Histiocitosis de células de Langerhans en su presentación focalizada en órbita. Revisión del tema a propósito de un caso / Langerhans cell histiocytosis in their orbitfocused presentation. Review of the topic regarding a case

Resumen Objetivo: Describir un caso de histocitosis de células de Langerhans en un paciente pediátrico. Método: Paciente de 4 años con proptosis, diplopía, fiebre y pérdida de agudeza visual, con imagen en tomografía computarizada evidente de masa orbitaria con erosión ósea. Se realizó biopsia excisional por craneotomía coronal, el resultado histopatológico fue histiocitosis de células de Langerhans. Resultados: El estudio anatomopatológico transoperatorio mostró una neoplasia de células pequeñas redondas y azules, compatible con rabdomiosarcoma. Las pruebas de inmunohistoquímica concluyeron el diagnóstico de histiocitosis de células de Langerhans. Conclusión: La histiocitosis de células de Langerhans es una enfermedad poco frecuente que requiere de una sospecha clínica y diagnóstico oportuno, adecuar el tratamiento y mejorar la sobrevida de los pacientes.

Abstract Objective: To describe a case of Langerhans cell histiocytosis in a pediatric patient. Method: A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis. Results: The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.

Schwannoma of the Orbit

BACKGROUND: A schwannoma is a benign, slow-growing peripheral nerve sheath tumor that originates from Schwann cells. Orbital schwannomas are rare, accounting for only 1% of all orbital neoplasms. In this study, we retrospectively review orbital schwannomas and characterize clinical, radiologic, and histologic features of this rare entity. METHODS: A retrospective review was performed to identify patients with histologically confirmed orbital schwannoma, among a list of 437 patients who had visited our hospital with soft tissue masses within the orbit as the primary presentation between 2010 and 2014. Patient charts and medical records were reviewed for demographic information, relevant medical and family history, physical examination findings relating to ocular and extraocular sensorimotor function, operative details, postoperative complications, pathologic report, and recurrence. RESULTS: Five patients (5/437, 1.1%) were identified as having histologically confirmed orbital schwannoma and underwent complete excision. Both computed tomography (CT) and magnetic resonance imaging (MRI) studies were not consistent in predicting histologic diagnosis. There were no complications, and none of the patients experienced significant scar formation. In two cases, patients exhibited a mild postoperative numbness of the forehead, but the patients demonstrated full recovery of sensation within 3 months after the operation. None of the five patients have experienced recurrence. CONCLUSION: Orbital schwannomas are relatively rare tumors. Preoperative diagnosis is difficult because of its variable presentation and location. Appropriate early assessment of orbital tumors by CT or MRI and prompt management is warranted to prevent the development of severe complications. Therefore, orbital schwannomas should be considered in the differential diagnosis of slow-growing orbital masses.

Transitional carcinoma with extensive invasion of the bony orbit in a dog / Carcinoma transicional com invasão dos ossos da órbita em um cão

A 12-year-old male English Pointer was examined due to a soft-tissue swelling at the medial canthus of the right orbital region, which was causing facial deformity. The dog had epiphora, purulent nasal discharge, epistaxis, dyspnea, and progressive weight loss. An intraoral mass was observed near the right maxillary premolars. Neoplastic disease was diagnosed based on ancillary tests, which included blood work, skull and intraoral radiographs, ocular ultrasonography and computed tomography. Histopathology revealed transitional carcinoma involving the nasal and oral cavities, maxilla, bony orbit and retrobulbar space. Nasal tumors represent approximately 2% of all tumors diagnosed in this species. Transitional carcinoma is the second most common type of malignant epithelial tumor in the nasal sinuses. This case illustrates the extensive destruction of the soft and bony tissues of the face, including the bony orbit that this type of tumor can cause.

Um cão da raça Pointer Inglês, de 12 anos de idade, foi encaminhado para avaliação clínica por apresentar aumento de volume no canto temporal (medial) da órbita direita, o qual estava causando deformidade facial. O cão apresentava epífora, secreção nasal mucopurulenta, epistaxe, dispnésia e perda de peso progressiva. Uma massa foi observada na cavidade oral, próxima aos dentes pré-molares, do lado direito da face. Suspeitou-se de proliferação neoplásica baseando-se nos testes auxiliares, entre os quais: radiografia craniana e oral, ultrassonografia ocular e tomografia computadorizada. A análise histopatológica da massa revelou tratar-se de carcinoma transicional envolvendo as cavidades oral e nasal, os ossos da maxila e da órbita, além do espaço retrobulbar. Os tumores da cavidade nasal representam aproximadamente 2% de todos os tumores diagnosticados nessa espécie. O carcinoma transicional é o segundo tipo de neoplasia epitelial maligna mais comum nos seios nasais de cães. Este trabalho descreve a destruição extensiva dos tecidos moles e dos ossos da face e chama atenção para o fato de que esse tipo de neoplasia deve ser considerado nas doenças da órbita.

