Extra-adrenal Retroperitoneal Paraganglioma Research Articles

P001 AN UNCOMMON CAUSE OF HIGH BLOOD PRESSURE IN YOUNG BASKETBALL PLAYER

Paragangliomas are rare neuroendocrine tumors that affect approximately 1.5–9 patients per million. They originate from chromaffin cells of the adrenal medulla or sympathetic and parasympathetic ganglia. Paragangliomas account for 10%–20% of retroperitoneal tumors and can manifest at any age. These tumors can secrete catecholamines, leading to symptoms such as hypertension, headaches, and diaphoresis.However, palpitations or tachycardia may also be present. Definitive treatment entails surgical resection, with acceptable rates of morbidity and mortality, necessitating careful patient preparation and intraoperative management. Case Presentation: We present the case of a 29-year-old man with a BMI 26,5 kg/m2, who is physically active and without other cardiovascular risk factors, episodic hypertension, headaches and blush. A contrast CT scan of the abdomen revealed a retroperitoneal intercavoaortic mass and 24-hour urine Normetanephrines were significantly elevated, indicating a functional paraganglioma. Laboratory tests revealed an elevated plasma normetanephrine level of 1838 pg/ml (reference values 0 - 180 pg/ml). The patient was prepared for surgery with alpha blockers, followed by beta blockers. Intraoperative blood pressure elevation was managed with intravenous beta blockers. Postoperatively, the patient did not require anti-hypertensives. The tumor, measuring 6x4x3.5 cm, histopathology and immunohistochemistry confirmed the diagnosis of a normetanephrine producing paraganglioma. The genetic study revealed a pathogenic variant in the SDHB gene, specifically the c.166_170delCCTCA (p.Pro56Tyrfs*5) in heterozygosity. This variant is associated with a 49% risk of Pheochromocytoma and Hereditary Paraganglioma, a 14% risk of Renal Carcinoma and susceptibility to Gastrointestinal Stromal Tumors. The c.166_170delCCTCA variant follows an autosomal dominant inheritance pattern, indicating a 50% risk of recurrence. Conclusions: The diagnosis of extra-adrenal retroperitoneal paragangliomas remains challenging due to the silent evolution of the condition. It is essential to consider screening patients who are young, and present with resistant arterial hypertension for secondary hypertension.

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.

Preoperative Alpha-Blocker Therapy in Patients with Missed Preoperative Diagnosis of Extra-Adrenal Retroperitoneal Paraganglioma Undergoing Resection: A Retrospective Study of 167 Cases at a Single Center

Introduction: Preoperative α-adrenergic blockade is thought to decrease perioperative risks and mortality in surgeries for adrenal pheochromocytoma. However, there are limited data on the efficacy of α-blockers in surgeries for retroperitoneal paragangliomas. The aim of this study was to evaluate the effects of the preoperative α-adrenergic blockade on outcomes in patients undergoing surgery for extra-adrenal retroperitoneal paraganglioma. Methods: We searched the clinical database for patients diagnosed with extra-adrenal retroperitoneal paraganglioma by postoperative histopathology in the General Hospital of People’s Liberation Army in China from 2000 to 2017. We compared the preoperative status of patients, preoperative examination, preoperative preparation, intraoperative and postoperative cardiovascular events, intake and output, length of stay in the intensive care unit, length of hospital stays, and short-term outcomes between patients who received preoperative treatment with α-adrenergic blockade and those who did not. Results: Of the 167 patients enrolled in the study, 61 received preoperative α-adrenergic blockade therapy. Intraoperative heart rate elevation and highest heart rate were higher in patients undergoing tumor manipulation with preoperative α-adrenergic blockade than those who did not (p < 0.05). However, there were no significant differences between these 2 groups in terms of intraoperative blood pressure elevation and systolic blood pressure decrease following tumorectomy (p > 0.05). Moreover, there were no significant differences in postoperative complications and outcomes (p > 0.05). Conclusion: Under the current medical practice, resection of extra-adrenal retroperitoneal paraganglioma can be successfully carried out with or without preoperative α-adrenergic blockade.

Retroperitoneal paraganglioma presenting as hypertension and paraparesis in young

Paragangliomas(PGL) are rare tumours that arise from extra-adrenal paraganglia. Extra-adrenal paragangliomas account for only 5 to 10% of all paragangliomas and may present incidentally as a mass. Typical triad of fluctuating hypertension, headache, and sweating is not always present which makes the diagnosis sometimes difficult. Extraadrenal retroperitoneal PGLs are functionally active more often than previously reported and that they are readily detected by soft tissue masses closely associated with the entire length of abdominal aorta. However, no CT feature was found that was unique for PGL.1 Herein we describe the clinical, radiological and pathological features in young patient presented for hypertension and paraparesis, and later on diagnosed as malignant abdominal paraganglioma with vertebral and lymph node metastasis.

A resected case of liver metastases from extra-adrenal retroperitoneal paraganglioma with von Recklinghausen's disease 16years after the initial surgery.

