Pheochromocytoma (English spelling)

Japanese: 褐色細胞腫 - かっしょくさいぼうしゅ（英語表記）Pheochromocytoma

What is the disease?

Adrenal glands ( Fukushima ) surrounds the outside cortex ( Crucifix ) And inside it Medulla ( Department ) Pheochromocytoma is classified into two types: Spinal cord ( Spinal cord ) In line with Sympathetic ganglion ( Exchange Commission ) Can be made into cells tumor ( Main ) Tumors secrete hormones called catecholamines, which cause various symptoms.

Most are benign, but sometimes they can be malignant. It is difficult to judge whether they are benign or malignant, so careful follow-up is required. Also, some families are genetically predisposed to this disease, and in such cases, regular testing may be required from childhood.

What is the cause?

As mentioned above, some patients have a genetic link, but in many cases the tumor develops without any clear cause.

How symptoms manifest

Typical symptoms include high blood pressure, headaches, excessive sweating, and metabolic disorders due to excessive secretion of catecholamines. Increased ( Public health ) , and an increase in blood sugar. Palpitations ( Excitement ) Other common symptoms include weight loss, constipation, chest pain, and vision problems.

Some patients always have symptoms such as high blood pressure, but more than half of the patients experience these symptoms in fits and starts. Attacks can be triggered by posture (lying on one's stomach or bending forward), eating, defecating, abdominal palpation, or other situations that put pressure on the tumor. In rare cases, attacks can cause a sudden rise in blood pressure or pulse rate, increasing the risk of heart failure or bleeding.

Testing and diagnosis

If symptoms suggest pheochromocytoma, hormone levels in the blood and urine are measured. Blood tests done during attacks can help make the diagnosis, but when attacks are not occurring, blood catecholamine levels may be normal.

For this reason, catecholamines and related substances are measured in urine immediately after an attack or in urine that has been collected throughout the day. Abdominal ultrasound and CT scans are also used to identify the location of the tumor, but MRI and nuclear medicine tests (MIBG scintigraphy) are often effective.

If genetic factors are suspected in pheochromocytoma based on family history, etc., genetic testing may be desirable. Although genetic testing has ethical issues, it is useful in determining subsequent testing and treatment plans.

Treatment methods

The definitive treatment is to remove the tumor. Before surgery, you will be prescribed a drug that blocks the action of catecholamines ( α ( alpha ) Receptor antagonists, etc. (Drugs generally used as antihypertensives) are administered to bring blood pressure closer to normal. In addition, if symptoms become severe during the course of the disease, the same drugs are administered to control blood pressure, etc.

What to do if you notice an illness

If you frequently experience sudden increases in blood pressure, sweating, palpitations, or headaches, please visit a hospital specializing in endocrinology.

Satoshi Sakihara

Pheochromocytoma
Pheochromocytoma
(Circulatory system disease)

What is the cause?

Pheochromocytoma is a type of tumor that causes catecholamine Adrenal medulla ( Fukushima Prefecture ) This is a disease caused by an excess of hormones secreted from the adrenal glands. As it is called the "10% disease," 10% of cases are caused by both adrenal glands, 10% are malignant, 10% are caused by extra-adrenal factors, and 10% occur within families. It accounts for 0.1-0.2% of all hypertension cases. There is no gender difference in the onset of hypertension, and it can be seen in a wide range of ages, from 10 to 80 years old.

How symptoms manifest

It is characterized by significant high blood pressure, headache, excessive sweating, hypermetabolism, and hyperglycemia. Two-thirds of pheochromocytoma cases are paroxysmal, usually asymptomatic but occasionally causing symptoms.

Testing and diagnosis

Excessive production of catecholamines is demonstrated by blood and urine tests. Generally, the diameter of the tumor is large when it is discovered, making imaging diagnosis such as CT, MRI, and scintigraphy easy. Recently, there has been an increase in cases where the tumor is discovered by chance (incidental tumor) during imaging diagnosis, and the patient is diagnosed with this disease. Coexistence of diabetes and hyperlipidemia is also common.

