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Background and Objectives: The aim of this study is to explore the long-term prognostic risk factors associated with patients diagnosed with retroperitoneal paraganglioma (RPGL) and examine their clinical and pathological characteristics., Methods: Expressions of biomarkers were identified using immunohistochemistry (IHC) and case databases were retrospectively searched. Survival analysis was performed using Kaplan-Meier and Cox risk regression to identify the factors that influence the postoperative progression-free survival of patients with RPGL., Results: A total of 105 patients, most of whom had tumors situated in the paraaortic region, and whose average tumor size was 8.6 cm, were enrolled in this study. The average follow-up duration was 51 months, with a mortality rate of 19% and a recurrence and metastasis rate of 41.9%. Tumors were assessed using the modified Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP), and SDHB, S-100, and Ki-67 were stained using IHC in all cases. Out of the total cases examined, negative in SDHB expression were observed in 18.1% of cases, S-100 expression was negative in 36.2% of cases, and endovascular tumor enboluswas present in approximately 25.7% of cases. The results of the univariate analysis indicated that several factors significantly influenced the progression-free survival of patients with PGL as follow: maximum tumor diameter (>5.5 cm), tumor morphological features, tumor grading (modified GAPP score > 6), SDHB negative, S-100 negative, and expression of proliferation index Ki-67 (>3%) (X 2  = 4.217-27.420, p < 0.05). The results of the multivariate analysis indicated that negative of S-100 (p = 0.021) and SDHB (p = 0.038), as well as intravascular tumor thrombus (p = 0.047) expression were independent risk factors for progression-free survival in patients., Conclusion: RPGL is characterized by diverse biological features and an elevated susceptibility to both recurrence and metastasis. Both SDHB and S-100 can be employed as traditional IHC indicators to predict the metastatic risk of PGL, whereas the tumor histomorphology-endovascular tumor enbolus assists in determining the metastasis risk of RPGL., (© 2024 Wiley Periodicals LLC.)

Paraganglioma, a catecholamine-producing tumor originating in extra-adrenal paraganglion cells, is rare in children. Although diagnosis of paraganglioma is based on the presence of catecholamine symptoms, some patients lack such symptoms. Delayed diagnosis is associated with tumor growth and invasion of surrounding vessels. Herein, we report a case of pediatric retroperitoneal paraganglioma invading the inferior vena cava. The tumor was completely resected and the patient has been free of recurrence for 2 years. Our findings suggest that complete resection can help prevent recurrence in cases of invasion of the inferior vena cava.

Background: Paragangliomas (PGLs) are extremely rare neuroendocrine tumours arising from extra-adrenal chromaffin cells. PGLs are clinically rare, difficult to diagnose and usually require surgical intervention. PGLs mostly present catecholamine-related symptoms. We report a case of Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal PGL. There has been only one similar case reported in literature., Case Presentation: We present a unique case of a 52-year-old female with acute abdomen induced by haemorrhagic retroperitoneal PGL. The patient had a 5-h history of sudden onset of serve right lower quadrant abdominal pain radiating to the right flank and right lumbar region. Patient had classic symptoms of acute abdomen. Abdominal ultrasound revealed a large abdominal mass with a clear boundary. A Computed Tomography Angiography (CTA) of superior mesenteric artery was also performed to in the emergency department. The CTA demonstrated a large retroperitoneal mass measured 9.0 × 7.3 cm with higher density inside. A provisional diagnosis of retroperitoneal tumour with haemorrhage was made. The patient received intravenous fluids, broad-spectrum antibiotics and somatostatin. On the 3 rd day of admission, her abdominal pain was slightly relieved, but haemoglobin decreased from 10.9 to 9.4 g/dL in 12 h suggesting that there might be active bleeding in the abdominal cavity. Thus, we performed a midline laparotomy for the patient. Haemorrhage was successfully stopped during operation. The retroperitoneal tumour with haemorrhage was completely removed. The abdominal pain was significantly relieved after surgery. The patient initially presented with acute abdomen instead of catecholamine-related symptoms. The diagnosis of retroperitoneal PGL with haemorrhage was finally confirmed by postoperative pathological and immunohistochemical results. The postoperative course was uneventful. At the 1-year follow-up visit, no tumour recurrence was observed by Single Photon Emission Computed Tomography. A literature review was performed to further understand and analyse the aforementioned disease., Conclusion: Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal paraganglioma is extremely rare. Abdominal Computed Tomography is essential to locate the lesion and differentiate between other causes of acute abdomen. PGLs are hypervascular tumours. We should be aware that ruptured retroperitoneal PGL with massive bleeding could be life threatening and require emergency laparotomy.

