Jpn J Intervent Radiol Vol.30 No.4 2015

State of the Art
Diagnosis and Treatment for Primary Aldosteronism

An Introduction
Yasutaka Baba

1. Anatomy and Pathology of Adrenal Glands for Understanding Primary Aldosteronism

Department of Pathology and Clinical Hypertension, Endocrinology & Metabolism1),
Tohoku University School of Medicine
Hironobu Sasano, Yuto Yamazaki, Yasuhiro Nakamura, Fumitoshi Satoh1)

Aldosterone is the mineralocorticoid exclusively produced and secreted from the zona glomerulosa in human adrenal cortex. Primary aldosteronism is defined as the cortical lesions associated with overproduction and secretion of aldosterone with suppressed plasma renin activity. It is important to note that not all the zona glomerulosa cells are involved in aldosterone production in more than half of general public and in these subjects, aldosterone producing zona glomerulusa cells are focally clustered. The adrenocortical lesions causing primary aldosteronism are classified into neoplastic and non-neoplastic ones. The former are predominantly benign adrenocortical adenoma but adrenocortical carcinoma associated with only overproduction of aldosterone could be present, which the clinicians should always consider as the differential diagnosis. In aldosterone producing adenomas, recently more cases of small or CT negative ones as well as bilateral adenomas have been detected with the advent of adrenal venous sampling and others. In non-neoplastic primary aldosteronism, the predominant form is IHA or idiopathic hyperaldosteronism or IHA. IHA is histopathologically characterized by diffuse hyperplasia of the zona glomerulosa involving overproduction of aldosterone. Others include dexamethasone suppressive hyperaldosteronism or UMN (unilateral hyperaldosteronemia due to multiple adrenocortical micronodules). They have been considered rare but UMN are currently considered more frequent than previously considered as a result of immunohistochemical evaluation of steroidenic enzymes involved in aldosterone biosynthesis.

Key words

  • Adrenal
  • Aldosteronism
  • Histology
  • Pathology

2. Surgery for Primary Aldosteronism

Department of Urology, Yokohama Rosai Hospital
Maki Nagata

Laparoscopic surgery has become a standard method for treatment of adrenal disease.Between June 1994 and June 2015, we performed 604 laparoscopic adrenalectomies includingprimary aldosteronism.
Recently there has been growing advocacy to perform partial adrenalectomy for aldosteroneproducing adenomas(APA), when proved to be causing hyperaldosteronism by SegmentalAdrenal Tributary Sampling(S-ATS).
We describe our technique with laparoscopic total and partial adrenalectomy using the transperitoneal approach for APA.
Laparoscopic total and partial adrenalectomy was complete in all cases without conversionto open surgery and blood transfusion, and most complications in our cohort were minor andthere was no mortality. These results show the procedure to be safe and effective.
Partial adrenalectomy compared favorably to total adrenalectomy for some perioperativeoutcomes. Operative duration was 123 and 96 minutes in total and partial adrenalectomyrespectively(P<0.01). Hypertension resolution rate was 39.3% and 65.9% in the total and partialcases respectively(P<0.05).
Laparoendoscopic single-site surgery(LESS) was introduced to our hospital in November2011. LESS is a good indication of adrenalectomy because the adrenal tumor is often small. InLESS, all of the instrument to used perform the procedure must be placed through a singleincision, often concealed within the umbilicus. It has evolved to improve upon cosmesisassociated with standard laparoscopic surgery. We show our experience with LESS.

Key words

  • Primary Aldosteronism
  • Adrenal Surgery
  • Laparoscopic Adrenalectomy

3. Adrenal Venous Sampling and Radiofrequency Ablation for Primary Aldosteronism

Department of Diagnostic Radiology, Tohoku University Graduate School of Medicine
Kei Takase

Hypertension has a high morbidity with approximately one of three individuals affected. Most hypertension is incorrectly assumed to be essential hypertension, and drug therapy is often selected without reasonable consideration. Secondary hypertension is assumed to account for approximately 20% of all hypertension cases. Particularly primary aldosteronism is a frequent disease underlying secondary hypertension, supposedly 5 to 20% of all hypertension cases. MDCT and MRI are not reliable methods in the differential diagnosis of primary aldosteronism because aldosterone may be produced on the side contralateral to the adrenal adenoma. Selective adrenal venous sampling is the standard to diagnose the laterality of aldosterone hypersecretion. If hypersecretion of aldosterone is confirmed in a unilateral adrenal gland by appropriate adrenal venous sampling (AVS), complete cure or improvement of hypertension can be expected by laparoscopic unilateral adrenalectomy. However, right adrenal vein sampling generally remains difficult with a success rate of approximately 70% because of unpredictability of the variable anatomy of the right adrenal vein in advance. MDCT enabled the identification of the adrenal vein and delineation of its anatomy. Use of adequate adrenal venous catheters based on the preoperative anatomical information facilitates accurate catheterization. Segmental sampling allowed the identification of the location of intraadrenal aldosterone hypersecretion, which has the potential to diagnose patients with bilateral aldosterone producing adenoma which otherwise might be misdiagnosed. An investigatorinitiated clinical trial of radiofrequency ablation of adrenal adenoma is ongoing.