Treatment methods

The principle of treatment is surgical removal of the tumor.

What to do if you notice an illness

If the tumor is completely removed, blood pressure will return to normal and the prognosis will be good. However, if the tumor is left untreated and blood pressure rises significantly, stroke ( That's a lie ) or heart failure.

Source: Houken “Sixth Edition Family Medicine Encyclopedia” Information about the Sixth Edition Family Medicine Encyclopedia

Japanese:

どんな病気か

　副腎(ふくじん)は、外側を取り巻く皮質(ひしつ)と、その内側にある髄質(ずいしつ)に分かれています。褐色細胞腫は、副腎髄質あるいは脊髄(せきずい)に沿った交感神経節(こうかんしんけいせつ)細胞にできる腫瘍(しゅよう)です。腫瘍からはカテコールアミンというホルモンが分泌され、このホルモンの作用でさまざまな症状が現れます。

　大部分は良性ですが、時に悪性の場合もあります。良性か悪性かの判断は難しく、慎重に経過を追う必要があります。また、遺伝的にこの病気になりやすい家系もあり、その場合は小児期から定期的な検査が必要になる場合もあります。

原因は何か

　前述のとおり、一部の患者さんに遺伝との関係が認められますが、多くは明らかな原因もなく腫瘍が発生します。

症状の現れ方

　代表的な症状はカテコールアミンが多く分泌されることによる高血圧、頭痛、発汗過多、代謝亢進(こうしん)、血糖の上昇です。そのほか、動悸(どうき)、やせ、便秘、胸痛、視力障害などもしばしば起こります。

　高血圧などの症状が常にある患者さんもいますが、半数以上の人はこれらの症状が発作的に現れます。発作は姿勢（腹ばいや前屈など）、食事、排便、腹部の触診など腫瘍が圧迫されるような状況で誘発されることがあります。まれに、発作のために急激に血圧が上がったり脈が速くなったりして、心不全や出血の危険性が高まることもあります。

検査と診断

　症状から褐色細胞腫が疑われれば、血液中および尿中のホルモンを測定します。発作中に血液検査ができる場合は診断に有用ですが、発作が起きていない時は、血液中のカテコールアミン濃度は正常値を示すこともあります。

　このため発作直後の尿中あるいは1日中ためた尿中のカテコールアミンやその関連物質を測定します。腫瘍の部位を明らかにするためには腹部の超音波やCTなども行われますが、MRIや核医学検査（MIBGシンチグラフィ）がしばしば有効です。

　家族歴などから、遺伝的要因が関係した褐色細胞腫が疑われた場合は、遺伝子の検査が望まれる場合があります。遺伝子の検査は倫理的な問題もありますが、その後の検査・治療方針を決めるうえで有意義です。

治療の方法

　根本的な治療は腫瘍を摘出することです。手術の前には、カテコールアミンの作用を阻害する薬（α(アルファ) 受容体拮抗薬など：一般的に降圧薬として使用している薬剤）を投与し、血圧を正常に近づけます。また経過中、症状が強い時も同剤を投与して血圧などをコントロールします。

病気に気づいたらどうする

　発作的な血圧上昇、発汗、動悸、頭痛などがしばしば起こる場合は、内分泌を専門とする病院に受診してください。

崎原　哲

褐色細胞腫
かっしょくさいぼうしゅ
Pheochromocytoma
(循環器の病気)

原因は何か

　褐色細胞腫は、カテコラミンという副腎髄質(ふくじんずいしつ)から分泌されるホルモンが過剰につくられる疾患です。“10％病”と呼ばれるように、両側副腎由来が10％、悪性が10％、副腎外由来が10％、家族内発症が10％を占めます。全高血圧の0.1～0.2％を占めます。発症に男女差はなく、年齢も10～80代まで幅広くみられます。