Retroperitoneal Paraganglioma Research Articles

An uncommon cause of high blood pressure in young people: retroperitoneal paraganglioma with vascular invasion.

An uncommon cause of high blood pressure in young people: retroperitoneal paraganglioma with vascular invasion.

Retroperitoneal Non-functioning Paraganglioma: A Case Report

Retroperitoneal paragangliomas are relatively rare endocrine tumours derived from the extra-adrenal paraganglia of the autonomic nervous system. They can be functional when they secrete catecholamines or non-functional. The diagnosis of a paraganglioma is infrequently made preoperatively unless the tumour is functional. In this paper, the authors report a new case of non-functional paraganglioma. A 54-year-old woman with a past medical history of viral hepatitis, presented with vague abdominal pain of five years\' duration. On examination, no abdominal mass was palpable and her blood pressure was 100/60 mm Hg. Abdominal computed tomography scan demonstrated a well-defined hypervascular retroperitoneal mass of heterogeneous density in the left para-aortic region measuring 3cm in diameter. As paraganglioma was suspected preoperatively, serum and urine cathecholamine levels were performed and were within normal range. Complete surgical excision of this mass was performed. Histological examination of the surgical specimen coupled with immunohistochemical study was consistent with the diagnosis of pararganglioma. Retroperitoneal paragangliomas are most commonly benign with good prognosis, but can be locally invasive and metastasize as well. The possibility for malignant transformation of paragangliomas makes surgical excision the treatment of choice.

Anesthetic management of a patient with undiagnosed paraganglioma -a case report-

Retroperitoneal paragangliomas are uncommon neuroendocrine tumors which are derived from extra-adrenal paraganglioma with various clinical signs and symptoms. Although most extra-adrenal paragangliomas are histologically benign, some tumors can synthesize and secrete excess catecholamine from the tumor. Excessive production of catecholamine causes numerous cardiovascular manifestations such as severe hypertension, cardiomyopathy, cardiac arrhythmias, and even multiorgan failure. It can lead to high risks of morbidity and mortality, especially in patients who are unrecognized or not adequately prepared. We present a female patient who was preoperatively undiagnosed of secreting retroperitoneal paraganglioma that caused cardiac tachyarrhythmia and severe intraopertive hypertension not controlled by usual antihypertensive agents. A secreting extra-adrenal paraganglioma should be included in differential diagnosis for patient who have incidentaloma and show wide range of hypertension with hemodynamic instability that is not well controlled by common antihypertensive drugs.

術中異常高血圧を契機に発見された副腎外paragangliomaの1例

61歳，女性．健診での腹部診察時に気分不良があり，心電図上，狭心症が疑われ救急搬送となった．胸腹部CTにて右乳癌と腹部大動脈左側に腫瘤を認め，腸間膜由来GISTの診断で右乳癌とともに一期的切除の方針となった．右胸筋温存乳房切除術後，開腹し，腫瘍切除操作を開始したところ著明な血圧上昇とVTの出現を認めた．一旦切除を断念した．追加検査により副腎外paragangliomaの診断を得た．後日，再手術を施行した．腹部大動脈左側の後腹膜に長径70mmの腫瘍を認めた．腫瘍に直接触れぬ様に栄養血管を結紮・切離し完全摘出した．術中，明らかな血圧上昇や不整脈の出現を認めなかった．病理組織診断はparagangliomaであった．Paragangliomaにおいて術中に診断されるのは2％程度である．術前にparagangliomaを念頭に置くべきであったと反省される点もあった．さらに術前未診断の術死率は36％との報告もあり，術前にα遮断薬を投与することが極めて重要であり，教訓的な症例として呈示する．

Retroperitoneal Paraganglioma Presenting as a Chest Pain: A Case Report

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. Definitive diagnosis is usually made with histologic findings and surgery is the treatment of choice. We report a case of a 53-year-old male who presented with chest pain and vomiting.

