Case Report

Retroperitoneal Paraganglioma: Clinical Presentation and Treatment Outcomes in Two Patients.

¹García Marchiñena Patricio, ¹Jurado Alberto, ¹Uria Luis, ¹Costabel José, ²Mariana Isola, ¹Damia Oscar, ¹Gueglio Guillermo.

¹ Urology Department, Hospital Italiano de Buenos Aires. ² Pathology Department, Hospital Italiano de Buenos Aires.

Submitted Tuesday, July 16, 2013

Accepted Tuesday, September 10, 2013

Published Friday, October 18, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction

Paragangliomas are rare neuroendocrine tumors that arise from extra-adrenal autonomic ganglia. The aim of this paper is to analyze the presentation, diagnosis and treatment outcomes of two patients with retroperitoneal paraganglioma treated in our hospital.

Case Presentation

The first case is a 34 year old female, ASA status 1, who underwent elective surgery for removal of retroperitoneal tumor. Laparoscopic surgical resection was decided. Minimal tumor manipulation was followed with severe hypertension. Due to high suspicion of the presence of a catecholamine-secreting tumor, intravenous antihypertensive drugs were given. The patient coursed with hypotension and acute pulmonary edema after the surgery. Transthoracic echocardiogram showed apical ballooning with impaired ejection fraction of the left ventricle. After 3 days in Cardiac Care Unit the patient was discharged. The second case is a 25 years old male, with large history of refractory hypertension who was admitted in our hospital with sweating, palpitations, dizziness and hypertension. Catecholamine dosage was performed in plasma and urine and the results were inconclusive. The CT scan showed a right hypoplastic kidney and a 2.5 x 1.9 cm nodular mass located between his left renal artery and vein. Because of the high suspicion of paraganglioma we decided the tumor excision. The patient recovered without complications, and was discharged 4 days after surgery. Final surgical pathology demonstrated in both cases a paraganglioma.

Conclusions

The paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively.

Key words

Extra-adrenal pheochromocytoma, Retroperitoneal mass, diagnosis.

Introduction

Paragangliomas are rare neuroendocrine tumors that arise from extra-adrenal autonomic ganglia (sympathetic or parasympathetic). They are closely related to pheochromocytomas (also called intra-adrenal paraganglioma) and are histologically indistinguibles [1]. Most of these tumors are hormonally active (up to 60 percent) and have a high incidence of malignancy (13 to 26 percent) [2]. The aim of this paper is to analyze the presentation, diagnosis and treatment outcomes of two patients with retroperitoneal paraganglioma located in the renal hilium treated in our hospital.

Case report

The first case is a 34 year old female, American Society of Anesthesiologist (ASA) status 1, underwent elective surgery for removal of retroperitoneal tumor. Our patient have no important clinical history, ande the tumor was discovered during a routine examination. Before the surgery, she underwent a percutaneous biopsy of the lesion, which courses without complications, reporting the pathological study insufficient material. The CT scan showed a 6x4x 3.5 cm latero aortic solid mass which contacts with left renal hilum. She underwent a percutaneous biopsy of the lesion, which courses without complications, reporting the pathological study insufficient material. With a diagnosis of retroperitoneal mass of unknown origin, the laparoscopic surgical resection was decided. Preoperative findings, electrocardiogram and blood pressure measured were normal. Retroperitoneoscopic access was performed using previously described technique3. Minimal tumor manipulation was followed with severe hypertension measuring 320/110 mmHg and tachycardia up to 120 beats/minute. Surgery was temporally interrupted, until hemodynamically compensation of the patient. Due to high suspicion of the presence of a catecholamine-secreting tumor, intravenous dose of propranolol and phentolamine was given. Once the tumor was excised the patient coursed with hipotension and acute pulmonary edema. Transthoracic echocardiogram showed hypokinesia of medial and apical segments (apical ballooning) with moderately impaired ejection fraction of the left ventricle. A coronary angiography was performed post operatively to rule out coronary lesions. The patient improved with decreased inotropic requirements and was discharged at third day of the surgery. The pathological diagnosis (see Figure 1)was retroperitoneal paraganglioma with negative surgical margins. We performed a positron emission tomography (PET / CT FDG) whole body, for staging, with no evidence in this study associated injuries. One year after surgery the patient is free of disease and symptoms.

Figure 1. Pathology: paraganglioma. Macroscopy (A) tumor nodule brownish lobulated surface and soft consistency of 60 x 45 x 31 mm. Microscopy (B) tumor proliferation of spindle cells with ovoid nuclei chromatin stippling "salt and pepper" and large finely granular basophilic cytoplasm.

The second case is a 25 years old male, with large history of hypertension refractory to medical treatment who was admitted in our hospital with sweating, palpitations, dizziness and high blood pressure records 180/120mmHg. Antihypertensive treatment was begun with intravenous nitroprusside. Once the patient was hemodynamically stable, oral antihypertensive treatment with four drugs started (doxazosin, propranolol, nebivolol and nifedipine). Catecholamine dosage was performed in plasma and urine and the results were inconclusive. The CT scan showed a right hypoplastic kidney and a 2.5 x 1.9 cm nodular mass located between his left renal artery and vein (see Figure 2).

