A clinical approach to brown adipose tissue in the para-aortic area of the human thorax.

BACKGROUND: Human thoracic brown adipose tissue (BAT), composed of several subdivisions, is a well-known target organ of many clinical studies; however, the functional contribution of each part of human thoracic BAT remains unknown. The present study analyzed the significance of each part of human thoracic BAT in the association between regional distribution, cellularity, and factors involved in the functional regulation of thoracic BAT. METHODS: We analyzed 1550 healthy adults who underwent medical check-ups by positron-emission tomography and computed tomography (PET-CT) imaging, 8 cadavers, and 78 autopsy cases in an observational study. We first characterized the difference between the mediastinum and the supraclavicular areas using counts of BAT detection and conditions based on PET-CT outcomes. The measurable important area was then subjected to systematic anatomical and immunohistochemical analyses using anti-uncoupling protein 1 (UCP1) antibody to characterize the cellularity in association with age and sex. RESULTS: In PET-CT scanning, the main site of thoracic BAT was the mediastinum rather than the supraclavicular area (P < 0.05). Systemic macroanatomy revealed that the thumb-sized BAT in the posterior mediastinal descending para-aortic area (paBAT) had feeding vessels from the posterior intercostal arteries and veins and sympathetic/parasympathetic innervation from trunks of the sympathetic and vagus nerves, respectively. Immunohistochemical analysis indicated that the paBAT exhibited immunoreactivity for tyrosine hydroxylase and vesicular acetylcholine transporter located in the pericellular nervous fibers and intracellular UCP1. The brown adipose cells of paBAT showed age-dependent decreases in UCP1 expression (P < 0.05), accompanied by a significant increase in vacuole formation, indicating fat accumulation (P < 0.05), from 10 to 37 years of age (P < 0.01). CONCLUSIONS: paBAT may be one of the essential sites for clinical application in BAT study because of its visible anatomy with feeding vessels and sympathetic/parasympathetic innervation functionally affected by outer condition and senescence.

Paraganglioma of the Organ of Zuckerkandl.

Paragangliomas are tumors of the sympathetic and parasympathetic paraganglia. While most paraganglioma are of parasympathetic origin and present as benign palpable masses of the neck, sympathetic paraganglioma are often secretory, presenting with symptoms related to excess catecholamines. Such symptoms include hypertension, headache, palpitations, and diaphoresis. Most sympathetic paraganglioma form within the adrenal medulla, the largest sympathetic paraganglia, and are commonly known as pheochromocytomas. However, sympathetic paragangliomas may present extra-adrenally, carrying a significantly higher risk of malignancy. In this manuscript, we examine a case of a young man with an extra-adrenal sympathetic paraganglioma of the Organ of Zuckerkandl. Furthermore, we discuss appropriate diagnostic workup and treatment of pheochromocytomas and sympathetic paragangliomas.

Double inferior vena cava does not complicate para-aortic nodal dissection for the treatment of pancreatic carcinoma.

Duplication of the inferior vena cava (IVC) involves large veins on both sides of the aorta that join anteriorly at the level of the renal arteries to become the suprarenal IVC. We report CT scan and intraoperative images of a patient with duplication of the IVC who underwent pancreaticoduodenectomy with para-aortic lymphadenectomy for carcinoma of the pancreatic head: nodal dissection along the left caval vein was not carried out. The anatomical background of the lymphatic flow to the para-aortic lymph nodes and the theoretic basis for lymph node dissection of the para-aortic area in cases of double IVC are highlighted. Lymphadenectomy along the left caval vein is not necessary in patients with double IVC who undergo pancreaticoduodenectomy with extended lymphadenectomy for carcinoma of the pancreatic head in the absence of preoperative appearance of para-aortic disease.

High accuracy of multislices computed tomography (MSCT) for para-aortic lymph node metastases from gastric cancer: a prospective single-center study.

BACKGROUND: The purpose of this study is to analyze the diagnostic accuracy of MSCT in the identification of para-aortic lymph node metastases from gastric cancer. METHODS: A total of 92 consecutive patients with primary gastric cancer were prospectively submitted to preoperative MSCT staging according to a standard protocol in the period 2003-2010. All diagnostic procedures were performed by dedicated radiologists who were unaware of the final pathological nodal status. Subsequently all patients underwent potentially curative (R0) resection with extended lymphadenectomy plus para-aortic nodal dissection. Lymph node mapping in different stations and retrieval of single lymph nodes were performed by the surgeon on the fresh specimen and then submitted for pathological examination. Clinical, radiological, and pathological data were prospectively stored on database. RESULTS: A median number of 47 (range: 18-114) total lymph nodes and 7 (range: 3-29) para-aortic lymph nodes were removed. In 13 of 92 included patients (14%), histological examination demonstrated para-aortic nodal metastases; MSCT was correctly positive in 11 of these cases (sensitivity: 85%). In 79 patients para-aortic nodes were not involved, and MSCT resulted correctly negative in 75 of these patients (specificity: 95%). Positive (PPV) and negative (NPV) predictive values were 73 and 97%, with a global accuracy of 93%. CONCLUSIONS: MSCT performed according to a standard protocol by dedicated radiologists demonstrated high accuracy in preoperative identification of para-aortic nodal metastases from gastric cancer. These results may be useful in planning surgical approach or during clinical staging before neoadjuvant chemotherapy.

