Hallazgo casual de un paraganglioma paraaórtico / An incidentally discovered paraaortic paraganglioma

Introducción: los paragangliomas son tumores neuroendocrinos derivados de células cromafi nes. Son entidadesmuy infrecuentes que se localizan típicamente en el cuello o en la base del cráneo. presentamos el caso de unparaganglioma paraaórtico, una ubicación inusual que cuenta con escasos reportes en la literatura. Caso clínico: mujer de 78 años que presenta una masa retroperitoneal hallada de forma casual durante el estudiopreoperatorio de una neoplasia ginecológica. ante la sospecha de un tumor del estroma gastrointestinal o unschawnnoma retroperitoneal, se indica su extirpación quirúrgica. Durante la operación se objetiva una masa muyvascularizada adherida a la cara anterior de la aorta, cuya resección provoca en la paciente un cuadro de crisishipertensiva y taquicardia asociadas a inestabilidad hemodinámica, que cede tras completar su exéresis. el estudioanatomopatológico posterior confi rma la sospecha diagnóstica intraoperatoria de paraganglioma paraaórtico. Discusión: los paragangliomas localizados en el abdomen suelen ser productores de catecolaminas y puedendesarrollar clínica adrenérgica de forma espontánea o inducida por fármacos, ejercicio o manipulación quirúrgica,como sucedió en nuestro caso. Un diagnóstico certero es clave para planificar un tratamiento adecuado quepermita reducir las posibles complicaciones en este tipo de pacientes.(AU)

Introduction: paragangliomas are neuroendocrine tumours derived from chromaffin cells. they are very rareentities that are typically located in the neck or at the base of the skull. We present the case of a para-aortic para-ganglioma, an unusual location that has few reports in the literature. Case report: a 78-year-old woman with a retroperitoneal mass found incidentally during the preoperative study fora gynecological neoplasm. Given the suspicion of a gastrointestinal stromal tumour or a retroperitoneal schawn-noma, surgical removal of it was indicated. Intraoperatively, a highly vascularized mass adhered to the anteriorface of the aorta was observed, whose resection caused a hypertensive crisis and tachycardia associated withhemodynamic instability, which subsided after completing the exeresis. the subsequent anatomopathologicalstudy confirmed the intraoperative diagnostic suspicion of paraaortic paraganglioma. Discussion: paragangliomas located in the abdomen are usually catecholamine producers and can develop adr-energic symptoms spontaneously or induced by drugs, exercise, or surgical manipulation, as occurred in our case.an accurate diagnosis is the key to planning an appropriate treatment that allows reducing possible complicationsin this type of patient.(au)

Co-occurrence of pheochromocytoma and paraganglioma of the organ of Zuckerkandl resected simultaneously by laparoscopy: a rare case report and literature review.

Pheochromocytomas (PHEOs) and paragangliomas are generally grouped as rare chromaffin cell tumors. The co-occurrence of PHEOs and paragangliomas of the organ of Zuckerkandl (POZ) is extremely rare. The most common symptom of pheochromocytoma-paraganglioma (PPGL) is hypertension, and open surgery is still recommended for the treatment of large PPGLs. Herein, we report a case of a successful simultaneous laparoscopic resection of a large PHEO accompanied by POZ in a 40-year-old man with normal blood pressure. DNA analysis revealed a mutation in the succinate dehydrogenase subunit B in both the PHEO and the POZ. To the best of our knowledge, this is the first report of tumors occurring simultaneously in these two locations. We believe that the co-occurrence of PHEO and POZ is extremely rare, and the possibility of PPGL cannot be ruled out in patients with normal blood pressure. The decision to perform laparoscopic surgery remains questionable for patients with a large PHEO and POZ. In addition, a genetic examination should be performed to identify the existence of PPGL-related inherited syndromes.

Paraaortic dissection in "total mesopancreas excision" and "mesopancreas-first resection" pancreaticoduodenectomies for pancreatic cancer: Useless, optional, or necessary?A systematic review.

