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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.4 Rio de Janeiro July/Aug. 2011
Maria Christina Lopes Araujo de OliveiraI; Luciana Baptista PereiraII; Priscila Cezarino RodriguesIII; Keyla Cunha SampaioIV; Benigna Maria de OliveiraV; Marcos Borato VianaVI
IPhD. Associate Professor, Department of Pediatrics, Federal University of Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil
IIMaster's Degree. Assistant Professor, Department of Clinical Medicine/Dermatology, Federal University of Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil
IIIMaster's Degree. Physician, Hematology Department, Teaching Hospital of the Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
IVPhD. Physician, Hematology Department, Teaching Hospital of the Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
VPhD. Adjunct Professor, Department of Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
VIPhD. Full Professor, Department of Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
Cutaneous lymphomas comprise a heterogeneous group of lymphoproliferative disorders with skin involvement and are classified as a subgroup of non-Hodgkin lymphomas. From 1981 to 2007, 100 children with non-Hodgkin lymphomas were admitted to the Hematology Unit of the Federal University of Minas Gerais Teaching Hospital. In nine of these children, the skin was involved at the onset of the disease. Three patients were classified as having primary cutaneous lymphoma, while in six the disease was systemic with cutaneous involvement. In seven patients, the immunophenotype was T-cell, in one it was B-cell, and in the remaining case the immunophenotype was indefinable. No deaths occurred in any of the children with primary cutaneous lymphoma.
Keywords: Lymphoma, T-cell, cutaneous; Pediatrics; Prognosis
Lymphomas involve the skin either primarily or secondarily and are classified as a subgroup of non- Hodgkin lymphomas (NHL). They can only be considered primary cutaneous lymphomas (PCL) when the initial presentation is in the skin and there is no evidence of extracutaneous involvement at diagnosis, following complete staging. 1,2 In children, NHLs represent 6 to 10% of malignant neoplasms. After the gastrointestinal tract, the skin is the extranodal site most affected. 3,4
The objective of this study was to describe the clinical course of children with skin manifestations of lymphoma being followed-up at the hematology department of the Teaching Hospital of the Federal University of Minas Gerais. In a retrospective study of 100 children with NHL admitted to the department between 1981 and 2007, nine patients had skin involvement at diagnosis. The study was approved by the Internal Review Board of the Federal University of Minas Gerais.
Of the nine patients, four were boys and five were girls (Figure 1). The median age at diagnosis was 4.9 years (range 1.5 to 12.8 years). These nine patients all had skin lesions at diagnosis, located principally on the upper and lower limbs, followed by the face and scalp. In seven children, multiple lesions were present, while two children had single lesions (Figures 1A and 1B). In four patients, there was spinal involvement, affecting the central nervous system in one and causing lymphadenomegaly in another. Therefore, the disease was considered systemic with skin involvement in six patients and primary cutaneous in three. All were submitted to systemic chemotherapy, which differed in accordance with the presentation and histological type.
The median time of follow-up was 3.4 years, ranging from 3 months to 17.4 years. The three patients initially diagnosed as PCL (patients 5, 8 and 9) achieved complete remission and remained in remission until the end of the study. Of the six children with the systemic disease, three are still in remission and three have died, two due to complications of the treatment, while the third, who was human immunodeficiency virus (HIV)-positive, died from the primary disease (patient #2). All the deaths occurred during an active phase of the disease.
Unlike systemic NHL in which the most com-mon cell lineage is B, cases of PCL are generally T-cell related. Cutaneous T-cell lymphomas constitute a heterogenous group of lymphoproliferative diseases characterized by a clonal expansion of mature postthymic T-cells that infiltrate the skin. Cutaneous B-cell lymphomas are rare in children except in the uncommon cases of precursor B-cell lymphoblastic lymphoma (B-LBL) in which the initial manifestation of the disease is in the skin. 5 In the present sample, T cell lineage was the most prevalent, occurring in seven patients. Only one case of B lineage was found and in one case the immunophenotype could not be defined (negative for B and T markers).
Mycosis fungoides (MF) is the most common form of cutaneous lymphoma in children and adolescents, accounting for 40% of cases. Clinical progression is slow. 1,6, 7 In the present sample, no cases of MF were found, which may reflect a true epidemiological variation or, more probably, a variation related to the sample population.
Anaplastic large cell lymphomas (ALCL) may be primary to the skin (patient #5) or may be systemic (patient #2). In children, the systemic form is more common and prognosis is poorer when the skin is also affected. 8, 9 Prognosis is better in cases of primary cutaneous ALCL and cytogenetic alterations such as t(2;5)(p23;q35) translocation and the product of the resulting fusion, anaplastic lymphoma kinase (ALK) protein, which occur in the systemic form, are not generally found. Therefore, although the morphological status is identical, the hypothesis has been raised that primary cutaneous CD 30-positive ALCL and systemic ALCL may represent different diseases that differ in their clinical behavior and in their pathogenesis.10
One patient was classified as having subcutaneous panniculitis-like T cell lymphoma (Patient #8), since the neoplastic infiltrate was diffusely affecting the subcutaneous tissue. This is a less common subtype of primary cutaneous T-cell lymphoma.1
With respect to the patient with the B immunophenotype, the histological type consisted of a precursor B-cell lymphoblastic lymphoma. As reported in the literature, precursor B-cell neoplasias generally present as acute lymphoblastic leukemia. Precursor B-cell lymphoblastic lymphoma is rare. It occurs in young, female patients and tends to affect the skin and long bones. 5 The patient in the present sample was also young and female, with spinal involvement at diagnosis, later developing lymphoblastic leukemia. She died three months after the beginning of treatment.
It is interesting to note that in three patients in the present sample the initial diagnosis was not cutaneous lymphoma but rather several different conditions that clinically resemble lymphoma: bacillary angiomatosis (patient #2), American tegumentary leishmaniasis (patient #5) and mastocytosis (patient #8). This reinforces the need to take a diagnosis of cutaneous lymphoma into consideration even in children despite the fact that it is rare in this age-group.
In relation to prognosis, patients with primary cutaneous lymphoma generally have a greater probability of disease-free survival than patients with secondary cutaneous lymphoma, despite the fact that the histological and immunohistochemical features of the secondary skin lesions are identical to those of the systemic disease.
In conclusion, non-Hodgkin lymphoma (NHL) with primary skin manifestation is a rare disease in childhood, accounting for around 1% of cases of NHL in children and adolescents. It may be confused with other non-neoplastic conditions. Although small, the present sample suggests that in cases of primary cutaneous lymphoma prognosis is indeed favorable; however, this is not the case with the systemic form of the disease, which should probably be considered to belong to the group of NHL in which the risk of recurrence and death is high.
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Mailing address: Received on 23.05.2010. * Study conducted at the Pediatric Hematology Unit, Teaching Hospital of the Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
Maria Christina Lopes A Oliveira
Rua Engenheiro Amaro Lanari,389 - Apt.501
CEP: 30.320-580 Belo Horizonte - MG, Brazil
Approved by the Advisory Board and accepted for publication on 07.06.10.
Conflict of interest: None
Financial funding: None
Received on 23.05.2010.
* Study conducted at the Pediatric Hematology Unit, Teaching Hospital of the Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.