Key words

  • Adrenal gland
  • Primary aldosteronism
  • Adrenal venous sampling

4. Endovascular Treatment for an Aldosterone Producing Adrenal Adenoma

Department of Radiology, Faculty of Medicine, Kagoshima University1), Ryokusenkai Yonemori Hospital2)
Yasutaka Baba1, 2), Sadao Hayashi1), Kohei Nagasato1), Takashi Yoshiura1)
Department of Radiology, Imakiire Hospital
Hirofumi Hokotate
Department of Radiology, Kagoshima Medical Association Hospital
Kazuto Ueno

Traditionally, endovascular treatment for adrenal tumors (Cushing syndrome, Pheochromocytoma, Adrenal carcinoma) has been done for the purpose of mitigating symptoms associated with hormonal hypersecretion. The aim of endovascular treatment for an aldosterone producing adrenal adenoma (APAA) is to mainly achieve a normalization of serum aldosterone and renin activity. Another goal of endovascular treatment for APAA is to achieve normalization of the blood pressure.
In this article, we will illustrate our experience of endovascular treatment for APAA.
Especially, we will also emphasize and discuss not only the technique of endovascular treatment for APAA, but also the outcome of patients with APAA.

Key words

  • Aldosterone producing adrenal adenoma(APAA)
  • Ethanol
  • Endovascular treatment

Case Reports

Preoperative Embolization of a Hyaline Vascular
Castleman Disease Tumor Located in the Mediastinum

Departments of Radiology and General Thoracic Surgery1), Juntendo University Faculty of Medicine
Akihiro Hotta, Akihiko Shiraishi, Yuki Yamashiro, Tatsuro Inoue
Mari Aida, Kazuya Takamochi1), Kenji Suzuki1), Ryohei Kuwatsuru

Castleman disease is a rare lymphoproliferative disorder that is classified pathologically as either hyaline vascular or plasma cell. Hyaline vascular Castleman disease most commonly presents as a solitary mediastinal mass. Although surgical resection is the standard treatment for these tumors, it can result in excessive blood loss due to their hypervascular nature. However, the dominant feeding vessels of Castleman disease tumors are amenable to preoperative radiographic embolization. Here we present a case of a Castleman disease tumor treated with preoperative embolization as an adjunct to operative management.

Key words

  • Casteleman disease
  • Embolization
  • Bronchial artery


Case Reports

Bleeding Arterioenteric Fistula between a Right Internal
Iliac Artery Aneurysm After an Exclusion Operation and Sigmoid Colon Treated by Collateral Embolization: A Case Report

Departments of Diagnostic Radiology and Emergency1), National Center for Global Health and Medicine
Ryuji Uehara, Tsuyoshi Tajima, Tomoyuki Noguchi
Toshifumi Masuda, Takashi Okafuji, Tatsuya Wada
Hiroko Tsukano, Shingo Gima, Reiko Ito, Kentaro Kobayashi1)

Arterioenteric fistulas (AEFs) are rare but often fatal. Conventionally, AEFs have been surgically treated. In recent years, however, more and more cases treated by interventional radiology (IR) have been reported and now IR is the first-line treatment for AEFs. We here describe the case of a fistula between a right internal iliac artery aneurysm after an exclusion operation and sigmoid colon, which was successfully treated by embolization.
A man in his 80s man who had undergone vascular graft replacement for an abdominal aortic aneurysm and right internal iliac artery aneurysm 9 years earlier was admitted to our hospital with fever. Contrast-enhanced computed tomography (CT) showed a fistulous connection between a right internal iliac artery aneurysm, which was excluded by the surgery, and the sigmoid colon, which was infected due to diverticulitis. On the 10th day after his admission, the patient showed bloody stool and anemia. Contrast-enhanced CT demonstrated blood flow within the excluded internal iliac artery aneurysm, suggesting gastrointestinal bleeding via the fistula. An angiographic examination and IR were performed to stop the bleeding. After searching the access routes for the aneurysm, we performed a selective arterial embolization of the aneurysm, medial circumflex femoral artery and superior gluteal artery using a mixture of NBCA and iodized oil. The patient remained stable with no rebleeding or infection for 50 days after the treatment.

Key words

  • Embolization
  • Arterioenteric
  • Fistula