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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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 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.

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.

Incidence and Prognostic Value of Metastases to "Posterior" and Para-aortic Lymph Nodes in Resectable Gastric Cancer.

BACKGROUND: The purpose of this retrospective study was to evaluate the incidence and prognostic value of metastases to "posterior" (8p, 12b/p, 13) and para-aortic lymph nodes in a large cohort of Western patients submitted to D2 plus lymphadenectomy. METHODS: Removal of "posterior" nodes was performed in 743 patients, and para-aortic lymphadenectomy in a subgroup of 390 patients. After lymph node mapping and retrieval on the fresh specimen, a median number of 41 total lymph nodes were analyzed. The median follow-up period was 37 months for the entire series and 68 months for survivors. RESULTS: Of 743 included patients, 23 (3.1%) had metastases in station 8p, 12 (1.6%) in station 12b/p, and 19 (2.6%) in station 13. On the whole, 47 of 743 patients (6.3%) had positive "posterior" nodes. Para-aortic metastases were present in 42 of 390 patients (10.8%). Metastases to "posterior" stations were significantly related to depth of invasion, number of positive nodes, and surgical radicality. Distal tumors showed higher trend to metastasize to "posterior" nodes than upper third, whereas for para-aortic metastases it was the reverse. 5-year survival in patients with positivity to "posterior" nodes was 17%, with no significant difference according to 8p, 12b/p, and 13 stations; long-term outcome was overlapping to pN3b stage. 5-year survival in para-aortic positive cases was 11%, and a trend to better outcome was observed in proximal tumors. CONCLUSIONS: Although metastases to "posterior" and para-aortic nodes are expression of an advanced nodal stage, not negligible survival rates are observed in subgroups of patients.

Resección laparoscópica de paraganglioma en el órgano de Zuckerkandl guiado por sonda gamma con 123I-metayodobencilguanidina / Laparoscopic resection of a paraganglioma in the organ of Zuckerkandl 123I-metaiodobenzylguanidine guided by gamma probe

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Paraganglioma del órgano de Zuckerkandl / Paraganglioma of Zuckerkandl's organ

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Should Systematic Infrarenal Para-aortic Dissection Be the Rule in the Pretherapeutic Staging of Primary or Recurrent Locally Advanced Cervix Cancer Patients With a Negative Preoperative Para-aortic PET Imaging?

BACKGROUND: Extended-field chemoradiation is the usual management of patients with locally advanced cervical cancer (LACC) and para-aortic node metastases (PA pN1). It is efficient but not without morbidity. Assessment of PA lymph node positivity by PA lymphadenectomy is the most accurate method to select the candidates for this treatment. Hence, to clarify the dissection pattern, we wanted to test the true incidence of isolated/skip node metastasis, above the level of the inferior mesenteric artery (IMA). MATERIALS AND METHODS: All patients with LACC and negative magnetic resonance imaging and positron emission tomography-computed tomography imaging at the PA level were offered a laparoscopic staging encompassing a diagnostic laparoscopy followed, if negative, by an extraperitoneal PA lymphadenectomy. All nodes were removed from both common iliac bifurcations up to the left renal vein. Node groups, below and above the IMA, were separately sent to the pathologist for definitive examination. RESULTS: From January 2010 to December 2013, 196 stage IB1 with pelvic pN1, IB2, to IVA LACC patients from 2 cancer centers who fulfilled the criteria were included in this institutional review board-approved study after informed consent. Thirty patients (15%) had PA pN1. Only 1 patient had positive nodes exclusively located above the IMA (3.3% of the pN1 group; 95% confidence interval, 0%-9.7%). Complications were observed in 15 (7.6%) of 196 patients. CONCLUSIONS: Given the very low rate of skip metastases above the IMA and the potential additional morbidity of a systematic extended dissection, a bilateral ilioinframesenteric dissection seems to be an acceptable pattern of PA lymphadenectomy in LACC patients.