The mesopancreas does not have well-defined boundaries but is continuous and connected through its components with the paraaortic area. The mesopancreatic resection margin has been indicated as the primary site for R1 resection after PD in pancreatic head cancer and total mesopancreas excision has been proposed to achieve adequate retropancreatic margin clearance and to minimize the likelihood of R1 resection. However, the anatomy of the mesopancreas requires extended dissection of the paraaortic area to maximize posterior clearance. The artery-first surgical approach has been developed to increase local radicality at the mesopancreatic resection margin. During PD, the artery-first approach begins with dissection of the connective tissues around the SMA. However, the concept of the mesopancreas as a boundless structure that includes circumferential tissues around the SMA, SMV, and paraaortic tissue highlights the need to shift from artery-first PD to mesopancreas-first PD to reduce the risk of R1 resection. From this perspective the "artery-first" approach, which allows for the avoidance of R2 resection risk, should be integrated into the "mesopancreas-first" approach to improve the R0 resection rate. In total mesopancreas excision and mesopancreas-first pancreaticoduodenectomies, the inclusion of the paraaortic area and circumferential area around the SMA in the resection field is necessary to control the tumour spread along the mesopancreatic resection margin rather than to control or stage the spread in the nodal basin.

Open abdominal aortic aneurysm repair with incidental finding of an extra-adrenal pheochromocytoma of the organ of Zuckerkandl.

OBJECTIVES: Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The most common location of these tumors is within the adrenal medulla. Extra-adrenal pheochromocytomas (EAPs) may occur in any portion of the paraganglion system. The most common location of EAPs is at the organ of Zuckerkandl, which is a collection of chromaffin cells near the origin of the inferior mesenteric artery. METHODS: We present a case of an EAP of the organ of Zuckerkandl incidentally discovered and resected during urgent open repair of a symptomatic 6.7-cm juxtarenal abdominal aortic aneurysm (AAA). RESULTS: The patient underwent successful open surgical repair of a juxtarenal AAA and resection of the pheochromocytoma. CONCLUSIONS: Concomitant pheochromocytomas and abdominal aortic aneurysms are rare, with a small number described in the literature. We describe the case of a simultaneous EAP of the organ of Zuckerkandl and AAA repair. This case demonstrates that these lesions can be safely resected in the same setting as AAA repair.

Para-aortic haemangioma mimics paraganglioma on MRI.

Here, we report a case of a 70-year-old man referred for an incidentally discovered left renal lesion with peri-aortic lymphadenopathy following a CT scan for back pain. A follow-up MRI scan demonstrated a Bosniak IIF left renal cyst and a T2-hyperintense para-aortic lesion concerning for extra-adrenal paraganglioma (EAP). [131I] Metaiodobenzylguanidine scintigraphy of the para-aortic lesion and urine catecholamines were equivocal. The mass was resected via a robotic approach. Histological examination revealed a haemangioma. Haemangiomas are benign vascular tumours frequently identified on imaging of the liver. Intra-abdominal haemangiomas outside of the liver, however, are rare and may have imaging characteristics that mimic EAP.

Paraganglioma of the organ of Zuckerkandl and FH gene mutation.

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Laparoscopic resection of a large clinically silent paraganglioma at the organ of Zuckerkandl: a rare case report and review of the literature.

BACKGROUND: Large paraganglioma of the Zuckerkandl organ (POZ) is extremely rare. The patient can occasionally be paucisymptomatic, further obscuring the diagnosis and carrying high mortality. Recommended treatment for large paraganglioma (PGL) is open surgical removal. We report a case of successful laparoscopic resection of a large POZ with normal blood pressure in a 45-year-old man. CASE PRESENTATION: A 45-year-old man was hospitalized because of hyperglycemia. Computed tomography of the abdomen and the serum and urinary catecholamine levels confirmed the diagnosis of large POZ. But his blood pressure was normal and he underwent laparoscopic tumor excision successfully. During 6 months follow-up after laparoscopy, serum and urinary catecholamines were normal but glycaemia remained high level. DNA analysis of the succinate dehydrogenase complex subunits B (SDHB) and SDHD revealed no mutation. CONCLUSIONS: POZ is an unusual mass and preoperative diagnosis can be difficult in clinically silent cases. PGL cannot be excluded in patients with normal blood pressure. Even a large POZ can be excised laparoscopically by following proper techniques.

Para-aortal Ganglion Mimicking a Lymph Node Metastases on 68Ga-PSMA PET/CT.

PSMA-based nuclear medicine imaging impacts increasingly the clinical decision process in prostate cancer patients. A well-known PSMA pitfall is uptake into autonomic ganglia. The intensity of uptake, the shape, and the exact location of the correlating structure in CT are supposed to aid discriminating between ganglia and lymph node metastases. In this patient, we found intense uptake in a nodular shaped para-aortal soft tissue lesion suspicious of a lymph node metastases at staging as well as restaging. After secondary resection, the lesion was histologically proven an autonomic ganglion with intense PSMA expression.

