Retroperitoneal Paraganglioma Research Articles

Subclinical paraganglioma of the retroperitoneum: A case report

BACKGROUND Paraganglioma (PGL) located in the retroperitoneum presents challenges in diagnosis and treatment due to its hidden location, lack of specific symptoms in the early stages, and absence of distinctive manifestations on imaging. CASE SUMMARY A 56-year-old woman presented with a left upper abdominal mass discovered 1 wk ago during a physical examination. She did not have a history of smoking, alcohol consumption, or other harmful habits, no surgical procedures or infectious diseases, and had a 4-year history of hypertension. Upon admission, she did not exhibit fever, vomiting, or abdominal distension. Physical examination indicated mild percussion pain in the left upper abdomen, with no palpable enlargement of the liver or spleen. Laboratory tests and tumor markers showed no significant abnormalities. Enhanced computed tomography and magnetic resonance imaging of the upper abdomen revealed a cystic solid mass in the left epigastrium measuring approximately 6.5 cm × 4.5 cm, with inhomogeneous enhancement in the arterial phase, closely associated with the lesser curvature of the stomach and the pancreas. The patient underwent laparoscopic resection of the retroperitoneal mass, which was successfully removed without tumor rupture. A 12-month postoperative follow-up period showed good recovery. CONCLUSION This case report details the successful laparoscopic resection of a retroperitoneal subclinical PGL, resulting in a good recovery observed at the 12-month follow-up. Interestingly, the patient also experienced unexpected cure of hypertensive disease.

Pulmonary and retroperitoneal paragangliomas in a young woman: about a case report and literature review

Pulmonary and retroperitoneal paragangliomas in a young woman: about a case report and literature review

An apparently non-functional retroperitoneal SDHB-related paraganglioma

An apparently non-functional retroperitoneal SDHB-related paraganglioma

Paraaortic Extra-Adrenal Paraganglioma: Challenging Robotic Resection.

Up to 41% of intra- and extra-adrenal paragangliomas are linked to germline mutations with autosomal dominant transmission, which necessitates genetic testing for patients and their relatives.1-4 Certain alterations, such as the succinate dehydrogenase (SDH) subunit B gene mutation, are associated with a significant risk of extra-adrenal, malignant, and metastatic disease forms.4-7 This highlights the need for routine genetic counseling and diligent surveillance, as well as surgeon awareness of hereditary paraganglioma-pheochromocytoma syndrome (HPPS). We present a multimedia article featuring a step-by-step video of a complex retroperitoneal resection, enriched with perioperative management insights. A 17-year-old female presented with episodes of hypertension, tachycardia, and diffuse diaphoresis. CT revealed a paraaortic mass adjacent to the left renal hilum later confirmed by a SPECT/CT with iodine-123 meta-iodobenzylguanidine.8 Additional imaging with gallium-68 DOTATATE was not performed then due to unknown mutation status. The patient underwent robotic removal of the tumor and adjacent lymph nodes. Pathology confirmed a poorly differentiated paraganglioma with 0/6 lymph node metastases. Genetic tests revealed SDHB gene mutation, indicative of HPPS.9,10 At 12 months, the patient remained disease-free on CT with normalized metanephrines levels and no detectable circulating tumor DNA. Familial screening detected her mother, maternal uncle, and maternal grandfather to be SDHB mutation carriers, although phenotypically silent. Robotic-assisted resection can be safe and effective for retroperitoneal malignant paragangliomas. However, management extends beyond surgery and requires cascade genetic testing to address familial risks. Because of the high probability of cancer associated with SDHB mutation, lifelong patient surveillance is imperative.

A rare case of extra adrenal retroperitoneal paraganglioma: case report

Extra adrenal retroperitoneal paragangliomas are neuro endocrine neoplasms with extremely rare incidence and a wide plethora of clinical presentations. They originate from the neural crest cells interspersed throughout the body. They can present with vague symptoms of pain abdomen, hypertension, palpitations and in severe cases with renal failure, and shock owing to catecholamine excess. On the other end of the spectrum they can be totally asymptomatic and detected incidentally. The multitude of clinical presentations and lack of specific diagnostic tests hence pose a great difficulty in the pre-operative diagnosis of the disease. We are presenting a case of a 20-year-old female with vague symptoms to highlight the management and clinical diagnosis of extra adrenal retroperitoneal paraganglioma.

