Extra-adrenal Pheochromocytoma Research Articles

Ectopic Cushing's syndrome due to retroperitoneal ACTH-producing paragangliomas.

Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup.

Ikerpárban előforduló örökletes phaeochromocytoma

Phaeochromocytoma is a tumor of the catecholamine-producing cells of the adrenal gland. Extraadrenal phaeochromocytomas are frequently called paragangliomas. The majority of phaeochromocytomas are sporadic, however, about 25-30% are caused by genetic mutation. These tumor are frequently referred as hereditary phaeochromocytomas/paragangliomas. Their incidence increases continuously which can be attributed to availability of genetic examination and to the discovery of novel genes. The 47-year-old female patient underwent abdominal computed tomography which revealed bilateral adrenal gland enlargement. Abdominal magnetic resonance imaging, the 131-I- metaiodobenzylguanidine scintigraphy, urinary catecholamines and serum chomogranin A measurements confirmed the diagnosis of bilateral phaeochromocytomas. The genetically identical twin sister of the patient was also diagnosed with hormonally active bilateral phaechromocytoma, suggesting the genetic origin of phaeochromocytoma. Mutation screening confirmed a germline mutation of the transmembrane protein 127 tumorsupressor gene in both patients. Both patients underwent cortical-sparing adrenalectomy. The adrenal gland with the larger tumor was totally resected, while in the opposite side only the tumor was resected and a small part of the cortex was saved. After the operation urinary catecholamines and serum chromogranin A returned to normal in both patients. Adrenocortical deficiency was absent in the first patient, but her sister developed adrenal insufficiency requiring glucocorticoid replacement. To the best of the authors' knowledge phaeochromocytoma affecting twins has never been described earlier. Genetic examination performed in siblings confirmed the presence of the mutant gene through four generations. Orv. Hetil., 2016, 157(33), 1326-1330.

Pheochromocytoma of the pancreas: A report of three cases and a literature review.

Pheochromocytoma is primarily derived from the adrenal medulla. The majority of extra-adrenal pheochromocytoma cases occur in the superior para-aortic region and para-adrenal area. However, pheochromocytoma originating from the pancreas is rare. The present study reports the cases of three patients who had no history of hypertension but were post-operatively diagnosed with pheochromocytoma located in the pancreas. Of the three patients, two were admitted to hospital due to abdominal pain, and imaging examinations revealed a soft-tissue lesion in the head of pancreas. Local resection of the pancreatic tumor was successfully performed and a diagnosis of pheochromocytoma derived from the pancreas was subsequently made by pathologists. The third patient was admitted to hospital for surgical treatment due to the identification of a continuously growing lesion in the tail of pancreas during physical examinations. Distal resection of the pancreas was stopped during surgery when the patient's blood pressure and heart rate suddenly increased to 180/110 mmHg and 140 beats/min, respectively. Due to a marked rise in noradrenaline and adrenaline levels in the blood subsequent to surgery, the patient was diagnosed with pancreatic pheochromocytoma. The present study additionally reviewed the associated literature concerning pheochromocytoma in order to improve the understanding of this rare clinical phenomenon. The aim of the present study is to highlight to surgeons that although patients may not present with typical clinical manifestations due to the non-functional status of the tumor, undiagnosed pheochromocytoma of the pancreas should be considered when surgeons observe an unexpected hypertensive crisis during pancreatic tumor surgery.

A rare presentation of clinically intractable hypertension: Pancreatic paraganglioma

Summary Paraganglioma is a rare extra-adrenal pheochromocytoma which originates from chromaffin cells within the ganglia of the sympathetic trunk and of the celiac, renal, suprarenal, and hypogastric plexuses. Pancreatic paragangliomas are rarer still. And even then, paragangliomas are mostly reported to be nonfunctional. We report a case of a 64-year-old woman with underlying disease of hypertension who presented with biliary colic. Contrast-enhanced computer tomography showed an enhancing mass in the uncinate process of the pancreas. Pylorus-sparing Whipple procedure was performed for complete tumor excision. Hypertensive crisis developed after Whipple, which improved after continuous intravenous nicardipine infusion. Pathology revealed a paraganglioma. A 24-h catecholamine urine test showed increased norepinephrine and vanillylmandelic acid level. Functional paraganglioma was diagnosed.

