Extra-adrenal Pheochromocytoma Research Articles

Unveiling three accessory spleens in one patient: a rare case report and literature review.

During embryogenesis, the spleen undergoes intricate developmental processes, originating from mesenchymal cells in the dorsal mesentery. An accessory spleen, a common anomaly found in autopsies and abdominal CT scans, can often be mistaken for different types of tumors. To the best of our knowledge, this is the first case in Syria documenting the occurrence of 3 accessory spleens in a patient who had previously undergone splenectomy. A 33-year-old male presented with right hypochondrium pain, sharp and radiating to the right flank, exacerbated by movement and large meals. Past medical history included mild Irritable Bowel Syndrome (IBS) and splenectomy due to a traumatic accident in childhood. On admission, vital signs were stable, with abdominal tenderness in the right upper quadrant. Laboratory investigations showed normal values. Ultrasound revealed a lobulated mass at the right adrenal gland (4.5 × 5cm) with an isoechoic to hypoechoic texture. Multi-slice computed tomography (MSCT) Scan showed multiple nodules in the right adrenal gland, regular in shape, exerting a compressive mass effect, and significant lymphadenopathy around the abdominal aorta. Elevated metanephrine levels raised suspicion of an extra-adrenal pheochromocytoma. Laparoscopic surgery was performed, revealing accessory spleens and normal adrenal tissue with no malignancy. Healthcare providers should consider accessory spleens as a differential diagnosis for masses near the adrenal glands. Multiple accessory spleens in the adrenal region can complicate cases. Accessory spleens in uncommon locations like the adrenal glands can be challenging to diagnose and manage.

8204 Rare Asymptomatic Presentation Of A Large Pheochromocytoma

Abstract Disclosure: A. Lorin: None. C.K. Persaud: None. M.H. Kemm: None. Background: Pheochromocytoma is a rare neuroendocrine tumor arising from chromaffin cells of the adrenal medulla. It is associated with excessive release of catecholamines causing symptoms of hypertension, palpitations, diaphoresis, and headaches. These symptoms are particularly prominent with larger pheochromocytomas. We present the case of a large 13 cm pheochromocytoma (PCC) without the typical associated symptoms. Clinical Case: A 45-year-old male with medical history of osteoarthritis and gastroesophageal reflux presented to his primary care doctor with 2 months of intermittent right upper quadrant abdominal pain associated with nausea. Work up with CT abdomen/pelvis found a 9.3 cm heterogeneous right adrenal mass with mass effect on the right liver lobe, superior pole of the right kidney, duodenum, and pancreatic head. Hormonal testing was notable for plasma metanephrine 257.8 pg/mL (0-88), plasma normetanephrine 12063.7 pg/mL (0.0-218.9), 24 hr urine metanephrine 159 mcg/24 hr (58-276), 24 hr urine normetanephrine 22575 mcg/24 hr (156-729), and chromogranin A 5387 mg/mL (0-101.8). Given lab evaluation, diagnosis was made of pheochromocytoma, and patient was initiated on alpha blockade with doxazosin. Patient’s blood pressure and heart rate were at goal prior to doxazosin initiation and patient needed to be decreased from 1 mg daily to 0.5 mg daily due to side effects of low blood pressure and orthostatic hypotension. Patient had an uncomplicated open right adrenalectomy with pathology showing a 13 cm pheochromocytoma with invasion through the capsule into the periadrenal adipose tissue. Genetic testing revealed no known mutation variants associated with PCC. Labs drawn two weeks postoperatively showed resolution of elevated plasma metanephrine, normetanephrine, and chromogranin A. Conclusion: While the patient had an elevated plasma metanephrine, the normetanephrine was predominantly elevated. Such a hormone profile is more common in extra-adrenal PCCs, consistent with cluster 1 associated genes. This noradrenergic phenotype is likely a reason the patient did not have typical symptoms, despite the size of his pheochromocytoma. A proposed pathophysiologic mechanism for this is the dissociation of renal medulla from the cortex given the large size of the tumor. This would lead to lower local availability of corticosteroid to potentiate the conversion of norepinephrine to epinephrine, thus decreasing likelihood of classical PCC symptoms. Despite lack of expected symptoms and an imaging initially concerning for adrenocortical carcinoma, we should recognize the potential for a large adrenal tumor to represent PCC and evaluate accordingly. Presentation: 6/1/2024

