Linfoma cutâneo primário difuso de grandes células B, tipo perna ­ rápida evolução e desfecho desfavorável: relato de caso / Diffused primary cutaneous lymphoma of large B cells, leg type - quick evolution and unfavorable outcome: case report

Linfomas cutâneos primários são a segunda forma mais comum de linfomas extranodais, sendo os linfomas de células B, (CBCLs) representantes de 20 a 30% dos casos. O linfoma cutâneo difuso de grandes células B, Tipo Perna (PCDLBCL-LT), representa o tipo mais agressivo de CBCLs. Na maioria dos casos, a apresentação clínica é caracterizada por placas ou tumores solitários, ora ulcerados, em uma ou ambas as pernas, de rápido crescimento. O diagnóstico é confirmado através do estudo histopatológico e imunohistoquímico. O tratamento é realizado por meio de quimioterapia e seu prognóstico é reservado com uma sobrevida de 50% a 60% em 05 anos. O objetivo deste trabalho é relatar um caso atendido de linfoma cutâneo primário difuso de grandes células B, tipo perna em um paciente de 75 anos, do sexo masculino com apresentação clínica clássica e desfecho desfavorável, realizar uma revisão bibliográfica do período de 2010 a 2020 na base de dados PUBMED sobre o assunto, dada sua raridade e agressividade ímpar. As informações foram obtidas através de revisão do prontuário, registro fotográfico e revisão da literatura. Por tudo isso, pode-se concluir a importância de estudos multidisciplinares, envolvendo dermatologistas, hematologistas, oncologistas e patologistas para que o diagnóstico e tratamento sejam instituídos o mais precoce possível, visto a raridade e agressividade do PCDLBCL-LT. [au]

Primary cutaneous lymphomas are the second most common form of extranodal lymphomas; with B cell lymphomas (CBCLs) representing 20 to 30% of cases. Diffuse cutaneous large B cell lymphoma, leg type (PCDLBCL-LT), represents the most aggressive type of CBCLs. In most cases, the clinical presentation is characterized by solitary plaques or tumors, sometimes ulcerated, on one or both legs, of rapid growth. The diagnosis is confirmed through histopathological and immunohistochemistry studies. Treatment is carried out through chemotherapy and its prognosis is reserved with a 50% to 60% survival in 5 years. The objective of this work is to report a case of diffuse primary B-cell cutaneous lymphoma, leg type in a 75-year-old male patient with a classic clinical presentation and unfavorable outcome, to perform a literary review from 2010 to 2020 in the PUBMED database on the subject, given its rarity and unique aggressiveness. The data was obtained by reviewing the medical record, photographic record and literature review. For all this, it is possible to conclude the importance of multidisciplinary studies, involving dermatologists, hematologists, oncologists and pathologists so that the diagnosis and treatment are instituted as early as possible, given the rarity and aggressiveness of the PCDLBCL-LT. [au]

Secondary Cutaneous Follicular B-Cell Lymphoma Associated with Erythematous Swelling on the Leg: A Case Report / 대한피부과학회지

Cutaneous follicular B-cell lymphoma (CFBCL) is defined as the neoplastic proliferation of germinal center cells confined to the skin. Secondary CFBCL demonstrates a more aggressive clinical course compared to the primary form. We report the case of a 45-year-old man who presented with a 15-day history of lesions on his right leg. Clinical examination revealed multiple erythematous miliary/agminated papules on the right proximal thigh with erythematous swollen patches on the right lower leg. Biopsy of a thigh lesion revealed a massive dermal lymphocytic infiltrate with a follicular pattern. Immunohistochemical staining revealed atypical lymphocytes, which strongly expressed CD20, CD10, Bcl-2, Bcl-6 and Ki-67 proteins, but not CD3 and Cyclin D1. Additionally, further studies revealed that this cutaneous lesion had originated from a retroperitoneal lymph node. We treated the patient with systemic chemotherapy using a cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) regimen and anti-CD20 monoclonal antibodies. This case illustrates a rarely reported example of secondary cutaneous follicular B-cell lymphoma showing peculiar clinical manifestations.