Retroperitoneal ekstraadrenal paraganglioma: Olgu sunumu

Paraganglioma (PG), also known as extra-adrenal pheochromocytoma, is a rare neuroectodermal tumor. The incidence of extra-adrenal paraganglioma is between 00.1-0.1%. Paragangliomas originating from the retroperitoneum are generally functional. 40-50% of them are malignant and they generally have a slow progression. Surgical resection and radiotherapy are the preferred methods for the local treatment of these tumors. A case with a tumor localized in the retroperitoneal space that was totally resected by surgical method was evaluated.

Case report of extra -adrenal pheochromocytoma

The first reported case of pheochromocytoma is attributed to Frankel in 1886. Pheochromocytoma is a neuroendocrine tumour that usually develops ahead of the chromaffin cells of the adrenal medulla. Embryonic rests of chromaffin tissue arising from sympathetic plexus within bladder wall are believed to be the source of these tumors. Extra-adrenal pheochromocytomas usually are located within the abdomen in association with the celiac, superior mesenteric, inferior mesenteric ganglia and Organ of Zuckerkandl (chromaffin body derived from neural crest located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery).

Paraganglioma masquerading as acute myocardial infarction and cardiogenic shock

Paragangliomas, extra-adrenal pheochromocytomas, are rare catecholamine-secreting tumor. A 34-year-old lady admitted with diagnosis of ST elevation acute myocardial infarction with cardiogenic shock. Left ventricular function, severely depressed, returned to normal after initial stabilization. Coronary angiogram was normal. A para-aortic paraganglioma was diagnosed during the patient's work-up with biochemical studies, computed tomography of abdomen and functional radioisotopes imaging and was eventually surgically resected.This case shows that acute myocardial infarction may be the initial manifestation of these neuroendocrine tumors. Hypertensive emergency, much less elevated blood pressure may not be present at time of presentation.

妊娠中に発見された褐色細胞腫の2症例

(Introduction) A pheochromocytoma diagnosed during pregnancy is rare, and also its fetal and maternal mortality is high if undiagnosed. We report two cases with successful pregnancy outcome with pheochromocytoma. (Case 1) A 31-years-old previously healthy woman presented at 19 week period of gestation with high blood pressure. High urine catecholamine level and a left adrenal mass recognized by magnetic resonance imaging (MRI) confirmed adrenal pheochromocytoma. The patient had good blood pressure control after we started doxazosin 4 mg daily, and Caesarean section was performed at 35 week period of gestation. Two months after delivery, laparoscopic adrenalectomy was performed and she was discharged in the absence of hypertension. (Case 2) A 31-years-old previously healthy woman presented at 29 week period of gestation with exacerbation of headache and palpitation. High urine catecholamine level and a mass recognized close to the inferior vena cava by MRI confirmed extraadrenal pheochromocytoma. The patient had good blood pressure after we started doxazosin 4 mg daily and labetalol hydrochloride 200 mg daily. Caesarean section was performed at 35 week period of gestation. One month after delivery, laparotomic resection of the tumor was performed. Seven years later, hypertension reoccurred and there was an extraadrenal pheochromocytoma recurrence. We had a laparotomic resection for the recurrent tumor, and now she is in the absence of hypertension. (Conclusion) Pheochromocytoma should be considered as a differential diagnosis in a case of high blood pressure occurred during pregnancy. Early detection and appropriate treatment is crucial to reduce fetal and maternal mortality.

