Assuntos
Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Neoplasias Orbitárias/patologia , Sarcoma Alveolar de Partes Moles/patologia , Adulto , Biomarcadores Tumorais/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Exenteração Orbitária , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/metabolismo , Neoplasias Orbitárias/cirurgia , Sarcoma Alveolar de Partes Moles/diagnóstico por imagem , Sarcoma Alveolar de Partes Moles/metabolismo , Sarcoma Alveolar de Partes Moles/cirurgiaRESUMO
In this retrospective study of patients with overt orbital retinoblastoma, we evaluated minimally disseminated disease (MDD) in bone marrow and cerebrospinal fluid (CSF) using CRX and/or GD2 synthase as markers. Ten patients were evaluated-five (50%) at diagnosis and five upon relapse. MDD was detected in four cases (one in the bone marrow, two in the CSF, and in one case in both sites). All patients received chemotherapy and four received orbital radiotherapy. Seven patients relapsed or progressed and all of them died. Three patients remain in complete remission. There was no apparent correlation between MDD and the outcome.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medula Óssea/metabolismo , Proteínas de Homeodomínio/metabolismo , N-Acetilgalactosaminiltransferases/metabolismo , Neoplasia Residual/mortalidade , Neoplasias Orbitárias/mortalidade , Retinoblastoma/mortalidade , Transativadores/metabolismo , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/líquido cefalorraquidiano , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Medula Óssea/patologia , Feminino , Seguimentos , Proteínas de Homeodomínio/líquido cefalorraquidiano , Proteínas de Homeodomínio/genética , Humanos , Masculino , N-Acetilgalactosaminiltransferases/líquido cefalorraquidiano , N-Acetilgalactosaminiltransferases/genética , Recidiva Local de Neoplasia/metabolismo , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Neoplasia Residual/metabolismo , Neoplasia Residual/patologia , Neoplasia Residual/terapia , Neoplasias Orbitárias/metabolismo , Neoplasias Orbitárias/patologia , Neoplasias Orbitárias/terapia , Prognóstico , Estudos Prospectivos , Dosagem Radioterapêutica , Neoplasias da Retina/metabolismo , Neoplasias da Retina/mortalidade , Neoplasias da Retina/patologia , Neoplasias da Retina/terapia , Retinoblastoma/metabolismo , Retinoblastoma/patologia , Retinoblastoma/terapia , Estudos Retrospectivos , Taxa de Sobrevida , Transativadores/líquido cefalorraquidiano , Transativadores/genética , Adulto JovemRESUMO
BACKGROUND: To describe a case series of nasal natural killer (NK)/T-cell lymphoma (NKTL) with orbital and central nervous system extension. METHODS: This is a retrospective study. The medical records of 9 patients with a diagnosis of NKTL were reviewed. All patients had a positive biopsy for CD3, CD56, T-cell-restricted intracellular antigen-1, and granzyme expression and CT imaging of nose, sinuses, orbits, and brain. Five patients were also examined with MRI. RESULTS: Orbital extension occurred in 6 patients. All had extraocular muscle enlargement and 5 showed signs of perineural spread. CONCLUSIONS: NKTL is a polymorphous disease. Extraocular muscle involvement and perineural spread are overlooked features that explain dissemination of the tumor to both the orbit and the central nervous system.
