Review of Paraneoplastic Syndromes in Children with Malignancy.

Mitur-Lesiuk M, Dubaj M, Raniewicz M, Bigosiński K, Dembowska-Kupień A
Medical science monitor : international medical journal of experimental and clinical research · 2025 · doi:10.12659/msm.947393 · PMID:40159669 · PMC11970335
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This review outlines the pathophysiology, clinical manifestations across various organ systems, diagnostic approaches, and treatment strategies for paraneoplastic syndromes in children with malignancy.

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This review article examines paraneoplastic syndromes in pediatric patients with malignancies, focusing on cutaneous, rheumatological, and hepatic manifestations that arise from immune cross-reactions or hormone release rather than direct tumor infiltration. The authors highlight conditions such as pruritus associated with lymphomas, alopecia areata linked to Hodgkin lymphoma, and dermatomyositis preceding cancer diagnosis, noting that treatment primarily targets the underlying neoplasm. A significant limitation noted is the scarcity of specific statistical data for these syndromes in children compared to adults, making early recognition through oncological vigilance critical for improving curability rates. Relevance to endometriosis: listed as one indication for GnRH antagonists, though the paper's main focus is uterine fibroids.

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Abstract

A paraneoplastic syndrome (PNS) is a set of symptoms and signs that can accompany the formation of a cancer but is not due to its direct infiltration or metastasis. PNS results from the secretion of peptides or hormones by tumor cells or from an immunological cross-reaction between the tumor antigens and host antigens. In the adult population, PNS affects up to 15% of patients with cancer, but data on pediatric patients remain lacking. The remarkable fact is that PNS can precede an oncology diagnosis, even by months or years. PNS can involve virtually any organ of the human body. In children, the most commonly involved are the nervous system (encephalitis, opsoclonus-myoclonus syndrome), skin (pemphigus, alopecia areata, pruritic skin, pyoderma gangrenosum, skin nevi), rheumatologic (dermatomyositis, vasculitis), liver (atrophic biliary syndrome, idiopathic cholestasis), endocrine system (hypercalcemia, syndrome of inadequate secretion of antidiuretic hormone), kidney (nephrotic syndrome), or hematopoietic system (hemolytic anemia, thrombocytopenia, eosinophilia, thrombotic macroangiopathy, leukomoid reaction). PNS can accompany all childhood cancers, but is most common in Hodgkin lymphoma, acute lymphoblastic and myeloid leukemia, neuroblastoma, Wilms tumor, and sarcoma. Diagnosis of PNS should begin as early as the suspicion of its unusual course, lack of response to standard treatment, or prolonged duration. Diagnosis should include typical disease-specific tests and simultaneous imaging of the head and neck, abdomen, and pelvis, as well as a bone marrow biopsy to look for malignancy. PNS treatment mainly includes anti-tumor therapy and sometimes additional immunosuppressive therapy. This article aims to review PNS in children with malignancy.
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Intro

Each year, approximately 400 000 children worldwide and 1200 in Poland receive a diagnosis of cancer, and there is a consistent increase in incidence [ 1 , 2 ]. The most common childhood cancers are hematological diseases, primarily leukemias [ 3 ]. Conditions that can accompany or even precede the development of cancer by many months and years are classified as paraneoplastic syndromes (PNS). PNS are defined as a group of symptoms occurring together with oncological disease, but not resulting from direct infiltration or the development of metastases, but rather from the release of hormones and peptides that affect host metabolic pathways or from immune system cross-reactions with neoplastic cells [ 4 ]. PNS is estimated to occur in up to 15% of oncological patients, being the second most common cause of mortality in this group (27%). Unfortunately, exact statistics for pediatric patients are not known [ 4 ]. Studies describing PNS in adults are common, but publications regarding pediatric patients are rare. Recognizing PNS is extremely important, as cancers in the pediatric group are curable in as much as 85.7% of patients [ 5 ]. PNS in children can involve all organs but most commonly involve the nervous system, skin, and hematopoietic system. PNS can accompany all cancers, but the largest number of described cases are Hodgkin lymphoma (HL), acute leukemia, and neuroblastoma. Oncological diagnosis of PNS should begin simultaneously with the diagnosis of the symptom itself, as part of so-called oncological vigilance. PNS treatment mainly includes treatment of the underlying disease and immunosuppressive therapy [ 6 , 7 ]. This article aims to review PNS in children with malignancy.

Other

PNS in pediatric patients are extremely rare. Epidemiological studies on larger groups of patients are definitely lacking on this topic. Further case reports and reviews are also needed to identify risk factors and effective therapeutic approaches in pediatric patients with PNS. It would also be useful to have guidelines that address precisely the diagnostic algorithms for each syndrome, as well as the features that would prompt the moment when oncologic diagnosis should be initiated.

Conclusions

PNS are not common phenomena but have remarkable diagnostic value. They can affect almost any system of the body. They often precede overt symptoms and cancer diagnosis by up to several months or years. Therefore, any symptom or, even more so, a set of symptoms that do not respond to the typical treatment or occur in high-risk patients should alert the physician to the need for extended early diagnosis in search of cancer. This is extremely important among pediatric patients because of the incredibly high cure rate of more than 80% for all types of cancer and the higher success of therapy if the disease is detected quickly.

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