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December 2012 Coding Corner

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Diseases of the Adrenal Cortex: Adrenal Cancer

What are the adrenal glands? The human body has two adrenal glands, located atop each of the two kidneys. They are critical to managing hormone levels and the other bodily systems that require these hormones to function.

The adrenal cortex is the outer part of the adrenal gland and makes cortisol, aldosterone, and dehydroepiandrosterone (DHEA), which carefully controls metabolism, hair growth and body shape.

The adrenal medulla is the inner part of the gland and produces epinephrine (adrenaline), norepinephrine, and dopamine which control the body’s responses to stress, including blood pressure.

Adrenal gland tumors develop when normal cells change and grow uncontrollably, and can be benign or malignant.

There are four main types of Adrenal Gland Tumors:

Adenoma - This is noncancerous and the most common type of adrenal gland tumor that affects the adrenal cortex. This type of tumor usually does not present with symptoms; and if small in size, often does not require treatment. (ICD-9-CM code 227.0).

Adrenocortical carcinoma (also adrenal cortical carcinoma) - This is a rare, cancerous type of adrenal gland tumor affecting one or two people in one million. This is a particularly aggressive form of cancer, and can grow for years without a person knowing and spread to other organs or a system before it is discovered. (ICD-9-CM code 194.0 if it is the primary site).

Neuroblastoma - This is a childhood cancer that forms when immature neuroblasts don’t mature properly. Neuroblastoma can form before the baby is born.  Although it can sometimes be discovered through a pre-natal ultrasound, many times, it is found after the cancer has already spread. (ICD-9-CM 194.0 if it is the primary site).

Pheochromocytoma - This is a cancerous neuroendocrine tumor which usually begins in the adrenal medulla.  (ICD-9-CM 194.0 if it is the primary site).

Characteristics of Adrenal Cortical Cancer

  • Typically an aggressive cancer  
  • Most (~60%) are found because excess hormone production causes symptoms which prompt patients to seek medical attention  
  • Most (60-80%) actually secrete high amounts of one or more adrenal hormones  
  • Many will present with pain in the abdomen and flank (nearly all that don't present with symptoms of hormone excess will seek medical attention because of pain)  
  • Spread to distant organs (metastasis) occurs most commonly to the abdominal cavity, lungs, liver, and bone  

Evaluation of a Suspected Adrenal Cortical Cancer

The initial evaluation should include blood tests to measure the amount of adrenal hormones in the circulation. Since the vast majority of these cancers make too much hormone (cortisol, testosterone, estrogen, aldosterone, etc.), this is an obvious place to start. Keep in mind, however, that most non-cancerous tumors of the adrenal glands (benign adenomas and hyperplasia) will also secrete too much hormone. Therefore, demonstrating overproduction of adrenal hormones helps establish the presence of an adrenal tumor, yet it does not always help distinguish between benign and malignant tumors. Extremely high levels, however, are more commonly produced by malignant tumors.

Some adrenal tumors will require special studies of the blood supply to help define the extent of the tumor, whether it is impinging on the blood supply to other nearby organs, and to help the surgeon decide on which operative approach to use. These tests are referred to as selective angiography and adrenal venography. They also may be helpful in distinguishing tumors of the adrenal gland from tumors of the upper pole of the kidney

Adrenal Syndromes Caused by Excess Hormone Secretion

Many patients will seek medical attention with some sort of bodily change which typically comes on quite slowly (usually over 1 to 3 years). When excess female hormones are produced in a female it can be hard to detect, except at extremes of age such as early puberty in a child, or the return of vaginal bleeding in a post-menopausal woman. The same is true for excess testosterone in a male. The opposite, however, will often make the presentation easier such as when a woman begins to develop male characteristics (deeper voice, excess body hair) or when a man begins to develop enlarged breasts. Some of these hormone overproduction problems have specific names and are listed below.

  • Hypercortisolism - Excess cortisol produced (ICD-9-CM code 255.3)
  • Cushing’s Syndrome – Excess cortisol produced, typically from long term use of medications containing cortisol (ICD-9- CM code 255.0)
  • Adrenogenital Syndrome - Excess sex steroids produced) (ICD-9-CM code 255.2)
  • Virilization - Acquisition of male traits in a female because of excess testosterone production (ICD-9-CM code 255.2)
  • Feminization - Acquisition of female traits in a male because of excess estrogen production (ICD-9-CM code 259.51)
  • Precocious Puberty - Puberty occurring too early because of excess sex steroids produced. (ICD-9-CM code 259.1)
  • Hyperaldosteronism (Conn's syndrome) - Excess aldosterone leading to hypertension and low potassium. (ICD-9-CM code 255.10)

In short, in women, many symptoms of adrenal gland disorders and cancers mimic the signs of menopause and go undiagnosed until widespread metastasis has already occurred. 