Bilateral exudative retinal detachment in a case of paraganglioma

Bilateral exudative retinal detachment due to extra-adrenal pheochromocytomas in a pediatric age group has been rarely reported. We report a case of a 12-year-old female child with exudative retinal detachment and hypertensive retinopathy changes secondary to hypertensive emergency due to extra-adrenal pheochromocytoma. The patient presented with sudden painless diminution of vision in both eyes since 15 days. Fundus examination showed exudative retinal detachment with Grade 4 hypertensive retinochoroidopathy changes. Supine blood pressure of 190/140 mm of Hg was recorded. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a heterogeneously enhancing right para-aortic mass likely representing extra-adrenal pheochromocytoma. Hypertensive ocular damage represents target organ damage in humans with systemic arterial hypertension. Fundus examination is a rapid and low cost modality for detecting previously existing and new onset vascular damage. Prompt recognition and timely referral of such cases have prodigious implications for both ocular and general health of a person.

Atypical Presentation of Vesical Paraganglioma

Paragangliomas are extra adrenal pheochromocytoma arising from the chromaffin tissue of sympathetic nervous system. It accounts for 15% of all the pheochromocytomas. Paraganglioma of urinary bladder is an extremely rare entity and difficult to diagnose in absence of typical symptoms of catecholamines excess. They usually have typical symptoms of hypertensive episodes specifically during voiding or sexual activity. Calcification within paraganglioma is even rare entity and often mimics vesical calculus. Partial cystectomy remains the treatment of choice and confirmation of diagnosis requires immunohistochemical staining. Here we report a case of calcified paraganglioma of urinary bladder with atypical presentation and review of pertinent literature.

Neurofibromatosis Type 1 With Ampullary Somatostatinoma, Small Bowel GISTs, and Extraadrenal Composite Pheochromocytoma-Ganglioneuroma

Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary tumor syndrome. Increased risk of GI tumors including neurofibromas, GISTs, and periampullary carcinoids is reported. Periampullary somatostatinomas as well as true neurogenic tumors including extra-adrenal pheochromocytomas (paragangliomas) and ganglioneuromas have also been reported. This case study reviews a patient with NF1 and multiple intra-abdominal neoplasms. Case: A 32 year old male presented with six months of jaundice, dark urine, and weight loss. CT showed dilated intrahepatic ducts, and elevated bilirubin and ALP. ERCP with biopsy of the ampulla was positive for invasive malignancy with neuroendocrine differentiation. Pancreaticoduodenectomy showed lowgrade somatostatinoma of the ampulla and an incidental duodenal GIST. CT scan at year two found an enlarging upper abdominal mass concerning for nodal metastasis. Ex-lap removal found a paraganglioma in association with ganglioneuroma. Given the pathology, the diagnosis of NF1 was confirmed with mutation analysis showing a truncating mutation in the NF1 gene. His exam showed multiple café au lait spots on the trunk and upper extremities, multiple neurofibromas, and axillary freckling. Years later he had a progressive right adrenal nodule and elevated plasma/urine metanephrines. Resection of a paracaval functional paraganglioma and partial right adrenalectomy with postoperative XRT to the right adrenal bed was performed. One year later, CT and OctreoScan showed a tumor corresponding with nodularity in the jejunal limb and pancreaticobiliary anastomosis. Surgery confirmed proximal and distal jejunal GISTs. Systemic octreotide treatment is under consideration. Discussion: Alterations in the NF1 gene (tumor suppressor) encodes for the protein neurofibromin. Unlike sporadic GISTs, NF1 associated GISTs do not usually harbor c-KIT or PDGFRA mutations. Pheochromocytomas reportedly affect 0.1-6% of NF1 patients. 3/14 reported cases of composite pheochromocytoma-ganglioneuroma were associated with NF1. The combination of pheochromocytoma and GIST has been described in 14 NF1 case. Periampullary tumors in NF1 patients are most commonly carcinoid, but most do not develop somatostatinoma syndrome. To our knowledge this if the first case of a patient with NF1 diagnosed with ampullary somatostatinoma, small bowel GISTs, and extra-adrenal composite pheochromocytoma-ganglioneuroma occurring together.Figure 1

Primary malignant hepatic paraganglioma mimicking liver tumor: A case report.