In front of a primary retroperitoneal tumour, it is necessary to have in mind all possible diagnoses in order to specify the diagnostic strategy and the treatment. According to the World Health Organization (WHO) classification of tumours, mesenchymal benign and malignant tumours (including sarcomas and, currently, neurogenic tumours), parasympathetic tumours, extragonadal germ cell tumours, and lymphoid tumours have been identified. By definition, primary retroperitoneal tumours start independently from the retroperitoneal organs. Secondary lesions, carcinoma metastasis, and adenopathy are excluded from this definition, but they can also develop in the retroperitoneal space and lead to misdiagnoses. In the absence of positive tumour markers or an evocative biology, percutaneous biopsy is necessary. Pathological diagnosis is necessary to decide whether surgery must be done, its timing among the other treatments, and its extension. This paper summarizes all the diagnostic possibilities., Competing Interests: Declaration of competing interest The authors have no potential conflicts of interest to disclose., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Paragangliomas (PGLs) arise from chromaffin cells of the extra-adrenal sites along the sympathetic and/or the parasympathetic chain. Majority of the PGLs occur in the abdomen. When retroperitoneal PGLs produce symptoms of compression or invasion of adjacent organs, they could be misdiagnosed as a tumor of adjacent organs. Here, we report a rare case of ureteral obstruction caused by an extra-adrenal retroperitoneal PGL that mimicked a ureteral tumor. A 46-year-old female presented with a 2-year history of hypertension. Routine laboratory tests were unremarkable. The computed tomography (CT) scan showed a hypervascular mass located in the left mid-ureteral section. Ureteropyelography revealed left ureteral obstruction at the level of L4 to L5 vertebrae and hydroureteronephrosis. Cystoscopy confirmed left ureteral obstruction. Based on the clinical and imaging findings, a left ureteral tumor was suspected preoperatively. The tumor was completely resected, and the involved segment of the ureter was removed. No significant enlarged lymph nodes were seen in the pelvic cavity and retroperitoneum. A histopathological examination of the tumor confirmed retroperitoneal PGL partly infiltrating the ureter. The patient's blood pressure returned to the normal range postoperatively. Our case demonstrates the importance of considering extra-adrenal PGL in the differential diagnosis of retroperitoneal tumors.

Background: Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other non-sarcomatous PRN are very rare and less familiar., Objectives: To evaluate the clinicopathologic and radiologic features of non-sarcomatous PRN, as well as the outcome of complete tumor resection (TR)., Methods: Retrospective data were collected on consecutive patients (June 2006 to January 2015) who underwent resection of retroperitoneal lesions at our department. Final pathology of non-sarcomatous PRN was included., Results: The study population included 36 patients (26% with PRN). PRN were neurogenic (17%), fat-containing (3%), and cystic (6%). The preoperative diagnosis was correct in only 28%. All patients underwent TR via laparotomy (72%) or laparoscopy (28%), for mean operative time of 120 ± 46 minutes. En bloc organ resection was performed in 11%. Complete TR was achieved in 97%. Intra-operative spillage occurred in 8%. Intra-operative, 90-day postoperative complications, and mortality rates were 11%, 36%, and 0%, respectively. The mean length of stay was 6.5 ± 5.5 days. The median overall survival was 53 ± 4.9 months., Conclusions: Familiarity with radiologic characteristics of PRN is important for appropriate management. Counter to STS, other PRN are mostly benign and have an indolent course. Radical surgery is not required, as complete TR confers good prognosis. Expectant management is reserved for small, asymptomatic, benign neoplasms.