Navigating the Diagnosis and Treatment of Extra-Adrenal Pheochromocytoma Presenting with Chronic Abdominal Pain: A Clinical Conundrum

Background: Extra-adrenal pheochromocytomas, or paragangliomas, are rare neuroendocrine tumors that present diagnostic challenges due to their nonspecific symptoms and radiologic features.Objective: To report a case of extra-adrenal pheochromocytoma presenting as chronic abdominal pain and hepatic masses, emphasizing the diagnostic and therapeutic challenges.Methods: A 36-year-old male with a history of diabetes and hypertension presented with weight loss and abdominal pain. Initial evaluation included comprehensive physical examination, laboratory tests, ultrasonography, and CT imaging of the abdomen and chest. Partial hepatectomy was performed, and the diagnosis was confirmed through histopathological and immunohistochemical analysis.Results: Imaging identified three mixed echogenic hepatic masses, with the largest measuring 5.6 x 5.4 cm. Enlarged para-aortic lymph nodes were observed, the largest being 4.0 x 3.5 cm. CT chest revealed a mass on the chest wall (7.0 x 3.0 cm) with metastatic lung nodules. Immunohistochemistry confirmed paraganglioma with positivity for chromogranin A and neuron-specific enolase.Conclusion: This case underscores the importance of considering paragangliomas in differential diagnoses of atypical abdominal masses and highlights the critical role of histopathological confirmation in guiding management.

Laparoscopic Management of Adrenal and Extra-Adrenal Pheochromocytoma

Background and Purpose Laparoscopic management of extra-adrenal pheochromocytoma requires meticulous surgical procedures because of the variable anatomic position and/or proximity to major blood vessels and the potential catecholamine surge during operation. We present our experiences with laparoscopic management of extra-adrenal pheochromocytoma and compare the intraoperative hemodynamics with those during laparoscopic resection of adrenal pheochromocytoma. Patients and Methods We retrospectively reviewed the medical records of 3 patients who underwent laparoscopic management of extra-adrenal pheochromocytoma (retrocaval, n=1; interaortocaval, n=1; periadrenal, n=1) and 24 patients who underwent laparoscopic management of adrenal pheochromocytoma between June 2005 and June 2023. We also evaluated fluctuations in blood pressure (BP) reported during both surgeries. Results The tumors were successfully resected under laparoscopic guidance in both groups, and one patient had converted to open surgery. Intraoperative hypertension (BP>200 mm Hg) was observed in 1 extra-adrenal pheochromocytoma and 13 adrenal pheochromocytoma patients, whereas intraoperative hypotension (BP <80 mm Hg) was observed in 1 extra-adrenal pheochromocytoma and 12 adrenal pheochromocytoma patients. Conclusion Laparoscopy is the method of choice for extra-adrenal resection because it is feasible and reproducible with appropriate preoperative planning, similar to adrenal resection.

