Granuloma eosinófilo craneal solitario: caso clínico / Solitary eosinophilic granuloma of the skull: a clinic case

La histiocitosis de las células de Langerhan (HCL), es una enfermedad Granulomatosa de etiología no bien precisada todavía, la cual puede tener diferentes manifestaciones y localizaciones,1 aunque algunos la consideran una proliferación neoplásica de las células de Langerhan que aparecen en el escenario de sitios nodulares o no.2 La Histiocytosis X incluye tres componentes: El Granuloma Eosinófilo, la enfermedad de Hand-Schüller-Christian y el síndrome de Letterer-Siwe.5 El Granuloma Eosinófilo es un tumor óseo benigno muy raro, que se presenta en más del 90 por ciento de los casos en niños menores de 10 años, 6 teniendo además cierta predilección por el sexo masculino. Esta lesión es usualmente encontrada en los huesos largos y planos. Se trata de una paciente femenina de 50 años de edad con antecedentes de procesos alérgicos y asma bronquial grado II y fumadora inveterada, que hacía 8 meses, previo a su ingreso, había comenzado a presentar cefalea holocraneal, fue notando irregularidades en su cuero cabelludo y fue hospitalizada porque al realizarle una radiografía simple de cráneo se evidenció una imagen radiotransparente de bordes mal definidos, lo que fue constatado por tomografía simple de cráneo, la paciente fue operada, realizándole una craniectomía y excéresis de un tejido Granulomatoso sobre la duramadre con colocación de injerto de cemento quirúrgico en el área de la craniectomía. Se le diagnosticó un Granuloma Eosinófilo. Posterior a su recuperación fue tratada con oncología donde se le aplicó radioterapia. La evolución de la paciente fue satisfactoria, ahora se encuentra asintomática.

The Langerhans cells hystiocitosis is a Granulomatous ill, it has unknown etiology today. This disease can be many places and clinical manifestations, although some authors consider it a neoplasic proliferation of the Langerhans cells which can be on nodular region scenery or not. X Hystiocitosis include three diseases: Eosinophilic Granuloma, Hand-Schuller- Chritian disease and Letterer-Siwe syndrome. Eosinophilic Granuloma is a very uncommon benign bone tumor, which may be present in more than 90 percent in childhood under 10 years old. They have predominance on male patients. Usually it is found on long and flat bones. The case report is about 50 years old, female patient, with allergic history and bronchial asthma II degree, she come to the hospital because she don’t filled well since 8 months ago and she complained mild headache, furthermore she noticed irregularity over her skull, on vertex region, at her admission she was screening with cranial X ray, where was found a radio lucid images over the painful area, moreover it had irregular edge whit more than 10 cm long. After that she was screening on CT-scan, where was found a very large osteolytic lesion without intracranial grow. She was approached with craniectomy and graft of surgical cement. She was diagnosed Eosinophilic Granuloma on pathologic department. After her discharge she was treated by oncology, here she received holocraneal radiotherapy. Her outcome has been satisfactory because she is asymptomatic and her CT-scan showed not evidences of recidivism.

Granuloma de células de Langerhans intraorbitário extraocular: abordagem cirúrgica primária. Por quê? - Relato de caso / Intraorbital extraocular Langerhans' cell granuloma: primary surgical approach. Why? - Case report

Apresentamos um caso no qual foi realizada orbitotomia de urgência em paciente com granuloma de células de Langerhans intraorbitário o qual apresentava comprometimento anatômico e funcional da função visual devido à hipertensão intraorbitária. Paciente masculino, 2 anos, apresentou exoftalmia, proptose conjuntival, olho vermelho, oftalmoplegia completa com midríase não fotorreativa e ptose palpebral direita. À fundoscopia apresentava engurgitamento venoso com edema de papila à direita. Foi realizada abordagem cirúrgica com orbitotomia via intracraniana sendo removida a lesão com remissão total da clínica tanto anatômica quanto funcionalmente. O manejo do granuloma de células de Langerhans pode variar desde a abordagem cirúrgica primária, passando pela terapia com esteróides até quimioterapia e radioterapia. No caso, a cirurgia foi a primeira opção devido ao e iminente risco de perda da função visual.