Assuntos
Neoplasias do Sistema Nervoso Central/patologia , Células Matadoras Naturais/patologia , Linfoma de Células T/patologia , Neoplasias Orbitárias/patologia , Adolescente , Adulto , Biomarcadores Tumorais/metabolismo , Complexo CD3/metabolismo , Antígeno CD56/metabolismo , Neoplasias do Sistema Nervoso Central/diagnóstico por imagem , Neoplasias do Sistema Nervoso Central/metabolismo , Criança , Feminino , Granzimas/metabolismo , Humanos , Células Matadoras Naturais/metabolismo , Linfoma de Células T/diagnóstico por imagem , Linfoma de Células T/metabolismo , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/metabolismo , Proteínas de Ligação a RNA/metabolismo , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
BACKGROUND: Mesenchymal chondrosarcoma (MC) is a subtype of chondrosarcoma, with an incidence varying from 1 to 8% of all chondrosarcomas. It is an aggressive neoplasm with a high tendency for late recurrence and occasional delayed distant metastasis. Orbital MC is very rare, and only approximately 30 cases have been described in the literature. We describe here one case of primary orbital MC. CASE REPORT: A 14-year-old boy without a past medical history presented with a 1-month history of progressive proptosis on the right eye. Computed tomography (CT) scans of the orbit revealed a right intraconic lesion, with areas of calcification. The lesion was excised. Histopathological analysis revealed that the tumor had a biphasic pattern, showing a combination of small cell malignancy and well-differentiated cartilage. Immunohistochemistry examination revealed a diffuse membrane expression of CD99 on the small cell malignancy; S-100 was positive only within the cartilage component. The patient received chemotherapy, and no metastatic disease was found at the 2-month follow-up. CONCLUSION: Although rare, MC should be considered in the differential diagnosis of a well-circumscribed orbital lesion in young adults, especially when CT scans reveal areas of calcification within the tumor.
Assuntos
Condrossarcoma Mesenquimal/diagnóstico , Neoplasias Orbitárias/diagnóstico , Antígeno 12E7 , Adolescente , Antígenos CD/análise , Antígenos CD/biossíntese , Calcinose/diagnóstico , Cartilagem/metabolismo , Cartilagem/patologia , Moléculas de Adesão Celular/análise , Moléculas de Adesão Celular/biossíntese , Condrossarcoma Mesenquimal/metabolismo , Condrossarcoma Mesenquimal/patologia , Condrossarcoma Mesenquimal/terapia , Diagnóstico Diferencial , Tratamento Farmacológico , Humanos , Imuno-Histoquímica , Masculino , Células-Tronco Mesenquimais/metabolismo , Células-Tronco Mesenquimais/patologia , Neoplasias Orbitárias/metabolismo , Neoplasias Orbitárias/patologia , Neoplasias Orbitárias/terapia , Proteínas S100/análise , Proteínas S100/biossíntese , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND: Orbital idiopathic inflammation, lymphoid hyperplasia, and lymphoma may all present clinically in the same manner. Histopathology and especially immunohistochemistry play a major role in the differential diagnosis. The purpose of this study was to determine the immunophenotypic features of these lesions. METHODS: Fifty-five orbital lymphoid lesions were retrieved from the ophthalmic pathology registries at McGill University, Montreal, Canada, and the Federal University of São Paulo, São Paulo, Brazil. Formalin-fixed, paraffin-embedded, histopathologic sections were stained with hematoxylin and eosin and periodic acid-Schiff. The sections were also immunostained for B-cell (CD20) and T-cell (CD43) markers and for immunoglobulin light chains kappa and lambda. Two pathologists determined the histopathologic and immunohistochemical pattern of each lesion in a masked fashion. RESULTS: Of the 55 lesions, 11 (20%) were idiopathic chronic inflammations, 22 (40%) were lymphoid hyperplasias and 22 (40%) were lymphomas. Idiopathic inflammation displayed a predominance of T cells and all lesions expressed polyclonal light chains. Lymphoid hyperplasia displayed a mixture of B cells and T cells, with a slight predominance of the former and all lesions expressed polyclonal light chains. Lymphoma showed a striking predominance of B cells and all lesions expressed monoclonal light chains, usually kappa (63.7%). The differences in the mean percentages of B cells among the orbital lymphoid lesions (inflammation, 35%; hyperplasia, 65.9%; lymphoma, 87.3%) were statistically significant (p < 0.001). INTERPRETATION: Orbital lymphoid lesions can be differentiated based on the percentages of B cells and T cells and the monoclonal or polyclonal expression of immunoglobulin light chains.
