Histiocitoses: uma revisão focada em achados de
                        neuroimagem

Histiocitose é uma doença sistêmica que, frequentemente, acomete o sistema nervoso central. Este trabalho propõe discutir as características de neuroimagem da histiocitose de células de Langerhans (HCL), mais comum; e as histiocitoses não-Langerhans (HNL), como a síndrome hemofagocítica, doenças de Erdheim-Chester e Rosai-Dorfman.

Método:

Revisão de literatura, com demonstração de casos confirmados por anatomopatológico.

Resultados:

Na HCL os achados mais comuns são: 1) lesões ósseas craniofaciais e da base do crânio; 2) lesões intracranianas, extra-axiais; 3) acometimento parenquimatoso intra-axial na substância cinzenta ou branca; 4) atrofia. Dentre as HNL, o diagnóstico, em geral, exige correlação com critérios clínico-laboratoriais. O espectro de apresentação das mesmas inclui acometimento intraparenquimatoso, lesões meníngeas, envolvimento orbitário e das cavidades paranasais.

Conclusão:

É importante o reconhecimento dos padrões de imagem mais comuns, para que HCL e HNL sejam incluídas no diagnóstico diferencial, sempre que pertinente.

Histiocitose de células de Langerhans; histiocitoses não-Langerhans; Rosai-Dorfman; síndrome hemofagocítica; Erdheim-Chester

Autoria

Larissa Barcessat Gabbay

Instituto de Radiologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;Universidade de São PauloBrazilSao Paulo, SP, BrazilInstituto de Radiologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;

Cláudia da Costa Leite

Departamento de Radiologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;Universidade de São PauloBrazilSao Paulo, SP, BrazilDepartamento de Radiologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;

Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill NC, USA;University of North Carolina at Chapel HillUSAChapel Hill, NC, USADepartment of Radiology, University of North Carolina at Chapel Hill, Chapel Hill NC, USA;

Ranieli Saraiva Andriola

Paula da Cunha Pinho

Leandro Tavares Lucato

Centro de Diagnósticos Brasil (CDB), Sao Paulo SP, Brazil.Centro de Diagnósticos Brasil (CDB)BrazilSao Paulo, SP, BrazilCentro de Diagnósticos Brasil (CDB), Sao Paulo SP, Brazil.

Correspondence:
Larissa Barcessat Gabbay; Av. Dr. Enéas de Carvalho Aguiar, s/n° / Rua 1 Cerqueira César; 05403-900 São Paulo SP, Brasil; E-mail: larissagabbay@hotmail.com

Conflict of interest: There is no conflict of interest to declare.

SCIMAGO INSTITUTIONS RANKINGS

Centro de Diagnósticos Brasil (CDB), Sao Paulo SP, Brazil.Centro de Diagnósticos Brasil (CDB)BrazilSao Paulo, SP, BrazilCentro de Diagnósticos Brasil (CDB), Sao Paulo SP, Brazil.

Figuras

Figuras (10)

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Figure 1
. Langerhans cell hystiocitosis in a 19-year-old patient. MR images in the axial plane, sequences FLAIR (A) and postcontrast T1WI (B) disclose a lesion in the occipital bone, with intermediate signal intensity in FLAIR (arrow in A) and intense contrast enhancement (arrow in B).

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Figure 2
. Langerhans cell hystiocitosis in a patient with diabetes insipidus. Sagittal T1WI image demonstrates a thickened pituitary infundibulum and absence of the normal T1-hyperintense posterior pituitary lobe (A). Enhanced sagittal T1WI image demonstrates intense contrast enhancement of the thickened infundibulum (B and arrow).

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Figure 3
. Patient with systemic Langerhans cell hystiocitosis. Axial T1WI (A), T2WI (B) and postcontrast T1WI (C). Extra-axial, bilateral space-occupying lesions originating from the meninges (mainly dura mater) in the high convexity. There is marked hypointensity in T2WI and intense contrast enhancement.