SAT529 An Uncommon Case Report Of A Paraaortic Paraganglioma

Abstract Disclosure: E. Askar: None. J.R. Fredrick: None. S. Anantha: None. K.G. Reddy: None. Background: Extraadrenal pheochromocytomas, also known as paragangliomas are uncommon neuroendocrine tumors that develop from sympathetic ganglia chromaffin cells. These tumors rarely cause hypertension, in less than 0.2% of patients. Although pheochromocytomas are known to be secreting catecholamines, paragangliomas are less likely to be functional. Here we report a rare case of Catecholamine secreting Paraganglioma presenting with hypertension. Clinical Case: A 28 -year-old female with past medical history of Goldenhar syndrome presented to our outpatient institution complaining of headache, palpitations and high blood pressure measurements at home with systolic blood pressure ranging between 160-200 mm Hg for a few months prior to presentation. Her only medication was propranolol for hypertension. At presentation, blood pressure was 126/72 mm Hg, heart rate was 76 beats per minute. Physical examination revealed no significant findings. Abdominal exam did not reveal any palpable masses. The patient denied any history of substance, tobacco, or alcohol abuse. No family history was found for any similar masses or hypertension. Laboratory findings were significant for elevated normetanephrines at 785 pg/mL (18 to 111) with normal metanephrines level. CT abdomen was significant for 2.5 cm enhancing left paraaortic mass separate from kidneys and adrenal glands. The patient was started on doxazosin and propranolol was switched to atenolol. After her blood pressure was adequately controlled with the alpha and beta blockade, she was referred to a tertiary facility for excision of the mass. Robotic excision of the mass was performed successfully without any significant blood pressure variations noted intraoperatively. The diagnosis of a well differentiated extraadrenal pheochromocytoma was confirmed by pathology, and the patient was referred for outpatient genetic testing and endocrinology follow up. Conclusion: This is an extremely rare case of paraganglioma given its presentation and location of the mass. The previously reported paragangliomas usually present as ER visits with severe sympathetic symptoms but it is uncommon to present as an outpatient for secondary hypertension workup. In the literature, we have found case reports of catecholamine secreting paragangliomas which guided the management of this uncommon case as mentioned above. Clinicians must be aware of this rare presentation of paraganglioma in order to properly diagnose and manage the condition.

Uncovering the Uncommon: Institutional Insights Into the Clinical and Epidemiological Characteristics of Rarer Forms of Bladder Cancer Beyond Transitional Cell Carcinoma.

Introduction Non-transitional cell carcinomas of the bladder (NTCCB) represent a significant clinical challenge due to their rarity, heterogeneity, and poor prognosis. Despite their poor prognosis, the treatment of NTCCB has historically been based on the same principles used for transitional cell carcinomas (TCCs). Our study focuses on the management of non-transitional cell carcinomas and aims to identify areas where treatment outcomes can be improved based on our institutional experience. Materials and methods A retrospective analysis of patients with NTCCB who presented at Kasturba Hospital Manipalwas conducted between 2012 to 2021. Patient data were collected, and demographic characteristics, presenting symptoms, history of other primary malignancies, comorbidities, location of the tumour, stage at presentation, histopathological subtype, site of systemic metastasis, and primary treatment given were analyzed descriptively. Median overall survival was determined by calculating the time from the initial diagnosis to the date of death. Results Among 31 patients with NTCCB, 15 (48%) presented with metastatic disease, five (16%) with locally advanced disease, and 11 (36%) with localized disease. The most common histopathological subtypes were squamous cell carcinoma and adenocarcinoma, as noted in 14 (45.2%) and 13 (41.9%) patients, respectively, followed by neuroendocrine tumours in two (6.5%), extra-adrenal phaeochromocytoma in one (3.3%), and sarcomatoid carcinoma in one (3.3%) patient, respectively. The lung was the most frequent site of systemic metastasis as noted in six (40%) patients, followed by the liver and skeletal system in three (20%) patients each, peritoneum in two (13.3%), cerebral cortex in one (6.7%), and non-regional lymph nodes in one (6.7%) patient. The primary treatment given included palliative chemotherapy in 14 (45.2%) patients, radical cystectomy with ileal conduit in 10 (32.3%), neoadjuvant chemotherapy only in four (12.9%), partial cystectomy in one (3.2%), pelvic exenteration with ileal conduit in one (3.2%), and peritoneal debulking with palliative chemotherapy in one (3.2%) patient. The overall median survival was 15 months, with a one-year survival rate of 67.4%. Conclusion NTCCB exhibits aggressive clinical behaviour and presents with nonspecific clinical features in the early stages, often leading to late diagnosis and an advanced tumour stage at presentation. Multi-institutional studies with larger patient cohorts are needed to recommend best clinical practices for early detection and optimal treatment strategies to improve patient survival.