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neuroendocrine tumors that express somatostatin receptors (SSTRs), a phenomenon that constitutes a basis for tumor imaging and treatment with somatostatin analogues and peptide receptor radionuclide therapy. We studied the immunohistochemical expression of SSTR1-5 in 151 primary tumors, including 14 metastasized and 16 SDHB-deficient tumors. SSTR2 and SSTR3 were most abundantly present in these tumors, whereas the tumors were mostly negative for SSTR1, SSTR4, and SSTR5. All metastasized PGLs (9/9), but only one metastasized PHEO (1/5), were strongly SSTR2 positive. SSTR3 expression was lower in metastatic tumors and tumors with a high proliferation rate (MIB1 ≥ 5%), but tumors had variable individual SSTR profiles. No correlation was found between SDHB status and SSTR expression. Our results suggest that new SSTR analogues with affinity for several SSTRs could be relevant for a subgroup of patients with these tumors. Better knowledge of tumor SSTR profiles could open the door for personalized imaging and treatment in the future. Because SSTR profiles vary in PHEOs and PGLs, individual analysis is required for each tumor., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Paragangliomas, defined as extra-adrenal chromaffin-cells tumors, are rarely located in the retro-peritoneum. Clinical presentation is similar to pheochromocytoma, and mainly depends on the producing character of the tumor. Positive diagnosis requires plasmatic and urinary hormonal assays. Radiological and isotopic explorations are essential before surgery. The only curative therapeutic strategy is surgical, associated to peri-operative prevention and monitoring of the frequently reported hemodynamic and cardiovascular disorders. Outcome depends of the metastatic character of the tumor, the presence of tumor remnant after surgical resection. Genetic study is recommended; the risk of recurrence and association to other neoplasm is more described in genetic forms., Material and Methods: Authors report 5cases of retro-peritoneal paraganglioma, operated in the department of urology of Hospital, between 2013 and 2017. Observations are about 2men and 3women. Clinical presentation is not always specific and paraganglioma may be discovered fortuitously. Two patients have been operated by coelioscopic approach, midline incision was performed in two other cases, and dorsal lumbotomy associated to a Rutherford-Morrison incision in a patient., Results: Two patients presented resistant hypertension and palpitation associated to suspect retro-peritoneal masses in imagery and elevated urinary methoxylated derivates before surgery. One patient was asymptomatic and the tumor was discovered in imagery. Per-operative hypertensive crisis and sinus tachycardia occurred in a case. The average follow-up period is 22.8months. Hypertension and palpitation disappeared after surgery. There was no recurrence for all the operated patients., Conclusion: Retro-peritoneal paraganglioma is a rare condition. Symptoms are not specific and clinical presentation may be similar to pheochromocytoma. Abdominal CT-scan and MRI, in association with MIBG scintigraphy are strongly evocative. Histological examination ensures diagnosis. Per-operative cardio-vascular disorders are to consider and must prevented and managed by anesthesiologists. Complete surgical resection is the only curative treatment and avoids recurrences., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Introduction: Paragangliomas are the most common extra-adrenal pheochromocytoma. Symptomatic and growing tumors demand surgical extirpation. Minimally invasive techniques allow surgeons to perform the procedure without wide exposure and mobilization of intra-abdominal organs. To our knowledge, we present the fourth case of robotic excision of a large retroperitoneal paraganglioma., Materials and Methods: An 18-year-old unmarried female presented with occipital headache for past 3 years. She also had palpitations and sweating. Ultrasound abdomen revealed a presacral mass of 4 × 4 × 3 cm, and on further evaluation, CECT abdomen characterized 5 × 3.2 × 4 cm well-defined heterogenous mass with intense enhancement in retroperitoneum near presacral region. Functional work-up revealed increased normetanephrine of 3723 mcg/dl and metanephrine of 273 mcg/dl. Patient underwent transperitoneal robot-assisted excision of the mass by fourth-generation da Vinci robotic system. After locating the mass of size 7 × 5 × 3 cm in the presacral region, dissection was carried out all around the mass. Intra-operative hypertensive episodes were managed by esmolol infusion. Feeding vessels were identified, clipped and cut. Resected mass was entrapped in specibag and retrieved intact., Results: Blood loss was around 50 ml and operative duration was 120 min. In the postoperative period, her blood pressure became normal. Patient was discharged on POD 4. Histopathology was confirmatory of paraganlioma., Conclusion: Presacral tumors, especially those growing anterior, can be successfully removed with the da Vinci robotic system, and this approach can become the standard for manipulation of this kind of tumor irrespective of its size and malignancy.

Non-functional retroperitoneal paragangliomas are rare tumors. They are often asymptomatic and can reach very large sizes. We report the case of a 49-year old woman with retroperitoneal tumor detected during CT scan examination performed to find the cause of non-specific abdominal pain. Malignant forms, more frequent than benign forms, show locoregional invasion and are characterized by delayed-onset metastases. The treatment of these tumors is based on the most complete degree of surgical resection, since prognosis depends on it. There is no consensus on the usefulness of complementary therapies which may nevertheless constitute a supportive treatment., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Multiple endocrine neoplasia type 2 (MEN2) is an autosomal-dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2-associated extra-adrenal pheochromocytomas (paraganglioma) are rare. A 59-year-old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10-mm left adrenal mass and a 30-mm retroperitoneal mass. He had paroxysmal elevations in blood pressure and in urinary metanephrine and vanillylmandelic acid values. Laparoscopic excision of the left adrenal gland and retroperitoneal mass was performed. We experienced an extremely rare case of composite paraganglioma-ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with MEN2B., (© 2016 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.)

