Review of paraneoplastic syndromes in children

Pediatr Radiol. 2019 Apr;49(4):534-550. doi: 10.1007/s00247-019-04371-y. Epub 2019 Mar 16.


Paraneoplastic syndromes are defined as clinical syndromes that are not related to direct tumor invasion or compression but are secondary to tumor secretion of functional peptides/hormones or related to immune cross-reactivity with normal host tissue. Paraneoplastic syndromes have a wide range of presentations and can present before the primary malignancy or tumor recurrence is diagnosed. They can mimic non-neoplastic processes, making detection, diagnosis and treatment difficult. However, they can also provide clues to the presence of an underlying malignancy. In this paper, we reviewed a range of paraneoplastic syndromes that can occur in children including: (1) neurologic (opsoclonus-myoclonus, limbic, anti-N-methyl-d-aspartate [NMDA] and anti-Ma2 encephalitis and myasthenia gravis); (2) endocrine (neuroendocrine tumors, hypercalcemia, SIADH [syndrome of inappropriate antidiuretic hormone secretion], osteomalacia/rickets and ROHHAD [rapid onset of obesity, hypoventilation, hypothalamic dysfunction and autonomic dysregulation]); and (3) dermatologic/rheumatologic syndromes (hypertrophic osteoarthropathy and paraneoplastic pemphigus). Familiarity with these syndromes can aid in early diagnosis, treatment and imaging optimization.

Keywords: Children; Computed tomography; Magnetic resonance imaging; Malignancy; Oncology; Paraneoplastic syndrome.

Publication types

  • Review

MeSH terms

  • Child
  • Diagnosis, Differential
  • Humans
  • Paraneoplastic Syndromes / diagnostic imaging*
  • Risk Factors