Rare incidental presentation of urinary bladder paraganglioma and the anaesthetic challenges faced: A case report

Urinary bladder paraganglioma is an extra-adrenal pheochromocytoma. As it lacks specific symptoms and because of its rare occurrence, it presents incidentally while tumour handling. A forty-eight-year-old female who presented with nausea, vomiting, weight loss, pain abdomen and hematuria was diagnosed with a bladder mass. She was scheduled for transurethral resection of the bladder tumour. On tumour manipulation, it showed numerous fluctuations in heart rate and blood pressure, from where a differential diagnosis of urinary bladder paraganglioma was made. Histopathology revealed a zellbellan pattern of paraganglioma. This case was discharged successfully postoperatively. But if undiagnosed preoperatively, it can be a real challenge because of the fatal hypertensive crisis and life-threatening cardiopulmonary complications.

VON HIPPEL-LINDAU DISEASE, DESCRIPTION, GENETICS, MOLECULAR BASIS, CLASSIFICATION AND MANIFESTATIONS OF THE DISEASE

Introduction: Von Hippel-Lindau disease (VHL) is autosomal dominant tumor syndrome that debuts mostly in young adults , patients with this disease are linked to the triggering of various types of benign and malignant neoplasms in multiple locations, systems and organs, in particular affecting more the nervous system and other internal organs. Approximately this tumor syndrome shows an incidence rate of 1 in 36,000 live births with a penetrance greater than 90%. The molecular basis of VHL disease is the impairment of VHL protein function and the consequent clustering of hypoxia-inducible factors with subsequent consequences on cell differentiation and metabolism. Objective: to present current information related to Von Hippel-Lindau disease, description, genetics, molecular basis, classification and manifestations of the disease. Methodology: a total of 33 articles were analyzed in this review, including review and original articles, as well as clinical cases, of which 26 bibliographies were used because the other articles were not relevant to this study. The sources of information were PubMed, Google Scholar and Cochrane; the terms used to search for information in Spanish, Portuguese and English were: Von Hippel-Lindau, VHL, tumor suppressor gene, pheochromocytoma, hemangioblastomas. Results: VHL has an incidence of approximately 1 in 36,000 live births, with a penetrance of over 90%. These present tumors are initiated by inactivation of biallelic VHL and are related to pathologic activation of hypoxic gene response pathways. Conclusions: Von Hippel-Lindau disease is an autosomal dominant disorder that is generated by mutations in the VHL tumor suppressor gene. Within the field intrafamilial variation may evidence correctly shaped genotype-phenotype connections for renal cancer and pheochromocytoma risks. Visceral cysts (renal, pancreatic and epididymal) are frequent, however organ function involvement is rare. They usually occur with hemangioblastomas of the central nervous system and retina, as well as renal cancers. Unusually it includes non-functioning pancreatic endocrine cancers, adrenal and extra-adrenal pheochromocytomas, endolymphatic sac tumors, as well as head and neck paragangliomas. KEYWORDS: Von Hippel-Lindau, VHL, gene, suppressor, tumor, pheochromocytoma, hemangioblastomas

Resistant hypertension - pheochromocytoma

We report a case of a man with a 30-year history of treatment-resistant hypertension, hydropoiesis, tachycardic spells and dysgeusia. Despite repeated visits to the emergency department and work-up in an out-patient clinic, the diagnosis was unknown. Three years prior to remittance to an endocrinologist, the hypertension worsened, and he developed diabetes type-II. Further work-up revealed a 3 cm extra-adrenal pheochromocytoma, a paraganglioma. After surgical removal of the tumor, he is without medication and symptom free. Pheochromocytoma and paraganglioma are rare causes of hypertension, estimated to explain 0.1-0.6% of all cases, but nonetheless an important diagnosis to make, due to serious side effects.