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Figure 4
. Langerhans cell hystiocitosis in a 5-year-old boy with exophthalmos, polyuria, polydipsia, and skin lesions. MR shows solid expansive lesions in the choroid plexus of both ventricular atria, characterized by hypointensity on T2WI (arrows in A) and intense contrast enhancement in corresponding T1WI (B). Notice some white matter edema near the atrium of the left lateral ventricle, associated to cystic areas more evident in the postcontrast T1WI.

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Figure 5
. Langerhans cell hystiocitosis patient with ataxia, diabetes insipidus and behavioral disturbances. Axial T1WI shows hypointense lesions in the deep white matter of both cerebellar hemispheres (A and arrows). Coronal T2WI discloses the hyperintensity of these lesions (B and arrows). Postcontrast axial T1WI demonstrates that the cerebellar lesions do not enhance; however, there is nodular enhancement of a lesion in the anterior medulla (C and arrow).

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Figure 6
. The same patient as in Figure 5, images obtained at the basal ganglia level. Axial T1WI demonstrates that these lesions are hyperintense (A and arrow). They are also hyperintense in a corresponding FLAIR image (B); and some of them clearly present enhancement in a postcontrast axial T1WI at the same level (C and arrow).

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Figure 7
. A 46-year-old patient diagnosed with Rosai Dorfman disease. Axial T2WI shows a large petroclival extra-axial lesion, with marked hypointensity, extending into the right temporal fossa, prepontine and right cerebellopontine cisterns (A). There is mass effect on the corresponding temporal lobe and brainstem, with associated vasogenic edema. Axial T1WI demonstrates isointensity (B). Postcontrast axial T1WI depicts marked enhancement of the mass (C).

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Figure 8
. A 35-year-old male patient diagnosed with Erdheim-Chester disease. Axial T2WI shows bilateral hyperintensity in the cerebellar white matter, middle cerebellar peduncles and pons, as well as low signal intensity filling the sphenoid sinus (A and arrow). Axial T1WI reveals that the lesions are hypointense (B). Gadolinium-enhanced axial T1WI shows enhancement of the lesions and inside the sphenoid sinus (C). Corresponding axial CT image from a paranasal sinus study reinforces the filling of the sphenoid sinus associated to the presence of thickened sclerotic walls (D). Bone scintigraphy reveals symmetrically increased radionuclide uptake at the level of the distal femoral diaphyses and metaphyses, a quite distinctive finding of the disease (E).

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Figure 9
. Patient with 6 months of life presenting bilateral paresis of the sixth cranial nerve, hepatomegaly, increased levels of ferritin and triglycerides, hypofibrinogenemia and pancytopenia, diagnosed as hemophagocytic lymphohistiocytosis. Axial FLAIR (A), diffusion-weighted (B) and enhanced T1WI (C) show subtle hyperintense symmetrical deep cerebellar white matter lesions without restricted diffusion or gadolinium enhancement.

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Figure 10
. Two-year-old patient with hemophagocytic lymphohistiocytosis. Axial FLAIR (A) and postcontrast T1WI (B) show lesions in the deep and periventricular white matter of the cerebral hemispheres, some of them associated to contrast enhancement (arrows in B). Axial T2WI (C) and corresponding postcontrast T1WI (D) demonstrate extensive involvement of cerebellar hemispheres and vermis, extending to the right middle cerebellar peduncle, associated to mass effect and heterogeneous contrast enhancement.

Figure 1 . Langerhans cell hystiocitosis in a 19-year-old patient. MR images in the axial plane, sequences FLAIR (A) and postcontrast T1WI (B) disclose a lesion in the occipital bone, with intermediate signal intensity in FLAIR (arrow in A) and intense contrast enhancement (arrow in B).

Figure 2 . Langerhans cell hystiocitosis in a patient with diabetes insipidus. Sagittal T1WI image demonstrates a thickened pituitary infundibulum and absence of the normal T1-hyperintense posterior pituitary lobe (A). Enhanced sagittal T1WI image demonstrates intense contrast enhancement of the thickened infundibulum (B and arrow).

