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Extra adrenal retroperitoneal paraganglioma.

We herein report a case of a 45-year-old Saudi lady not diabetic nor hypertensive who presented to the emergency room with a one day history of severe central and lower abdominal pain. On examination, she was hemodynamically stable and abdominal examination showed tenderness in the lower abdomen. Her hematological and biochemical investigations were normal. Computed tomography of the abdomen showed an 8 x 7 cm retroperitoneal mass located at the aortic bifurcation. The patient had exploratory laparotomy and complete excision of the mass. The histopathological study showed a paraganglioma. The patient had an uneventful postoperative period and follow up.

Female↗

The multidisciplinary management of paragangliomas of the head and neck, Part 2.

Paragangliomas most commonly occur in the carotid body, jugulotympanic area, and vagus nerve but have also been reported in other areas of the head and neck. These tumors are highly vascular and characteristically have early blood vessel and neural involvement, making their treatment particularly challenging. Surgery has traditionally been the preferred method of treatment, especially in light of recent advances in technique. However, compared to radiation therapy, it can result in a higher incidence of cranial nerve dysfunction. Radiation therapy has the advantage of avoiding the increased morbidity of surgery while offering an equal possibility of cure. Part 2 of this article discusses radiation therapy as primary treatment of patients who are ineligible for surgery and the elderly and infirm. Results with radiotherapy are comparable to those achieved with surgery. The efficacy of salvage therapy with either surgery or radiation is discussed, and a treatment algorithm for these tumors is proposed.

Combined Modality Therapy↗

Paraganglioma of the cauda equina.

A case of paraganglioma of the cauda equina is presented. Usually reported to be benign with good prognosis, the present patient had a somewhat different course. The behaviour of the tumour was unusual, with rapid recurrence seen within first month of surgery. The need for total surgical excision and role of adjuvant radiotherapy is highlighted.

Cauda Equina↗

[Nonfunctioning paraganglioma of the posterior mediastinum].

A 23-year-old man, complaining of a discomfort in the chest, saw a physician nearby and was pointed out chest abnormal shadow in chest X-ray. He was admitted to our hospital for surgery. As a result of a close check-up at this department, a giant tumor extending from above the right diaphragm to the posterior mediastinum was found. Physical findings in the preoperative examination and general examinations showed no abnormality. The operation was performed and was diagnosed as paraganglioma. This tumor is abundant in blood vessels. Thus, preoperative angiography and securing of the field of operation in treating the blood vessels were considered indispensable for the safety of operation.

Adult↗

Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis.

We report a case of duodenal gangliocytic paraganglioma (GP) in a 47-yr-old man. The GP arose in the second portion of the duodenum and was shown by histological examination to consist of epithelioid cells, spindle cells, and ganglion-like cells. Although most of the reported cases of GP have been regarded as benign, in the present case we found that a lymph node in the peripancreatic region contained a metastatic tumor. This is believed to be the third documented case of GP showing regional lymph node metastasis. The metastatic tumor consisted of only epithelioid cells, without spindle cells or ganglion-like cells, supporting the hypothesis that only epithelioid cells have malignant potential.

Ampulla of Vater↗

[Retroperitoneal paraganglioma].

OBJECTIVES: To report the radiological findings of retroperitoneal paragangliomas. METHODS: A patient who presents with colic pain and undergoes intravenous urography and CT scan. RESULTS: KUB x-ray revealed an increase of radiodensity on the left flank. The urography showed a partially obstructive urinary lithiasis in the proximal third of the left ureter with a mass effect laterally displacing pelvis and ureter. The study was completed with an i.v. contrast abdominal CT scan which showed a left para-aortic solid mass under the kidney with heterogeneous contrast uptake. CONCLUSIONS: The correlation of symptoms and catecholamine levels is the best way to guide the etiological diagnosis after CT scan.

Aged↗

Pelvic retroperitoneal paraganglioma mimicking an ovarian mass.

A 75-year-old hypertensive woman was referred with ultrasound findings of a 40 x 35 mm semi-solid right adnexal mass and right hydroureteronephrosis. She complained of headache and right-sided back pain. Computed tomography demonstrated a cystic adnexal mass that did not appear to originate from the right ovary and grade 2 hydroureteronephrosis. Magnetic resonance imaging indicated that the mass originated from the right ovary. Tumor markers were in the normal range. Exploratory laparotomy was performed to determine the origin of the lesion, and revealed a retroperitoneal mass obstructing the right ureter. The mass was completely removed and and the histopathologic diagnosis was paraganglioma.

Aged↗

Primary cardiac paraganglioma.