CT Imaging Findings of Extra-adrenal Abdominal Paragangliomas: A Case Series

Extra-adrenal paragangliomas are rare neuroendocrine tumours originating from paraganglia outside the adrenal glands. The present case series illustrates the Computed Tomography (CT) imaging features of three instances of extra-adrenal abdominal paragangliomas. The discussed cases include Retroperitoneal paraganglioma with Inferior Vena Cava (IVC) infiltration and associated hepatic, pancreatic, and skeletal metastasis; Inter-aortocaval paraganglioma; and Organ of Zuckerkandl Paraganglioma. The CT imaging features of the lesions and their relation with the adjacent vascular structures are described. The differential diagnosis and histopathological correlations are discussed. These cases underscore the importance of considering paragangliomas in the differential diagnosis of soft-tissue masses in the abdomen and highlight the role of Contrast-enhanced CT (CECT) imaging in their evaluation. Understanding the diverse imaging characteristics of extra-adrenal paragangliomas aids in accurate diagnosis and appropriate management. Further studies are needed to expand the authors knowledge of these rare tumours and optimise their imaging evaluation.

Development and internal validation of a novel predictive model for SDHB mutations in pheochromocytomas and retroperitoneal paragangliomas.

To develop and internally validate a novel predictive model for SDHB mutations in pheochromocytomas and retroperitoneal paragangliomas (PPGLs). Clinical data of patients with PPGLs who presented to Peking Union Medical College Hospital from 2013 to 2022 and underwent genetic testing were retrospectively collected. Variables were screened by backward stepwise and clinical significance and were used to construct multivariable logistic models in 50 newly generated datasets after the multiple imputation. Bootstrapping was used for internal validation. A corresponding nomogram was generated based on the model. Sensitivity analyses were also performed. A total of 556 patients with PPGLs were included, of which 99 had a germline SDHB mutation. The prediction model revealed that younger age of onset [Odds ratio (OR): 0.93, 95% CI: 0.91-0.95], synchronous metastasis (OR: 6.43, 95% CI: 2.62-15.80), multiple lesion (OR: 0.22, 95% CI: 0.09-0.54), retroperitoneal origin (OR: 5.72, 95% CI: 3.13-10.47), negative 131I-meta-iodobenzylguanidine (MIBG) (OR: 0.34, 95% CI: 0.15-0.73), positive octreotide scintigraphy (OR: 3.24, 95% CI: 1.25-8.43), elevated 24h urinary dopamine (DA) (OR: 1.72, 95% CI: 0.93-3.17), NE secretory type (OR: 2.83, 95% CI: 1.22- 6.59), normal secretory function (OR: 3.04, 95% CI: 1.04-8.85) and larger tumor size (OR: 1.09, 95% CI: 0.99-1.20) were predictors of SDHB mutations in PPGLs, and showed good and stable predictive performance with a mean area under the ROC curve (AUC) of 0.865 and coefficient of variation of 2.2%. This study provided a novel and useful tool for predicting SDHB mutations by integrating easily obtained clinical data. It may help clinicians select suitable genetic testing methods and make appropriate clinical decisions for these high-risk patients.

SAT329 Case Series Of Patients With FGFR-1 Related Pheochromocytoma With A Focus On Biochemical And Imaging Signatures