The patient was a 46-year-old man who had undergone resection for a bulky retroperitoneal tumor 16 years previously during a follow-up for von Recklinghausen’s disease. Histopathological examination of the resected specimen showed that the tumor was an extra-adrenal paraganglioma. After the surgery, he had survived without any recurrence of the tumor. However, 16 years after the initial surgery, liver tumors were identified, and he was referred to our hospital for further investigation and treatment. Abdominal imaging modalities showed three masses in the left lateral segment of the liver. Fluorodeoxyglucose-positron emission tomography/computed tomography showed an abnormal uptake of fluorodeoxyglucose corresponding to the mass lesions. The patient was diagnosed with a metastatic paraganglioma based on histopathological examination of a liver mass biopsy. The patient underwent left lateral sectionectomy of the liver. Histopathological examination of the resected specimen revealed proliferating cells with basophilic cytoplasm and oval densely stained nuclei arranged in an alveolar pattern, which was similar to the findings of the initial resection specimen. Immunohistochemical staining was positive for synaptophysin and chromogranin A. Based on these findings, the resected tumors were histopathologically diagnosed with liver metastases from the retroperitoneal paraganglioma. We concluded that this is an extremely rare case of liver metastases occurring long after the initial resection of extra-adrenal peritoneal paraganglioma with von Recklinghausen’s disease.

Peptide receptor radionuclide therapy with lutetium-177 DOTATATE in a case of recurrent extradrenal retroperitoneal malignant paraganglioma with nodal and bone metastasis

Extra-adrenal retroperitoneal paragangliomas (PGLs) are rare tumors causing considerable difficulty in both, diagnosis and treatment. They can be unicentric or multicentric, tend to be locally invasive and therefore have a high incidence of local recurrence. PGLs shows somatostatin receptor positivity, which can be imaged with technetium-99m (Tc-99m)-hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC) and can be treated with lutetium-177 (Lu-177) DOTATATE. We present a case of recurrent unresectable retroperitoneal PGL with nodal and bone metastases in a 27-year-old male, 6 months postsurgery detected with Tc-99m-HYNIC-TOC and was administered with peptide receptor radionuclide therapy using Lu-177 DOTATATE.

Bi-Focal Retroperitoneal Paraganglioma in a Young Patient: A Case Report and Review of the Literature

Extra-adrenal paraganglioma of the retroperitoneum are rare neuroendocrine tumors arising from specialized neural crest cells, intimately associated with sympathetic nervous tissue and distributed along the aorta. Here we present a case of multiple extra-adrenal retroperitoneal paraganglioma in a 16-year-old boy. The clinical presentation, imaging characteristics, and surgical pathologic findings were reported. A comprehensive review of the medical literature concerning multiple extra-adrenal paraganglioma in the pediatric and adult population is also discussed.

Extra-adrenal retroperitoneal paraganglioma in a dog

An extra-adrenal retroperitoneal paraganglioma was observed in a 10.5-year-old male Boxer dog. Additionally, the dog had an aortic base tumor, multiple thyroid adenomas, multiple testicular interstitial cell tumors, bilateral nodular adrenal cortical hyperplasia, and parathyroid gland hyperplasia. The hypothesis that the retroperitoneal mass represents a primary extra-adrenal paraganglioma rather than metastatic mass from the aortic body tumor is considered. Either primary or metastatic extra-adrenal retroperitoneal paragangliomas are rarely reported in dogs.

Adrenal Nodular Hyperplasia in Hereditary Leiomyomatosis and Renal Cell Cancer

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

Asymptomatic extra-adrenal paraganglioma masquerading as retroperitoneal sarcoma.

A 41-year-old man without clinical symptoms was referred for treatment of an enlarging retroperitoneal tumor. Enhanced computed tomography showed a well-defined and heterogeneously enhanced tumor, 4cm in size, in the dorsal portion of the pancreas. A low-density nodule was detected in the left adrenal gland, 10mm in diameter. Retroperitoneal sarcoma and nonfunctional left adrenal tumor were suspected, and surgical treatment was performed. During excision of the retroperitoneal tumor, blood pressure was extremely elevated when the tumor was compressed. Blood pressure normalized after excision of the tumor; thus, a diagnosis of paraganglioma was favored over that of retroperitoneal sarcoma. The left adrenal gland was resected together with the adrenal tumor. Microscopically, the tumor cells of the retroperitoneum had round to oval nuclei, and abundant granular amphophilic cytoplasm proliferated in nest-like fashion. Extra-adrenal retroperitoneal paraganglioma was considered, and the adrenal tumor was diagnosed as cortical adenoma. In patients with retroperitoneal tumor, even in the absence of clinical symptoms, we should keep in mind the possibility of extra-adrenal paraganglioma.

