a-novel-method-super-selective-adrenal-venous-sampling

A Novel Method: Super-selective Adrenal Venous Sampling

Primary aldosteronism (PA) and subclinical Cushing&#39;s syndrome (SCS) are conditions in which the adrenal glands autonomously produce excessive amounts ...

Yokohama Rosai Hospital

[A case with severe acute pancreatitis treated by regional arterial infusion of nafamostat mesilate and imipenem via both celiac artery and superior mesenteric artery].

The purpose of our study was to assess the usefulness of dynamic MRI in distinguishing high-flow vascular malformations from low-flow vascular malformations, which do not need angiography for treatment.

Dynamic MRI for distinguishing high-flow from low-flow peripheral vascular malformations.

We report a case in which C2 neurinoma was completely removed with preservation of the surrounding supportive tissue. A 47-year-old female was admitted to our hospital with gait disturbance and spinal tumor identified in other hospital. Neurological examination on admission revealed hyperreflexia in bilateral limbs and pathological reflexes. Cervical plain X-radiography showed erosion of the left C2 lamina. Axial Gd-enhanced T1 weighted magnetic resonance (MR) images showed partial enhancement of a dumbbell shaped tumor at the C1-2 level, revealing compression of the spinal cord to the right and extending to postero-laterally, and coronal MR images demonstrated that the tumor was clearly surrounded by the semispinalis capitis, semispinalis cervicis and inferior oblique muscles. The tumor was resected through a posterior approach without injuring the surrounding supportive tissue. Post operatively, she did not complain of nuchal pain during neck movement. After a 6-month follow-up period, cervical plain X-radiography showed preservation of cervical alignment, and coronal Gd-enhanced T1-weighted MR images demonstrated preservation of the posterior group of cervical muscles. It is desirable to preserve the surrounding supportive tissue as far as possible in order to minimize the postoperative neck pain and instability of cervical spine, like this case.

[A case involving complete removal of C2 dumbbell type neurinoma with preservation of the surrounding supportive tissue].

[Adrenal venous sampling].

The prognosis of metastatic or recurrent gastrointestinal stromal tumors (GISTs) accompanied by multiple hepatic metastases and peritoneal dissemination is very poor. We encountered a case of stage IV small intestinal GIST with multiple hepatic metastases and peritoneal dissemination that were observed after resection of the primary lesion. Multidisciplinary treatments were performed over time, including hepatic resection, radiotherapy, imatinib therapy, sunitinib therapy, and transcatheter arterial chemoembolization, and the disease had been brought under control following resection of a primary lesion 14 years ago. The patient was a 49-year-old woman diagnosed with hemorrhagic stool in July 1998, when a computed tomography scan revealed an 8-cm-diameter tumor in her small bowel. Partial resection of her small bowel was performed and the pathological diagnosis was a high-risk GIST showing 15 mitoses per 50 high power fields. Several metastases developed in the S4 and S5 segments of the patient's liver 3 years after resection of the primary lesion, and a central two-segmental resection of the liver was performed. Furthermore, 1 year after this procedure, peritoneal dissemination developed near the pancreas, for which radiotherapy was performed. Four months later, the patient again developed multiple liver metastases and was started on treatment with 400 mg imatinib per day, achieving a partial response(PR). Five years and 6 months after imatinib initiation, resistance emerged in one of the liver metastases. The patient was switched to sunitinib(50 mg per day), but was diagnosed with progressive disease at the end of the second course and the procedure was discontinued. Treatment with 400 mg of imatinib per day was resumed, and transcatheter arterial chemoembolization was performed twice over a 17-month period for the resistant hepatic region and a PR was achieved each time. We were able to maintain a PR in this patient; other metastases indicated the effectiveness of imatinib therapy. Therefore, a multidisciplinary team approach can be effective in achieving long-term disease control in patients with metastatic or recurrent GIST.

[A case of small intestinal stage IV gastrointestinal stromal tumor in which long-term disease control was maintained for more than 10 Years through a multidisciplinary team approach].

We report a case of non-familial juvenile primary aldosteronism (PA). Super-selective adrenal venous sampling identified less aldosterone production in the right inferior adrenal segment than others. Bilateral adrenalectomy sparing the segment normalized blood pressure and improved PA. Both adrenals had similar histologies, consisting of a normal adrenal cortex and aldosterone synthase-positive hyperplasia/adenoma. An aldosterone-driving KCNJ5 mutation was detected in the lesions, but not in the histologically normal cortex. After taking into account that the two adrenal glands displayed a similar histological profile, as well as the fact that hyperplastic lesions in both glands exhibited a common KCNJ5 mutation, we conclude that the specific mutation may have occurred at an adrenal precursor mesodermal cell, at an early stage of development; its daughter cells were mixed with non-mutant cells and dispersed into both adrenal glands, resulting into a form of the condition known as genetic mosaicism.

Somatic KCNJ5 mutation occurring early in adrenal development may cause a novel form of juvenile primary aldosteronism.

A stress reaction involving increased adrenal hormone release occurs when starting adrenal venous sampling (AVS). The purpose of the present study was to investigate the effect of single shot venography on adrenal hormone production during AVS.

Article	Year
[A case with severe acute pancreatitis treated by regional arterial infusion of nafamostat mesilate and imipenem via both celiac artery and superior mesenteric artery]. Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology\| PubMed ID: 12355899	2002
Dynamic MRI for distinguishing high-flow from low-flow peripheral vascular malformations. AJR. American journal of roentgenology\| PubMed ID: 16247121	2005
[A case involving complete removal of C2 dumbbell type neurinoma with preservation of the surrounding supportive tissue]. No shinkei geka. Neurological surgery\| PubMed ID: 16277222	2005
[Adrenal venous sampling]. Nihon rinsho. Japanese journal of clinical medicine\| PubMed ID: 21830595	2011
[A case of small intestinal stage IV gastrointestinal stromal tumor in which long-term disease control was maintained for more than 10 Years through a multidisciplinary team approach]. Gan to kagaku ryoho. Cancer & chemotherapy\| PubMed ID: 23267923	2012
Somatic KCNJ5 mutation occurring early in adrenal development may cause a novel form of juvenile primary aldosteronism. Molecular and cellular endocrinology\| PubMed ID: 27514282	2017
Adrenal hormones before and after venography during adrenal venous sampling: a self-controlled study. Japanese journal of radiology\| PubMed ID: 28074381	2017

Seishi Matsui

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