Adrenal Nodular Hyperplasia in Hereditary Leiomyomatosis and Renal Cell Cancer

The condition hereditary leiomyomatosis and renal cell carcinoma is characterized by cutaneous leiomyomas, uterine fibroids and aggressive papillary renal cell carcinoma. A number of our patients with hereditary leiomyomatosis and renal cell carcinoma had atypical adrenal nodules, which were further evaluated to determine whether these nodules were associated with hereditary leiomyomatosis and renal cell carcinoma. Patients with hereditary leiomyomatosis and renal cell carcinoma underwent a comprehensive clinical and genetic evaluation. We reviewed the clinical presentation, anatomical and functional imaging, endocrine evaluation, pathological examination and germline mutation testing results. Of 255 patients with hereditary leiomyomatosis and renal cell carcinoma 20 (7.8%) had primary adrenal lesions, including 4 with bilateral adrenal lesions and 4 with multiple nodules. Two patients had adrenocorticotropic hormone independent hypercortisolism. A total of 27 adrenal lesions were evaluated. The imaging characteristics of 5 of these lesions (18.5%) were not consistent with adenoma by noncontrast computerized tomography criteria. Positron emission tomography was positive in 7 of 10 cases (70%). A total of 12 nodules were surgically resected from 10 adrenal glands. Pathological examination revealed macronodular adrenal hyperplasia in all specimens. Unilateral and bilateral adrenal nodular hyperplasia was detected in a subset of patients with hereditary leiomyomatosis and renal cell carcinoma. A functional endocrine evaluation is recommended when an adrenal lesion is discovered. Imaging frequently reveals lesions that are not typical of adenomas and positron emission tomography may be positive. To date no patient has had adrenal malignancy, and active surveillance of hereditary leiomyomatosis and renal cell carcinoma adrenal nodules appears justified.

Intraoperative diagnosis of functional retroperitoneal multiple paraganglioma: A case report

Paragangliomas are extra-adrenal chromaffin tumors that arise from neuroectodermal cells of the autonomous nervous system. It is difficult to make an accurate preoperative clinical diagnosis of silent paraganglioma. The best choice of treatment is complete surgical resection. However, it is important to note that in patients with functional paragangliomas, the tumor's ability to produce catecholamines may cause abrupt changes in blood pressure. Thus, surgery may induce life-threatening complications. In the present study, we present a case of functional retroperitoneal multiple paraganglioma in a 39-year-old male patient who was diagnosed during surgery. Four years after the operation, the patient remains asymptomatic and free of disease.

Retroperitoneal paraganglioma manifesting as paralytic ileus: a case report

IntroductionRetroperitoneal neoplasms are rare and easily misdiagnosed. These tumors are often discovered incidentally during imaging studies performed for other reasons. Paragangliomas are tumors that arise from extra-adrenal medullary neural crest derivatives. They are usually located in the head and neck but can be found in various body sites, including the chest cavity, abdomen, pelvis and bladder. We report the case of a patient who had a retroperitoneal paraganglioma manifested as paralytic ileus, which is an unusual presentation of a paraganglioma.Case presentationA 63-year-old Taiwanese woman was admitted to the emergency department of our hospital with progressive abdominal fullness for two days. Her medical history included medically controlled hypertension for 10 years and type 2 diabetes mellitus. Plain abdominal radiography showed a solitary loop of the air-filled dilated small bowel. Abdominal computed tomography did not show a mechanical obstruction; however, a retroperitoneal mass was incidentally detected. Histological analysis of the mass led to a diagnosis of a paraganglioma.ConclusionsIn cases of patients with hypertension presenting with an intestinal pseudo-obstruction, a paraganglioma may be considered as a possible differential diagnosis of retroperitoneal tumors to avoid risky therapeutic procedures or medication that may produce severe adverse effects.

Criteria for Laparoscopic Advanced Surgery in Semi-Equipped Setup (CLASS): Feasibility Study Based on Institutional Experience.

Laparoscopic and Minimally invasive techniques have become a routine practice for various surgical disorders in present times. Though, advanced laparoscopic procedures are feasible they are largely restricted to fewer centers due to lack of advanced instrumentation, finances and expertise at most of them. In this context, we conducted a feasibility study to evolve definite criteria for performing advanced laparoscopic surgeries in resource restricted set-ups. We present our experience with 25 cases of advanced laparoscopic procedures using conventional laparoscopic instruments. We evaluated the clinico-investigative profile and operative details of all the patients. We classified the surgical expertise, laparoscopic instrumentation, surgical set ups and patient factors systematically to evolve the criteria for feasibility of advanced laparoscopicsurgery. Out of the 22 eligible patients for the study, various laparoscopic surgeries performed were - Fundoplication (4), Cystogastrostomy (3), Endoscopic thyroidectomy (7), Thoracoscopic Thyroidectomy (2), Adrenalectomy (5) and Retroperitoneal paraganglioma excision (1). There was no mortality and two morbidities in the form of hypercarbia and a tracheo-cutaneous fistula in 2 cases of endoscopic thyroidectomy. According to the criteria, we propose our surgical set up falls in to Grade 3, for which this criteria fits in. This study demonstrates the feasibility of advanced laparoscopic procedures in semi-equipped set-up, preferably by employing institute specific criteria of CLASS.

