Available online 10 February 2023, 101448

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Cutaneous lymphomas and lymphoid proliferations (LPD) in children, adolescents, and young adults (CAYA) are a heterogeneous group of lymphoid neoplasms that present formidable diagnostic challenges to clinicians and pathologists alike. Although rare overall, cutaneous lymphomas/LPD occur in real-world settings and awareness of the differential diagnosis, potential complications, and various therapeutic approaches will help ensure the optimal diagnostic work-up and clinical management. Lymphomas/LPD involving the skin can occur as primary cutaneous disease in a patient that characteristically has lymphoma/LPD confined to the skin, or as secondary involvement in patients with systemic disease. This review will comprehensively summarize both primary cutaneous lymphomas/LPD that occur in the CAYA population as well as those CAYA systemic lymphomas/LPD with propensity for secondary cutaneous involvement. Focus on the most common primary entities occurring in CAYA will include lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma, mycosis fungoides, subcutaneous panniculitis-like T-cell lymphoma, and hydroa vacciniforme lymphoproliferative disorder.

Section snippetsDefinition of primary cutaneous lymphomas/lymphoid proliferations

Primary cutaneous lymphomas/lymphoid proliferations (PCL/LPD) compromise a spectrum of B-cell and T/NK-cell derived neoplasias of varying aggressiveness confined to the skin at disease presentation [1,2]. PCL/LPD are very rare in children, adolescents, and young adults (CAYA) and analogous to systemic lymphomas, encompass a different spectrum of lymphoma subtypes in comparison with advanced-age adults.

Some general rules concerning the diagnosis of PCL/LPD are shared irrespective of age. PCL/LPD

Secondary skin infiltration by systemic lymphomas arising in CAYA

The dermotropism of different subtypes of systemic lymphomas mainly arising in this age group is highly variable (Fig. 1). Generally, the dermotropism of systemic mature T-cell lymphomas is far superior to mature B-cell lymphomas, whilst the inverse is true for immature/lymphoblastic T- and B-cell lymphoma/leukaemia. Table 1 summarizes a list of systemic lymphomas, which albeit rarely occur in CAYA, have a particular propensity for secondary skin involvement.

Incidence and distribution of subtypes of primary cutaneous lymphomas and lymphoid proliferations

Overall PCL/LPD are very rare diagnoses in CAYA. The estimated annual incidence—including both T-and B-cell neoplasias—can be estimated as <1/one million per year [14]. More recently published case series of PCL/LPD in CAYA (<15–20 years) report patient numbers ranging between 31 and 62 cases with collection time periods of 17–42 years [[15], [16], [17]].

PCL/LPD are diagnosed in different clinical settings and by specialties ranging from dermatologists, dermatopathologists, haematopathologist

Primary cutaneous CD30-positive T-cell lymphoproliferative disorders

Primary cutaneous CD30-positive T-cell lymphoproliferative disorders (CD30+ LPD) encompass a spectrum of diseases with overlapping histopathological and genetic features [1]. This group includes LyP and PC-ALCL; less frequently borderline cases are recognized [1,36,37]. CD30+ LPD share a proliferation of activated mature CD30+ T cells. Nevertheless, the amount of the name-defining pleomorphic/anaplastic CD30+ T cells and admixed inflammatory cells is variable in histopathology. Differing

Summary

PCL/LPD are very rare in the paediatric population and thus, it is challenging to capture extensive data on clinical trials to help guide consensus treatment guidelines. Most recommendations are made with the guidance of adult data based on higher prevalence in older patients. Because of the rarity of these diseases, an experienced pathologist should assist in diagnosis and every effort should be made to enrol these patients in biology and/or clinical trials. Multi-disciplinary collaboration

Practice points⁃

Differentiation between primary cutaneous lymphomas (confined to skin) versus secondary cutaneous lymphomas occurring in patients with systemic disease is essential.

⁃

The most common primary cutaneous lymphomas/lymphoid proliferations (LPD) in CAYA include the CD30+ LPD (lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, typically ALK-negative) and mycosis fungoides.

⁃

Although localised treatment strategies (eg topical therapies, phototherapy, surgical resection, or

Research agenda⁃

Due to the rarity of primary cutaneous lymphomas in CAYA, multi-disciplinary, international collaboration will be required for global capture of the true incidence and characteristics of disease in the CAYA population.

⁃

Future research agenda should be directed at defining the underlying disease biology of primary cutaneous lymphomas in the CAYA population to determine if differences or overlap exist in comparison to disease occurring in adults

⁃

Such research may enlighten opportunities for

Declaration of competing interest

The authors report no conflicts of interest.

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