Diseases of the Adrenal Cortex: Adrenal Cancer Article by James Norman MD, FACS, FACE

Adrenal Gland Tumors:  Facts, Symptoms and Treatments by Darlene Oakley

Coding Corner Q&A – December, 2012


Can we use secondary code 729.5, pain in limb with primary code 282.62, SS disease crisis?


It would not be appropriate to code 729.5 with 282.65.  Per Official ICD-9-CM Guidelines for Coding and Reporting, “Codes that describe symptoms and signs, as opposed to diagnoses, are acceptable for reporting when a diagnosis has not been established (confirmed) by the provider. …”  Sickle-cell pain crisis is an established diagnosis, so symptoms are not coded.

Pain is a symptom, usually caused by some other disorder.  In the case of sickle cell crisis, the pain is a symptom of the crisis and not coded separately.  The ICD-9-CM has a note under the crisis codes directing coders to “Use additional codes for type of crisis….”  This does not mean symptoms, but actual manifestations of the crisis such as acute chest syndrome or splenic sequestration.

Coding Clinic, 4th Quarter, 2003, pg 51-56 has an article of what sickle-cell is and the codes associated with it.  Coding Clinic 2nd Quarter, 1998, pg 8 has an article on sickle cell with acute chest syndrome (this was written before the new sickle-cell codes were created).

ICD-10 Spotlight

Chapter 16: Certain Conditions Originating in the Perinatal Period (P00-P96)  

This month, we are reviewing some of the coding guidelines for perinatal conditions.

In comparing the ICD-9-CM chapters with the ICD-10-CM blocks related to perinatal conditions, note the addition of many helpful subcategories. Though many of the same or similar diagnosis are found in ICD-9-CM and ICD-10-CM, there are more specific areas with the ability for future expansion of codes. 

Refer to the table for a review of differences between the two codes sets.  There are significant differences in just the number of chapters/blocks!


Chapter 15. Certain Conditions Originating in the Perinatal Period (760-779)

  • Maternal causes of perinatal morbidity and mortality (760-763)
  • Other conditions originating in the perinatal period (764-779)


Chapter 16. Certain Conditions Originating in the Perinatal Period (P00-P96)

  • P00-P04 Newborn affected by maternal factors and by complications of pregnancy, labor and delivery
  • P05-P08 Disorders of newborn related to length of gestation and fetal growth
  • P09 Abnormal finding on neonatal screening
  • P10-P15 Birth trauma
  • P19-P29 Respiratory and cardiovascular disorders specific to the perinatal period
  • P35-P39 Infections specific to the perinatal period
  • P50-P61 Hemorrhagic and hematological disorders of newborn
  • P70-P74 Transitory endocrine and metabolic disorders specific to newborn
  • P76-P78 Digestive disorder of newborn
  • P80-P83 Conditions involving the integument and temperature regulation of newborn
  • P84 Other problems of newborn
  • P90-P96 other disorders originating in the perinatal period

As with ICD-9-CM guidelines, the perinatal period is defined as before birth through the 28th day following birth.  The codes for the perinatal period are never allowed on the maternal record.  These codes are for the newborn only, but can be used throughout the life of the patient if the condition is still present.

Examples of this would be diagnoses that can prove to be long term such as P78.81, Congenital Cirrhosis of the liver or P96.0, Congenital Renal Failure.

The guideline regarding a diagnosis that could be either due to the birth process or be community acquired does not change in ICD-10-CM.  If the documentation does not clarify which, it is correct to make an assumption that the diagnosis is due to the birthing process.  If the documentation clearly identifies a community acquired cause, then do not use a code from Chapter 16. In other words, if the physician does not specify, the default coding will always be a code in Chapter 16. See coding guideline 16.a.5 for specific details.

When coding clinically significant conditions (secondary codes) it is important to note all of the similarities to ICD-9-CM with this rule.  Our guidance leads us to code all clinically significant conditions noted on routine newborn examinations.  As with ICD-9-CM, there remains the additional final point for newborn examinations that is different from the general coding guidelines. A condition is clinically significant if it requires:

  • Clinical evaluation; or
  • Therapeutic treatment; or
  • Diagnostic procedures; or
  • Extended length of hospital stay; or
  • Increased nursing care  and / monitoring; or
  • Has implications for future health care needs

If the diagnosis is questionable regarding the implications for future and possible healthcare needs, as always it is appropriate to query the physician. 

In many cases, a newborn will have a suspected condition that, after study is determined to not be a valid diagnosis.  In those cases, code the signs and symptoms from the appropriate chapter.

Example:   A healthy newborn is suspected of having sepsis due to increased temperature and listlessness for two days.  If after study it is determined that the infant does not have sepsis, only code the symptoms provided.

In ICD-9-CM, the area of newborn coding tends to be a challenge for many coders.  Most of the guidelines for ICD-10-CM are similar or the same to ICD-9-CM.  But with ICD-10-CM, it is a more detailed code set that will provide additional clarity and concisely explain the condition, while allowing for further expansion of other codes - a benefit that ICD-9-CM was not able to provide.