The ICD 10 code is one among many codes that are used by medical professionals in the diagnosis of various illnesses. The 2019 ICD 10 CM codes were implemented as of October 2018 and they involve new codes, changed codes, revised codes as well as deactivated codes. Under the ICD 10 code for the paraneoplastic syndrome, there is the G13.0, which takes into account the manifestation of an underlying disorder, but not the disorder itself. A paraneoplastic neurological disorder is defined as a remote effect of cancer that is not caused by the tumor, its spread, or even by an infection, ischemia, or metabolic disruptions.[1] [2]


What Is The ICD 10 Code For Paraneoplastic Syndrome?

There is no one code for paraneoplastic syndrome. However, there is a code for paraneoplastic neuromyopathy and neuropathy, that befits paraneoplastic syndrome. The ICD 10 code that is meant for this syndrome is dismissive, as it doesn’t mention the syndrome directly. In addition to that, the former code, i.e. the ICD 9 doesn’t collect all the paraneoplastic syndromes into separate sections. It is important to recognize clinical presentations that tend towards paraneoplastic syndromes even though they may not be labeled as such.


Some of the clinical indicators of the paraneoplastic syndrome are more common or rather well-recognized compared to other indicators. They are; encephalitis, which is the inflammations of the brain or spinal cord, endocrine disorders such as hyponatremia, peripheral nerve disorders including myasthenia gravis and paraneoplastic sensory neuropathy, and encephalomyelitis.

Adding to that, they sometimes occur as paraneoplastic syndromes, and they include;


  • Musculoskeletal disorder
  • Polyarthritis e.g. arthritis associated with Leukemia
  • Amyloidosis – indicating abnormal protein deposition affecting organs such as the heart, brain, kidney, and intestines.
  • Hypertrophic arthropathy – indicating painful bone deposition and joint effusions, in particular, of the fingers
  • Endocrine disorders which include; hypoglycemia, hypercalcemia, and Cushing’s syndrome
  • Connective tissue diseases including rheumatoid arthritis-like, scleroderma-like, and systemic lupus erythematosus-like diseases
  • Glomerular diseases and nephrotic syndrome among others[3] [4]

There are a number of new codes and other changes in Chapter 2 Neoplasm. Some of the new codes are inclusive of malignant melanoma of the right upper and right lower eyelid including canthus, as well as malignant melanoma of left upper and left lower eyelid including canthus.[5]

What Are Paraneoplastic Syndromes?

Paraneoplastic syndromes are rare disorders affecting less than 1 patient in 10,000 with cancer. They are triggered by an altered immune response to a neoplasm. In other words, neoplastic syndromes can be considered as symptoms of substances released by the tumor, but occur remotely from the tumor itself. These symptoms can range from endocrine, musculoskeletal, cutaneous, hematologic, renal, gastrointestinal, cardiovascular or even miscellaneous symptoms.

The Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small cell lung cancer. Other common clinical presentations of paraneoplastic syndromes include; subacute cerebral ataxia, opsoclonus-myoclonus limbic encephalitis, chronic gastrointestinal pseudoobstruction, sensory neuronopathy, retinopathies, stiff-person syndrome, dermatomyositis, encephalomyelitis, and the retinopathy.

Diagnosis And Treatment Of Paraneoplastic Syndrome

Paraneoplastic syndrome is a neurological disorder, which in many patients develops prior to cancer becoming clinically evident. Since they have high specificity, the best way of diagnosis is by identifying one of the well-characterized anti-onconeural protein antibodies in a patient’s serum. The best treatment for paraneoplastic syndromes is the treatment of the underlying condition, which in this cancer is a particular tumor. This is basically because the syndromes arise as a result of cancer, causing an autoimmune response towards the antibodies, common in cancer and nervous system. Therefore, due to this phenomena, suppression of the immune response is also considered as a treatment approach.[2] [6]


The ICD 10 code for paraneoplastic syndromes is a diagnosis guideline for the condition. For most patients, these syndromes arise due to the presence of a tumor, which causes an immune system response. The ICD 10 code should be detailed enough to allow for a proper medical diagnosis. However, in the absence of a confirmed diagnosis, the medical report made should be inclusive of symptoms, signs, and problems or reasons for the encounter.


Also Read:

Pramod Kerkar

Written, Edited or Reviewed By:


Pain Assist Inc.

Last Modified On: July 8, 2019

This article does not provide medical advice. See disclaimer


Sign Up for Our Newsletter

We'll help you live each day to the healthiest