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Nelson’s Syndrome

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Nelson’s syndrome affects individuals in whom one or more adrenal glands are surgically removed. Please read this article to know more.

Medically reviewed by

Dr. P. C. Pavithra Pattu

Published At December 16, 2021
Reviewed AtMarch 17, 2023

What Is Nelson’s Syndrome?

Nelson’s syndrome is a serious clinical condition in patients who have undergone surgery of adrenal glands to treat Cushing's disease or Cushing’s syndrome. This "post adrenalectomy" syndrome was first reported through case studies of patients in 1959 by Dr. Fon H Nelson, an endocrinologist, and physician who first elaborated on this pituitary tumor.

Nelson's syndrome is a clinical challenge to both physicians and patients due to its features’ complexity. This article elaborates the features of this pituitary tumor, the disorders of the adrenal gland that are indicated for surgery, and the management of Nelson’s syndrome.

What Are the Clinical Features of Nelson Syndrome?

In this pituitary tumor, multiple clinical symptoms arise, all of which start with hyperpigmentation of the skin, hyperpigmentation of the scrotum, the areolae, and of the gingiva. Gingival hyperpigmentation or the oral mucosal membrane hyperpigmentation that may be accompanied or preceded by gingival hypertrophy (enlargement or overgrowth of gingival tissue causing variations in its contour) is a very obvious oral characteristic feature of this disease, just like the hyperpigmented skin surface of the individual.

The serious clinical feature of this disease or tumor is hemianopia, wherein there may be partial loss of the visual field in one eye. Hemianopias can be temporary or permanent, usually depending on the extent of the pituitary tumor. Complete visual loss is not uncommon in very large pituitary tumors of this disease which can be progressive and also a hindrance to the patient other than accessory clinical symptoms like headaches, severe fatigue (malaise or weakness), the underlying reason mainly being the pituitary gland dysfunctionality.

The characteristic feature that distinguishes this syndrome is that it is seldom found in patients suffering from an adrenal disease and is instead found in those patients who have undergone surgical adrenalectomy or removal of one or both of the adrenal glands. In the past decades, the surgical procedure to avoid long-term complications as a result of Cushing's disease has led to the physician's and surgeon’s recommendation of it, that is, the bilateral adrenalectomy procedure. However, in the recent decade, this surgical modality is not preferred anymore as it can make the patients more prone to the risk of developing Nelson's syndrome.

What Adrenal Gland Disorders Necessitate Adrenalectomy?

The adrenal glands situated bilaterally above the kidneys are major endocrine hormonal organs that secrete for the purpose of growth and development, sexual function, and metabolism in the human body.

Though in normal individuals not suffering from any systemic disease, the problems of adrenal glands should be definitely rare, still, the conditions that impact the adrenal glands are varied, ranging from cancerous tumors to hypercortisolism (increased production of cortisol or stress hormone - Cushing’s syndrome or disease), hyperaldosteronism (excess production of aldosterone - the pituitary hormone that regulates salt metabolism as well as blood pressure), and pheochromocytoma (a condition characterized by overproduction or over secretion of hormones like catecholamines and metanephrines that severely causes an imbalance or an unregulated mechanism of blood pressure control).

Overproduction of steroid hormones or cancers of the adrenal gland themselves though less common, definitely is indicative for the patient to undergo adrenalectomy.

Why Is Adrenalectomy Done?

Adrenalectomy is a procedure done through an open incision and open surgery for large cancers of the adrenal glands. For mild to moderate-sized cancers, the adrenalectomy can be performed in smaller incisions by robotic surgery or through a laparoscopy that is minimally invasive in comparison (laparoscopic adrenalectomy).

Unilateral adrenalectomy usually allows the other existing adrenal gland in the body to cope. It allows the patient a better long-term prognosis from either cancer or Cushing’s syndrome or the above-enlisted conditions that interfere with the adrenal gland metabolism.

However, it is bilateral adrenalectomy that is of the most risk to the patient as both glands are bilaterally eliminated atop the kidneys, and medications replace the normal metabolic function of the glands. Hence it makes the patient more prone to Nelson's syndrome.

What Investigations Help Diagnose Nelson’s Syndrome?

Primarily tests for evaluation are conducted by the physician for assessing the plasma ACTH (adrenocorticotropic hormone) levels. An ACTH increase greater than 30 % and with a positive history of adrenalectomy is confirmative of diagnosis of the syndrome.

An MRI (magnetic resonance imaging) may show either a new tumor or enlargement of a long-standing or previously recurrent tumor. MRI and plasma ACTH levels with patients' medical history along with certain severe clinical features limited exclusively to pituitary tumor enlargements like sudden or progressive visual loss and hemianopias are very confirmative and hence gold standard diagnosis for detection and confirmation prior to surgical intervention.

Mainly diagnosis proves useful only in the long-term prognosis of these cases after surgery of the tumor as early diagnosis can help prevent the postoperative complications. This tumor, though slow, can often be clinically asymptomatic for a limited duration (also early detection can help the physician manage endocrine complications like diabetes insipidus or hypopituitarism in these individuals prior to surgery).

How Can Nelson’s Syndrome Be Treated?

  1. The mainline treatment is usually surgical resection of the tumor and is performed mainly by either craniotomy surgery or endoscopic or transsphenoidal route surgeries.

  2. Radiosurgery that usually directs the radiation specifically (using a gamma source and knife) to the enlarged or the cancerous tissue in the pituitary gland proves an effective option both as an alternative to surgery and if post-operative outcomes fail after surgery in these individuals. Radiosurgery is thus proven very effective as per current research and an effective alternate modality for improving long-term recovery outcomes in these cases. Radiotherapy, on the other hand, can be prophylactically used post-surgery in bilateral adrenalectomy cases.

