Marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue type is a peculiar variant of B-cell neoplasm showing distinct clinical and pathologic features. Its occurrence in children or adolescents is extremely uncommon. We describe the case of an 11-year-old boy who developed such a lymphomatous process involving primarily the skin. The tumor was characterized by a maturing plasma cell population that included immunoblasts, heavy-chain but not light-chain restriction suggestive of monoclonality, and a prevalent, focally granulomatous component of histiocytes. As an additional interesting feature, a trisomy 3 was demonstrated by fluorescence in situ hybridization analysis. The clinical, morphological, phenotypic, and genetic characteristics seen in our patient do not differ significantly from those described in primary cutaneous MZBCL occurring in adults.
WillemzeRJaffeESBurgG. WHO-EORTC classification for cutaneous lymphomas. Blood2005;105:3768–3785.
2.
Fink-PuchesRChottAArdigóM. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol2004;21:525–533.
3.
GhislanzoniMGambiniDPerroneTAlessiEBertiE. Primary cutaneous follicular center cell lymphoma of the nose with maxillary sinus involvement in a pediatric patient. J Am Acad Dermatol2005;52:S73–S75.
4.
IsaacsonPGMüller-HermelinkHKPirisMA. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: JaffeESHarrisNLSteinHVardimanJW, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Hematopoietic and Lymphoid Tissues. Lyon: IARC Press, 2001;157–160.
5.
HorstmannMErttmannRWinklerK. Relapse of MALT lymphoma associated with Helicobacter pylori after antibiotic treatment. Lancet1994;343:1098–1099.
6.
BleckerUMcKeithanTWHartJKirschnerBS. Resolution of Helicobacter pylori–associated gastric lymphoproliferative disease in a child. Gastroenterology1995;109:973–977.
7.
Teruya-FeldsteinJTemeckBKSloasMelisse M. Pulmonary malignant lymphoma of mucosa-associated lymphoid tissue (MALT) arising in a pediatric HIV-positive patient. Am J Surg Pathol1995;19:357–363.
8.
Teruya-FeldsteinJTemeckBKSloasMelisse M. Author's reply. Am J Surg Pathol1996;20:644.
9.
JoshiVVGagnonGAChadwickEG. The spectrum of mucosa-associated lymphoid tissue lesions in pediatric patients infected with HIV. A clinicopathologic study of six cases. Am J Clin Pathol1997;107:592–600.
10.
BerrebiDLescoeurBFayeAFaureCVilmerEPeuchmaurM. MALT lymphoma of labial minor salivary gland in an immunocompetent child with a gastric Helicobacter pylori infection. J Pediatr1998;133:290–292.
11.
KurugogluSMihmanliICelkanTAkiHAksoyHKormanU. Radiological features in paediatric primary gastric MALT lymphoma and association with Helicobacter pylori.Pediatr Radiol2002;32:82–87.
12.
Taddesse-HeathLPittalugaSSorbaraLBusseyMRaffeldMJaffeES. Marginal zone B-cell lymphoma in children and young adults. Am J Surg Pathol2003;27:522–531.
13.
DargentJLFersterAAndryG. Marginal zone B-cell lymphoma of the sinonasal tract in an eleven-year-old girl. Med Pediatr Oncol2003;40:393–395.
14.
LiangXStorkLCAlbanoEA. Primary ocular adnexal lymphoma in pediatric patients: report of two cases and review of the literature. Pediatr Dev Pathol2003;6:458–463.
15.
MoJQDimashkiehHMallerySRSwerdlowSHBoveKE. MALT lymphoma in children: case report and review of the literature. Pediatr Dev Pathol2004;7:407–413.
16.
SwerdlowSH. Pediatric follicular lymphomas, marginal zone lymphomas, and marginal zone hyperplasia. Am J Clin Pathol2004;122:(suppl 1):S98–S109.
17.
TiemannMHäringSHeidemannMReicheltJClaviezA. Mucosa-associated lymphoid tissue lymphoma in the conjunctiva of a child. Virchows Arch2004;444:198–201.
18.
ClaviezAMeyerUDominickCBeckJFRisterMTiemannM. MALT lymphoma in children: a report from the NHL-BFM Study Group. Pediatr Blood Cancer In press.
19.
IsaacsonPGDuMQ. MALT lymphoma: from morphology to molecules. Nat Rev Cancer2004;4:644–653.