An extra-adrenal pheochromocytoma is also known as a paraganglioma. The present study reported the case of a 47-year-old female patient with an extremely rare primary nonfunctioning hepatic paraganglioma without any clinical signs and symptoms. A computed tomography scan of the entire abdomen region revealed a hyper-enhanced, well-marginated round mass located in segment 3 of the liver. A preoperative diagnosis of hepatocellular carcinoma was established and a left lateral hepatic lobectomy was performed. During the removal of the mass, the patient experienced extreme fluctuations in blood pressure. Analysis of hepatic and peripheral venous blood test results confirmed the increase of noradrenaline secretion. Postoperatively, the patient's blood pressure and catecholamine level returned to the normal range. However, three years after surgery, a plasma catecholamine examination revealed a high noradrenaline level. Abdominal magnetic resonance imaging scans revealed two metastases, located in the spleen and below the right posterior lobe of the liver, which were identified as malignant paragangliomas. Therefore, the patient was diagnosed with primary malignant hepatic paraganglioma recurrence three years after hepatic resection.

Non-functioning paraganglioma occurring in the urinary bladder: A case report and review of the literature.

Paraganglioma, also termed extra-adrenal pheochromocytoma, may be observed at the base of the skull and neck as well as within the mediastinum and periaortic region. The clinical symptoms of paraganglioma of the urinary bladder include intermittent hypertensive attacks, micturition, headaches and palpitations due to high catecholamine levels; these types of paragangliomas are extremely rare. However, certain bladder paragangliomas do not present with any of these symptoms; thus, surgeons are not pre-warned on how to prepare for their resection. Surgery to remove pheochromocytomas may therefore result in an intraoperative hypertensive crisis and increase the mortality rate. This infrequent type of paraganglioma is only recognized through histological examination following surgery. The current study reports the case of a 61-year-old male with urinary bladder paraganglioma. The patient presented with hypertension, which was controlled to within a normal range using diovan and norvasc treatment; in addition, the patient's blood pressure was not altered with urination or postural changes. The patient was not administered an α-blocking agent or a blood volume expander prior to the surgery, and during the partial cystectomy no hypertensive crisis occurred.

Retroperitoneal composite pheochromocytoma/paraganglioma complicating pregnancy: a rare case report

Pheochromocytoma is a tumor of adrenal medulla. Pheochromocytoma arising outside the adrenal medulla is commonly called paraganglioma. These tumours arise from paraganglia of the autonomic nervous system, most commonly occurring in head and neck region, and much less frequently in the retroperitoneum. But when they occur below the diaphragm in the organ of zuckerkandl or retroperotoneum, they are also called as extra adrenal pheochromocytoma. Composite pheochromocytoma/paraganglioma is a tumor composed of pheochromocytoma/ paraganglioma along with another tumor of neural crest origin composed of either a ganglio-neuroma/ ganglio- neuroblastoma /neuroblastoma or schwannoma. They are mostly located in the adrenal gland, while extra adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the literature. Composite tumors of the adrenal medulla are rare tumors accounting for less than 3% of all sympatho-adrenal tumors. We report a rare case of retroperitoneal extra adrenal composite pheochromocytoma in a 19 year old female with a history of 3 months amenorrhoea.

Anaesthetic management of a case of adrenal and extra-adrenal phaeochromocytoma for preoperative embolisation.