Background: Due to variations in location and size, laparoscopic surgery for paraaortic or paracaval neurogenic tumors is challenging. We evaluated the surgical outcomes, as well as surgical tips and tricks., Methods: Between 2000 and 2015, 25 procedures were performed in 24 patients. One patient underwent second surgery due to the recurrence of paraganglioma. Data were collected on the tumor diameter, tumor location, perioperative outcomes, pathology, and last-known disease status. Regarding the operative procedures, we reviewed the operative charts or videos to identify surgical tips and tricks., Results: The median tumor diameter was 5.0 cm (range 1.5-10). The tumor location was suprahilar in 10, hilar in 6, and infrahilar in 9 cases. Regarding the approach, a transperitoneal approach was selected in 24 cases and retroperitoneal approach in 1. The median operative time and blood loss were 208 min (range 73-513) and 10 mL (range 0-1020), respectively. No patient required blood transfusion or conversion to open surgery. Pathological examination revealed paraganglioma in 12, ganglioneuroma in 7, and schwannoma in 6 cases. At the last follow-up, 23 patients were free of disease, while one patient developed metastatic multiple recurrence of paraganglioma 54 months after the second laparoscopic surgery. A review of the surgical records revealed several tips and tricks, including taping the vena cava/renal vein (n = 2) being helpful for detaching a retrocaval tumor from these great vessels, or rotating the kidney to provide a favorable operative view of tumors behind the renal hilum (n = 2). In recent cases, 3D-CT was helpful for preoperative planning., Conclusions: Laparoscopic resection of paraaortic or paracaval neurogenic tumors is feasible in experienced hands. Surgeons should be familiar with detaching maneuvers around great vessels and the mobilization of adjacent organs. Careful preoperative planning is mandatory.

We report a case of extra-adrenal retroperitoneal paraganglioma (RP) with extensive duodenal invasion and tumor thromboses both in the right testicular vein and in the inferior vena cava (IVC). Because there was rigid adherence between the RP and the abdominal aorta, pancreatoduodenectomy with replacement of the IVC and aorta was performed for complete surgical resection. In the present case, both the mode of progression of the RP and the surgical approach were extremely rare.

Objective: To assess the feasibility and safety of a modified retroperitoneoscopic technique in supine position for the primary tumors located below the level of renal pedicle., Materials and Methods: In this retrospective study, 27 patients with primary retroperitoneal tumors located below the level of renal pedicle were recruited from January 2009 till now. The mean age of patients was 49.0 years (36-66 years). The average body mass index was 21.1 ± 1.4 kg/m(2). The retroperitoneoscopic resections were performed in supine position for all patients. The perioperative outcomes were collected and analyzed., Results: All cases were performed by the modified technique without converting to open surgery. The mean operation time was 98.3 ± 12.8 min, with a mean estimated blood loss of 94.4 ± 42.5 mL. There were four patients suffering from intraoperative complications, one with the inferior vena cava (IVC) injury, one with the lumbar vein injury, and other two with the peritoneum breach. Postoperatively, the median bowel recovery time was 2 days (range: 1-3 days), and the mean length of stay after operation was 2.9 ± 1.0 days. Additionally, compared with the tumors located around the aorta (region B), a longer operation time was needed with tumors located around the IVC (region A) (A vs B: 105.8 ± 10.2 vs 91.2 ± 14.4, p = 0.02). Histopathology confirmed paraganglioma in 5 cases, schwannoma in 10 cases, and ganglioneuroma in 12 cases. After follow-up of 12-72 months, no recurrences have been identified., Conclusion: Our modified retroperitoneoscopic technique in supine position was proven to be safe and effective for the treatment of primary retroperitoneal tumors, especially suitable for the tumors located below the level of the renal pedicle.

Varicocele, the most important identifiable pathological cause of male infertility, is rarely observed on the right side. In isolated cases of right-sided varicocele, the presence of retroperitoneal masses compressing the inferior vena cava should be considered by further investigation. The retroperitoneal masses that are detected tend to grow rapidly due to their location and may be very large at the time of diagnosis. Soft tissue sarcomas are the most common cause of retroperitoneal masses, while paragangliomas are rare causes. Retroperitoneal paragangliomas should be diagnosed via detection of symptoms caused by hormonal activity, observation of the compression effect of the mass, or incidentally during imaging studies., Similar Cases Published: This study presents the first case of retroperitoneal paraganglioma in a 41-year old man as isolated right-sided varicocele that has been described in the published reports.

