Paraneoplastic Syndromes Treatment & Management

Updated: Dec 24, 2016
  • Author: Luigi Santacroce, MD; Chief Editor: Wafik S El-Deiry, MD, PhD  more...
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Treatment

Approach Considerations

Treatment varies with the type and location of the paraneoplastic disorder. Two general treatment options exist.

The first option is treatment of the underlying tumor. In general, the therapeutic protocols used are those that are applied to the disease in patients without paraneoplastic syndromes (ie, surgery, radiation, or chemotherapy, alone or in combination).

The second therapeutic option, in patients with clearly identifiable antibodies in their serum, is immunosuppression. This may be accomplished with intravenous immunoglobulins, [48, 49] steroids or other immunosuppressive drugs, [50] or plasma exchange. Some patients with paraneoplastic pemphigus [51, 52, 53, 54] seem to derive some benefit from rituximab, but further studies are necessary to confirm this observation.

Standard perioperative care and monitoring before and after radiotherapy or chemotherapy are required. Systematic psychotherapeutic and rehabilitative interventions may also be helpful.

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Surgical Care

Surgical treatment for patients with paraneoplastic syndromes is typically directed toward the underlying neoplasm. On the other hand, some paraneoplastic disorders may resolve rapidly without surgery on the primary tumor (eg, in patients with hypertrophic osteoarthropathy, resection of either the tumor or the ipsilateral vagus nerve leads to rapid remission of symptoms).

Selected surgical options are as follows:

  • In patients with ectopic adrenocorticotropic hormone (ACTH) syndrome, bilateral adrenalectomy [55] with hormone replacement is the most effective treatment
  • Patients with thymoma must have complete surgical resection of the tumor to obtain complete remission of paraneoplastic syndromes (ie, myasthenia gravis)
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