RESUMO
ABSTRACT Mantle cell lymphoma of the ocular and periorbital regions is extremely rare but should be considered in the differential diagnosis of lesions affecting the periorbital tissues. In this study, we present a rare case of mantle cell lymphoma of the lacrimal sac in a 65-year-old male presenting with a mass in the lacrimal sac region and epiphora. After clinical examinations and imaging studies, the mucocele was misdiagnosed. Considering the unexpected findings during external dacryocystorhinostomy, a frozen biopsy was performed, which confirmed the diagnosis of lymphoma.
RESUMO
Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.
RESUMO
Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.
Assuntos
Humanos , Masculino , Idoso , Ruptura Esplênica/patologia , Linfoma de Célula do Manto/epidemiologia , Esplenomegalia/complicações , Linfoma de Célula do Manto/fisiopatologia , Ciclina DRESUMO
Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.
RESUMO
Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.
Assuntos
Humanos , Masculino , Idoso de 80 Anos ou mais , Neoplasias de Cabeça e Pescoço/diagnóstico , Linfoma de Célula do Manto/diagnóstico , Manifestações Bucais , Diagnóstico Diferencial , Evolução Fatal , Neoplasias de Cabeça e Pescoço/patologiaRESUMO
El linfoma de células del manto hace parte del subgrupo de linfomas no Hodgkin (LNH). Este se manifiesta con adenopatías, esplenomegalia, síntomas B y compromiso cutáneo asociado a enfermedad diseminada. El compromiso esplénico se presenta como: Esplenomegalia sin lesión focal; lesiones sólidas únicas o múltiples e infiltración del bazo sin cambios morfológicos ni lesiones focales. La aparición de linfoma con lesiones quísticas es extremadamente rara, se encuentran solo unos cuantos casos en la literatura, ninguno de ellos en el bazo. Se expone el caso de una paciente de 59 años de edad, quien consultó por dolor abdominal intermitente. En los estudios diagnósticos se observó esplenomegalia con lesiones sólidas y quísticas. Se realizó esplenectomía con estudio histopatológico que confirmó compromiso por linfoma de células del manto.
Mantle cell lymphoma is a Non-Hodgkin Lymphoma (NHL). In cases of disseminated disease, lymphadenopathy, splenomegaly, B-symptoms and skin disease are present. Lymphoma affecting the spleen has several radiologic presentations, ranging from normal appearance, to splenomegaly or multiples focal solid lesions. Cystic presentation of lymphoma is rare and few cases have been reported, none of them involving the spleen. We report a case of a 59-year old female patient with cystic spleen lesions that after splenectomy were diagnosed as Mantle cell lymphoma.
Assuntos
Humanos , Linfoma de Célula do Manto , Tomografia Computadorizada por Raios X , LinfomaRESUMO
Here we report the case of a 73-year-old man who was diagnosed with metachronous, multiple primary tumors with non-Hodgkin B-cell mantle cell lymphoma involving the orbit on the basis of biopsy and immunohistochemistry in 2012. The patient had been diagnosed with non-Hodgkin small cell lymphoma and basal cell skin carcinoma in 2010 and intestinal adenocarcinoma with metastasis to the regional lymph nodes in 2011, thus representing a typical case of metachronous, multiple primary tumors. Mantle cell lymphoma is a rare disease and its prognosis is quite poor, particularly when it is associated with other metachronous malignancies. Therefore, physicians should consider mantle cell lymphoma as a differential diagnosis for neoplasms of the orbit.
Relatamos um caso de linfoma não-Hodgkin de células B subtipo células do manto acometendo região orbital, com o diagnóstico confirmado pela biópsia e testes imuno-histoquímicos em 2012. O paciente apresentava história de linfoma não-Hodgkin de pequenas células em 2010, carcinoma basocelular de pele no mesmo ano e adenocarcinoma intestinal com metástase para linfonodos regionais em 2011; caracterizando um caso de tumor primário múltiplo metacrônico. O linfoma de células do manto é uma doença rara, ainda mais quando associado a outras neoplasias metacrônicas, apresentando prognóstico bastante reservado. Dessa forma, deve estar entre os diagnósticos diferenciais de neoplasias da órbita.
Assuntos
Idoso , Humanos , Masculino , Linfoma de Células B/patologia , Linfoma de Célula do Manto/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Orbitárias/patologia , Biópsia , Imuno-Histoquímica , Imageamento por Ressonância MagnéticaRESUMO
Relata-se o caso clínico de paciente feminino, de 80 anos de idade, com o diagnóstico inicial de pênfigo vulgar. Após um ano de evolução, identificou-se linfoma de células do manto. Trata-se de linfoma não Hodgkin agressivo com resposta quimioterápica limitada e prognóstico ruim. A obtenção do diagnóstico exigiu extensa propedêutica inicial (biópsias de lesão de pele de medula óssea e de linfonodo inguinal e ultrassonografia abdominal), que não estabeleceu diagnóstica. Evoluiu com linfocitose, tendo sido submetida à imunofenotipagem, que permitiu o diagnóstico. A quimioterapia inicial consistiu de fludarabina, entretanto, em decorrência de efeito adverso (pancitopenia), necessitou de hemotransfusão e fator estimulador de colônia de granulócitos (G-CSF).
An eighty-year-old woman initially presented with skin lesions suggestive of Pemphigus vulgaris, one year later diagnosed as mantle cell lymphoma. Even after extensive investigation (skin lesion biopsy, abdominal ultra-sound, myelogram, bone marrow biopsy and inguinal lymph node biopsy) no diagnosis could still be firmed. At that time, however, the patient presented lymphocytosis, which leads to immunophenotyping, eventually defining the above-mentioned diagnosis. Fludarabine was selected for initial therapy, but the patient didn?t tolerate it well (pancytopenia) and needed to receive blood transfusion and granulocyte colony-stimulating factor (G-CSF). It is a non-Hodgkinïs agressive lymphoma with bad response to chemotherapy and poor prognoses.