Clinical manifestations of Pacak-Zhuang syndrome in a male pediatric patient.

We report an index case of a male patient who presented with all clinical manifestations of Pacak-Zhuang syndrome, including early-age polycythemia, multiple pheochromocytomas/paragangliomas, duodenal somatostatinoma, and ocular findings. Sequencing analysis detected an EPAS1 mutation in all tumors tested, but not in the germline.

Para-aortic lymph node involvement revisited in the light of the revised 2018 FIGO staging system for cervical cancer.

OBJECTIVE: This dual-institutional, retrospective study aimed to determine the clinicopathological risk factors for para-aortic lymph node (LN) metastasis among women who underwent radical hysterectomy with systematic pelvic and para-aortic lymphadenectomy for 2009 FIGO stage IB1-IIA2 cervical cancer. METHODS: Institutional cervical cancer databases of two high-volume gynecologic cancer centers in Ankara, Turkey were retrospectively analyzed. Women with 2009 FIGO stage IB1-IIA2 cervical cancer that had undergone radical hysterectomy with pelvic and para-aortic lymphadenectomy between January 2006 and December 2018 were included in the study. Patient data were analyzed with respect to para-aortic LN involvement and all potential clinicopathological risk factors for para-aortic LN metastasis were investigated. RESULTS: A total of 522 women met the inclusion criteria. Pelvic LN metastasis was detected in 190 patients (36.4%), para-aortic LN metastasis in 48 patients (9.2%), isolated para-aortic LN metastasis in 4 (0.8%), and both pelvic and para-aortic LN metastasis in 44 (8.4%) women, respectively. The independent risk factors identified for para-aortic LN involvement included parametrial invasion (odds ratio [OR]: 3.57, 95% confidence interval [CI]: 1.65-7.72; p = 0.001), metastasized pelvic LN size > 1 cm (OR: 4.51, 95% CI: 1.75-11.64; p = 0.002), multiple pelvic LN metastases (OR: 3.83, 95% CI: 1.46-10.01; p = 0.006), and common iliac LN metastasis (OR: 2.97, 95% CI: 1.01-8.68; p = 0.04). A total of 196 (37.5%) patients exhibited at least one risk factor for para-aortic nodal disease. CONCLUSION: Parametrial invasion, metastasized pelvic LN size > 1 cm, multiple pelvic LN metastases, and common iliac LN metastasis seem to be independent predictors of para-aortic LN involvement.

[Paraganglioma of Zuckerkandl's organ. Report of two pediatric cases]. / Paraganglioma del órgano de Zuckerkandl: reporte de dos casos pediátricos.

INTRODUCTION: Pheochromocytoma is an infrequent neuroendocrine tumor, originated from neural crest cells. 10% of them are extra adrenal, located at sympathetic nodules and are known as paragangliomas. The most common place is the Zuckerkandl organ, 10 to 26% are malign and has a mortality around de 26% of the cases. CASE REPORT: We present two cases of teenagers with Zuckerkandl's organ paraganglioma, who debut with high blood pressure, diaphoresis and dyspnea. Complete tumor resection was performed in both cases, during which they presented hipertensive crisis and, after vascular pedicle ligation, hypotension. CONCLUSIONS: Zuckerkandl's organ paraganglioma is a rare pathology in pediatric population, therefore represents an important diagnostic and therapeutic challenge. Surgery differs from other tumors because of catecholamine secretion that produces hemodynamic changes and demands prompt and accurate management from surgeon and anesthetist.

INTRODUCCION: El feocromocitoma es un tumor neuroendocrino infrecuente. El 10% son extraadrenales en los ganglios simpáticos, conocidos como paragangliomas. El sitio más común es el órgano de Zuckerkandl. Son malignos en un 10 a 26% con una mortalidad del 26%. REPORTE DE CASOS: Presentamos dos casos de paraganglioma del órgano de Zuckerkandl en adolescentes, que debutan con hipertensión arterial, diaforesis y disnea. Se realizó resección completa del tumor; durante ambas cirugías presentaron crisis hipertensiva y posterior a la ligadura del pedículo vascular del tumor presentaron hipotensión, con normalización progresiva de las cifras tensionales en el postoperatorio. CONCLUSIONES: El paraganglioma del órgano de Zuckerkandl es una patología infrecuente en la población pediátrica, representa un importante reto diagnóstico y terapéutico. La cirugía difiere de las de otros tumores ya que la secreción de catecolaminas genera cambios hemodinámicos transoperatorios que exigen al cirujano y anestesiólogo un manejo rápido, eficaz y oportuno.