PSUN11 Outcomes of Nonselective or Selective Alpha-Adrenergic Antagonist for Preoperative Management of Intra-adrenal Pheochromocytoma

Abstract Background Pheochromocytoma is a rare neoplasm of the adrenal medulla that leads to catecholamine excess. Adrenalectomy is the standard management for operative candidates. Preoperative alpha-adrenergic antagonism has been traditionally performed with the non-selective irreversible agent phenoxybenzamine. Recently, use of this agent has been limited due to availability and cost. We sought to determine whether type of preoperative antihypertensive management with nonselective alpha-adrenergic antagonist or selective alpha-1 antagonist was associated with differences in patient outcomes for those undergoing adrenalectomy. Methods We performed a retrospective review of adult patients at a single institution undergoing index adrenalectomy for initial diagnosis of intra-adrenal pheochromocytoma from 2012 through 2021. Exclusion criteria were prior adrenalectomy and extra-adrenal pheochromocytoma or paraganglioma. Clinical and demographic factors were assessed. Our primary outcome was intraoperative hemodynamic stability, secondary outcomes were postoperative hemodynamic instability, and surgical complications. Data were analyzed using Chi-squared, Fisher's exact and Mann Whitney U tests. Results During the study period, 38 patients underwent index adrenalectomy for initial diagnosis of intra-adrenal pheochromocytoma. Patient age was a mean of 57.8 years (SD 13.6) with 14 male (37%) and 34 (90%) white. On preoperative cross-sectional imaging, the mean tumor size was 3.9cm (IQR 2.5–5.2) with 15 (40%) left sided, 21 (58%) right sided and 1 (3%) bilateral with 32 (87%) undergoing minimally invasive laparoscopic or robotic assisted adrenalectomy. The mean pre-contrast density on computed tomography imaging was 35.4 Hounsfield units (SD 15). Preoperative plasma metanephrines were mean 0.65 (SD 6.7), plasma normetanephrines 11.1 (SD 14.3). For preoperative pharmacologic therapy, 16 (42%) were on phenoxybenzamine, 21 (55%) selective alpha-1 antagonist, and 1 (3%) patient on both who was excluded from comparative analysis. Nine patients overall (24%) had preoperative hypertensive crisis with no difference between the groups. There was no difference between the groups for intraoperative hemodynamic instability with 6 (38%) in the phenoxybenzamine group and 10 (48%) in the selective alpha blockade group (p = 0.74) or postoperative hemodynamic instability with 5 (31%) compared with 9 (45%) respectively (p = 0.50). Groups were also similar with regard to age, sex, body mass index, comorbidities, preoperative catecholamine levels, American Society of Anesthesiologists Physical Status classification, minimally invasive or open surgery, tumor size, estimated surgical blood loss, and postoperative complications. Follow up duration was longer for the phenoxybenzamine group at 55 months (SD 35) compared with 21 months (SD 25) for the selective alpha blockade group (p = 0.003). Conclusions Use of preoperative alpha adrenergic antagonism selective alpha-1 antagonists showed similar outcomes intraoperative and postoperatively compared with irreversible nonselective alpha-adrenergic antagonists in this cohort. Use of selective alpha-1 antagonists should be considered when nonselective agents are not accessible. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

