Recognize when clinical indicators point to paraneoplastic syndromes

Association of Clinical Documentation Improvement Specialists, June 1, 2011

The ICD-9 chapter-specific guidelines for neoplasms (Section 1.C.2) provides guidance for coding and sequencing of complications associated with malignancies. This section in the ICD-9-CM Manual offers direction for coding anemia and dehydration associated with malignancies, neoplasm-related pain, encounters for chemotherapy and radiotherapy, and complications resulting from surgical procedures performed to treat malignancies. However, there is no guidance for coding the multitude of other conditions that occur in association with malignancies, many of which are classified as paraneoplastic syndromes.

Paraneoplastic syndromes are a diverse group of conditions that result from an immunological response to an underlying malignancy, but they are not caused directly by the malignancy itself. Approximately 15% of patients diagnosed with cancer will have a paraneoplastic disorder at the time of initial cancer diagnosis. Almost half of all cancer patients will develop paraneoplastic syndrome at some time during the course of the cancer. Management approaches include treatment of the underlying malignancy, medications to suppress the immune system, and treatments directed to the affected body part.
The ICD-9 Alphabetic Index entry "paraneoplastic syndrome" includes the note "see condition." Sadly, the ICD-9 Manual does not collect all of the paraneoplastic syndromes into a separate section. The ICD-10 Alphabetic Index is even more dismissive; it never mentions paraneoplastic syndromes.
Paraneoplastic syndromes are very real and often very serious. Many are CC or MCC conditions under MS-DRGs. Capturing these conditions when physicians document them in the medical record can improve accuracy and specificity for correct code selection.
The following conditions may sometimes occur as paraneoplastic syndromes, although physicians don't always label them as such:
  • Musculoskeletal disorders (code 729.90), including
    • Hypertrophic arthropathy (code 716.9x), indicating painful bone deposition and joint effusions, especially of the fingers
    • Polyarthritis (i.e., variable inflammation of large and small joints), for example, arthritis associated with leukemia (code 208.9 [713.2])
    • Polymyalgia rheumatica (code 725), indicating pain and stiffness in the hip and shoulder girdles, often with high erythrocyte sedimentation rate blood test
  • Endocrine disorders (code 259.9), including:
    • Cushing’s syndrome, adrenal hyperactivity (code 255.0)
    • Hypoglycemia (code 251.2)
    • Hypercalcemia (code 275.42)
  • Connective tissue diseases (code 710.9), including scleroderma-like, rheumatoid arthritis-like, and systemic lupus erythematosis-like diseases.
  • Amyloidosis (code 277.30), indicating abnormal protein deposition affecting the heart, intestines, kidney, and brain
  • Kidney diseases (code 593.9)
  • Glomerular diseases and nephrotic syndrome (code 581.9)
  • Dermatomyositis (code 710.3) and other inflammatory muscle disorders (code 728.9)
  • Skin disorders (code 709.9), including
    • Pemphigus (code 694.4), a skin disorder with blisters
    • Acanthosis nigricans, hyperpigmented skin (code 701.2)
    • Leukocytoclastic vasculitis (code 446.29), painful ulcerations of the distal arms and legs
Recognize clinical indicators for paraneoplastic syndromes
Some paraneoplastic syndromes are more common than others. Well-recognized paraneoplastic syndromes include:
  • Encephalitis (code 323.9) (i.e., inflammations of the brain or spinal cord)
  • Encephalomyelitis (code 323.9)
  • Endocrine disorders, e.g. hyponatremia due to the syndrome of inappropriate antidiuretic hormone (code 253.6)
  • Peripheral nerve disorders, such as:
    • Myasthenia gravis (code 358.0x)
    • Myasthenia syndromes in diseases classified elsewhere ([358.1]) includes Eaton-Lambert syndrome (code 199.1, [358.1])
    • Paraneoplastic sensory neuropathy)(code 199.1, [357.3])
Look for paraneoplastic neuropathies in the ICD-9 Alphabetic Index, reference:
-malignant neoplasm not elsewhere classified (NEC) 199.1, [357.3]
If possible, replace code 199.1 with the most specific malignancy code that the medical record supports, and sequence that code before code 357.3 (polyneuropathy in malignant disease). The instructional note under ICD-9-CM code 357.3 reinforces this sequencing guideline, by directing us to code first the underlying disease (codes 140.0–208.9). Coding guidelines are similar for cerebellar degeneration, another paraneoplastic neurologic condition.