Histologic results of para-aortic lymphadenectomy in patients treated for stage IB2/II cervical cancer with negative [18F]fluorodeoxyglucose positron emission tomography scans in the para-aortic area.

PURPOSE: Histologic results of complete para-aortic lymphadenectomy were studied in patients treated for stage IB2/II cervical carcinoma who had no para-aortic uptake on [(18)F]fluorodeoxyglucose positron emission tomography combined with integrated computed tomography (FDG-PET/CT). PATIENTS AND METHODS: Patients were treated between 2004 and 2006 for stage IB2/II cervical cancer. Magnetic resonance imaging of the abdomen and pelvis and FDG-PET/CT were initially performed. Patients with no para-aortic abnormalities were treated with external pelvic radiation therapy and concomitant chemotherapy followed by utero-vaginal brachytherapy. Para-aortic lymphadenectomy was then performed. FDG-PET/CT images were reviewed by two nuclear medicine specialists. RESULTS: Thirty-eight patients were studied. Three patients had histologically proven para-aortic involvement (metastatic nodes with capsular rupture in the para-aortic area), leading to a negative predictive value of 92% for para-aortic nodal involvement. CONCLUSION: In this study, three of 38 patients with no para-aortic uptake on [(18)F]FDG-PET/CT imaging had histologically proven para-aortic node involvement. PET/CT imaging without histologic examination of the para-aortic area used to determine radiation therapy fields in stage IB2/II cervical cancer would overlook 8% of patients with histologic para-aortic nodal involvement.

Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction.

OBJECTIVE: To describe a case of a malignant pheochromocytoma located in the organ of Zuckerkandl that required aortic and vena caval resection and reconstruction. METHODS: We present a case report that includes clinical, laboratory, and radiographic data as well as photographs, results from pathology, and a brief review of the literature. RESULTS: A 46-year-old man was referred for evaluation of a 1.4-cm left adrenal mass incidentally discovered on an abdominopelvic computed tomography (CT) scan. Subsequent laboratory evaluation revealed the following values: urine norepinephrine, 252 microg/24 h; urine normetanephrine, 1122 microg/24 h; urine metanephrine, 162 microg/24 h; urine epinephrine, 7 microg/24 h; urine vanillylmandelic acid, 8 mg/24 h; and plasma metanephrine, 98 pg/ mL. Imaging characteristics of the left adrenal mass were consistent with a benign adenoma, but CT also demonstrated a hypervascular para-aortic mass. 123I-metaiodo-benzylguanidine scanning with fusion CT imaging demonstrated increased radiopharmaceutical uptake within the para-aortic mass consistent with a paraganglioma in the organ of Zuckerkandl. Findings from CT angiography of the abdomen and pelvis suggested aortic involvement and vena caval thrombus. The mass was excised en bloc, including portions of the aorta, inferior vena cava, and right ureter. The aorta and vena cava were reconstructed using Dacron grafts. The remaining right ureter and kidney were removed to avoid the possibility of a urine leak from an ureteroureterostomy. Final pathologic and operative findings confirmed a malignant pheochromocytoma of the organ of Zuckerkandl with invasion into the wall of the inferior vena cava and tumor thrombus extending into the lumen. CONCLUSION: Malignant pheochromocytoma of the organ of Zuckerkandl involving the aorta and inferior vena cava is exceedingly rare, and although surgical resection and reconstruction can be radical and aggressive, this treatment offers the only chance for cure.

Laparoscopic resection of a paraganglioma of the organ of Zuckerkandl in a patient with a carotid body tumor.

Paragangliomas of the organ of Zuckerkandl are rare tumors with only several case reports noted in the literature. Synchronous paraganglioma occur sporadically, and on occasion, in association with several genetic syndromes. Paraganglioma of the organ of Zuckerkandl with synchronous carotid body paraganglioma is described herein. Traditionally, surgical resection of abdominal paraganglioma involved an exploratory laparotomy. In this manuscript, we describe one of the first reported laparoscopic resections of this tumor and review the literature on multiple paragangliomas and their associated genetic syndromes.

Long-term efficacy of doxazosin plus atenolol in the management of severe and sustained arterial hypertension and reversibility of the cardiac damage induced by chronic cathecolamine excess. A case report in a young girl with recurrent, functioning paraganglioma.