  3. This would be a contraindication in patients who have already received radiation therapy (though previously). Also, as these individuals cannot produce cortisol anymore, the Glucocorticoids and Mineralocorticoids are administered permanently to compensate for the loss of natural cortisol production. Somatostatin analogs and Dopamine agonists are other drugs that have proved effective as per recent research alongside Hydrocortisone or Glucocorticoid/Mineralocorticoid administration every day.

Conclusion:

Nelson’s syndrome is a complex clinical entity and challenge. With current treatment modalities, though the prognosis is improved for these cases, early detection and recognition of symptoms are essential in adrenalectomy patients to combat this tumor.

Frequently Asked Questions

1.

What Are the Ways to Prevent Nelson Syndrome?

During bilateral adrenalectomy (removal of both adrenal glands), Nelson's syndrome can be prevented by prophylactic radiotherapy (protective effect). Temozolomide, Octreotide, and Pasireotide are the most promising pharmacological drugs. However, they are frequently delivered following transsphenoidal surgery.

2.

Is Cushing Syndrome the Same as Cushing's Disease?

A tumor growing in the pituitary gland, which is benign, situated near the base of the brain, releases an excess of adrenocorticotropic hormone (ACTH), which induces the adrenal glands to release more cortisol is called Cushing disease. On the other hand, Cushing syndrome is a collection of symptoms caused by an excess of cortisol in the body.

3.

How Quickly Do the Pituitary Tumors Grow?

The vast majority of pituitary adenomas (over 90 percent) are benign, slow-growing tumors that increase at one to three millimeters per year. In contrast, about five to ten percent of pituitary tumors are slightly more aggressive (atypical), and very than one percent develop into pituitary carcinoma.

4.

What Are the Characteristic Features of Nelson Syndrome?

The invasive tumor grows and causes vision loss, pituitary failure, and headaches. One distinguishing feature of Nelson's disease is dark skin pigmentation, which occurs due to skin pigment cells reacting to adrenocorticotropic hormone (ACTH) production.

5.

What Is the Accurate Code for Nelson’s Syndrome?

Accuracy code is important as it helps to ensure that patient care is tracked correctly. So that the healthcare providers can give the best possible treatment. ICD-10-CM Nelson's syndrome is coded as E24.1

6.

Is Pituitary Tumors a Life-Threatening Condition?

Pituitary gland lesions can cause a fatal imbalance in homeostasis. In particular, pituitary adenomas (noncancerous growth on the pituitary gland), necrosis (localized death of the cells), hypophysitis (inflammation of the pituitary gland), and abscesses (collection of pus in any body parts) can cause sudden death and may only be noticed at autopsy.

7.

Who Is Predisposed to a Pituitary Tumor?

The primary risk factors are age and a family history of specific genetic diseases. Pituitary tumors can occur at any age, although they are more frequent as a person ages. A pituitary gland tumor is a prevalent subtype diagnosis among those aged 15 to 19, accounting for 33 percent of all brain tumors in this age range. Women are more prone than males to getting these tumors.

8.

Can Stress Cause Pituitary Tumors?

Chronic stress can stimulate the hypothalamic-pituitary-adrenal axis and the sympathetic nervous system, producing endocrine hormones and inducing the formation and progression of cancers. In addition, scientific studies indicate that acute stress, childhood trauma, and physical trauma elevate the risk of developing a pituitary tumor.

9.

What Are the Symptom of a Pituitary Tumor Headache?

Pituitary tumor apoplexy is characterized by a constant, rapid onset, severe headache, either bifrontal or unilateral frontal pain (ipsilateral (same side) to the tumor). In certain cases, pain is localized in the middle of the face since the face is supplied by the branches of the trigeminal nerve second division or secondary to sinusitis.

10.

Should One Be Concerned About a Pituitary Tumor?

The majority of pituitary tumors are not malignant (benign). They do not spread to the other body parts. Instead, they cause the pituitary gland to produce little or excess hormones, producing difficulties in the body. Pituitary tumors that produce too many hormones stimulate other glands to produce additional hormones.

11.

Do Pituitary Tumors Have the Potential to Alter One's Personality?

Yes. Brain tumors can induce personality changes and mood swings. Although the intensity of these mood changes varies from person to person, it is usual for someone with a brain tumor to have increased aggression. Furthermore, individuals with pituitary tumors have various emotional issues, such as depression, anxiety, behavioral difficulties, and personality changes.

12.

What Happens If a Pituitary Tumor Is Left Untreated?

Some tumors grow large enough to push normal cells in the pituitary gland's small area. This can cause visual difficulties, headaches, and other problems. The tumor also compresses the normal pituitary gland, producing insufficient hormones.

13.

Which Organs Are Affected by Cushing's Disease?

 - Cushing's disease is a significant and independent risk factor for chronic kidney disease and microalbuminuria (moderate increase in the urine albumin level).
 - Cushing's disease causes brain shrinkage and cognitive impairments.
 - A rise in liver enzymes, perhaps due to hepatic steatosis, is a typical finding in severe Cushing's syndrome.

14.

What Are the Blood Tests Available to Detect Pituitary Tumors?

Many hormonal changes associated with pituitary tumors can be detected using blood testing. A blood test can detect excessive amounts of the hormone prolactin, which results in a disease known as hyperprolactinemia. This might be an indication of a pituitary tumor called a prolactinoma.
Dr. Achanta Krishna Swaroop
Dr. Achanta Krishna Swaroop

Dentistry

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pituitary tumornelson's syndrome
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