Objectives: To explore the feasibility and safety of retroperitoneal laparoscopic resection of paraganglioma (RLPG) in a large study population., Methods: In a six-year period, 49 patients with primary retroperitoneal paragangliomas (PG) underwent retroperitoneal laparoscopic surgery in a single center. Medical records were reviewed, and collected the following data, which were clinical characteristics, perioperative data (operative time, estimated blood loss, intraoperative hemodynamic changes, intraoperative and postoperative complications, and open conversions), and follow-up data (recurrence or distant metastases)., Results: All PGs were removed with negative tumor margin confirmed by postoperative histopathology. The operative time of RLPG was 101.59±31.12 minutes, and the estimated blood loss was 169.78±176.70ml. Intraoperative hypertensive and hypotensive episodes occurred in 25 cases and 27 cases, respectively. Two open conversions occurred. Two intraoperative complications occurred but were successfully managed endoscopically. Postoperative complications were minor and unremarkable. No local recurrence or distant metastasis were observed during the follow-up period., Conclusions: Our experience indicates the feasibility and safety of resection of PGs in a relatively large study population.

Paragangliomas are rare tumors arising from extra-adrenal chromaffine tissues. The diagnosis of non-functional retroperitoneal paraganglioma and its surgical management can be difficult. We report a case of a retroperitoneal paragangliomaof an unusual localization that renders the surgery more challenging. A 40 year-old woman presented to our department with a four-month history of upper quadrant pain with no vomiting, no fever, nor jaundice. Physical examination was normal. Ultrasonography showed a retro duodenal homogenous mass and computed tomographyscan showed a well-circumscribed round mass of heterogeneous density, which was in close contact with the aorta and the left kidney vein. Laboratory tests were normal. The patient underwent surgical management. The surgical exploration found a retroperitoneal tumor that was encapsulated and showing intimate contact with the abdominal aorta. We performed a complete resection of the tumor. Histological examination of the surgical specimen revealed a paraganglioma. The post operative course was uneventful. Paragangliomas are rare tumors. They can be asymptomatic for a long time and thus be diagnosed at late stage. A follow-up of patients is then essential. Surgical treatment is the only radical treatment and should be performed even in paragangliomas in close contact with the great vessels.

Material and Methods: Robot-assisted operations for non-organ retroperitoneal tumors were performed in 20 patients. The biggest tumor's diameter was 160 mm, the smallest--32 mm, mean value was 79.1±37.8 mm. According to morphological investigation data lymphangioma was diagnosed in 4 (20%) patients, lipoma--in 3 (15%) cases, peritoneal cyst--in 2 (10%) patients. Solitary lymphatic node, teratoma, leiomyosarcoma, accessory stomach, bronchogenic cyst, adenogenous cancer, ureterocele, megaureter, schwannoma, leiomyoma, malignant paraganglioma were observed in 1 (5%) case respectively., Results: Surgery duration was 138±55.9 min. Blood loss was 69±112.7 ml (range 0-500 ml). Intraoperative complications (bleeding) were diagnosed in 3 (15%) patients. There were no conversions. Robot-assisted variant has been demonstrated in case of non-organ retroperitoneal tumors with diameter not more than 100-120 mm.

Pheochromocytomas are rarely seen cathecolamine-secreting neuroendocrine tumors. Diagnosis, treatment, and prognosis of this rarely seen condition have been discussed. In this study, a retroperitoneal paraganglioma with a diameter of 27 mm was detected in a 29-years old nephrectomized male patient. In our case, in reevaluations performed after definitive establishment of the diagnosis, any abnormal radiological signs suggesting a metastatic lesion were not detected. During the postoperative period the patient was referred to the clinics of endocrinology, and oncology for medical follow-up. In the differential diagnosis of retroperitoneal mass lesions, pheochromocytoma/paraganglioma should not be forgotten. Besides, for malignant tumors chemoradiotherapy, and for benign functional tumors medical treatment should be applied.

Objective: Paraganglioma (PGL) is a rare type of tumor that arises from the extra-adrenal paraganglia. A PGL tumor hypersecretes catecholamines and causes symptoms identical to those in patients with hyper-functioning adrenal pheochromocytoma (PCC). In this study, we compared the surgical outcome of laparoscopic surgery, including laparoendoscopic single-site (LESS) surgery, in patients with PCC and patients with retroperitoneal solitary PGL., Methods: The records of 49 patients with PCC and 9 patients with unilateral retroperitoneal PGL at our institution from January 2001 to March 2013 were retrospectively reviewed. Multiple tumors, tumors suspected of being malignant preoperatively, and tumors operated on using a retroperitoneal approach were excluded from the study., Results: Each group was equivalent with respect to patient background, hemodynamic variables, and preoperative biochemical assessments, including plasma catecholamine levels and catecholamine levels in 24-hour urine samples. The mean operative time was significantly longer in the PGL group (149.4 ± 56.5 minutes v 189.8 ± 44.9 minutes, P=0.019). In univariate and multivariate analyses, tumor size ≥ 50 mm and PGL were statistically significant factors that predicted prolonged operative time. Intraoperative hypotension occurred in 15 patients in the PCC group and in 8 patients in the PGL group, and the difference was statistically significant (P=0.002). One postoperative complication in the PCC group and two postoperative complications (Clavien-Dindo grade II or higher) in the PGL group were observed, and the difference was statistically significant (P=0.012). Twenty-two patients in this series underwent LESS surgery (PCC: n=19; PGL: n=3), and there was no statistically significant difference in the perioperative outcomes between the two groups., Conclusions: The present results demonstrate that the operation for solitary extraperitoneal PGL required a longer operative time and had more hypotensive episodes and higher postoperative morbidity than the PCC group. Though the perioperative outcome of LESS surgery for PGL is comparable to that of PCC, we should treat the patients with PGL accordingly.