Herein we report on a young girl with recurrent, functioning paraganglioma of the organ of Zuckerkandl and severe and sustained arterial hypertension (systolic pressure >200, diastolic pressure >120 mmHg); with evidence of cardiac damage induced by chronic cathecolamine excess. She promptly and steadily improved after the institution of doxazosin (6 mg/day) plus atenolol (50 mg bid) treatment. This case demonstrates that a correct therapeutic strategy in the long-term management of patients with inoperable catecholamine-producing neuroendocrine tumors (pheochromocytomas and paragangliomas) can maintain arterial pressure in the normal range and reverse the cardiac damage induced by chronic cathecolamine excess.

[Pheochromocytoma of the organ of Zuckerkandl. Apropos of a case]. / Feocromocitoma del órgano de Zuckerkandl. A propósito de un caso.

INTRODUCTION: Extraadrenal pheochromocytomae represent 17-18% of pheochromocytomae, 85% of which are located infradiaphragmaticaly and generally wherever there is cromaffin tissue, as in this case, located at the organ of Zuckerkand. They can also appear in the context of family syndromes, i.e. phacomatosis and MEN (IIA and IIB). CLINICAL CASE: The authors present a case of pheochromocytoma located in the organ of Zuckerkand, diagnosed in the course of uncontroled HBP. Diagnosis was achieved by metanephrine determinations and radiological tests (ultrasound, CAT and MIBG). This patient was surgically treated by exeresis of the lession previous alpha and beta adrenergic blockade with phenoxybenzamine and propanolol. Currently this patient is asymptomatic with normal blood pressor and metanephrines blood levels. DISCUSSION: Extraadrenal pheochromocytomae are usually located in superior paraaortic infradiaphragmatic region. The grade of malignancy must be established by histopathological findings, local recidives and metastasis. After clinical suspicion and the finding of a retroperitoneal mass with ultrasound, CAT and/or MRI, MIBG must be performed to confirm the diagnosis as well as to leave out possible tumours at distance. Treatment is surgery previous blockade of cathecolamines secretion by the preoperative administration of alpha blockers, with or without beta blockers.

Multifocal malignant extra-adrenal paragangliomas of the Organ of Zuckerkandl and urinary bladder.

A case of multifocal malignant extra-adrenal paragangliomas involving the Organ of Zuckerkandl and urinary bladder is presented. The role and order of imaging investigations of functioning extra-adrenal paraganglioma is discussed.

Spectrum of catecholamine-secreting tumors of the organ of Zuckerkandl.

In a series of 24 pheochromocytomas, four were tumors in the organ of Zuckerkandl. Their presenting symptoms of headache, palpitation, diaphoresis, and anxiety were similar to symptoms of adrenal pheochromocytomas. Two patients had paroxysmal and two patients sustained hypertension. All four had elevated urinary vanillylmandelic acid, metanephrine or catecholamine levels. Two of the tumors were localized with angiography before the availability of computerized tomography. Two patients underwent initial computerized tomography, which was nondiagnostic. Later scans looking specifically for organ of Zuckerkandl tumors were positive. Organ of Zuckerkandl tumors, like other ectopic pheochromocytomas, have a higher rate of malignancy. Two of the four were malignant tumors. One patient died 4 years after operation, but the other patient remains stable despite metastatic disease 8 years after operation. The two patients with benign tumors remain well 2 and 3 years after resection and treatment with alpha-blockade.

[Pheochromocytoma of Zuckerkandl's organ. Value of angiographic study. Apropos of a case]. / Phéochromocytome de l'organe de Zuckerkandl. Intérêt de l'exploration angiographique. A propos d'un cas.

The authors report a case of pheochromocytoma of Zuckerkandl's organ revealed by permanent hypertension. Angiography, combined with the clinical and biological data suggesting a catecholamine secreting tumor, made the diagnosis of pheochromocytoma of Zuckerkandl's organ prior therapy.

[Paraganglioma of the organs of Zuckerkandl. A case report (author's transl)]. / Paragangliome de l'organe de Zuckerkandl. A propos d'un cas.

Endocrine manifestations were absent in a patient presenting with pain in the left peri-umbilical region from a paraganglioma of the organs of Zuckerkandl. The authors describe the embryology of paragangliomas and review the published literature concerning the noradrenaline-secreting forms of these tumors. Only 86 references to those of Zuckerkandl, situated on the side of the aorta in relation to the inferior mesenteric artery, were found. Diagnosis of non-secreting forms is confirmed by arteriography which in this present case demonstrated three pedicles arising from the inferior mesenteric, celiac, and one of the lumbar arteries respectively. The only evidence of malignancy is the presence of metastases. Their diagnosis is therefore difficult but essential as one-third of the localisations of malignant pheochromocytomas (10 p. cent of pheochromocytomas) outside of the adrenals affect the organs of Zuckerkandl.