Paraganglioma del órgano de Zuckerkandl: reporte de dos casos pediátricos / Paraganglioma of Zuckerkandl’s organ. Report of two pediatric cases

Introducción: El feocromocitoma es un tumor neuroendocrino infrecuente. El 10% son extraadrenales en los ganglios simpáticos, conocidos como paragangliomas(1,2). El sitio más común es el órgano de Zuckerkandl. Son malignos en un 10 a 26%(4) con una mortalidad del 26%(5). Reporte de casos: Presentamos dos casos de paraganglioma del órgano de Zuckerkandl en adolescentes, que debutan con hipertensión arterial, diaforesis y disnea. Se realizó resección completa del tumor; durante ambas cirugías presentaron crisis hipertensiva y posterior a la ligadura del pedículo vascular del tumor presentaron hipotensión, con normalización progresiva de las cifras tensionales en el postoperatorio. Conclusiones: El paraganglioma del órgano de Zuckerkandl es una patología infrecuente en la población pediátrica, representa un importante reto diagnóstico y terapéutico. La cirugía difiere de las de otros tumores ya que la secreción de catecolaminas genera cambios hemodinámicos transoperatorios que exigen al cirujano y anestesiólogo un manejo rápido, eficaz y oportuno

Introduction: Pheochromocytoma is an infrequent neuroendocrine tumor, originated from neural crest cells. 10% of them are extra adrenal, located at sympathetic nodules and are known as paragangliomas(1,2). The most common place is the Zuckerkandl organ, 10 to 26% are malign(4) and has a mortality around de 26% of the cases(5). Case report: We present two cases of teenagers with Zuckerkandl’s organ paraganglioma, who debut with high blood pressure, diaphoresis and dyspnea. Complete tumor resection was performed in both cases, during which they presented hipertensive crisis and, after vascular pedicle ligation, hypotension. Conclusions: Zuckerkandl’s organ paraganglioma is a rare pathology in pediatric population, therefore represents an important diagnostic and therapeutic challenge. Surgery differs from other tumors because of catecholamine secretion that produces hemodynamic changes and demands prompt and accurate management from surgeon and anesthetist

Lack of Survival Benefit of Para-Aortic Lymphadenectomy in Advanced Cervical Cancer.

INTRODUCTION: The presence of positive para-aortic lymph nodes in advanced cervical cancer remains the most important prognostic factor for survival and also defines the treatment. Our aim was to define the influence of staging para-aortic lymphadenectomy in patients' survival. MATERIAL AND METHODS: The medical records of 74 patients with advanced cervical cancer (FIGO IIB-IVA) were reviewed. In 31 patients (41.9%), the assessment of lymph nodes was performed with imaging test (group 1) and in 43 (58.1%) within a surgical staging para-aortic lymphadenectomy (group 2). We compared both groups according to stage of disease, treatment, progression-free survival (PFS), and overall survival (OS). RESULTS: The extended-field radiotherapy was performed in 44.2 and 19.4% of patients in surgical and imaging staging group, respectively (p = 0.045). The disease-free survival rate was 17.4 ± 17.4 months in group 1 and 14.4 ± 12.6 months in group 2 (p = 0.456). No differences in OS were found between these 2 groups (p = 0.676). CONCLUSIONS: Despite the higher diagnostic accuracy of surgical staging and the higher number of patient who received extended field radiotherapy, we did not find differences between the overall and PFS rates in both the studied groups. Further prospective study on a higher number of patients would be necessary.

Laparoscopic paraaortic surgical staging in locally advanced cervical cancer: a single-center experience

