Linfoma T subcutâneo do tipo paniculite: relato de um caso acometendo paciente pediátrico

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Abstract

Os autores relatam um caso de linfoma T subcutâneo do tipo paniculite em uma paciente feminina de 3 anos, apresentando havia um mês múltiplos nódulos subcutâneos, indolores, disseminados no abdome, região peitoral e cervical. Ao exame histopatológico, evidenciou-se um linfoma T infiltrando tecido adiposo subcutâneo. Os linfomas T subcutâneos representam uma entidade clinicopatológica distinta, sendo raro o acometimento pediátrico.

Translated abstract

We report a case of lypotrophic lymphoma affecting a 3-year-old child with 1-month history of nontender subcutaneous nodules disseminated throughout the abdomen, chest and cervical region. Histological examination showed a T-cell lymphoma affecting the subcutaneous tissue. Subcutaneous panniculitis-like T-cell lymphoma is an unusual cutaneous T-cell lymphoma that rarely affects pediatric patients.

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Most cited references 19

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T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome.

Claudia Gonzalez, R Braziel, L. Jeffrey Medeiros … (1990)

Eight cases of T-cell lymphoma localized primarily to the subcutaneous adipose tissue are described, five of which were referred in consultation with a benign diagnosis having been made or suggested. All patients presented with 1-12-cm-diameter subcutaneous nodules, which preferentially involved the extremities in six individuals. Histologically, the lesions were reminiscent of panniculitis and were composed of a mixture of small and large atypical lymphoid cells (large cells predominated in four cases) infiltrating between adipocytes. Focally, sheets of tumor cells were found. Karyorrhexis, fat necrosis, and benign histiocytes were present in all cases. Involvement of small blood vessels was found in seven cases, but the infiltrates were not primarily angiocentric, and angiodestruction was minimal or absent. Immunophenotypic analysis (paraffin or frozen sections) in all cases showed that the atypical cells were of T-cell phenotype. Frozen-section studies demonstrated a mature T-cell phenotype with evidence of pan-T-cell antigen loss in two of five lesions. Genotypic analysis demonstrated a rearrangement of the T-cell receptor beta-chain gene in one (possibly two) biopsies of three cases studied. All patients had some evidence of hemophagocytosis during their clinical course. Six patients developed a florid hemophagocytic syndrome, fatal in five patients. Autopsies were done in all of the expired patients, and all had residual subcutaneous lymphoma and a hemophagocytic syndrome. Dissemination to nonsubcutaneous sites did not occur. Three patients are currently alive without evidence of lymphoma after aggressive chemotherapy (mean follow-up, 12 months). These results suggest that T-cell lymphomas that are primarily localized to the subcutaneous tissue may represent a distinct clinicopathologic entity. Initial biopsy findings may be misinterpreted as benign. A hemophagocytic syndrome commonly supervenes that may be secondary to lymphokine production by the malignant cells or related to the destruction of normal cells at subcutaneous sites.

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Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes.

A. Kumar, Gwendolyn Greiner, M Raffeld … (1998)

Subcutaneous panniculitic T cell lymphoma (SCPTCL) is characterized by primary involvement of the subcutaneous fat in a manner mimicking panniculitis. We studied 16 cases of this lymphoma to define its immunophenotypical profile as well as cellular origin. Involvement of the subcutaneous fat in a lacelike pattern with neoplastic cells rimming individual fat spaces was present in all cases. All 16 cases were of T cell phenotype. Thirteen of the 16 cases were CD8+, whereas three were negative for both CD4 and CD8. Twelve cases were stained for betaF1; of these, eight were betaF1+ and four were betaF1-. Focal staining for CD56 and CD30 was seen in 2 of 13 and two of eight cases, respectively. Intense diffuse positivity for the cytotoxic granular proteins T cell intracellular antigen-1 (TIA-1) and perforin was present in all cases, indicating an origin from cytotoxic T lymphocytes. Ten cases studied for Epstein-Barr viral sequences were negative. Eight of 9 cases with amplifiable DNA showed a clonal TCR gamma gene rearrangement by polymerase chain reaction. Controls included seven cases of benign panniculitis and seven other peripheral T cell lymphomas involving the skin and subcutaneous tissues: two peripheral T cell lymphomas, not otherwise specified (PTL,NOS), four anaplastic large cell lymphomas (ALCL), one T/NK cell lymphoma. The seven cases of panniculitis lacked cytological atypia and were characterized by an admixture of CD4+ and CD8+ cells with interspersed aggregates of L26+ B cells. Only infrequent cells showed staining for TIA-1 and perforin. In the control cases of T cell lymphoma, the infiltrate had a tendency for dermal and sometimes even epidermal involvement, with sheeting out of malignant cells, in contrast to the characteristic subcutaneous localization and rimming of fat spaces noted in SCPTCL. The two PTL, NOS were CD4+ and negative for both TIA-1 and perforin. Although the remaining controls expressed TIA-1 and perforin, in keeping with their cytotoxic T or natural killer (NK) cell origin, histological and other immunophenotypical features allowed distinction from SCPTCL. Five cases of SCPTCL were also stained for apoptosis using a tdt-mediated end labeling kit. All cases showed numerous positive apoptotic bodies, suggesting apoptosis as the mechanism of cell death in these tumors. Our study indicates that SCPTCL constitutes a distinctive clinicopathological entity derived from cytotoxic T lymphocytes and should be differentiated from other benign and malignant lymphoid infiltrates involving the subcutis. The apoptosis seen in these tumors may be mediated by release of cytotoxic granular proteins.