Abstract Disclosure: S. Jhawar: None. J. Del Rivero: None. K. Pacak: None. Introduction: Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of adrenal gland and neural crest, respectively. 40% of cases are associated with germline mutations and 30-40% somatic driver mutations. Here, we present two patients with PCC who were found to have somatic FGFR1 mutation. FGFR signaling is involved in regulation of tissue repair, angiogenesis, and inflammation. Patient 1: A 70-year-old female with a history of resistant hypertension, episodic palpitations and sweating presented with diffuse abdominal pain and chest tightness. She was noted to have a blood pressure of 220/160 mm Hg. CT scan showed a 4.2 x 3.9 cm left adrenal mass, suspicious for malignancy and a <1 cm retroperitoneal mass. Blood work revealed elevated plasma normetanephrine (NM) 3543 pg/mL (normal 0- 145) and metanephrine (MN) 871 pg/mL (normal 0-62). 18F-FDG PET/CT showed a left adrenal mass without abnormal FDG activity. 68Ga-DOTATATE PET/CT was compatible with left PCC (SUVmax 28). 18F-DOPA PET/CT showed left PCC and a small retroperitoneal PGL. Two weeks after initiating phenoxybenzamine, she underwent total left adrenalectomy. Pathology was consistent with PCC. Molecular testing revealed FGFR1 pathogenic somatic mutation (c.1638C>G p.Asn546Lys). Patient 2: A 73-year-old male with known left renal cell carcinoma presented to our institution for management of recurrent right PCC. He was found to have a 3.8 cm right adrenal incidentaloma eight years ago and elevated plasma NM 6.3 nmol/L (normal <0.9), MN 1.5 nmol/L (normal <0.5). He underwent total right adrenalectomy, and the diagnosis of PCC was confirmed on histopathology. Two years later, he developed a 5.1 x 4.7 cm nodular mass in the right adrenal fossa and a <1 cm liver lesion on CT scan. The adrenal lesion showed intense avidity on 68Ga-DOTATATE PET/CT and 18F-DOPA PET/CT and mild avidity on 18FDG-PET/CT. 18F-DOPA PET/CT also showed avidity towards the liver lesion. He was started on doxazosin and underwent laparoscopic right adrenalectomy. Pathology demonstrated PCC. Whole exome sequencing of the tumor showed pathogenic FGFR1 mutation (c.1638C>G, p.Asn546Lys). Discussion: Activating FGFR1 mutations are associated with sporadic PCC. The biochemical phenotype of our patients showed higher levels of NM than MN. The imaging profile revealed that 18F-DOPA PET/CT was more sensitive than 68Ga-DOTATATE PET/CT and 18F-FDG PET/CT as it showed avidity towards other extra-adrenal lesions. Further studies in larger cohorts are needed to enhance understanding of pathogenesis, biochemical and imaging phenotype, which will help tailor the treatment and potential development of novel targeted therapies. Presentation: Saturday, June 17, 2023

Functional Retrocaval paraganglioma: Case Report and Review of Literature

Functional Retrocaval paraganglioma: Case Report and Review of Literature

Differentiation of retroperitoneal paragangliomas and schwannomas based on computed tomography radiomics

The purpose of this study was to differentiate the retroperitoneal paragangliomas and schwannomas using computed tomography (CT) radiomics. This study included 112 patients from two centers who pathologically confirmed retroperitoneal pheochromocytomas and schwannomas and underwent preoperative CT examinations. Radiomics features of the entire primary tumor were extracted from non-contrast enhancement (NC), arterial phase (AP) and venous phase (VP) CT images. The least absolute shrinkage and selection operator method was used to screen out key radiomics signatures. Radiomics, clinical and clinical-radiomics combined models were built to differentiate the retroperitoneal paragangliomas and schwannomas. Model performance and clinical usefulness were evaluated by receiver operating characteristic curve, calibration curve and decision curve. In addition, we compared the diagnostic accuracy of radiomics, clinical and clinical-radiomics combined models with radiologists for pheochromocytomas and schwannomas in the same set of data. Three NC, 4 AP, and 3 VP radiomics features were retained as the final radiomics signatures for differentiating the paragangliomas and schwannomas. The CT characteristics CT attenuation value of NC and the enhancement magnitude at AP and VP were found to be significantly different statistically (P < 0.05). The NC, AP, VP, Radiomics and clinical models had encouraging discriminative performance. The clinical-radiomics combined model that combined radiomics signatures and clinical characteristics showed excellent performance, with an area under curve (AUC) values were 0.984 (95% CI 0.952–1.000) in the training cohort, 0.955 (95% CI 0.864–1.000) in the internal validation cohort and 0.871 (95% CI 0.710–1.000) in the external validation cohort. The accuracy, sensitivity and specificity were 0.984, 0.970 and 1.000 in the training cohort, 0.960, 1.000 and 0.917 in the internal validation cohort and 0.917, 0.923 and 0.818 in the external validation cohort, respectively. Additionally, AP, VP, Radiomics, clinical and clinical-radiomics combined models had a higher diagnostic accuracy for pheochromocytomas and schwannomas than the two radiologists. Our study demonstrated the CT-based radiomics models has promising performance in differentiating the paragangliomas and schwannomas.