Background: One aim of this study was to assess the efficacy and safety of laparoscopic paraaortic lymphadenectomy for paraaortic lymph node staging in locally advanced cervical carcinoma. The second aim was to identify prognostic factors in the evolution of this disease and to evaluate how the results of the surgery modify the oncological treatment of patients. Materials and methods: We analyzed 59 patients diagnosed with locally advanced cervical cancer International Federation of Gynecology and Obstetrics stage IB2-IVA who underwent laparoscopic paraaortic lymphadenectomy at our hospital between 2009 and 2015. Depending on the results of the paraaortic lymphadenectomy, treatment consisted of pelvic- or extended-field chemoradiotherapy. Results: The mean age at diagnosis was 52.3 years. The median operative time was 180 min. The mean hospital stay was 1.7 days. The mean number of paraaortic lymph nodes excised was 16.4. Eight patients (13.5%) had positive paraaortic lymph nodes. Thirteen patients (22%) underwent surgery via the transperitoneal route, and 46 (78%) underwent surgery via the retroperitoneal route. The sensitivity and specificity of computerized axial tomography (CT) scanning for detecting paraaortic lymph node involvement was 75 and 86%, respectively. The statistically significant prognostic factors that affected survival were surgical paraaortic lymph node involvement, radiological pelvic lymph node involvement, and radiological tumor size as assessed with nuclear magnetic resonance. The rate of serious complications was 1.7%. Conclusions: Pretherapeutic laparoscopic paraaortic lymphadenectomy for locally advanced cervical carcinoma allows the adaption of radiotherapy fields to avoid false-positive and false-negative imaging results

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Risk factors related to metastasis of para-aortic lymph nodes in pancreatic ductal adenocarcinoma: A retrospective observational study.

This study was designed to explore the risk factors related to metastasis of para-aortic lymph node (PALN).Clinicopathologic data of 241 patients with resectable or borderline resectable pancreatic cancer who underwent pancreaticoduodenectomy with extended lymphadenectomy between January 2008 and December 2015 were collected, potential factors related to metastasis of PALN were analyzed.Positive rate of PALN was 19.5% (47/241). Univariate analysis showed that back pain (P = .028), preoperative CA19-9 level (P < .001), tumor size (P < .001), portal vein (PV)/superior mesenteric vein (SMV) invasion (P < .001), superior mesenteric artery (SMA) invasion (P < .001), and diameter > 1.0 cm were in correlation with PALN involvement, multivariate analysis revealed that preoperative CA19-9 level, PV/SMV invasion, SMA invasion and diameter > 1.0 cm were independent risk factors to metastasis of PALN. Patients with LN8+ had a higher positive rate of PALN than with LN8- (38.1% vs 15.6%, P = .001), similar results could be found when LN12+ (35.8% vs 13.2%, P < .001) and LN14+ (41.2% vs 11.0%, P < .001), multivariate analysis showed that LN8+ and LN14+ were closely in correlation with PALN metastasis.Several factors were related to the status of PALN, preoperative CA19-9 level, PV/SMV invasion, SMA invasion and diameter > 1.0 cm were 4 independent risk factors to PALN metastasis. LN8+ and LN14+ were 2 strong predictors of PALN metastasis. A comprehensive analysis covering all possible risk factors related to metastasis of PALN should be given before design of treatment plan whenever involvement of PALN was suspected.

Stage- and Histologic Subtype-Dependent Frequency of Lymph Node Metastases in Patients with Epithelial Ovarian Cancer Undergoing Systematic Pelvic and Paraaortic Lymphadenectomy.

PURPOSE: Tumor stage and distinct histological subtypes in epithelial ovarian cancer (EOC) show different prognostic outcome. The aim of this study is to evaluate whether the frequency of lymph node (LN) metastases in patients with different tumor stages and histological subtypes undergoing systematic pelvic and paraaortic lymphadenectomy is coincidentally divergent. METHODS: Patients with EOC treated with upfront staging or debulking surgery between January 2000 and December 2016 were included. Systematic lymphadenectomy was performed in all consecutive patients with optimal debulking and without medical contraindications. RESULTS: Seven hundred sixty-two patients including 27.2% with early-stage EOC were included. The median number of removed LNs was 69, and metastases to LNs were found in 54.7%. No LN metastases were found in patients with low-grade endometrioid carcinoma, independently of tumor stage. LN metastases in early-stage low-grade serous (N = 5), mucinous (N = 31), and clear cell (N = 28) EOC were found in one (20%), zero, and one (3.6%) patient, respectively. LN metastases were detected in more than 10% of patients with all other histological subtypes. On multivariate analyses, overall survival was significantly impaired in patients with LN metastases, as compared with patients without LN metastases (p = 0.001). CONCLUSIONS: The risk of LN metastases in patients with EOC is dependent on stage and histological subtype. Patients with incidental finding of early mucinous or low-grade endometrioid EOC are at very low risk of retroperitoneal lymph node metastases. Reoperation for lymph node staging only should be discussed individually with caution.