PMON88 A Rare Case of Composite Paraganglioma in the Bladder

Abstract Background Paragangliomas are neuroendocrine tumors that arise from embryonic neural crest cells; they are essentially extra-adrenal pheochromocytomas most frequently found in the head and neck. Composite tumors are rare and consist of the presence of two histologically distinct tissues within a single tumor. Additionally rare is the presence of a composite paraganglioma in the retroperitoneum and urinary bladder, of which there are few reported cases. Case Report We present a case of a 57 year old female who was found to have an incidental pelvic mass noted to progressively increase in size over the years on subsequent imaging. The patient had a cystoscopy and retrograde pyelogram that were both unremarkable. MRI Pelvis showed a 1.8×1.8×1.9 cm rounded, well-circumscribed lesion at the left posterior lateral bladder extending exophytically from the serosa at the ureterovesicular junction. As she had associated hypertension, palpitations, and anxiety, the patient was evaluated for pheochromocytoma.Laboratory workup showed elevated plasma levels of free Metanephrines at 130 (<57 pg/mL), free Normetanephrines at 247 (<148 pg/mL), and a free Total level at 377 (<205 pg/mL). 24-hour urine collection was consistent with mild elevation of Metanephrines at 330 (90-315 mcg/24 h), normal levels of Normetanephrines at 469 (122-676 mcg/24 h), and normal levels of Total urine Metanephrines of 799 (224-832 mcg/24 h). 24-hour urine free Catecholamines showed: Epinephrine of 6 (2-24 mcg/24 h), Norepinephrine of 77 (15-100 mcg/24 h), and a Total (Epi+NE) of 83 (26-121 mcg/24 h), with Dopamine of 220 (52-480 mcg/24 h).Due to mildly elevated metanephrine levels, the patient was started on the alpha-blocker doxazosin. She ultimately underwent a laparoscopic removal of the bladder mass, with pathology reports confirming presence of a composite paraganglioma with atypical features. Additionally, further immunohistochemistry was negative for the SDHB gene mutation in the tissue samples obtained. Conclusion Composite paragangliomas of the urinary bladder are very rare. Certain genetic pathologic variants carry an increased risk for malignancy and possible metastasis; therefore, these neuroendocrine tumors usually require targeted mutation testing. At this time, the pathogenesis of composite paragangliomas is still largely unknown, with little information available about treatment options and clinical outcomes. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Metastatic Extra-Adrenal Pheochromocytoma with Single Kidney and Renal Compromise: A Case Report of Excellent Response, Tolerability, and Outcome to a Modified Regimen of 131I-mIBG, and Decision-Making between 131I-mIBG Therapy and PRRT

AbstractDetermining the choice and the goal is key element for decision-making of a systemic radionuclide therapy. They should be clearly defined in deciding and individualizing the dose and regimen. For iodine-131 metaiodobenzylguanidine (131I-mIBG) therapy, the important considerations during dose fractionation include disease burden, tumor biology, functional symptoms, and associated comorbidities, all of which are important determinants for the intent and course of treatment. Herein, we present the case of a 67-year-old elderly female with highly functional metastatic recurrent extra-adrenal pheochromocytoma (presenting 42 years after the primary surgery and 32 years following excision of pararenal recurrence) with multiple comorbidities including single kidney and borderline renal compromise, treated successfully with a relatively lower dose of 131I-mIBG (cumulative dose of 22.2 GBq in four cycles with a mean dose of 5.7 GBq per therapy cycle). The excellent tumor burden reduction, hormonal tumor marker response, and most importantly asymptomatic status could be achieved with the administered dose. On follow-up, none of the pretherapeutic parameters (including renal function) showed any further derangement compared with the baseline during next 24 months following the treatment. All cycles were well tolerated with only reversible hematological toxicity that normalized without any active intervention. The report is intended to provide some guidance for future therapeutic regimens.

Laparoscopic resection of pheochromocytoma (paraganglioma) of the organ of Zuckerkandl in a pediatric patient

Pheochromocytoma is a rare neuroendocrine tumor that secretes catecholamines. Classically located in the adrenal gland, a small percentage of pheochromocytomas arise along the sympathetic chain. The majority of cases are diagnosed among adults age 20–50 years old. Laparoscopic adrenalectomy has become standard treatment for pheochromocytoma of the adrenal gland. The safety and efficacy of laparoscopic approach to resection of extra-adrenal pheochromocytoma in the pediatric population has not been established. We present an interesting case of a 12-year-old female diagnosed with a functional pheochromocytoma of the Organ of Zuckerkandl. Following preoperative optimization of blood pressure, she underwent successful tumor resection via a minimally invasive laparoscopic approach. The operation was completed without intraoperative complication. She was discharged home the following day with resolution of symptoms. She continues to do well in follow up. We demonstrate the successful laparoscopic resection of pheochromocytoma localized to the Organ of Zuckerkandl in a pediatric patient.