Assuntos
Linfoma/patologia , Neoplasias Orbitárias/patologia , Pseudotumor Orbitário/patologia , Pseudolinfoma/patologia , Adolescente , Adulto , Idoso , Antígenos CD20/metabolismo , Antígenos CD34/metabolismo , Linfócitos B/patologia , Biomarcadores Tumorais/metabolismo , Feminino , Humanos , Cadeias kappa de Imunoglobulina/metabolismo , Cadeias lambda de Imunoglobulina/metabolismo , Imuno-Histoquímica , Imunofenotipagem , Linfoma/metabolismo , Masculino , Pessoa de Meia-Idade , Neoplasias Orbitárias/metabolismo , Pseudotumor Orbitário/metabolismo , Pseudolinfoma/metabolismo , Linfócitos T/patologiaRESUMO
Infantile myofibromatosis is a rare disorder of infancy that can provoke osteolytic lesions. A 15-day-old infant presented with three round, firm lesions located on the forehead, shoulder, and back. Excisional biopsy of the forehead lesion revealed that the tumor was composed of spindle cells resembling normal smooth muscle arranged in short fascicles. Immunohistochemical staining was positive for vimentin and actin. Five months later, the child presented with three new lesions, including one in the superolateral aspect of the left orbit. It is important to recognize the multicentric form of infantile myofibromatosis because, despite its aggressive clinical presentation, the disease is benign and usually does not require extensive surgery or chemotherapy.
Assuntos
Miofibromatose/diagnóstico por imagem , Miofibromatose/patologia , Órbita/diagnóstico por imagem , Órbita/patologia , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/patologia , Actinas/metabolismo , Biópsia , Humanos , Imuno-Histoquímica/métodos , Recém-Nascido , Miofibromatose/metabolismo , Miofibromatose/cirurgia , Órbita/metabolismo , Órbita/cirurgia , Neoplasias Orbitárias/metabolismo , Neoplasias Orbitárias/cirurgia , Coloração e Rotulagem , Tomografia Computadorizada por Raios X , Vimentina/metabolismoRESUMO
PURPOSE: Leiomyoma is a benign tumor derived from smooth muscle, most frequently occurring in the uterus and gastrointestinal system. This report discusses clinical and pathologic findings in a 56-year-old man with orbital leiomyoma. METHODS: Case review. RESULTS: A lateral orbitotomy was performed. Immunohistochemical staining for actin and desmin was positive result. There was no evidence of recurrence during a two-year follow-up interval. CONCLUSIONS: Leiomyoma is a benign tumor of low incidence because there is little smooth muscle in the orbit. The differential diagnosis must include any fully encapsulated orbital tumor. Prognosis after surgical excision is favorable.
Assuntos
Leiomioma/diagnóstico , Neoplasias Orbitárias/diagnóstico , Actinas/metabolismo , Biomarcadores Tumorais , Desmina/metabolismo , Diagnóstico Diferencial , Humanos , Leiomioma/metabolismo , Leiomioma/cirurgia , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Oftalmológicos , Neoplasias Orbitárias/metabolismo , Neoplasias Orbitárias/cirurgia , Tomografia Computadorizada por Raios XRESUMO
A 10-year-old girl developed a lump in the lateral aspect of the right eyebrow over a 3-week period. Computed tomography and magnetic resonance imaging revealed a soft-tissue mass in the superolateral aspect of the right orbit associated with zygo-maticofrontal bone erosion and hyperostosis. An incisional biopsy specimen was studied using light microscopy, immunohistochemical staining, and electron microscopy, resulting in a diagnosis of peripheral primitive neuroectodermal tumor. The results of an extensive evaluation for systemic involvement were negative. The patient was subsequently treated with chemotherapy and radiation therapy. Primary primitive neuroectodermal tumor of the orbit is rare. The differential diagnosis and the diagnostic features of this entity are discussed herein.