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.

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.

Paraganglioma of the organ of Zuckerkandl and FH gene mutation.

Not required for Clinical Vignette.

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.

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.

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.

Natural Orifice-Assisted Laparoscopic Meckel Diverticulectomy Incidentally Found During Para-Aortic Mass Resection.

STUDY OBJECTIVE: To show a surgical video in which an incidentally found Meckel diverticulum was resected with a natural orifice-assisted laparoscopic approach during para-aortic resection of a retroperitoneal schwannoma. DESIGN: Case report (Canadian Task Force classification III). SETTING: Tertiary referral center in New Haven, Connecticut. INTERVENTIONS: This is a step-by-step illustration for resection of a retroperitoneal para-aortic schwannoma and of an incidentally found Meckel diverticulum. The patient was a 39-year-old white woman diagnosed with stage IV choriocarcinoma with metastasis to the lungs and left para-aortic area. She received chemotherapy in the form of etoposide, methotrexate, actinomycin-D, cyclophosphamide, oncovine (EMA-CO) and had an excellent clinical response with resolution of all metastatic disease except for the para-aortic mass. Therefore, she was taken to the operating room for laparoscopic resection of the persistent left para-aortic mass. After placement of four 5-mm abdominal ports, the pelvis and abdomen were explored and revealed an incidental Meckel diverticulum as well as the 5 cm left para-aortic mass. The peritoneum overlying the para-aortic mass was incised and the retroperitoneum explored. Given the proximity to the mass, left ureterolysis was performed. The retroperitoneal attachments were resected, and the left para-aortic mass was removed without any complications. At this point attention was turned to the Meckel diverticulum. In order not to extend the abdominal incisions, a posterior colpotomy was performed in the cul-de-sac equidistant from the uterosacral ligaments. Endo-GIA (Covidien, New Haven CT) was introduced through the 10-mm port site at the posterior colpotomy. Meckel diverticulum was resected without narrowing the lumen of the distal ileum. The specimen was removed in a contained manner through posterior colpotomy. MEASUREMENTS AND MAIN RESULTS: The procedure was performed without any complications. The patient had an uneventful postoperative course and was discharged home on postoperative day 0. Pathology revealed a retroperitoneal schwannoma with negative margins and benign Meckel diverticulum without ectopic gastric or pancreatic tissue. The patient has been disease-free since the completion of surgery. CONCLUSION: Laparoscopic resection of the retroperitoneal schwannoma and Meckel diverticulum were successfully performed in this patient with history of stage IV choriocarcinoma. To our knowledge, this is the first report describing a natural orifice-assisted laparoscopic approach for resection of Meckel diverticulum. Natural orifice-assisted laparoscopy should be considered when the surgeon needs to remove a large specimen and/or to introduce >5-mm diameter instruments into the peritoneal cavity without having to extend the abdominal incisions.

Compromiso ganglionar para-aórtico en estadios tempranos de cáncer de endometrio de acuerdo con el algoritmo de Kumar y col / Para-aortic ganglionic involvement in early stages of endometrial cancer according to the algorithm of Kumay et al

Introducción: La linfadenectomía para-aórtica en pacientes con estadios tempranos de cáncer de endometrio continúa siendo controversial, debido a que algunos estudios sugieren mayor morbilidad y costo sin claro beneficio en la sobrevida global. Objetivo: determinar el compromiso ganglionar para-aórtico en pacientes con cáncer de endometrio en estadios tempranos de acuerdo con él algoritmo de Kumar y col. Método: estudio descriptivo retrospectivo tipo serie de casos que incluyó pacientes llevadas a cirugía clasificatoria por vía abierta o laparoscópica (histerectomía total, salpingo-oforectomía bilateral más linfadenectomía pélvica y para-aórtica) con diagnóstico de adenocarcinoma de endometrio tipo endometriode, aplicación del algoritmo y evaluación de las diferencias que resultaran. Resultados: se incluyeron 15 pacientes, de las 9 clasificadas por el algoritmo de Kumar y col. como de alto riesgo en 2 (28.5%) hubo compromiso ganglionar para-aórtico (GPA) y en ninguna en el grupo de bajo riesgo. Cuatro (26,6%) pacientes presentaron complicaciones derivadas del procedimiento, 75% fueron tempranas, una ingresó a la unidad de cuidado intensivo (UCI) y no hubo muertes asociadas con los procedimientos quirúrgicos. Conclusión: las pacientes de bajo riesgo según el algoritmo de Kumar y col. fueron negativas para compromiso GPA, sin embargo, no ocurrió lo mismo para las de alto riesgo, por lo cual no podemos recomendar el uso rutinario de la realización de la linfadenectomía para-aórtica. Tendrán que realizarse estudios prospectivos para validar este algoritmo con el número de pacientes con el que fue planteado.

Introduction: Controversy persists on the benefit of para-aortic lymphadenectomy in patients with early-stage endometrial cancer, for some trials suggest increased morbidity and costs showing no clear benefit for overall survival. Objective: to determine para-aortic lymph node metastases (LNM) in patients with early-stage endometrial cancer according to the Kumar et al. risk classification algorithm. Method: a case series descriptive retrospective study was conducted in patients who underwent open or laparoscopic staging surgical procedures (total hysterectomy, bilateral salpingo-oophorectomy and pelvic and para-aortic lymphadenectomy), diagnosed with endometrioid endometrial adenocarcinoma, classified by the risk algorithm and analysis of their outcomes. Results: a total of 15 patients were included, of the 9 patients classified as high risk by the Kumar et al algorithm, 2 (28.5%) had para-aortic LNM, and no patients in the low risk group had LNM. Four (26.6%) patients had postoperative complications, 75% were early complications, one patient was admitted to the intensive care unit (ICU) and there were no deaths related to the surgical procedures. Conclusion: low risk patients classified by the Kumar et al algorithm had no para-aortic LNM however high risk patients had different results. We cannot therefore recommend routine para-aortic lymphadenectomy. Further prospective clinical trials must be conducted for validation of this algorithm with the same number of patients with which it was proposed.

Implications of para-aortic lymph node metastasis in patients with endometrial cancer without pelvic lymph node metastasis.

OBJECTIVE: The aim of this study was to confirm the incidence and implications of a lymphatic spread pattern involving para-aortic lymph node (PAN) metastasis in the absence of pelvic lymph node (PLN) metastasis in patients with endometrial cancer. METHODS: We carried out a retrospective chart review of 380 patients with endometrial cancer treated by surgery including PLN dissection and PAN dissection at Hokkaido Cancer Center between 2003 and 2016. We determined the probability of PAN metastasis in patients without PLN metastasis and investigated survival outcomes of PLN-PAN+ patients. RESULTS: The median numbers of PLN and PAN removed at surgery were 41 (range: 11-107) and 16 (range: 1-65), respectively. Sixty-four patients (16.8%) had lymph node metastasis, including 39 (10.3%) with PAN metastasis. The most frequent lymphatic spread pattern was PLN+PAN+ (7.9%), followed by PLN+PAN- (6.6%), and PLN-PAN+ (2.4%). The probability of PAN metastasis in patients without PLN metastasis was 2.8% (9/325). The 5-year overall survival rates were 96.5% in PLN-PAN-, 77.6% in PLN+PAN-, 63.4% in PLN+PAN+, and 53.6% in PLN-PAN+ patients. CONCLUSION: The likelihood of PAN metastasis in endometrial cancer patients without PLN metastasis is not negligible, and the prognosis of PLN-PAN+ is likely to be poor. The implications of a PLN-PAN+ lymphatic spread pattern should thus be taken into consideration when determining patient management strategies.