Intraoperative Corrected Diagnosis of a Right Renal Para-Hilar Paraganglioma, Taken Preoperatively for a Renal Tumor

Nonfunctional retroperitoneal paragangliomas are rare tumors. They are defined as extra-adrenal chromaffin tumors and represent about 20% of chromaffin tumors. They are often asymptomatic and can grow to large dimensions. We report the observation of a 67-year-old patient admitted to the operating room for right total nephrectomy for right hilar renal tumor and whose intraoperative dissection of the renal pedicle could demonstrate an exorenal mass. Anatomopathological analysis was in favor of a paraganglioma. Malignant forms, more frequent than benign forms, present a locoregional invasion and metastasize late. The management of paragangliomas must be multidisciplinary but only surgical treatment is curative. However, there is no consensus on the usefulness of complementary therapies, which can nevertheless be used as a symptomatic adjunct. Paragangliomas are genetic in 25% of cases. A genetic investigation must be systematically proposed.

Anesthetic management of an emergency cesarean section in a parturient with inoperable paraganglioma: A challenging case report

Paraganglioma is a neuroendocrine tumor characterized by small organs comprising neuroendocrine cells that can produce catecholamines. Because of the nature of such tumors, hemodynamic instability can pose a management challenge. This condition can influence the provision of medical care across different clinical settings. The aim of this case report is to highlight anesthesia management in parturient patients with abdominopelvic paraganglioma, particularly during an emergency cesarean section. At 34 weeks of gestation, the 33-year-old female patient with three pregnancies and two past deliveries had a known case of inoperable large pelvic and lower abdominal retroperitoneal paraganglioma and poor radiotherapy response. The 15.5 × 4.2 × 22 cm tumor encased the aorta, inferior vena cava, and bilateral iliac vessels, compressing the lumbar vertebrae. The patient had an uneventful and successful cesarean delivery with a multidisciplinary team effort. Paragangliomas are rarely reported in a parturient and its optimization course. Despite being interrupted by an emergency cesarean section, which was handled safely and effectively, both mother and fetus had a safe delivery and postoperative recovery without complications.

Mass size is a major predictor of hypertensive attack during surgery in patients with paraganglioma of retroperitoneum.

Surgical manipulation of paraganglioma can induce a massive release of catecholamines leading to hypertensive attack. But it has been not known about risk factors to cause hypertensive attack because paragangliomas of urinary bladder and retroperitoneum are notably rare tumors and have been recorded as case report or series. We investigated the relationship between mass size and hypertensive attack during surgery in patients with paraganglioma. Our retrospective chart review included 32 patients who had a pathological diagnosis of paraganglioma between March 2006 and May 2021, in single center. We analyzed the risk factors such as age, sex, height, weight, blood pressure before surgery, history of hypertension, pre-operative symptoms, mass location, and mass in 24 patients with retroperitoneal paragangliomas including urinary bladder. Hypertensive attack was defined as systolic blood pressure >180 mmHg during excision of the mass from the electric medical chart. The predictive power was assessed by the area under the curve of the receiver operating characteristic curve. There were 19 retroperitoneal, 5 urinary bladder, 2 middle-ear cavity, 2 mediastinal, 2 neck, 1 spinal cord and 1 duodenal paraganglioma. Seven (29.2%) of the 24 patients had preoperative symptoms such as pain, fluctuation of blood pressure, and palpable mass. Hypertensive attack during surgery occurred in 11 patients (45.8%). There was a significant difference in mass size between groups with (n=11) and without (n=13) fluctuation of blood pressure (P=0.007). The area under curve for predicting surgical complications according to mass size was 0.808 (cutoff size 4.25 cm, sensitivity 72.7%, specificity 76.9%, 95% CI: 0.635-0.981). Mass size impacted occurrence of hypertensive attack during surgery in patients with retroperitoneal paraganglioma. Surgeons have to be watchful regarding of intraoperative hypertension during resection of retroperitoneal masses exceeding 4.25 cm, which are suspected as paraganglioma.