We present a case in which a patient with intraorbital Langerhans' cell granuloma, with anatomical and functional skills compromised by intraorbital hypertension, which was treated with urgency orbitotomy by cranial approach. Male, two years-old, presented conjuntival proptosis, red eye, complete ophthalmoplegy, exophthalmos, with non-fotoreactive mydriasis and blepharoptosis of the right eye. Fundoscopy presented venous enlargement with optic disc edema. A cranial approach with orbitotomy was carried out, removed the lesion with total remission of clinical presentation and anatomic appearance as well. The handling of Langerhans' cell granuloma can vary from primary surgical approach as corticotherapy to chemotherapy and radiotherapy. In this case, the surgery was the first option due to the imminent risk of loss of visual function.

Solitary eosinophilic granuloma of parietal bone in an adult patient

Eosinophilic granuloma [EG] is a well-recognized benign form of Langerhans-cell histiocytosis. The estimated incidence of EG is 3-4 per million of the population. More patients are children and adolescents between the ages of 1-15 years. In this report, we present an adult patient. A 37-year-old male was admitted with headache and right parietal swelling that was present for 2 months together with epileptic attacks. Cranial CT scan revealed a right parietal osteolytic lesion with large epidural and subcutaneous mass, causing brain compression. Total removal of the mass was carried out with cranioplasty. Histopathological examination disclosed Langerhans" cell histiocytosis and immunohistochemical detection of S-100 antigen. At follow-up, he had no neurological deficits, and control CT was normal. For symptomatic solitary calvarial EG, surgical excision is the main treatment option. Surgical treatment is simple, quick, and allows histological diagnosis of the osteolytic lesions

Granuloma eosinofílico: presentación de un caso clínico / Eosinophilic granuloma: presentation of a case

El granuloma eosinofílico constituye una forma leve de histiocitosis de X, se caracteriza por lesión inflamatoria ósea única o múltiple, es poco frecuente de etiología desconocida. La edad de presentación ocurre entre los 5 y 20 años con predominio en varones. Se describe el caso de masculino de 21 años ingresado al Servicio de Neurocirugía del Hospital Dr. Patrocinio Peñuela Ruíz, San Cristóbal, Estado Táchira en julio del 2003. Los hallazgos clínicos positivos son: cefalea y dolor a la palpación a nivel de región occipito-parietal izquierda. Los estudios de diagnóstico por imagen sugieren lesión de naturaleza lítica con reacción a partes blandas. Se plantea como diagnóstico de ingreso: osteomielitis occipital izquierda. Hallazgo intraoperatorio describe LOE de partes blandas tumoral que penetra el diploe y se adhiere a la duramadre. En vista de la forma de presentación clínica del caso, dada por lesión ósea única y sin compromiso de otros órganos y sistemas, se correlaciona con el estudio histológico, corroborado a su vez por inmunohistoquímica y se concluye: Histiocitosis de Células de Langerhans, variedad Granuloma eosinofílico.

Tumores pared torácica / Thoracic wall tumours

Los tumores de la pared torácica son entidades raras de encontrar, entre las que seincluyen lesiones de hueso y partes blandas, pueden ser primarias o secundarias (metástasis, infecciosas) incluyendo también tumores de estructuras adyacentes al tórax que comprometen la pared torácica como mama, pulmón, pleura y mediastino. Se presentan dos casos clínicos, que consultan por dolor costal, diagnosticándose en ambos un tumor costal, tratados con exéresis costal. La evolución postoperatoria fue satisfactoria y las biopsias demostraron una Displasia Osea Fibrosa y con Granuloma Eosinofilo.

Unusual location of eosinophilic granuloma

Eosinophilic granuloma EG is a well-recognized benign form of Langerhans cell histiocytosis, most commonly involving the skull bones. In this paper, we report an 8-year-old girl with EG of posterior element of vertebra; she had complete resolution with surgical curettage and bone grafting

Granuloma eosinofilo solitario de vertice de orbita y senos paranasales en un adulto: comunicacion de un caso y revision de la literatura / Adult solitary eosinophilic granuloma of the orbital vertex and paranasal sinuses: case report and review

Objective: to report a rare localization of Langerhans cell histiocytosis and to define its differential diagnosis and therapeutic options. Description: a 32 year-old male with derecasing visual acuity, headache and epistaxis. MRI: parasellar lytic lesion extending to nasal cavities. Transnasal biopsy: proliferation of S100 positive cells and eosinophilic granulocytes. Intervention: radiation therapy was followed by reission. Conclusion: Langerhans cell histiocytosis can present as a unifocal (eosinophilic granuloma) or multifocal sidorder. Usually affects children. The skull base is rarely affected. Surgery with or without radiotherapy is the treatment of choice for solitary accessible lesions. Isolated radiotherapy and intralesional steroids are valid options. Systemic disease requires chemoterapy

Patologías excepcionales en estructuras de cabeza y cuello / Exceptional pathologies in head and neck structures

De 4711 enfermos con lesiones patológicas de cabeza y cuello tratadas, 14 de ellas (0,29 por ciento) han sido, en nuestra experiencia, variedades únicas en sus respectivos órganos. Siete son presentadas en este artículo.