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Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes.

J. Choi, K Salhany, Lawrence S. Lessin … (1998)

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon cutaneous lymphoma that has been proposed as a distinct clinicopathologic entity, but studies of SPTCL are limited. We studied the clinicopathologic, immunophenotypic, and genetic features of 11 SPTCLs. All cases had a variable admixture of pleomorphic small, medium, or large lymphocytes and histiocytes infiltrating the subcutis in a lobular panniculitis-like pattern. A granulomatous reaction was seen in three cases and erythrophagocytosis in four. Karyorrhexis and fat necrosis were present in all cases. Angioinvasion was seen in seven SPTCLs; four had areas of coagulation necrosis. All cases expressed T-cell-associated antigens (CD3epsilon, CD45RO, or CD43) and T-cell receptors (TCR); nine expressed alphabeta TCRs and two expressed gammadelta TCRs. T-cell receptor-gamma, TCRbeta, or TCRdelta genes were clonally rearranged in 8 of 10 cases studied. Both gammadelta SPTCLs expressed Vdelta2+ TCRs and were CD4-, CD8- and CD56+. CD56 was negative in seven of nine alphabeta SPTCLs and inconclusive in the other two. Six of nine alphabeta SPTCLs were CD8+; the CD4/CD8 phenotypes were indeterminate in the other three. Cytolytic granule-associated proteins were expressed by all SPTCLs (11 of 11 were TIA-1+, 4 of 4 were perforin+). In situ hybridization for Epstein-Barr virus-encoded RNA (EBER-1) was negative in all cases. Most patients responded to systemic chemotherapy or local radiation therapy. Seven patients are alive: four without disease (19-73 months) and three with disease (32-72 months); four died: three of disease (3-25 months) and one without disease (42 months). We conclude that SPTCLs are clonal, EBV-, cytotoxic T-cell lymphomas derived from alphabeta T-cells or gammadelta T-cells. The gammadelta SPTCLs appear to be preferentially derived from the Vdelta2+ subset. Subcutaneous panniculitis-like T-cell lymphoma may be rapidly fatal or indolent; local therapy may be appropriate for some patients.

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Author and article information

Contributors

Lúcia de Noronha: Role: ND

Fabiola Medeiros: Role: ND

Luciano Monte Serrat Prevedello: Role: ND

Vanessa Dello Monaco Martins: Role: ND

Flora Watanabe: Role: ND

José Fillus Neto: Role: ND

Journal

Journal ID (publisher): jbpml

Title: Jornal Brasileiro de Patologia e Medicina Laboratorial

Abbreviated Title: J. Bras. Patol. Med. Lab.

Publisher: Sociedade Brasileira de Patologia Clínica (Rio de Janeiro )

ISSN: 1678-4774

Publication date (Print): 2001

Volume: 37

Issue: 4

Pages: 287-291

Affiliations

[1 ] Universidade Federal do Paraná Brazil

[2 ] Hospital Infantil Pequeno Príncipe

Article

Publisher ID: S1676-24442001000400011

DOI: 10.1590/S1676-24442001000400011

SO-VID: 4b64c52d-3af7-4624-a0a6-fe0c165a308d

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http://creativecommons.org/licenses/by/4.0/

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Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=1676-2444&lng=en

Linfoma T subcutâneo do tipo paniculite: relato de um caso acometendo paciente pediátrico Translated title: Subcutaneous panniculitic T cell lymphoma: a case report affecting a child

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Abstract

Translated abstract

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Most cited references 19

T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome.

Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes.

Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes.

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