A retropancreatic celio-mesenteric paraganglioma

Paragangliomas are extra-adrenal pheochromocytomas that arise from chromaffin cells in the sympathetic or parasympathetic neural paraganglia. Surgery remains the only curative treatment, although prominent vascularity can make excision difficult. We have recently encountered a patient with a retropancreatic celio-mesenteric paraganglioma unusually located between celiac trunk (CT) and superior mesenteric artery.

Bladder paraganglioma: CT and MR imaging characteristics in 16 patients.

BackgroundBladder paraganglioma (BPG) is a rare extra-adrenal pheochromocytoma with variable symptoms and easy to be misdiagnosed and mishandled. The aim of the study was to document the imaging features of BPG using computed tomography (CT) and magnetic resonance imaging (MRI).Patients and methodsWe retrospectively enrolled consecutive patients with pathology-proven BPG, who underwent CT or MRI examinations before surgery between October 2009 and October 2017. The clinical characteristics, CT, and MRI features of the patients were described and analysed.ResultsA total of 16 patients with 16 bladder tumours (median age 51 years, 9 females) were included. Among them, 13 patients underwent CT examinations and eight patients underwent MRI examinations preoperatively. Tumour diameters ranged from 1.6−5.4 cm. Most of the tumours grew into the bladder cavity (n = 11) with oval shapes (n = 10) and well-defined margins (n = 14). Intratumour cystic degeneration or necrosis (n = 2) was observed. Two lesions showed peripheral tissue invasion, suggesting malignant BPGs. All 13 lesions imaged with CT exhibited slight hypoattenuation and moderate to marked enhancement. Compared to the gluteus maximus, all lesions showed slight h yperintensity in T2-weighted images, hyperintensity on diffusion-weighted images (DWI), hypointensity on apparent diffusion coefficient maps, hyperintensity on T1-weighted images and a “fast in and slow out” enhanced pattern on contrast-enhanced MRI images.ConclusionsBPGs are mostly oval-shaped, broadly-based and hypervascular bladder tumours with hypoattenuation on non-contrast CT, T2 hyperintensity, slight T1 hyperintensity compared to the muscle, marked restricted diffusion on DWI. Peripheral tissue invasion can suggest malignancy of the BPGs. All of these features contribute to preoperative decision-making.

Laparoscopic removal of a large pheochromocytoma-paraganglioma of the Zuckerkandl organ

Paragangliomas represent 15 to 20% of all chromaffin tissue tumors. Most often, paragangliomas are located in the abdominal cavity along the large vessels — in the para-aortic region from the diaphragm to the aortic bifurcation. One of the most common extra-adrenal pheochromocytomas is the Zuckerkandl tumor, originating from the para-aortic accumulation of sympathetic tissue located in the area of the inferior mesenteric artery’s origine or in the zone of the aortic bifurcation itself. Due to the technical difficulty in performing laparoscopic removal of paragangliomas, conversion to laparotomy is frequent and reaches 80%. The article describes a clinical case of a patient suffering from this type of neoplasm, with diagnosis details and treatment by a successful radical laparoscopic intervention with a large chromaffinnoma located in a difficult anatomical zone removal.

Extra-adrenal pheochromocytoma with initial symptom of haemoptysis: a case report and review of literature

BackgroundPheochromocytoma is a catecholamine-secreting tumour that leads to various symptoms. Haemoptysis is rarely caused by a pheochromocytoma occurring outside the bronchus or thoracic cavity. Here, we report the case of an extra-adrenal abdominal pheochromocytoma initially manifesting as haemoptysis/dyspnoea during exercise without classic symptoms.Case presentationA 22-year-old man with a history of severe dyspnoea experienced difficulties in breathing following a marathon owing to haemoptysis that required ventilator management 1 year before presentation. His father had undergone surgery for ectopic pheochromocytoma. Computed tomography (CT) revealed a 30-mm tumour between the inferior vena cava and pancreatic head while urinalysis revealed abnormally high noradrenaline levels. He was clinically diagnosed with an extra-adrenal abdominal ectopic pheochromocytoma. After controlling blood pressure, surgery was performed, and the tumour was successfully removed. Histopathology revealed chromogranin A (+), synaptophysin (+), S100 protein (+), and MIB-1 index of 1%. Therefore, the patient was finally diagnosed with extra-adrenal abdominal ectopic pheochromocytoma.ConclusionsHaemoptysis is a rare manifestation of abdominal ectopic paraganglioma. Prompt consideration of pheochromocytoma/paraganglioma when patients experience haemoptysis without any other possible aetiology may prevent inappropriate diagnosis and treatment and ultimately fatalities.