A Rare Case of a Retroperitoneal Paraganglioma: A Case Report

Retroperitoneal paraganglioma is a rare neoplasm that develops from chromaffin cells that secrete catecholamines in the sympathetic ganglia. They are frequently functional and may be benign or malignant depending on the presence of metastasis and/or the occurrence of recurrence. Different imaging modalities play a crucial role in detecting these tumors. The typical CT appearance of PG is that of a well limited isodense mass with an intense contrast enhancement. On MRI, the mass presents an isosignal or hyposignal in T1 and hypersignal in T2 with an early and intense enhancement. The final diagnosis is based on histological and immunohistochemical findings and the surgical excision is the mainstay of the treatment. However, these tumors are often difficult to diagnose and treat. We report a case of a 30-year-old female patient who presented with a 3 months history of constipation, revealing on computed tomography (CT) a retroperitoneal mass lateralized on the right with a close connection to the inferior vena cava. Urine normetadrenaline (metanephrine) was elevated at 451 ug/24h. Neck and chest CT scan and scintigraphy with 123-I labeled metaiodobenzylguanidine (MIBG) were negative. Complete resection of the tumor was performed. The histological examination and immunohistochemical analyses concluded the diagnosis of a paraganglioma.

Minimally invasive management for multifocal pelvic retroperitoneal malignant paraganglioma: a neuropelveological approach

BackgroundPheochromocytoma and Paraganglioma (PGL) are rare neuroendocrine tumors, with an estimated incidence of about 0.6 cases per 100.000 person/year. Overall, 3–8% of them are malignant. These tumors are characterized by a classic triad of symptoms (headaches, palpitations, profuse sweating) due to hypersecretion of catecholamines. Despite several advantages of minimally invasive surgery (MIS) for PGL debulking, the surgical approach is not standardized yet. In this scenario, we aimed to report a case of a multiple recurrent PGL with metastatic retroperitoneal localization involving the pelvic sidewall, excised with MIS.Case presentationWe performed complete laparoscopic-assisted neuronavigation (LANN technique) with isolation of the sacral routes and the sciatic nerve to obtain complete exposure of the main anatomic landmarks. Robotic surgery was used to perform neurolysis of sacral plexus, and partial resection of left splanchnic nerves was needed. After the resection of the first mass, extensive neurolysis of all sacral routes, obturator nerve, pudendal nerve till the entrance of the pudendal (Alcock) canal, and sciatic nerve was performed. Finally, the mass was identified after trans gluteal incision and dissection of the maximum gluteal muscle, and a partial resection of the superior gluteal nerve and slicing of the sciatic nerve were needed to obtain a radical excision of the mass. Then neurorrhaphy of the sectioned nerve fibers of the superior gluteal nerve was performed, and nerve protection was obtained using a collagen nerve wrap. After 18 months of follow-up, the patient is free of disease at the MRI imaging and 123I-metaiodobenzylguanidine scintigraphy.ConclusionsMinimally invasive gynecological surgery with neuropelveological approach could be considered as a feasible option in case of multifocal pelvic retroperitoneal malignant paraganglioma of the pelvic side wall.

An incidental finding of retroperitoneal paraganglioma during the coronavirus disease 2019 pandemic: a case report

BackgroundExtra-adrenal paraganglioma of the retroperitoneum is a very rare neoplasm arising from cells of the primitive neural crest. Although paragangliomas are considered benign and are often found incidentally, they have the potential to metastasize.Case presentationWe report the case of a 68-year-old Caucasian woman with an incidental diagnosis of retroperitoneal paraganglioma that was discovered on chest computed tomography performed for high suspicion of coronavirus disease 2019 pneumonia. The patient showed no metastasis and was successfully treated by complete surgical removal of the tumor.ConclusionAs the diagnosis of paragangliomas is often delayed because of absent clinical symptoms, they represent a significant diagnostic challenge. Although surgery may exacerbate coronavirus disease 2019 infection, surgical resection of this tumor is prioritized, given its malignancy potential, and it must be performed as soon as no infection is detected.