The ICD-9 Alphabetic Index states the following for paraneoplastic cerebellar degeneration:
-in neoplastic disease NEC 239, [331.7]
In the interest of greater specificity, replace ICD-9-CM code 239 (neoplasm of unspecified nature, site unspecified) whenever possible with a code that identifies the patient's neoplasm more specifically.
Subcategory code 331.7 (cerebral degeneration in diseases classified elsewhere) includes the following note:
"Code first underlying disease, as . . . neoplastic disease (140.0–239.9)."
The principal diagnosis for a patient admitted for treatment of paraneoplastic cerebellar degeneration would be the code for the neoplasm itself, followed by the code for the paraneoplastic cerebellar degeneration.
Physician documentation of ataxia due to cerebellar degeneration may provide an opportunity for more specific code selection. The Alphabetic Index in the ICD-9-CM Manual provides several codes for ataxia. The index directs "Ataxia–acute" to code 718.3 (lack of coordination) and "Ataxia–brain (or cerebral)" to code 331.89 (cerebral degeneration). Only the search sequence "Ataxia–cerebellar" will result in assignment of the most specific term available—cerebellar ataxia (code 334.3). Consider a physician query if the physician has not provided sufficient documentation to associate the patient's ataxia and cerebellar disease.
Secondary hypercoaguable states are also common in patients with underlying malignancies; so common that attending physicians often assume that the association is obvious. Unfortunately, many physicians are unaware that we cannot code an association between two conditions unless the physician documents that association (with rare exceptions, like diabetes and osteomyelitis or renal insufficiency and hypertension).
Patients with underlying malignancies are significantly more likely to suffer deep vein thromboses or pulmonary emboli than people without cancer, and often are on anticoagulants or have an inferior vena cava filter placed. Secondary hypercoaguable state (code 292.82) is a CC condition under MS-DRGs, in recognition of the seriousness of this condition. Consider querying the physician if clinical indicators suggest the presence of a secondary hypercoaguable state due to underlying malignancy, but the physician does not explicitly document the association.
What if the physician indicates that the patient has a medical condition due to an underlying malignancy, but the physician does not specify the medical condition?
In the absence of a confirmed diagnosis, documentation in the medical record may describe the patient’s condition using terminology that includes signs, symptoms, and problems or reasons for the encounter. Medical record documentation may correspond to codes in ICD-9-CM chapter 16: Symptoms, Signs, and Ill-Defined Conditions (codes 780–799).
The ICD-9-CM Official Guidelines for Coding and Reporting (section I.C.2.g.) state,
 “Symptoms, signs, and ill-defined conditions listed in Chapter 16 characteristic of, or associated with, an existing primary or secondary site malignancy cannot be used to replace the malignancy as principal or first-listed diagnosis.”
The malignancy itself would have sequencing priority over codes from chapter 16 in the ICD-9-CM Manual. Physician documentation of a "paraneoplastic condition of the brain" without further description might support a code from chapter 6, Diseases of the Nervous System and Sense Organs, such as code 348.89 (other conditions of brain).
Sequencing for conditions outside of chapter 16 will depend on the circumstances of admission, especially whether treatment of the malignancy itself was the focus of admission versus treatment of the associated condition. Consider a query if the circumstances of admission require clarification.
The conditions described above are only a start. Hopefully, this discussion will help you assign the most accurate and specific codes for paraneoplastic syndromes that are documented or supported by clinical indicators in the medical records.
Editor’s Note: Joel Moorhead MD, PhD, CPC, is an adjunct assistant professor at the Rollins School of Public Health at Emory University in Atlanta. He is also clinical director of research and development, for FairCode Associates in Baltimore, MD. E-mail him at jmoorhead@faircode.com.
Diana Williams, BS, CPC, CCS-P, CPMA, is an adjunct regulatory specialist and an instructor for HCPro’s Certified Coder Boot Camp®, E-mail her at nlwilliams123@netzero.com.

This article was first published on the JustCoding web site www.justcoding.com.

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