Sir, A 40-year-old asymptomatic patient was found to have a blood pressure (BP) of 170/110 mmHg during routine medical examination. His systemic examination was essentially normal and routine investigations did not reveal any abnormality. However, his 24 h urine normetanephrine levels were elevated to 1977.37 μg/g of creatinine (normal: 46-256 μg/g). Magnetic resonance imaging of the abdomen showed right adrenal, superior mesenteric and left paraaortic phaeochromocytoma. Biochemical screening for multiple endocrine neoplastia was negative. Antihypertensive drugs were used to manage hypertension and to prepare the patient for surgery; the patient was started on oral amlodipine 5 mg once daily, prazosin 5 mg once daily and carvedilol 25 mg twice daily and continued for 4 months while embolisation of tumour was planned to reduce vascularity before surgery. The technique of anaesthesia decided upon was sedation with monitored anaesthesia care. On the day of the procedure, patient was shifted to interventional radiology centre. A valid informed consent was obtained. All emergency equipments and drugs, including those required for general anaesthesia were checked and kept ready. Standard monitoring including pulse oximetry (SpO2), electrocardiogram (ECG) and non-invasive BP were applied. Baseline BP recorded was 120/80 mmHg with heart rate (HR) 64/min and ECG monitor showed normal sinus rhythm. Vasoactive drugs like sodium nitroprusside (SNP), nitroglycerin (NTG), noradrenaline and dopamine were kept ready and loaded in syringes in infusion pumps. A large bore 16 gauge intravenous (IV) cannula was inserted in left upper limb under local anaesthesia. Premedication consisted of ondansetron 4 mg, midazolam 2 mg and fentanyl 100 μg, all IV. Oxygen was started at 5 L/min through a facemask. The right radial artery was cannulated using a 20 gauge arterial catheter, and right subclavian vein was cannulated with a 7.5 French triple-lumen central venous catheter under strict asepsis under local anaesthesia. A baseline infusion of SNP was started at 0.3 μg/kg/min. Right femoral artery was canulated by the radiologist under local anaesthesia using 5 F femoral sheath. Flush aortogram was done and selective catheterisation and polyvinyl alcohol embolisation done in the right middle and inferior suprarenal and bilateral lumbar arteries at L1 level. Coils were placed in branches of left lumbar and paravertebral branch of middle suprarenal artery. After embolisation of main feeding vessel of tumour, BP rose to 172/110 mmHg with a drop in HR to 44/min. SNP infusion was increased stepwise till 1.2 μg/kg/min and NTG was started at 1.5 μg/kg/min. Both infusions were carefully titrated to maintain BP of 140/90 mmHg. Blood sugar levels were checked and were found to be 46 mg/dl and 50 ml of 50% dextrose was administered. The blood sugar value was checked 1/2 hourly thereafter during the procedure, which were normal. Arterial blood gas analyses obtained during the procedure were normal. Intraoperative central venous pressure (CVP) was maintained at 10 cm of water. Ringer's lactate, 2.5 L was infused during the procedure. Total duration of procedure was 3 h. Once procedure was over, patient was shifted to the Intensive Care Unit (ICU) under close monitoring where SNP and NTG were tapered down slowly. Patient was then closely monitored for hypotension and hypoglycaemia. Throughout ICU stay BP remained 130/90 mmHg with a HR of 80–90/min. He was taken up for laparotomic excision of the tumour next day. Previous reports have described the use of transcatheter arterial embolisation (TAE) in patients with phaeochromocytoma. Pre-operative embolisation in both adrenal and extra-adrenal location[1] has been shown to reduce the risk of catecholamines release during manipulation of the tumour at the time of surgery. Treatment of malignant phaeochromocytoma[2] or its metastatic lesions,[3] particularly liver metastasis has been performed too with TAE. Marked decrease in circulating levels of catecholamines following TAE may be associated with lower risks of haemodynamic fluctuations during surgical manipulation of the tumour and in the post-operative period. Hence, TAE may have an important role in the pre-operative management of large adrenal and extra-adrenal pheochromocytomas.[4,5] There are many anaesthetic choices available for this procedure. It can be performed under sedation with monitored anaesthesia care as in our case.[6] It can also be performed using supraglottic airway devices with general anaesthesia with spontaneous ventilation. Epidural anaesthesia can alleviate ischaemic pain after TAE of tumour, after ensuring stringent haemodynamic monitoring. Irrespective of the type of technique, it is useful to have invasive monitoring, including intra-aortic balloon pump and CVP so as to have constant haemodynamic monitoring, which is of prime importance in such a case.