Gastrointestinal Stromal tumor of the stomach and retroperitoneal paraganglioma in Saudi woman, “A case report”

Gastrointestinal stromal tumor (GIST) and paraganglioma are uncommon tumors; mostly occur in a sporadic and isolated form. They are occasionally components of 3 clinical syndromes, familial paraganglioma and gastric GIST, neurofibromatosis type 1 and Carney triad. The latter does not show genetic mutations and affects young women predominantly. This article reports the occurrence of GIST and retroperitoneal paraganglioma in young Saudi woman aged 22 years. The initial clinical presentation was acute severe gastric bleeding. Gastric mass with retroperitoneal mass was discovered during surgical exploration. Histological and immunohistochemical study of both masses showed features of GIST and paraganglioma. The combination of GIST and retroperitoneal paraganglioma in the reported case may be incomplete expression of Carney triad or be coincidental occurrence.

Gastrointestinal Stromal tumor of the stomach and retroperitoneal paraganglioma in Saudi woman, “A case report”

Gastrointestinal stromal tumor (GIST) and paraganglioma are uncommon tumors; mostly occur in a sporadic and isolated form. They are occasionally components of 3 clinical syndromes, familial paraganglioma and gastric GIST, neurofibromatosis type 1 and Carney triad. The latter does not show genetic mutations and affects young women predominantly. This article reports the occurrence of GIST and retroperitoneal paraganglioma in young Saudi woman aged 22 years. The initial clinical presentation was acute severe gastric bleeding. Gastric mass with retroperitoneal mass was discovered during surgical exploration. Histological and immunohistochemical study of both masses showed features of GIST and paraganglioma. The combination of GIST and retroperitoneal paraganglioma in the reported case may be incomplete expression of Carney triad or be coincidental occurrence.

Nonfunctional Retroperitoneal Paraganglioma: A Histopathological Surprise

ABSTRACT Paragangliomas are the tumors arising from chromaffin tissue which is distributed along the autonomic nervous system. Most of these tumors secrete and store catecholamines. They can be suspected by clinical symptoms and confirmed by biochemical tests. Retroperitoneal paraganglioma mostly arise from adrenal medulla and are also known as adrenal pheochromocytoma. About 10% of abdominal paragangliomas occur at extraadrenal locations and where they are known as extraadrenal paraganglioma or pheochromocytoma (EAP). Like their adrenal counterparts, most EAPs are functional tumors as they secrete and store catecholamines. Very few EAPs do not secrete clinically detectable amount of hormones and present themselves with the pressure symptoms related to their size. These nonfunctional EAPs mimic more commonly occurring tumors of the retroperitoneum and true diagnosis is made only after microscopic examination of the tumor tissue. We had a patient who was suspected to have a pancreatic tumor but found to have a nonfunctional primary EAP completely separate from the pancreas. How to cite this article Goel N, Gupta N, Nag HH, Saran RK, Mishra PK, Agarwal AK. Nonfunctional Retroperitoneal Paraganglioma: A Histopathological Surprise. World J Endocr Surg 2012;4(2):60-62.