Figure 3 . Patient with systemic Langerhans cell hystiocitosis. Axial T1WI (A), T2WI (B) and postcontrast T1WI (C). Extra-axial, bilateral space-occupying lesions originating from the meninges (mainly dura mater) in the high convexity. There is marked hypointensity in T2WI and intense contrast enhancement.

Figure 4 . Langerhans cell hystiocitosis in a 5-year-old boy with exophthalmos, polyuria, polydipsia, and skin lesions. MR shows solid expansive lesions in the choroid plexus of both ventricular atria, characterized by hypointensity on T2WI (arrows in A) and intense contrast enhancement in corresponding T1WI (B). Notice some white matter edema near the atrium of the left lateral ventricle, associated to cystic areas more evident in the postcontrast T1WI.

Figure 5 . Langerhans cell hystiocitosis patient with ataxia, diabetes insipidus and behavioral disturbances. Axial T1WI shows hypointense lesions in the deep white matter of both cerebellar hemispheres (A and arrows). Coronal T2WI discloses the hyperintensity of these lesions (B and arrows). Postcontrast axial T1WI demonstrates that the cerebellar lesions do not enhance; however, there is nodular enhancement of a lesion in the anterior medulla (C and arrow).

Figure 6 . The same patient as in Figure 5, images obtained at the basal ganglia level. Axial T1WI demonstrates that these lesions are hyperintense (A and arrow). They are also hyperintense in a corresponding FLAIR image (B); and some of them clearly present enhancement in a postcontrast axial T1WI at the same level (C and arrow).

Figure 7 . A 46-year-old patient diagnosed with Rosai Dorfman disease. Axial T2WI shows a large petroclival extra-axial lesion, with marked hypointensity, extending into the right temporal fossa, prepontine and right cerebellopontine cisterns (A). There is mass effect on the corresponding temporal lobe and brainstem, with associated vasogenic edema. Axial T1WI demonstrates isointensity (B). Postcontrast axial T1WI depicts marked enhancement of the mass (C).

Figure 8 . A 35-year-old male patient diagnosed with Erdheim-Chester disease. Axial T2WI shows bilateral hyperintensity in the cerebellar white matter, middle cerebellar peduncles and pons, as well as low signal intensity filling the sphenoid sinus (A and arrow). Axial T1WI reveals that the lesions are hypointense (B). Gadolinium-enhanced axial T1WI shows enhancement of the lesions and inside the sphenoid sinus (C). Corresponding axial CT image from a paranasal sinus study reinforces the filling of the sphenoid sinus associated to the presence of thickened sclerotic walls (D). Bone scintigraphy reveals symmetrically increased radionuclide uptake at the level of the distal femoral diaphyses and metaphyses, a quite distinctive finding of the disease (E).

Figure 9 . Patient with 6 months of life presenting bilateral paresis of the sixth cranial nerve, hepatomegaly, increased levels of ferritin and triglycerides, hypofibrinogenemia and pancytopenia, diagnosed as hemophagocytic lymphohistiocytosis. Axial FLAIR (A), diffusion-weighted (B) and enhanced T1WI (C) show subtle hyperintense symmetrical deep cerebellar white matter lesions without restricted diffusion or gadolinium enhancement.

Figure 10 . Two-year-old patient with hemophagocytic lymphohistiocytosis. Axial FLAIR (A) and postcontrast T1WI (B) show lesions in the deep and periventricular white matter of the cerebral hemispheres, some of them associated to contrast enhancement (arrows in B). Axial T2WI (C) and corresponding postcontrast T1WI (D) demonstrate extensive involvement of cerebellar hemispheres and vermis, extending to the right middle cerebellar peduncle, associated to mass effect and heterogeneous contrast enhancement.

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Métricas

Histiocitoses: uma revisão focada em achados de neuroimagem

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Mensagem

[1] Correspondence:
Larissa Barcessat Gabbay; Av. Dr. Enéas de Carvalho Aguiar, s/n° / Rua 1 Cerqueira César; 05403-900 São Paulo SP, Brasil; E-mail: larissagabbay@hotmail.com