Secondary Cutaneous Follicular B-Cell Lymphoma Associated with Erythematous Swelling on the Leg: A Case Report / 대한피부과학회지

Cutaneous follicular B-cell lymphoma (CFBCL) is defined as the neoplastic proliferation of germinal center cells confined to the skin. Secondary CFBCL demonstrates a more aggressive clinical course compared to the primary form. We report the case of a 45-year-old man who presented with a 15-day history of lesions on his right leg. Clinical examination revealed multiple erythematous miliary/agminated papules on the right proximal thigh with erythematous swollen patches on the right lower leg. Biopsy of a thigh lesion revealed a massive dermal lymphocytic infiltrate with a follicular pattern. Immunohistochemical staining revealed atypical lymphocytes, which strongly expressed CD20, CD10, Bcl-2, Bcl-6 and Ki-67 proteins, but not CD3 and Cyclin D1. Additionally, further studies revealed that this cutaneous lesion had originated from a retroperitoneal lymph node. We treated the patient with systemic chemotherapy using a cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) regimen and anti-CD20 monoclonal antibodies. This case illustrates a rarely reported example of secondary cutaneous follicular B-cell lymphoma showing peculiar clinical manifestations.

Linfoma cutáneo primario de células B centrofolicular: un desafío diagnóstico / Primary cutaneous follicular B-cell lymphoma: a diagnostic challenge

El linfoma cutáneo primario de células B (LCPCB) centrofolicular corresponde a una proliferación neoplásica infrecuente e indolente de células del centro germinal confinadas a la piel. Se reporta y revisa un caso de LCPCB centrofolicular. Paciente femenino de 76 años, con antecedentes de hipertensión arterial y resistencia a la insulina. Consultó por aumento de volumen frontal de dos años de evolución. Al examen físico destacaba un nódulo único en región frontal derecha. La ecografía de partes blandas fue compatible con quiste epidérmico. La histopatología demostró en dermis profunda, tejido adiposo subcutáneo y tejido muscular estriado una proliferación linfoide sólida dispuesta en patrón nodular y difuso. A la inmunohistoquímica (IHQ), los linfocitos fueron CD20 y BCL-6 positivo, con un Ki-67 de 60% y BCL-2, CD3, CD5 y CD10 negativo. El estudio de diseminación tumoral resultó negativo. El LCPCB centrofolicular concentra el 60% de todos los LCPCB. Se presenta en promedio a los 51 años. Se ha descrito asociación con Borrelia burgdorferi, VIH, virus hepatitis C y virus Epstein-Barr. Clínicamente corresponde a un nódulo eritematoso y asintomático, localizado preferentemente en cabeza, cuello y tronco. La IHQ es fundamental para diferenciarlo de otros tipos de LCPCB. Habitualmente, el tratamiento es con radioterapia o cirugía escisional. La supervivencia es de un 95% a 5 años. Se presenta este caso dado que el LCPCB centrofolicular corresponde a un tumor cutáneo infrecuente, con múltiples diagnósticos diferenciales, que requiere de un alto índice de sospecha para lograr un diagnóstico y tratamiento oportuno.

The primary cutaneous follicle center lymphoma (PCFCL) corresponds to an infrequent and indolent neoplastic proliferation of germinal center cells confined to the skin. A case of PCFCL is reported and revised. Results: A female patient, 76 years old, with arterial hypertension and insulin resistance. Sough attention for an increase in size of the frontal region over the course of two years. Upon physical examination, a single nodule was noted in the right frontal region. A soft tissue ultrasound identified results indicative of an epidermal cyst. The histopathology revealed a proliferation of solid lymphoid arrayed in a diffuse and nodular pattern in the deep dermis, subcutaneous adipose tissue, and striated muscular tissue. Immunohistochemistry (IHQ) revealed CD20 and BCL-6 positive lymphocytes, with 60% of Ki-67 and BCL-2, CD3, CD5, and CD10 negative. A study of tumor dissemination resulted negative. The PCFCL concentrates 60% of all primary cutaneous B cell lymphomas (PCBCL). It presents at an average of 51 years of age. It has been described association with Borrelia burgdorferi, HIV, hepatitis C virus and Epstein-Barr virus. Clinically it corresponds to an erythematous and asymptomatic nodule, found frequently on the head, neck and trunk. The IHQ is essential to differentiate it from other types of PCBCL. It is usually treated with radiotherapy or excisional surgery. Survival is 95% over 5 years. This case is presented because the PCFCL corresponds to an infrequent cutaneous tumor, with multiple differential diagnoses, requiring a high index of suspicion to achieve an opportune diagnostic and treatment.

