RESUMO
One of the pathogenic causes of cutaneous inflammatory pseudotumors is chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV), a vasculitic reaction pattern seen in granuloma faciale (GF), a localized vasculitis, and erythema elevatum diutinum (EED), a generalized vasculitis. Patients with myelodysplastic syndromes (MDSs) are at risk for a diverse spectrum of cutaneous neutrophilic dermatoses such as EED. Herein, we report a 74-year-old man who presented with a large ulcerative, fungating tumor affecting the right flexor ankle caused by CLFLCV. During his workup and management, MDS and Philadelphia chromosome-negative chronic myeloid leukemia was diagnosed. Surgical excision of the inflammatory mass promptly triggered tumor recurrence, whereas antineutrophil therapy (dapsone coupled with hydroxyurea, and prednisone) lead to tumor regression. Histopathologic examination revealed an eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis (GF-like). In the regions of fibrosis, dilated lymphatic and vascular spaces were numerous, some of which were congested with small CD3-positive lymphocytes (intralymphatic and intravascular lymphocytosis). These findings indicate coexisting localized lymphedema. By direct immunofluorescence, IgM and C4d vessel deposits were detected. The pathogenesis of the fibrotic nodules and plaques of CLFLCV is suspected to be due to recurring bouts of immune-complex vasculitis, creating a cycle of vessel damage followed by reparative granulation tissue. Poor lymphatic drainage may be the underlying factor initiating and maintaining recurrent, localized episodes of immune-complex vasculitis and progressive fibrosis. Although his tumor histopathology resembled GF-eosinophilic rich CLFLCV-the clinical context points to a solitary and paraneoplastic case of EED.
Assuntos
Granuloma de Células Plasmáticas/patologia , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/patologia , Linfedema/patologia , Linfocitose/patologia , Síndromes Paraneoplásicas/patologia , Vasculite Leucocitoclástica Cutânea/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Dapsona/uso terapêutico , Evolução Fatal , Granuloma de Células Plasmáticas/imunologia , Granuloma de Células Plasmáticas/terapia , Humanos , Hidroxiureia/uso terapêutico , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/tratamento farmacológico , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/genética , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa/imunologia , Linfedema/imunologia , Linfedema/terapia , Linfocitose/imunologia , Linfocitose/terapia , Masculino , Síndromes Paraneoplásicas/imunologia , Síndromes Paraneoplásicas/terapia , Pentoxifilina/uso terapêutico , Prednisona/uso terapêutico , Indução de Remissão , Resultado do Tratamento , Vasculite Leucocitoclástica Cutânea/imunologia , Vasculite Leucocitoclástica Cutânea/terapiaRESUMO
El tumor miofibroblástico inflamatorio es una lesión infrecuente, cuya importancia radica en ser diagnóstico diferencial de otras neoplasias. Se le ha relacionado con Infecciones, traumas, cirugías previas e ínmunosupresión, pero se cree que éstos sólo serían el evento ínidal que daría lugar a una serie de cascadas autoinmunes que perpetuarían al cuadro. Afecta a población infantil y adulta joven, con una edad media de aparición de 10 años, sin distinción de género. A continuación se presenta el caso de una mujer de 45 años de edad con diagnóstico de tumor miofibroblástico inflamatorio de arteria carótida interna, resecado con embolización arterial selectiva previa. Además, se revisa la literatura al respecto.
The inflammatory myofibroblastic tumor is a rare condition, and its' relevant for being a differential diagnosis of many other neoplasms. It has been related with different infections, traumas, previous surgeries and immune deficiencies, but there are studies that support the theory that these factors are only the initial event of an autoimmune reaction that perpetuates the disease. It affects childs and young adults, with an average of 10 years old at the moment of presentation, without differences between genre. In this case, we reporta 45 year old woman, with an histological diagnosis of inflammatory myofibroblastic tumor related to the internal carotid artery, extirpated after an arterial selective embolization. Besides, we review the literature.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doenças das Artérias Carótidas/cirurgia , Granuloma de Células Plasmáticas/cirurgia , Embolização Terapêutica , Doenças das Artérias Carótidas/terapia , Granuloma de Células Plasmáticas/terapia , Resultado do TratamentoRESUMO
El granuloma de células plasmáticas se encuentra comprendido dentro del grupo de los pseudotumores inflamatorios. Es una rara lesión tumoral benigna descrita en diversos órganos, pero su localización en cavidad oral y labio es muy rara. Su etiología es desconocida y por su aspecto tumoral se hace necesario establecer diagnósticos diferenciales, en especial para no confundirlos con neoplasias malignas. Su tratamiento es quirúrgico y eventualmente puede haber recidivas. Se presenta el caso de una paciente mujer de 60 años, con una lesión tumoral en labio inferior de 3 meses de evolución. El propósito de este artículo es mostrar una localización poco habitual de esta afección relacionándola a un traumatismo local previo.
The plasma cell granuloma is included in the group of inflammatory pseudotumors. It is a rare benign tumor, described in various organs, but its location on the lip and oral cavity is very rare. Its aetiology is unknown. His appearance tumor like makes necessary to establish differential diagnosis, especially with malignancies. Their treatment is surgical and eventually may have relapses. A female patient 60 year old is presented with an injury tumor on the lower lip of 3 months of evolution. The purpose of this article is to increase the casuistry of plasma cell granuloma in this location and to correlate the presence of this lesion to a local trauma.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Granuloma de Células Plasmáticas , Granuloma de Células Plasmáticas/terapiaRESUMO
O tumor trofoblastico do sitio placentario (TTSP) e neoplasia derivada de celulas trofoblasticas intermediarias presentes nos vilos coriais e sitios extravilosos durante a gravidez. Entre 1976 a 1980, o TTSP era conhecido como pseudotumor trofoblastico. Apos reconhecimento do seu ...