Successful outcome after resection of liver metastasis arising from an extraadrenal retroperitoneal paraganglioma that appeared 9 years after surgical excision of the primary lesion

A 54-year-old man underwent surgery for excision of a retroperitoneal tumor that measured 12 cm in diameter, and histopathological examination revealed the tumor was an extraadrenal retroperitoneal paraganglioma. He presented 9 years later with epigastric discomfort. Abdominal ultrasound showed a solitary liver tumor. The diagnosis, based on radiological workup, was metastatic paraganglioma. The tumor was surgically resected and the histological findings resembled those of the primary tumor. The patient has been followed up for 3 years and remains recurrence-free. Surgical resection is an effective treatment approach for primary and secondary paragangliomas, but the resectability of liver metastatic lesions is usually low, although complete resection with a wide surgical margin was possible in this patient. This case suggests that a good prognosis after the resection of hepatic metastasis depends not only on the curative resection of the metastatic lesion but also on the tumor characteristics, such as slow growth or low aggressiveness.

Malignant Paraganglioma of the Retroperitoneum with Lung Metastases: A 13-year Survivor After Radical Surgery

We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.

Fluorine-18 DOPA PET Imaging of Paraganglioma Syndrome

A 12-year-old boy was admitted after recently having had a resection performed of an extraadrenal retroperitoneal paraganglioma and left adrenalectomy for pheochromocytoma. Despite these procedures, the blood pressure and urinary noradrenalin were elevated. Screening with fluorine-18 DOPA PET demonstrated increased tracer uptake in the right adrenal gland, in a second abdominal lesion, which was prevertebral, and 2 cervical hot spots near the carotid bifurcation, one on each side of the neck. The patient carries a mutation of the gene Succinate dehydrogenasis subunits D (SDHD) and is thus classified with the paraganglioma syndrome type 1.

Recommendations for Reporting of Extra-Adrenal Paragangliomas

The Association of Directors of Anatomic and Surgical Pathology (ADASP) has proposed recommendations for surgical pathology reporting of malignant tumors. Recommendations for reporting of extra-adrenal paragangliomas are outlined herein. The Association of Directors of Anatomic and Surgical Pathology (ADASP) has requested various committees to propose recommendations for the content and scope of the surgical pathology report for common or uncommon tumors. The following recommendations were developed by an ad hoc committee composed of Ernest E. Lack, MD, (Chair), Ricardo V. Lloyd MD, PhD, J. Aidan Carney, MD, PhD, James M. Woodruff MD, and a group of pathologists with expertise or special interest in paragangliomas. These recommendations are reviewed and approved by the council of the ADASP and then circulated to the entire membership of the ADASP for their input. Thus, an interim consensus on reporting is reached among those with interest and expertise in a particular area or field of pathology; clearly, it is recognized that classification of tumors, diagnostic criteria, and terminology may undergo change in the future.

Extraadrenal Retroperitoneal Paraganglioma with Osseous Metaplasia

We report a case of extraadrenal retroperitoneal paraganglioma in a 24-year-old female. This case is unusual in that the neoplasm showed extensive calcification, osseous metaplasia, and marrow elements. Although calcification may occur in paragangliomas, to the best of our knowledge, osseous metaplasia with marrow elements have not been previously reported in the English literature. Int J Surg Pathol 4(3):00-00, 1997

Extraadrenal retroperitoneal paraganglioma: clinical, pathologic, and CT findings.

Paragangliomas of the retroperitoneum arise from specialized neural crest cells distributed along the aorta in association with the sympathetic chain. In order to ascertain characteristic CT features of extraadrenal retroperitoneal paragangliomas to differentiate them from other retroperitoneal tumors, 31 discrete tumors and two cases of paragangliomatosis in 28 patients were reviewed retrospectively, and the CT features were correlated with clinical and pathologic findings. There were 16 men and 12 women. Average age was 37 years (range, 11-70 years). Twenty-four patients (86%) had hypertension. Of these, catecholamine levels were elevated in all 18 patients who had biochemical studies. Four patients (14%) had malignant paragangliomas. The discrete tumors were classified by location as suprarenal (26%), renal hilar (32%), or infrarenal (42%). Suprarenal paragangliomas could not be distinguished from the ipsilateral adrenal gland on CT. The average size of functional tumors was smaller (7.0 cm) than that of nonfunctional tumors (12.0 cm), but the sizes of the two groups overlapped. Smaller tumors were more likely to be homogeneous and have well-defined margins than were larger tumors. Our findings indicate that extraadrenal retroperitoneal paragangliomas are functionally active more often than previously reported and that they are readily detected by CT as soft-tissue masses closely associated with the entire length of the abdominal aorta. However, no CT feature was found that was unique for paraganglioma.

Extra-Adrenal Non-Functioning Paraganglioma

Extra-adrenal non-functioning retroperitoneal paragangliomas are rare tumors. We herein report a case of retroperitoneal tumor which posed a diagnostic and management problem. Review of the literature shows only 16 previously reported cases of extra-adrenal paraganglioma which underwent complete surgical excision.