Intraoperative electron-beam radiation therapy with or without external-beam radiotherapy in the management of paraaortic lymph-node oligometastases from gynecological malignancies

Purpose. To analyze long-term outcomes and prognostic factors in patients with paraaortic lymph-node oligometastases (LNO) from gynecological malignancies treated in a multimodal protocol. Methods. Patients with a histological diagnosis of LNO gynecological cancer [uterine cervix (n = 14, 40 %), endometrial (n = 18, 51 %), ovarian (n = 3, 9 %)] who underwent surgery with radical intent and intraoperative radiotherapy (IORT), median dose 12.5 Gy) were considered eligible for participation in this study. Additionally, 51 % received external-beam radiotherapy (EBRT). Results. From 1997 to 2012, a total of 35 patients from a single institution were analyzed. With a median follow-up time of 55 months (range 2–148), 5-year loco-regional control (LRC), disease-free survival (DFS) and overall survival (OS) were 79, 44 and 49 %, respectively. On multivariate analysis, no EBRT treatment to the LNO (p = 0.03), and time interval from primary tumor diagnosis to LNO <24 months (p = 0.04) remained significantly associated with locoregional recurrence (LRR). We found on multivariate analysis that only R1 margin status (p = 0.01) was significantly associated with OS. Conclusion. From the current series of patients with gynecological LNO, it emerges the fact that EBRT promotes local control. Future prospective studies might be designed according to the predicted risk of LRR focusing on different subgroups (AU)

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Impact of Pelvic and Para-aortic Lymphadenectomy in Advanced Ovarian Cancer After Neoadjuvant Chemotherapy.

AIM: The aim of our study was to evaluate the impact of systemic pelvic and para-aortic lymphadenectomy on survival in patients with advanced ovarian cancer after neoadjuvant chemotherapy. PATIENTS AND METHODS: This multi-centric descriptive study included patients with initially inoperable advanced ovarian cancer, undergoing neoadjuvant chemotherapy followed by cytoreductive surgery with no residual tumor between 1998 and 2012. They were distributed into two groups depending on if they underwent lymphadenectomy or not during the interval surgery. RESULTS: Among the 101 included patients, 54 underwent lymphadenectomy and 47 did not. The multivariate analysis for overall survival showed no significant difference between the two groups [hazard ratio (HR)=1.88, confidence interval (CI)=0.89-3.94; p=0.08]. The multivariate analysis for progression-free survival showed no significant difference (HR=1.43, 95% CI=0.86-2.39; p=0.17). CONCLUSION: In patients with advanced ovarian cancer, treated by neoadjuvant chemotherapy and interval surgery with no residual tumor, lymphadenectomy does not seem to improve the survival rate.

Zuckerkandl Tubercle: An Important Landmark in Thyroid Surgery.

OBJECTIVE: To find out the frequency of Zuckerkandl Tubercle (ZT) and the position of recurrent laryngeal nerve with reference to ZK. STUDY DESIGN: Cross-sectional observational study. PLACE AND DURATION OF STUDY: Surgical Ward-3, Jinnah Postgraduate Medical Centre (JPMC), Karachi, from June 2010 to August 2011. METHODOLOGY: Patients having goiter, aged between 12 - 60 years were admitted through OPD and operated after informed written consent. After admission, they underwent surgery. ZT and recurrent laryngeal nerve position i.e. whether medial, lateral or posterior to ZT was identified during surgery. Data was collected on pre-designed proforma and analysed on SPSS version 15 for descriptive statistics. RESULTS: Out of the 84 patients ZT was found in 52 (61.90%) patients. Nineteen patients had grade-I (less than 0.5 cm), 22 (42.3%) had grade-II (0.5 - 1 cm) and 11 (21.1%) had grade-III (more than 1 cm) ZT. During surgery, 33 (63.4%) patients had recurrent laryngeal nerve medial to ZT, 15 (28.8%) patients had lateral and in 4 (7.6%) patients recurrent laryngeal nerve was engraved posteriorly. CONCLUSION: Zuckerkandl tubercle was found in about 62% cases; recurrent laryngeal nerve was located pre-dominantly medial to ZT.