Paraganglioma: A cause of hypertension in a young patient

Collections of neuroendocrine cells dispersed throughout the body are known as paraganglia and the tumor arising from these paraganglia are known as paragangliomas. Paragangliomas located along side the aorta are associated with the pheochromocytoma and they secrete and store catecholamines. We reported a 29-year-old woman with episodes of headache, palpitations, dizziness and sweats, associated with hypertension. Elevated urine catecholamines were consistent with pheochromocytoma. Nuclear magnetic resonance (NMR) was performed and the symptoms were caused by associated mediastinal and retroperitoneal paragangliomas. The tumors were surgically removed. In a 3-year follow-up period the patient had two recidivate lesions, one of them was surgically removed, and for the last one a 6-month follow-up was recommended, because urine cathecholamine level was not significantly elevated and blood pressure was normal. Clinical and imaging data of patients with extra adrenal paragangliomas are not specific. Many of them may be asymptomatic even when the lesion is large, but if tumor is functional, diagnosis may be easier. Patients should be initially evaluated by determining catecholamine level, followed by computerized tomography (CT) or NMR to locate the primary lesion. Since there are no definite microscopic criteria for the distinction between benign and malignant tumors, radical excision and prolonged follow-up is necessary.

An exclusively dopamine secreting paraganglioma in the retroperitoneum: a first clinical case in Korea

Exclusively dopamine producing retroperitoneal paragangliomas are extremely rare. We have experienced the first Korean case managed successfully based on the proper evaluation. A 26-year-old female patient came to our attention after the accidental detection of an adrenal mass. She had no symptoms and denied any family history. Laboratory evaluations were normal but serum dopamine (425 ng/L) and 24-hour urine dopamine levels (1,565.3 µg/day) were elevated. She underwent laparoscopic right adrenalectomy. Histopathological diagnosis was a paraganglioma. After operation, dopamine levels in serum and 24-hour urine dropped to 0.09 ng/L and 388.4 µg/day. Dopamine producing paraganglioma elicit no clinical symptoms. Only the dopamine level is elevated in serum and 24-hour urine samples. Surgical resection without using preoperative alpha blockage is the treatment of choice. The prognosis for patients with this tumor tends to be poor because the diagnosis is usually delayed due to lack of symptoms.

術後逆行性射精をきたした後腹膜傍神経節腫の１例

術後逆行性射精をきたした後腹膜傍神経節腫の１例

小腸GISTとの鑑別が必要であった後腹膜原発paragangliomaの１例

小腸GISTとの鑑別が必要であった後腹膜原発paragangliomaの１例

Paraganglioma with intracranial metastasis: a case report and review of the literature

Paragangliomas are rare neuroendocrine tumors of neural crest origin. They are mostly benign, however; malignant tumors with aggressive behavior and distant metastasis can also occur. Intracranial involvement is extremely rare and has been sporadically reported in the literature. Here we report a case who presented with progressive neurologic deficits due to multiple intracranial lesions found to be metastasis from an occult retroperitoneal malignant paraganglioma.

Pheochromocytoma multisystem crisisをきたした後腹膜paragangliomaの1剖検例

pheochromocytoma multisystem crisisをきたした後腹膜paragangliomaの1剖検例

Sporadic Retroperitoneal Paragangliomas; A Diagnostic and Prognostic Challenge

Introduction: Sporadic retroperitoneal paragangliomas are rare tumours of the chromaffin cells of the autonomic ganglia. We report a case series of non-familial retroperitoneal paragangliomas presenting to our unit, highlighting the features aiding diagnosis, and the challenges of management and predicting long-term survival.

Long-term follow-up after en bloc resection and reconstruction of a solitary paraganglioma metastasis in the first lumbar vertebral body: a case report

IntroductionParagangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia. They metastasize infrequently. Malignancy can only be demonstrated by the presence of chromaffin tissue at sites where it usually is not present, such as bone, lung or liver, or local recurrence after total resection of a primary mass. Paragangliomas within the central nervous system are usually intradural near the conus medullaris. The metastatic spread of a retroperitoneal paraganglioma to a vertebral body is extremely rare, and there are only a few cases reported in the literature.Case presentationWe report the case of a 16-year-old Caucasian girl who had undergone resection of a retroperitoneal paraganglioma that measured 15 × 11.5 × 9.5 cm. After further staging, a solitary metastatic paraganglioma was detected in the first lumbar vertebral body. After initial chemotherapy, marginal en bloc resection and reconstruction were performed followed by radiotherapy. Histologic examination of the specimen revealed that the tumor cells did not show any response to preoperative chemotherapy, which is in line with a few other reports in the literature. Ten years after operative treatment, the patient is free of complaints, very satisfied with the result and without signs of local recurrence or distant metastases.ConclusionWe recommend en bloc spondylectomy and local radiotherapy in the treatment of solitary spinal metastatic paragangliomas.