A primary cardiac paraganglioma was identified in a 59-year-old Caucasian male who complained of palpitations and chest pain. The neoplasm (4.7 cm in diameter) was surgically excised from the left posterior atrium. Adriamycin-based adjuvant chemotherapy was administered. The patient returned to his normal activities. There is no evidence of recurrence or metastasis after two years and nine months.

Antibiotics, Antineoplastic↗

[Paraganglioma of the jugular foramen presenting with initial isolated vertigo].

The more common symptoms of the jugulotympanic paragangliomas are hearing loss, tinnitus and palsy of cranial nerves. Diagnosis is often delayed due to the slow growth of the tumour. We report one case with affectation of jugular foramen which clinically debuted as a peripheral vertigo and the diagnosis was made by CT, IRM and angiography. The patient was treated by Neurosurgery using Gamma Knife (radiosurgery) with good result.

Adult↗

[Unsuspected posterior mediastinal paraganglioma--anesthetic risk].

A 70-year old female patient was admitted to the hospital because of scheduled thoracotomy and biopsy of posterior mediastinal retrocardiac tumor of unrecognized etiology. The patient had no complaints regarding the tumor. Routine anesthesiological preoperative examination revealed status ASA III. Induction in anesthesia was usually stable. At the moment when the surgeon intraoperatively touched the tumor, hemodinamic instability started and arterial blood pressure and heart rate dramatically increased. Our first reaction was to deepen the anesthesia. Very soon it was obvious that blood pressure increased by the surgeon's manipulation of the tumor and we started to doubt on catecholamine-secreting tumor. During the course of the operation there were several hypertensive episodes and we managed them by alternately using atenolol, glyceroltrinitrate and anesthetic drug. The patient was extubated 5 hours after transfer to the intensive care unit. Postoperative period was hemodynamically stable. The level of catecholamines in 24-hour urine collection was significantly increased. Pathohistologic diagnosis was mediastinal paraganglioma.

Aged↗

Suprasellar and intrasellar paragangliomas.

Neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. Immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.

Adenoma↗

[Significance of neuronal acetylcholine receptors as differentiation antigens in neuroblastomas and paragangliomas].

Using a panel of monoclonal antibodies to various epitopes of the alpha-, beta- and gamma-subunit of the muscle acetylcholine receptor (AchR), two different immunohistochemical reactivity patterns--corresponding to different neuronal AchRs--were identified in ganglia of the peripheral and central nervous system. The immunoreactivity pattern of neuroblastomas and paragangliomas was identical to the pattern found in the peripheral nervous system and has not been encountered in any other tumor type. Both the intensity of neuronal AchR immunoreactivity and the transcription of the neuronal AchR alpha-gene seem to correlated with a higher degree of neuroblastoma differentiation.

Antibodies, Monoclonal↗

[Imaging of non-functional retroperitoneal paragangliomas. Apropos of a case].

The non functional extraperitoneal paragangliomas are rare tumors (less than 50 cases reported). We report a case with ultrasound and CT scan imaging. The tumor is usually large with important necrotic areas leading to a cystic aspect. The malignant tumors more frequent than the benign tumors present with a regional extension and delayed metastasis.

Female↗

[A case of asymptomatic paraganglioma diagnosed by abnormal shadow on chest X-ray].

A 65-year-old man was noted to have an abnormal shadow on chest X-ray at mass screening examination, and was seen at Douhoku Byoin National Sanatorium. Chest X-ray showed a homogeneous mass shadow and a lytic lesion of the right 5th rib with extra-pleural tumor sign at the right upper and middle lung fields. Retroperitoneal tumor of 5 cm in diameter was detected in the right adrenal area by ultrasonography. Because of increasing plasma levels of norepinephrine and dopamine, the diagnosis of malignant pheochromocytoma with rib metastasis was made. Histologically, chromogranin staining was positive in each resected specimen. The retroperitoneal tumor was separate from the right adrenal gland. We report a rare case of malignant paraganglioma which was asymptomatic in spite of increasing plasma levels of catecholamines.

Aged↗

[131I]metaiodobenzylguanidine treatment of a malignant paraganglioma.

The bone metastases of a malignant, non-secreting paraganglioma were treated with [131I]metaiodobenzylguanidine (131I-MIBG) over a 10-year period. Initial treatment (131I-MIBG: 9.6 GBq) resulted in a decrease in the number of bone metastases from 16 to 2. At three years, a relapse with primary tumor regrowth and liver metastasis was again treated with 131I-MIBG (22.2 GBq). A decrease in the number of bone metastases and MIBG uptake was again observed.

3-Iodobenzylguanidine↗