GrønbækKRalfkiaerEKallaJSkovgaardGLGuldbergP. Infrequent somatic Fas mutations but no evidence of bcl10 mutations or t(11;18) in primary cutaneous MALT-type lymphoma. J Pathol2003;201:134–140.
22.
StreubelBLamprechtADierlammJ. T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. Blood2003;101:2335–2339.
23.
StreubelBSimonitsch-KluppIMüllauerL. Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. Leukemia2004;18:1722–1726.
24.
SchreuderMIHoefnagelJJJansenPMvan KriekenJHJMWillemzeRHebedaKM. FISH analysis of MALT lymphoma-specific translocations and aneuploidy in primary cutaneous marginal zone lymphoma. J Pathol2005;205:302–310.
25.
StreubelBVinatzerULamprechtARadererMChottA. T(3;14)(p14.1;q32) involving IGH and FOXP1 is a novel recurrent chromosomal aberration in MALT lymphoma. Leukemia2005;19:652–658.
26.
DierlammJRosenbergCStulM. Characteristic pattern of chromosomal gains and losses in marginal zone B cell lymphoma detected by comparative genomic hybridization. Leukemia1997;11:747–758.
27.
DierlammJ. Genetic abnormalities in marginal zone B-cell lymphoma. Haematologica2003;88:8–12.
28.
GazzoSBaseggioLCoignetL. Cytogenetic and molecular delineation of a region of chromosome 3q commonly gained in marginal zone B-cell lymphoma. Haematologica2003;88:31–38.
29.
AamotHVMicciFHolteHDelabieJHeimS. G-banding and molecular cytogenetic analyses of marginal zone lymphoma. Br J Haematol2005;130:890–901.
30.
YazdiASPuchtaUFlaigMJSanderCA. Helicobacter pylori not detected in cutaneous mucosa-associated lymphoid tissue (MALT) lymphomas. Arch Dermatol Res2003;294:447–448.
31.
PratiDZanellaADe MatteiC. Chronic hepatitis C virus infection and primary cutaneous B-cell lymphoma. Br J Haematol1999;105:839.
32.
ViguierMRivetJAgbalikaF. B-cell lymphomas involving the skin associated with hepatitis C virus infection. Int J Dermatol2002;41:577–582.
33.
MichaelisSKazakovDVSchmidMDummerRBurgGKempfW. Hepatitis C and G viruses in B-cell lymphomas of the skin. J Cutan Pathol2003;30:369–372.
34.
NagoreELedesmaEColladoCOliverVPérez-PérezAAliagaA. Detection of Epstein-Barr virus and human herpesvirus 7 and 8 genomes in primary cutaneous T- and B-cell lymphomas. Br J Dermatol2000;143:320–323.
35.
MerinoRde InocencioJGarcía-MiguelPGarcía-ConsuegraJ. Lymphoproliferative disorders in paediatric rheumatic diseases. A report of two cases. Clin Exp Rheumatol2004;22:649–650.
36.
HsiEDSingletonTPSwinnenLDunphyCHAlkanS. Mucosa-associated lymphoid tissue-type lymphomas occurring in post-transplantation patients. Am J Surg Pathol2000;24:100–106.
37.
BaldassanoMFBaileyEMFerryJAHarrisNLDuncanLM. Cutaneous lymphoid hyperplasia and cutaneous marginal zone lymphoma. Comparison of morphologic and immunophenotypic features. Am J Surg Pathol1999;23:88–96.
38.
LeachIHMacLennanKA. Gastric lymphoma associated with mucosal and nodal granulomas: a new differential diagnosis in granulomatous gastritis. Histopathology1990;17:87–89.
39.
FliederDBYousemSA. Pulmonary lymphomas and lymphoid hyperplasias. In: KnowlesDM, ed. Neoplastic Hematopathology. Philadelphia: Lippincott Williams & Wilkins, 2001;1263–1301.
40.
VaílloAGuttiérrez-MartínABallestínCRuiz-LisoJM. Marginal zone B-cell lymphoma of the parotid gland associated with epithelioid granulomas. Report of a case with fine needle aspiration. Acta Cytol2004;48:420–424.
41.
ScarabelloALeinweberBArdigóM. Cutaneous lymphomas with prominent granulomatous reaction. A potential pitfall in the histopathologic diagnosis of cutaneous T- and B-cell lymphomas. Am J Surg Pathol2002;26:1259–1268.
