioma (PG) and ganglioneuroma (GN) in the retroperitoneum are rare. In the present case, dynamic computed tomographic (CT) findings showed a 30 × 22 × 20 mm tumor that was located in the retroperitoneum and which was dissociated from pancreatic tissue and the left adrenal gland. The markedly reddish tumor showed a clear margin and central multicystic changes on the cut surface. The tumor was composed of two major components, the PG and the GN. The paraganglionic cells in the PG component, which were arranged in a nested pattern, occupied the main and central part of the tumor. Both ganglionic cells and Schwann cells in the GN were located in a unorganized pattern in the periphery. The paraganglionic cells exhibited a Zellballen pattern, which consisted of an association of edematous vascular-rich stroma and focal hemorrhage, resulting in multicystic changes. These centrally located tumor cells were pleomorphic in part and did not have mitotic figures. In the periphery, Schwann cells, which were arranged in an obscure and fascicular pattern that was intermingled with large ganglionic cells, were located adjacent to the PG component with a mostly sharp margin. With higher magnification, the border was not as sharp, as revealed especially with chromogranin-A immunostain, in which both the PG and GN components were focally intermingled with each other. The histogenesis of the composite PG and GN was thought to be extraadrenal neural crest cells in the retroperitoneum because the tumor was not located in the adrenal gland or the Zuckerkandl organ, according to the CT findings. The immunohistochemical findings of this rare case of a composite PG and GN in the retroperitoneum are reported with a focus on the peculiar arrangement of these two components.

Cervical intradural paraganglioma presenting as progressive cervicodynia: Case report and literature review

Paraganglioma (PGL) is a neuroepithelial tumor that arises from he paraganglia cells (PCs) of the neuroendocrine system. Although t always develops in the sites of normal paraganglia, mainly intrabdominally and in the glomus jugulare and carotid bodies, it may ccur in other parts of the body. PGLs are rare in the spinal canal and sually present as the intradural tumor of the cauda equine. Here, e present a patient with a primary nonfunctioning intradural araganglioma of the cervical canal and review the clinical, radilogical and histopathological presentation of this unusual tumor.

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.

The immunomorphological analysis of innervation of paraganglian chromaffin cells of mammalian arteries and heart

Innervation of chromaffin cells of paraganglia of the wall of mammalian large arterial vessels and heart (in rat, cat, and human) was studied by neuromorphological and immunohistochemical methods. There is established similarity in structure of specialized, “basket“-like nerve endings of the chromaffin cells (ChC) with pericellular nerve apparatuses of sympathetic and parasympathetic autonomic neurons. It is proposed to use immunohistochemical reaction for synaptophysin as method of selective detection of ChC of paraganglia and adrenal medulla. The conclusion is made that synaptophysin-positive terminals (SPPT) found on bodies of ChC and postganglionic neurons represent efferent, rather than afferent, synapses formed by myelinated axons of preganglionic fibers. It is suggested that ChC of paraganglia alongside with their characteristic endocrine function participate in complex mechanisms of chemoreceptor regulation of tissue homeostasis of mammalian blood vessels and heart.

Transarterial Embolization and Doxorubicin Eluting Beads-Transarterial Chemoembolization (DEB-TACE) of Malignant Extra-Adrenal Pheochromocytoma

Extra-adrenal pheochromocytomas (EAPs) are neuroendocrine tumors that arise from paraganglion cells of the sympathetic component of the autonomic nervous system. The paraganglia are chromaffin tissue complexes that extend along the paravertebral axis [1]. As part of the autonomic nervous system, these paraganglia are the dominant source of catecholamine production during early childhood [2]. Failure of involution of these paraganglia leads to the development of extra-adrenal pheochromocytomas. The majority of intra-abdominal paragangliomas present at the organ of Zuckerkandl (to the left of the aorta near the inferior mesenteric artery takeoff). Tumors below the diaphragm are typically functional with symptoms relating to excess catecholamine secretion. Generally, patients report nonspecific symptoms, such as headaches, sweating, palpitation, anxiety, and tremors. Biochemical workup demonstrates elevated levels of plasma and urinary catecholamines and their metabolites. Provided there is no contraindication, a contrastenhanced CT should be the initial imaging modality because it is readily available and highly sensitive. Scintigraphy with I-labelled metaiodobenzylguanidine (I-MIBG) is the most common functional study used. The treatment traditionally consists of open or laparoscopic exploration and resection with preoperative a and b blockade [3]. It is now known that EAPs demonstrate potential malignant change in up to 50% of patients, far greater than the 10% reported for pheochromocytomas [4]. Because there are no pathognomonic histological findings that distinguish benignity from malignancy, the diagnosis of malignant transformation is made on the basis of the development of recurrence or metastases and presence of lymph nodes.

Human laryngeal ganglia contain both sympathetic and parasympathetic cell types

The presence of ganglia associated with the laryngeal nerves is well documented. In man, these ganglia have been less well studied than in other species and, in particular, the cell types within these ganglia are less well characterized. Using a panel of antibodies to a variety of markers found in the paraganglion cells of other species, we were able to show the existence of at least two populations of cells within human laryngeal paraganglia. One population contained chromogranin and tyrosine hydroxylase representing a neurosecretory population possibly secreting dopamine. A second population of choline acetyltransferase positive cells would appear to have a putative parasympathetic function. Further work is needed to characterize these cell populations more fully before it will be possible to assign functions to these cell types but our results are consistent with the postulated functions of these ganglia as chemoreceptors, neurosecretory cells, and regulators of laryngeal mucus secretion.

Necessity of adequate monitoring during carotid body tumors

Carotid body tumors are rare, benign, vascular, slow growing and invasive to adjacent structures. The tumor originates from the neural crest paraganglion cells (1). Infiltration into the adjacent tissues and into the jugular vascular area requires surgical excision (2). During surgery intraluminal shunt procedure is usually required (3). The goal in the presentation of these case reports is to discuss preoperative angiographic findings and different monitoring options for cerebral ischemia prevention during removal of carotid body tumors (3, 4).

Paraganglia Mimicking Metastatic Seminomatous Tumor

Two cases are presented in which microscopic groups of retroperitoneal paraganglionic cells simulated metastatic seminomatous tumor. Both patients had histories of mixed testicular germ cell tumor with abdominal metastases and had been treated with chemotherapy. Persistent retroperitoneal disease was favored on follow-up imaging studies. Subsequent retroperitoneal lymph node dissection disclosed multifocal epithelioid cell groups with clear/vacuolated cytoplasm in the fibroconnective and adipose tissue, ranging from 1.0 to 3.0 mm in size. These cell groups were initially interpreted as recurrent metastatic seminoma, but were later reinterpreted as paraganglionic cells, which were confirmed by immunohistochemical analysis. The pathologic features for distinguishing paraganglionic cells from metastatic seminoma are discussed. Awareness of the presence of paraganglia and their distinction from metastatic disease is of practical importance in avoiding an overdiagnosis of malignancy and assuring proper patient management.