Figure 2. CT scan shows an hypertrophied left kidney. (A) Just below the renal artery a nodular formation. (B) caudal extension laterally to the aorta (B).

The metaiodobenzylguanidine scintigraphy was normal without uptake at the level of the lesion. We decided to make the tumor excision by open surgery through a left subcostal incision. The patient recovered without complications during both intra-and postoperatively. Discharge was given 4 days after surgery with 3 antihypertensive drugs. Final surgical pathology demonstrated 2,2x1,5x0,4 cm paraganglioma.

Discussion

Pheochromocytomas are rare tumors usually arising from the adrenal medulla. In 14% to 25% of cases, they may arise from embryological adrenal remnants referred as extra-adrenal paragangliomas [4]. The estimated annual incidence of pheochromocytoma / paraganglioma is 0.8 per 100,000 people per year. Most paragangliomas are diagnosed in the third to fifth decades of life. Patients with hereditary paragangliomas tend to develop disease a decade earlier than do those with sporadic disease. The male to female ratio is equal in patients with hereditary paraganglioma, while sporadic tumors are much more common in women (71% versus 29%) [5]. In addition to variability in relation to location, paragangliomas refute the "10% rule" applied to pheochromocytomas, since about 26% of paragangliomas are multiple, 30-50% are associated with a hereditary syndrome and about 20% of paragangliomas secreting (abdominal and mediastinal) are malignant [6]. The four types of presentation that describes, in descending order of frequency are: mass effect (usually the head and neck), hypersecretion of catecholamines, asymptomatic and an incidental finding on imaging study [7]. Biochemical diagnosis of paraganglioma is done by measurements fractionated metanephrines and catecholamines in urine and / or plasma. Imaging studies provide information about the location of the tumor, vascularization and relationship to adjacent organs. The methods used are computed tomography (CT) and magnetic resonance imaging (MRI). If the CT or abdominal and pelvic MRI are negative in the presence of clinical and biochemical evidence of a catecholamine-secreting tumor, the next step is to apply functional studies with radioisotopes images using metaiodobenzylguanidine (MIBG) or 18F-fluoro-2-deoxyglucose (FDG) -PET [8]. The differential diagnosis of an abdominal mass includes carcinomas, lymphomas and soft tissue sarcomas, including gastrointestinal stromal tumors and desmoid tumors. The definitive diagnosis is achieved at the time of resection. A biopsy (incisional or fine needle aspiration) is contraindicated in a patient with suspected paraganglioma because it can trigger a severe hypertensive crisis punctures can be confused with many different malignancies [9-10].

Once a paraganglioma is suspected, the patient should undergo surgical resection after adequate medical preparation. Surgical resection is a high-risk procedure that requires an experienced team (surgeon / anesthesiologist). Preoperative medical treatment is to control blood pressure (including the prevention of hypertensive crisis during surgery). The medical preparation with alpha and beta blockers is indicated in all preoperative patients with catecholamine-secreting tumors in order to control intraoperative blood pressure and prevent hypertensive crises. Even with modern genetic testing, most paragangliomas appear to be sporadic [11]. Paragangliomas most have been linked to inherited mutations in the genes that encode different subunits of succinate dehydrogenase (SDH). Furthermore, pheochromocytoma /paraganglioma are a component of four genetic syndromes: multiple endocrine neoplasia type 2A and 2B (MEN2), neurofibromatosis type 1 (NF1), von Hippel Lindau disease (VHL), and Carney-Stratakis dyad. Most cases of hereditary paraganglioma posted by mutations in SDHD, SDHB and SDHC, VHL and NF1 [12].Genetic screening is recommended for all patients with a diagnosis of paraganglioma. The presence of multiple paragangliomas, diagnosis in childhood, history of associated tumors, family history and the location of the head and neck should make us consider hereditary disease [13].The histologic features such as nuclear pleomorphism, necrosis, mitotic index, local invasion and can be seen in benign paragangliomas are not diagnostic of malignancy. According to the World Health Organization 2004 (WHO) the only real indicator of malignant behavior metastatic spread is [14].

Conclusion

The paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively. Is essential to rule out the existence of paraganglioma in all retroperitoneal tumors, even in asymptomatic and hemodynamically stable patients. Preoperative diagnosis helps to plan treatment and prevent complications.

Competing interests

The authors declare that there are no competing interests

Authors' Contribution

GMP: Concept and design, preparation of manuscript

JA: Concept and design, preparation of manuscript

UL: Concept and design, preparation of manuscript

CJ: Concept and design, preparation of manuscript

MI: Concept and design, preparation of manuscript

DO: Concept and design, preparation of manuscript

GG: Concept and design, preparation of manuscript

Acknowledgement

None

References

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