Retroperitoneal Paraganglioma often Atypical: Short Case Series and Review of the Literature

Retroperitoneal Paraganglioma often Atypical: Short Case Series and Review of the Literature

Imaging features of retroperitoneal extra-adrenal paragangliomas in 10 dogs.

Retroperitoneal paragangliomas are rare tumors of the neuroendocrine system. Only a few canine case reports are available with rare descriptions of their imaging features. The objectives of this multi‐center, retrospective case series study were to describe the diagnostic imaging features of confirmed retroperitoneal paragangliomas and specify their location. Medical records and imaging studies of 10 affected dogs with cytological or histopathologic results concordant with retroperitoneal paragangliomas were evaluated. Dogs had a median age of 9 years. Four of them had clinical signs and laboratory reports compatible with excessive production of catecholamines. Six ultrasound, four CT, four radiographic, and one MRI studies were included. The paragangliomas did not have a specific location along the aorta. They were of various sizes (median 33 mm, range: 9–85 mm of length). Masses had heterogeneous parenchyma in six of 10 dogs, regardless of the imaging modality. Strong contrast enhancement was found in all CT studies. Encircling of at least one vessel was detected in six of 10 masses, clear invasion of a vessel was identified in one of 10 masses. In five of 10 cases, the masses were initially misconstrued as lymph nodes by the on‐site radiologist. Retroperitoneal paragangliomas appear along the abdominal aorta, often presenting heterogeneous parenchyma, possibly affecting the local vasculature, and displaying strong contrast enhancement on CT. Clinical signs can be secondary to mass effects or excessive catecholamine production. Underdiagnosis and misdiagnosis of this tumor are suspected as they can be silent, of small size, or confused with other structures.

A diagnostic dilemma of hormone-secreting paraganglioma presenting as secondary diabetes

Paragangliomas are rare neuroendocrine neoplasms that arise from chromaffin cells. They most commonly arise in adrenal medulla and are called as pheochromocytoma. About 10% of paragangliomas arise at extra-adrenal sites. Of the functioning extra-adrenal paragangliomas, majority present with intermittent hypertensive attacks. Very rarely, paragangliomas secreting other hormones including adrenocorticotropic hormone, endorphins, pancreatic polypeptide, androgens, and insulin have been reported. Patients should be evaluated with imaging studies along with appropriate hormonal assays keeping in mind the variety of hormones paragangliomas may secrete in turn leading to diagnostic dilemmas. This case report describes a patient with uncontrolled high blood glucose levels and an abdominal mass initially suspected for a glucagonoma which was later discovered to be a functioning retroperitoneal paraganglioma.

Unclear retroperitoneal tumors, an interdisciplinary challenge - A case report and review of the literature.

Introduction and importanceUnclear retroperitoneal tumors impose major challenges for clinicians. Tumors can originate primarily from retroperitoneal tissue or secondarily invade into the retroperitoneum. While benign lesions also occur, malignant tumors are far more common. Clinical presentation depends on replacement or invasion of other organs and is therefore highly variable. The heterogeneous tumor composition makes a definitive preoperative diagnosis difficult. Surgical resection is the gold standard for treatment but often proves challenging due to frequent involvement of large retroperitoneal vessels.Case presentationWe present the case of a 70-year old woman diagnosed with a large, unclear retroperitoneal tumor. Initial clinical symptoms were increasing dyspnea and dysphagia in our clinic. Gastroenterologic and cardiologic workup was unremarkable. Computed Tomography (CT) revealed a large retroperitoneal mass in the right upper abdomen with severe displacement of the inferior vena cava and renal veins. The patient was scheduled for primary tumor resection. The procedure was challenging due to the vessel involvement and large blood pressure alterations during tumor mobilization. The post-op pathologic workup then revealed the rare finding of a completely resected paraganglioma. The post-surgical course was uneventful. One year after diagnosis, the patient is relapse-free.Clinical discussionAmong retroperitoneal tumors, paragangliomas and pheochromocytomas are rare tumor entities. Asymptomatic, sporadic disease is hard to identify preoperatively and can cause unexpected complications in the OR. An experienced team is crucial in achieving best short- and long-term outcomes.ConclusionThis case impressively shows the challenges of retroperitoneal tumors and the importance of interdisciplinary work in these cases.