Ten-year follow-up of a case series of primary epithelial neoplasms of the lacrimal gland: clinical features, surgical treatment and histopathological findings / Dez anos de acompanhamento de uma série de casos de neoplasias epiteliais primárias da glândula lacrimal: características clínicas, tratamento cirúrgico e achados histopatológicos

PURPOSE: To describe and analyze the features of a cases series of patients with primary epithelial neoplasms of the lacrimal gland, its surgical treatment, and histopathological findings. METHODS: Retrospective evaluation of files from patients with primary epithelial neoplasms of the lacrimal gland in the period from 1997 to 2007. All patients with primary epithelial tumors of the lacrimal gland were included in this study. Data on gender, age, clinical features, surgical treatment, histopathological findings and follow-up were collected. The slides with histological sections of the tumors were reviewed by the same pathologist. RESULTS: During the study period, there were 12 patients, 5 (41.7 percent) with benign tumors, all pleomorphic adenomas (benign mixed tumor) and 7 (58.3 percent) with malignant neoplasms, thus distributed: four cases of adenoid cystic carcinoma, two of mucoepidermoid carcinoma and one carcinoma expleomorphic adenoma. Globally, patients mean age was 54.1 years-old (ranging from 14 to 70 years-old), with mean age of 52.4 years-old (ranging from 14 to 65 years-old) for benign neoplasms, and 55.3years-old for malignant neoplasms (ranging from 26 to 70 years-old). Clinical follow-up information, ranging from 2 to 10 years-old, was available for all patients. Three patients developed distant metastasis and died of disease. CONCLUSIONS: The most frequent primary epithelial neoplasms of the lacrimal gland were pleomorphic adenoma and adenoid cystic carcinoma during the study period. Malignant tumors were more frequent than benign tumors. The histopathological diagnosis and the disease initial stage can play a significant role in patient's survival.

OBJETIVO: Descrever e analisar as características de uma série de casos de portadores de neoplasias epiteliais primárias da glândula lacrimal, o tratamento cirúrgico, assim como os achados histopatológicos. MÉTODOS: Avaliação retrospectiva dos arquivos de pacientes com neoplasias epiteliais primárias da glândula lacrimal, no período de 1997 até 2007. Todos os pacientes com tumores epiteliais primários da glândula lacrimal foram incluídos neste estudo. Foram analisados os dados sobre sexo, idade, características clínicas, tratamento cirúrgico, achados histopatológicos e seguimento dos pacientes. As lâminas com secções histológicas dos tumores foram revisadas pelo mesmo patologista. RESULTADOS: No período do estudo, foram encontrados 12 pacientes, sendo 5 (41,7 por cento) portadores de tumores benignos, todos adenomas pleomórficos (tumor benigno misto), e 7 (58,3 por cento) com neoplasias malignas, assim distribuídos: quatro casos de carcinoma adenóide cístico, dois de carcinoma mucoepidermóide e um de carcinoma ex-adenoma pleomórfico. Analisando-se de modo global, a idade média dos portadores foi de 54,1 anos (variando de 14 a 70 anos); com média de idade de 52,4 anos (variando de 14 a 65 anos) para neoplasias benignas, e 55,3 para neoplasias malignas (variando de 26 a 70 anos). Informações do seguimento, variando de 2 a 10 anos, estavam disponíveis para todos os pacientes. Três pacientes desenvolveram metástases distantes e morreram devido à doença. CONCLUSÕES: A maioria das neoplasias epiteliais primárias da glândula lacrimal foi o adenoma pleomórfico e o carcinoma adenóide cístico no período de estudo. Os tumores malignos foram mais frequentes que os benignos. O diagnóstico histopatológico e o estadiamento inicial da doença podem desempenhar uma papel significante na sobrevida do paciente.