Various types of neurogenous tumours occur in the retroperitoneum. Hormonally inactive tumours are often asymptomatic and their diagnosis is mostly accidental. Authors are presenting their recent experience with three cases. They discuss diagnostic options and the therapy of retroperitoneal schwannoma, ganglioneuroma and paraganglioma.

Reports of a composite paraganglioma (PG) and ganglioneuroma (GN) in the retroperitoneum are rare. In the present case, dynamic computed tomographic (CT) findings showed a 30 × 22 × 20 mm tumor that was located in the retroperitoneum and which was dissociated from pancreatic tissue and the left adrenal gland. The markedly reddish tumor showed a clear margin and central multicystic changes on the cut surface. The tumor was composed of two major components, the PG and the GN. The paraganglionic cells in the PG component, which were arranged in a nested pattern, occupied the main and central part of the tumor. Both ganglionic cells and Schwann cells in the GN were located in a unorganized pattern in the periphery. The paraganglionic cells exhibited a Zellballen pattern, which consisted of an association of edematous vascular-rich stroma and focal hemorrhage, resulting in multicystic changes. These centrally located tumor cells were pleomorphic in part and did not have mitotic figures. In the periphery, Schwann cells, which were arranged in an obscure and fascicular pattern that was intermingled with large ganglionic cells, were located adjacent to the PG component with a mostly sharp margin. With higher magnification, the border was not as sharp, as revealed especially with chromogranin-A immunostain, in which both the PG and GN components were focally intermingled with each other. The histogenesis of the composite PG and GN was thought to be extraadrenal neural crest cells in the retroperitoneum because the tumor was not located in the adrenal gland or the Zuckerkandl organ, according to the CT findings. The immunohistochemical findings of this rare case of a composite PG and GN in the retroperitoneum are reported with a focus on the peculiar arrangement of these two components.

Paragangliomas and pleomorphic undifferentiated sarcoma/malignant fibrous histiocytomas are examples of uncommon tumours in retroperitoneum. Where malignant fibrous histiocytoma is to be treated aggressively to save the life, long-term follow-up after surgery is the basic management for paraganglioma. Hence an inappropriate diagnosis of malignant fibrous histiocytoma in place of a paraganglioma which in most cases behave as benign tumour, can lead to a significant complication related to postoperative therapy. Here in this case a patient of retroperitoneal tumour having discrepancies between pre-operative cytological and postoperative histopathological diagnosis is reported. After careful review of clinical, radiological and histopathological features, the appropriate diagnosis of paraganglioma was rendered and the patient was kept for long-term close follow-up instead of aggressive therapy. The appropriate clinicopathologic correlation of the case by a group of experienced oncologists and pathologists revealed the diagnostic enigma and offered the appropriate management which may otherwise lead to iatrogenic complications.

Unlabelled: The Authors report a case of a voluminous abdominal paraganglioma and a review of the literature on the surgical management of paragangliomas., Case Report: A 58 years old woman was admitted to our Institution with acute endocrine impairment and simultaneous intestinal necrosis resulting from the mass effect of the tumor on the mesenteric vessels. Clinical and biochemical preoperative work up posed the suspect of paraganglioma. Previous intensive care treatment, the patient, underwent surgical resection of the tumor and left hemicolectomy, with immediate remission of both endocrine and abdominal syndromes. Histological examination confirm the preoperative clinical hypothesis. Paragangliomas are rare tumors of the extra-adrenal chromaffin tissue. A paraganglioma may be discovered in the absence of any symptom. When symptomatic, the clinical findings are related to the hypersecretion of catecholamines or to the compression of several anatomical structures from a growing mass. Combined acute syndromes of hormonal impairment and contemporaneous mass effect, as in our case, are exceptional. The diagnosis of the secreting forms may be obtained on the basis of biochemical dosage of serum and urinary catecholamines and metanephrines. The assessment of malignancy for paragangliomas is not always feasible. Several imaging, cytological and histological parameters have been proposed as predictors of malignancy, but the only element widely accepted is the existence of distant metastasis. Surgery represents the treatment of choice for paragangliomas. Benign forms, catecholamine secreting forms and tumors compressing neighbouring anatomical structures can be treated surgically with success. In general, an accurate follow-up after complete resection must be considered.