Laparoscopic Resection of a Large Functional Paraganglioma of the Organ of Zuckerkandl

Background: Paragangliomas of the organ of Zuckerkandl (PZ) are rare extra-adrenal pheochromocytomas, and according to a recent systematic review,1 only 135 cases have been reported so far. Detection and appropriate treatment of these tumors is important because PZ have a mortality rate of 43% when presenting acutely. While the utility of the laparoscopic approach in adrenal pheochromocytoma is well established, information on the safety and effectiveness of this approach in PZ is still very sparse. Materials and Methods: Here, we describe the successful laparoscopic resection of a large functional PZ in a 20-year-old woman with infrequent, but severe, hypertensive crises. Urinary metanephrines were three to five times higher than the upper limit of the normal range, and a CT scan revealed a large spherical (10 cm in diameter) abdominal mass, overlying the vena cava and the aorta, from the subpancreatic area to iliac bifurcation. We excised the tumor by the transperitoneal laparoscopic approach. The patient has been placed in the flank position, elevated at ~60°. Trocars were placed following the classical right nephrectomy scheme, with the camera in a lower position to allow a better visualization of the interaortocaval groove. Laparoscopy produced excellent exposure, allowing proper identification of the tumor origin and its relation to adjacent organs. Initially, we did a Kocher maneuver to mobilize the whole cefalo-duodenal-pancreatic bloc and the third and fourth part of duodenum to totally free the anterior vena cava and aorta. Next, we begin preparing the tumor, by leaving laterally the right ureter and clipping large vessels arising from the right iliac axis. We freed the interaortocaval groove by clipping and sectioning two or three retrocaval vessels. Then, we resected the inferior mesenteric artery and divided the blood vessels coming, directly, from the aorta and vena cava. At the end, full resection was completed uneventfully, and the tumor was retrieved in a bag through an enlarged incision of the periumbilical port. Results: Postoperative course was uneventful, and the patient was symptom-free and normotensive. After 1 month, urinary metanephrine dosage was in the normal range. Discussion: Available experience, although limited, suggests that laparoscopic exploration of suspected extra-adrenal pheochromocytoma is an appropriate approach to estimate the extension of the tumor and its resectability. Conclusions: The postoperative follow-up showed the success of the described surgical approach. Appropriate patient preparation and intraoperative and postoperative monitoring appear critical for the successful clinical management of Zuckerkandl paragangliomas. No competing financial interests exist. Running time: 6 mins, 29 secs

A rare case of extra-adrenal pheochromocytoma localized to the ovary and detected via abdominal computed tomography angiography.

Extra-adrenal pheochromocytomas are rare tumors that originate from the chromaffin tissue of the sympathetic nervous system. Ovarian extra-adrenal pheochromocytoma is even more rare. The present study reports a rare case of an extra-adrenal pheochromocytoma that was localized to the right ovary, but was gynecologically asymptomatic. Computed tomography angiography (CTA) detected the tumor and indicated that it was well defined, highly vascularized and obtained its blood supply from the right ovarian artery. This is the second case of ovarian extra-adrenal pheochromocytoma reported in the literature, and the first description of the CTA manifestations in the ovary. Gynecologists and radiologists should consider the possibility that an ovarian mass could be an extra-adrenal pheochromocytoma, which would allow time to prepare appropriately for the surgical removal of the mass.