Cervical spine cord compression by eosinophilic granuloma: case report

Eosinophilic granuloma is a term reserved for the most often and benign form of disorder known as Langerhans cells histocytosis. It is a disease of children and adolescents that very rarely affects adults, representing the localized form of a pathological proliferation of histocytes in bones, like skull and long bones Bertebral involvement is uncommon, approximately 8 per cent of the cases, being the cervical localization the least affected. Moreover, the involvement of the spinal cord and roots remains a rare occurrence. Only five cases characterized by signs of cervical spinal cord compression have been reported. We report the sixth case in a 42-year-old-man who evolved with resolution of symptoms, and has remained asymptomatic after treatment. The clinical, radiological and histological features and, also the value, in selected cases, of surgical treatment followed by low-dose radiation therapy is discussed. A review of the pertinent literature is also presented.

Granuloma eosinófilo em coluna cervical com manifestaçäo neurológica / Eosinophilic granuloma of cervical spine with neurological manifestation

Relato de paciente de 11 meses com história de compressao medular cervical por histiocitose X. Os achados clínicos e radiológicos sao revistos, assim como o tratamento cirúrgico e radioterápico em pacientes portadores de granuloma eosinófilo é discutido.

Compressäo medular por granuloma eosinófilo de origem na lâmina e processo espinhoso vertebrais: relato de caso / Spinal cord compression due to eosinophilic granuloma originating at the lamina and spinous process of the vertebra: report of case

Os autores relatam um caso de granuloma eosinófilo da coluna dorsal (T9), em um paciente com 32 anos de idade, com história de paraparesia crural de 3 meses de evoluçao; a mielografia demonstrou obstruçao extradural em T9 e o trtamento constituiu em laminectomia descompressiva, biópsia e radioterapia complementar.

Granuloma eosinófilo da coluna cervical: relato de caso / Cervical spine eosinophilic granuloma: case report

E relatado o caso de um doente com granuloma eosinófilo da coluna cervical com sintomas de quadriplegia pré-operatória. O paciente foi submetido a cirurgia por via anterior com remoçäo do corpo vertebral comprometido e enxerto ósseo. No pós-operatório, ele teve uma excelente recuperaçäo

Granuloma Eosinófilo de la Columna Cervical

Se presenta un caso de Granuloma Eosinófilo, localizado en la columna cervical (C5) en un hombre de 15 años con sintomatologçía radicular bilateral a quien se le practicó resección total de la lesión y tratamiento complementario con dosis mínimas de radioterapia. Se realiza ubna revisión actualizada de la literatura

Granuloma eosinofílico gástrico / Gastric eosinophilic granuloma

Se presenta un caso clínico de granuloma eosinofílico gástrico tratado quirúrgicamente con excelente evolución posterior. Debido a la rareza de cuadro se revisa la bibliografía, concluyendo que se trata de una patología de causa poco clara y de difícil diagnóstico. El tratamiento debe ser quirúrgico y el pronóstico es bueno, no habiéndose descrito hasta ahora casos de evolución maligna

Granuloma eosinófilo ósseo: estudo baseado em 19 casos / Eosinophilic granuloma of bone: study of 19 cases

Os autores estudam clínica e radiograficamente 19 casos comprovados por biópsia de granuloma eosinófilo ósseo. Näo houve nenhum caso de transiçäo para a doença de Hand-Schuller-Christian ou a de Letter-Siwe. Os achados radiográficos näo säo característicos, sendo que as lesöes podem ser confundidas com neoplasias malignas. A biopsia é imperativa para o diagnóstico definitivo. Todas as lesöes analisadas regrediram completamente, independente do tratamento instituído. Nas lesöes de fácil acesso cirúrgico, a curetagem simples pôde ser utilizada, com bons resultados. Nas lesöes de difícil abordagem cirúrgica, a administraçäo de corticoterapia oral teve igualmente bom resultado