Retroperitoneal recurrence of clear cell renal cell carcinoma (CCRCC) after surgical resection is often investigated by needle biopsy and frequently requires immunohistochemistry to distinguish from other lesions with similar histology. This study explores the diagnostic utility of a panel of immunohistochemical markers and emphasizes potential pitfalls in dealing with this differential diagnosis. A tissue microarray with 1 mm tissue cores was constructed to include 21 CCRCC, 19 adrenocortical lesions, and 15 retroperitoneal or mediastinal paragangliomas. Triplicate cores were used for each case. The tissue microarray was then immunostained with epithelial, RCC, adrenocortical, and neuroendocrine markers. Pancytokeratins AE1/3, CAM5.2, and epithelial membrane antigen were positive in 52.4%, 66.7%, and 61.9% of CCRCC cases. Three (14.2%) CCRCC cases were negative for all 3 epithelial markers. AE1/3 and epithelial membrane antigen were negative in all adrenocortical lesions and paraganglioma cases, whereas CAM5.2 was positive in 78.9% of adrenocortical lesions and 6.7% of paragangliomas. RCC markers, including RCC Ag, CA9, and CD10, were positive in 76.2%, 85.7%, and 100% of CCRCC cases and were negative in all adrenocortical lesions and paragangliomas. Calretinin and Melan-A were positive in 100% and 94.7% of adrenal, 0% and 14.3% of CCRCC, and 26.7% and 26.7% of paragangliomas. Epithelial markers may be entirely negative in CCRCC, whereas pancytokeratin CAM5.2 is often positive in adrenocortical lesions. Furthermore, neuroendocrine markers are frequently positive in adrenocortical lesions. Therefore, a panel of, rather than single, epithelial, "CCRCC-specific," adrenocortical and neuroendocrine markers should be applied in the differential diagnosis of CCRCC, adrenocortical lesions, and paragangliomas.

Paraganglioma is a rare neuroendocrine tumor which arises from extra adrenal paraganglionic cells of the autonomic nervous system. We report a case of a giant retroperitoneal paraganglioma. A 43-year-old man referred to our hospital for further examination of a retroperitoneal mass. The patient had neither familial nor past medical history. The blood and urine test, laboratory examinations including cathecolamines, were unremarkable. Abdominal computed tomography showed an enhancing solid mass 13 cm in diameter on the left kidney. The invasion to the left kidney was suspected. Angiography showed the left renal, splenic, middle suprarenal and left inferior diaphragmatic artery feeding the tumor. The splenic and left inferior diaphragmatic artery were embolized before surgical treatment. The tumor, left kidney, adrenal gland and spleen were surgically resected. Histological examination revealed extra-adrenal paraganglioma, and there was no invasion of the tumor to the left kidney, adrenal gland and spleen. The patient has now survived more than 10 months following the surgery without tumor recurrence.

Paraganglioma, a sporadically occurring rare tumor should be included in the differential diagnosis of retroperitoneal tumors, such as malignant lymphomas, gastrointestinal stromal tumors, sarcoma and carcinoma of unknown primary site. A 58-year-old Japanese woman presented with a large retroperitoneal tumor detected by ultrasonography (US). She had no medical history of hypertension. Computed tomography showed a mass, 7 cm in diameter, located between the pancreas and the inferior vena cava. It was unclear whether the mass originated from the duodenum or the mesentery. Endoscopic ultrasonography (EUS) demonstrated a large solid paraduodenal mass. Doppler US revealed sparse vascularity in the tumor. With the differential diagnosis of retroperitoneal tumor, we carried out EUS-FNA. At the time of the third needle puncture, transient severe hypertension was noted, with a blood pressure measurement of 269/130 mmHg. Data obtained from urine and blood examinations after EUS-fine-needle aspiration indicated a diagnosis of paraganglioma.

Paragangliomas are very rare tumors arising from extraadrenal chromaffin cells. Clinical presentation of benign retroperitoneal nonfunctional paraganglioma is unspecific. Symptoms may occur when tumor attains a remarkable size or when complications arise. This article reports a case of nonfunctional retroperitoneal paraganglioma as a cause of acute upper gastrointestinal hemorrhage which represents the unusual urgent clinical manifestation of these tumors. The presented case emphasizes the necessity to include extraadrenal paraganglioma in the differential diagnosis in all patients with retroperitoneal mass found even in the presence of at first appearance non-related emergency condition like acute upper gastrointestinal bleeding.

Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.

Objective: To study the detailed cytologic features of paragangliomas (PGs) and delineate features helpful in predicting behavior., Study Design: We performed retrospective analysis of 12 cases cytologically suggested as PG over a period of 10 years. Smears were reviewed for cellularity, pattern, shape, cytoplasm, nuclear features and background. Cytologic diagnosis was correlated with clinicoradiologic features, histologic features and immunocytochemistry., Results: Patient age ranged from 12 to 65 years, with male preponderance; they presented with a mass lesion. Sites involved were head and neck (5), retroperitoneum (5) and mediastinum (2). Smears were cellular; cells were round to oval, plasmacytoid and spindled and seen as scattered, in clusters, acinar and perivascular pattern. The most consistent feature was presence of pink granules. Pleomorphism was present in all but 1 case and less prominent in tumors with metastasis. Mitoses were more frequently seen in malignant cases., Conclusion: Cytologic features of PG are diverse. Although there are certain main features, they are not diagnostic unless combined with data on site, histology and ancillary studies. Presence of granules, mild pleomorphism, frequent mitoses and necrosis needs careful evaluation for malignancy potential and warrants close follow-up.

Paragangliomas are rare neuroendocrine tumors that arise from hyperplastic paraganglionic cells and occur in or near the ganglia of the autonomic nervous system. Paragangliomas are classed as either functional or nonfunctional based on production of catecholamines. We report a case of retroperitoneal nonfunctional paraganglioma, which was successfully resected. A 26-year-old man presented with complaints of a mass palpable in his left lateral region. Blood and urine studies, including catecholamines, were within normal limits. Computed tomography and magnetic resonance imaging showed a solid cystic mass in the left retroperitoneal space without any distant metastasis. On exploration, there was an encapsulated, vascular mass 10 cm in diameter. There was no pedicle and no connection of the tumor to the abdominal or retroperitoneal organs. Complete excision of the tumor was performed smoothly. The postoperative period was uneventful. Histological findings were of paraganglioma, and there was no surgical margin. The patient did not receive any further treatment. Nine months after surgery, there were no signs of tumor recurrence.

A 41-year-old man was referred to our hospital for a left renal mass detected by screening computed tomography for hypertension. He had undergone surgery for left pheochromocytoma at 16 years of age in 1981, and the last follow-up was in 1986. Although a 131I-metaiodebenzylguanidine (MIBG) scintigram did not show abnormal uptake, elevated levels of serum cathecholamines were demonstrated. The left renal mass positioned in the upper pole and inside of the kidney had invaded the pancreas, colon and spleen. Surgical resection was done under a diagnosis of recurrent pheochromocytoma, and the tumor was removed with other organs, including normal left adrenal gland that was confirmed histologically. Histologically and immunohistochemically, the resectioned tumor was invasive malignant paraganglioma and diagnosed as the local recurrence or metastasis of previously resectioned retroperitoneal paraganglioma, which was believed to have grown slowly.

Primary and recurrent retroperitoneal tumors can involve the aortoiliac vasculature. They are often considered inoperable or incurable because of the locally advanced nature of the disease or the technical aspects involved in safely resecting the lesion. Safe resection of these lesions requires experience and extensive preoperative planning for success. A retrospective database review of 76 patients with retroperitoneal tumors identified tumors involving major vascular structures in the abdomen and pelvis undergoing resection of tumor en bloc with the aortoiliac vasculature. Preoperative planning and intraoperative technical maneuvers are reviewed. Patients were followed until time of this report. Four patients with retroperitoneal tumors involving the aortoiliac vessels underwent surgery: two patients with sarcoma (one primary and one recurrent), one with metastatic renal cell carcinoma, and one with a paraganglioma. All patients had resection of the aorta and vena cava or the iliac artery and vein. Arterial reconstruction (anatomic or extra-anatomic) was performed in all cases. The patient with renal cell carcinoma also required venous reconstruction to support a renal autotransplant. Veno-venous bypass was required in one patient. Local control was achieved in 3 of 4 cases. Surgery for retroperitoneal tumors involving major vascular structures is technically feasible with appropriate planning and technique. Multiple disciplines are required, including general surgical oncology, vascular surgery, and possibly, cardiothoracic surgery.

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior para-aortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131MIBG (metaiodobenzylguanidine) scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.

A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 x 32 x 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.