Unusual and rare cause of PUJ obstruction with a brief review of the literature

Paragangliomas or extra adrenal pheochromocytoma of the genitourinary tract are infrequent and arise from chromaffin cells of the autonomic nervous system. They have been described in the kidney, bladder, prostate, urethra and spermatic cord. However, the occurrence of paragangliomas in the ureter is very unusual. We present one such case in a patient who presented with signs and symptoms of pelvi-ureteric junction obstruction, wherein the initial clinical and imaging findings were deceptive. The patient underwent right-sided open end-to-end uretero-ureterostomy with excision of the strictured segment. Histopathological examination of excised tissue revealed a tumour with clear cell morphology, and in conjunction with immunohistochemistry a final diagnosis of paraganglioma of the ureter was rendered. The case is discussed here with differential diagnosis in the context of its location.

An unusual and interesting case of phaeochromocytoma

Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla. The common age of onset is between the third and fifth decades of life. Commonly adrenal phaeochromocytoma secretes epinephrine (its metabolite metanephrine). Extra-adrenal phaeochromocytoma secretes norepinephrine and normetanephrine. Herein, we present the case of a 13-year-old male patient who presented with palpitations, giddiness and increased appetite for 3 days. He was found to have de novo hypertension. Imaging showed bilateral adrenal masses. Biochemical analysis revealed normal levels of plasma and 24-h urinary metanephrines. On further evaluation, serum metanephrine-fractionated assay showed marked elevation in normetanephrine levels. The patient was initially managed medically with alpha- and beta-blockers. After controlling blood pressure, adrenalectomy was done. The histopathological examination was suggestive of phaeochromocytoma. Adrenal phaeochromocytoma-secreting normetanephrines are a rare entity. On reviewing literature, only a few cases of this type were published.

Paraganglioma of urinary bladder – A rare case report

Background: Extra-adrenal chromaffin related tumors are called Paraganglioma. These tumors constitute 18% of extra-adrenal pheochromocytomas and are exceptionally rare in the bladder accounting for of bladder tumors. Case report: A 45-year-old female came with history of painless hematuria of 3 days duration. On MRI scan, she was found to have a bladder tumor arising from the posterior wall. Transurethral resection of the bladder tumor (TUR-BT) was done. During the procedure, the patient had a sudden increase in blood pressure with acute left ventricular failure and pulmonary edema. Multiple grey brown TUR-BT specimen bits on microscopy showed tumor cells in “Zellballen” pattern. These cells were round to polygonal with abundant granular cytoplasm, round, regular nuclei with stippled chromatin. Mitotic figures were seen occasionally. Immunohistochemistry for Chromogranin A showed strong positivity confirming the diagnosis to be paraganglioma of the bladder. Follow-up cystoscopy of the patient didn’t show any evidence of the tumor, also her urinary and plasma vanillylmandelic acid (VMA) levels were within normal range post-operatively. Conclusion: Paraganglioma of the bladder is extremely rare. Our case presenting with only painless gross hematuria was a close mimicker of urothelial carcinoma. It has a benign coarse but has a very high recurrence rate. It is essential to differentiate it from its histological differentials for proper management and follow-up. Keywords: Chromaffin tumors, Extraadrenal Pheochromocytoma, Paraganglioma, Bladder tumor, Painless hematuria, Chromogranin A.