A Histopathologic and Immunogenetic Analysis of Primary Cutaneous B-cell Lymphomas in Korea / 대한피부과학회지

BACKGROUND: Despite the advent of molecular biology and immunogenetics, the biologic behaviors and disease entities of primary cutaneous B-cell lymphomas(pCBCL) have been undetermined. Moreover, rarity of pCBCL cases and the conflicting datas of current issues have contributed to the dilemmas in understanding of the biology of pCBCL. Until now, a study of the overall features of pCBCL in Korea has been rarely presented. OBJECTIVE: We performed this study in order to identify the histopathologic and immunogenetic characteristics of pCBCL in Korea. METHODS: The histopathologic, immunophenotypic and molecular analysis of preserved specimens of 15 cases with pCBCL were conducted. RESULTS: 1. Of the 15 patients with pCBCL, most common types are follicle center cell lymphomas(73.3%). In REAL classification, diffuse large B-cell lymphoma is most common(66.6%). 2. In bcl-2 immunohistochemical staining, 3 cases(20%) were positive. 3. Only one of 15 cases of pCBCL denoted bcl-2 gene rearrangement by t(14;18) in minor cluster region. 4. Immunohistochemical staining demonstrated overexpression of p53 protein in 3(20%) of 15 cases. 5. 2 cases(13.3%) with point mutations(one for exon 5; the other for exon 8) in p53 DNA sequencing analysis. CONCLUSION: t(14;18) translocation may be rare in pCBCL in Korea. This finding indicates that bcl-2 expression by tumor cells in pCBCL without t(14;18) may occur by different genetic dysregulation. It seems to be that overexpression of p53 protein might not correspond with p53 mutations in pCBCL.

A Case of Cutaneous Diffuse Large B-Cell Lymphoma / 대한피부과학회지

We report a case of cutaneous B cell lymphoma in a 65-year-old male who had several bizarre shaped erythematous to brownish plaques on the both shins for 2 months. An abdominal CT scan revealed gastrohepatic ligament, retroperitoneal, mesenteric and bilateral external iliac lymphadenopathies. Histologic examinations revealed diffuse dense infiltration of the large atypical cells with vesicular nucleus and prominent nucleoli in the entire dermis. They showed a positive reaction to the LCA, CD20 and kappa light chain in the immunohistochemical study, suggesting that these neoplastic lymphoid cells are B cell lineage. We treated him with COPBLAM-V regimen. In the course of treatment, new skin lesions developed on both upper arms and herpes zoster on the left thigh. So we changed the regimen into IMVP-16. No relapses have been found up to the present date for 9 months.

A Case of Cutaneous B Cell Lymphoma / 대한피부과학회지

We report a case of cutaneous B cell lymphoma in a 68-year-old male who had primarily developed two 2.0 * 2.0 * 0,6cm sized, dome shaped, dark red colored tumors on the medial side of his left thigh about 4 months previously. His general condition was good and laboratory results were within normal limits, except for the enlargement of aortocaval, paraaortic and left inguinal lymph nodes. Histopathological examinations revealed diffuse dense infiltration of large atypical cells with vesicular nuclei and large prominent nucleoli in the entire dermis. They were largely composed of centroblast and immunoblast-like cells and showed positive reactions to the leukocyte common antigen, and L26 and CD22 was related to peripheral B-cell lineage in the immunohistochemical study. The skin lesions improved completely with an m-BACOD regimen. There was no relapse over a 3-year follow-up period.

Primary Cutaneous B Cell Lymphoma

A 7-year-old girl had a 7 × 6cm sized dark red colored tumor on the left elbow and several erythematous macules on the face, right upper arm, and both thighs. Her general health was good and all laboratory findings were within normal limit. Histopathologic examination revealed an infiltration of atypical mononuclear cells in the dermis and subcutis. L26 and leukocyte common antigen were positively stained in an immunohistochemical study. According to clinical, histological and immunohistochemical findings, we diagnosed the disease as primary cutaneous B cell lymphoma. The patient's skin lesions markedly improved after a surgical excision and chemotherapy.

Primary Cutaneous B Cell Lymphoma

We report a case of B-cell lymphoma primarily involving the skin in a 12-year-old boy. The histopathologic findings were compatible with those of small lymphocytic type of non-Hodgkin's lymphoma. A cutaneous lesion was the sole manifestation of his disease without any other organ involvement. Immunophenotypic studies and immunoglobulin gene rearrangement with Southern blot analysis determined its lineages and monoclonality with result of B-cell lineage neoplasm, i. d. CD20⁺, C1323⁺, CD35⁻ and rearranged band on JH probe. We treated him with surgical excision and CVP regimen of chemotherapy (cyclophosphamide, vincristine, prednisolone). There is no recurrence or metastasis during the last six months.