PARAGANGLIOMA E ANGIOEDEMA

Paragangliomas ou Feocromocitomas extra-suprarrenais são tumores raros derivados do tecido cromafim que tendem a ocorrer esporadicamente ou no contexto de doenças genéticas complexas. São tumores com apresentação clínica variada, dependente do seu perfil secretório ou do efeito de massa determinado pela neoplasia. Sintomas dermatológicos são raros, tendo sido classificados como agudos/paroxísticos ou crónicos. É apresentado caso de uma Eurocaucasiana de 40 anos de idade que referia, nos últimos 2 anos, episódios auto-limitados de angioedema, diarreia, toracalgia, hiper ou hipotensão arterial, dispneia e ansiedade. Estudos laboratoriais e, posteriormente, avaliação imagiológica e exame patológico permitiram identificar um paraganglioma drenante para a veia renal esquerda, o qual foi extirpado com sucesso. Durante a cirurgia, a manipulação do tumor desencadeou uma nova crise grave, a qual exigiu, com carácter de emergência, a instituição de medidas de suporte avançado de vida. A recuperação decorreu sem incidentes, tendo-se verificado uma rápida regularização dos valores laboratoriais a par de uma remissão clínica completa, sem novos episódios verificados durante 17 anos.

Preliminary experience of the robot-assisted laparoscopic excision of a retroperitoneal mass: A case report.

The aim of the present study was to report the initial clinical experience of adopting the da Vinci Surgical System (Intuitive Surgical, Inc., Sunnyvale, CA, USA) to perform a retroperitoneal tumor resection. The patient was a 56-year-old female who presented with a five-year history of hypertension. Abdominal dynamic computed tomography (CT) and positron emission tomography-CT scans revealed a mass measuring ~6 cm in diameter that was located anterior to the abdominal aorta, and between the abdominal aorta and the inferior vena cava (at the level of the third lumbar vertebra). The tumor was excised via a five-port, robot-assisted, transperitoneal laparoscopic approach. Careful dissection of the tumor away from the abdominal aorta and the inferior vena cava was accomplished without resulting in major vascular injury. There were no complications and the patient was discharged in a good condition on the eleventh postoperative day. Pathological analysis of a tumor specimen demonstrated a benign pheochromocytoma (PHEO). During the three-month follow-up, no recurrence was identified through CT scans or measurement of the patient’s endocrine hormone levels. Thus, the da Vinci robot-assisted laparoscopic system may be safely employed in the treatment of extra-adrenal PHEOs that occur in difficult locations for which a laparoscopic surgical excision may be challenging.

Catecholamine-secreting carotid body paraganglioma: successful preoperative control of hypertension and clinical symptoms using high-dose long-acting octreotide.

SummaryA 48-year-old hypertensive and diabetic patient presented with a 10-year history of progressive right facial pain, tinnitus, hearing loss, sweating, and palpitations. Investigations revealed a 5.6 cm vascular tumor at the carotid bifurcation. Her blood pressure (BP) was 170/110, on lisinopril 20 mg od and amlodipine 10 mg od and 100 U of insulin daily. A catecholamine-secreting carotid body paraganglioma (CSCBP) was suspected; the diagnosis was confirmed biochemically by determining plasma norepinephrine (NE) level, 89 000 pmol/l, and chromogranin A (CgA) level, 279 μg/l. Meta-iodobenzylguanidine and octreotide scanning confirmed a single tumor in the neck. A week after giving the patient a trial of octreotide 100 μg 8 h, the NE level dropped progressively from 50 000 to 25 000 pmol/l and CgA from 279 to 25 μg/l. Treatment was therefore continued with labetalol 200 mg twice daily (bid) and long-acting octreotide-LA initially using 40 mg/month and later increasing to 80 mg/month. On this dose and with a reduced labetalol intake of 100 mg bid, BP was maintained at 130/70 and her symptoms resolved completely. CgA levels returned to normal in the first week and these were maintained throughout the 3 month treatment period. During tumor resection, there were minimal BP fluctuations during the 10 h procedure. We conclude that short-term high-dose octreotide-LA might prove valuable in the preoperative management of catecholamine-secreting tumors. To the best of our knowledge, this is the first report on the successful use of octreotide in a CSCBP.Learning points The value of octreotide scanning in the localization of extra-adrenal pheochromocytoma.Control of catecholamine secretion using high-dose octreotide.This is a report of a rare cause of secondary diabetes and hypertension.