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Ординатура / Офтальмология / Английские материалы / Imaging of Orbital and Visual Pathway Pathology_Muller-Forell_2005

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236

W. Müller-Forell and S. Pitz

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Fig. 6.94a–h. A 37-year-old woman with Graves’ disease persisting for several years, currently presenting with proptosis of the right eye (31 mm), progressing slowly over the past few months. No other known history of diseases. Diagnosis: neurofibromatosis in (previously unknown) NF 1. CT: a Axial medial orbital view, where in addition to the inhomogeneous mass infiltrating the retrobulbar fat and the lacrimal gland, the right lateral sphenoid wing is missing. MR: b Axial proton density-weighted view, demonstrating the primarily intraconal but also extraconal mass. c Axial T1-weighted native view. d Corresponding T1-weighted, contrast-enhanced (FS) view with infiltration of the largest part of the retrobulbar fat by the irregularly shaped tumor. Note the bright enhancement of the temporopolar dura. e Coronal, T1-weighted, contrast-enhanced (FS) view where the entire tumor, sparing the external rectus muscles, is seen. Note the normal size of the conal muscles without any sign of

Orbital Pathology

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Graves’... ... disease. f Coronal, T1-weighted, contrast-enhanced (FS) of the orbital apex, demonstrating dural enhancement of the entire middle cranial fossa region. Note asymmetry of the middle cranial fossa as well as of the orbit. Histology: g (×280) several pseudomesserian corpuscles (arrows), surrounded by a dense fibrillary connective tissue, diffusely proliferating in the orbital tissue, confirm the diagnosis of neurofibroma. h (×140): focal lymphatic infiltration (corresponding to Graves’ disease) in the neighborhood of some blood vessels surrounded by a dense fibromatous tissue. (CT image with permission of Radiologie Brüderkrankenhaus Trier, histology with permission of Dr. Bohl, Department of Neuropathology, Medical School, Mainz)

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W. Müller-Forell and S. Pitz

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Fig. 6.95a–d. A 78-year-old man presenting with epistaxis and numbness of the left cheek. Diagnosis: carcinoma of the left maxillary sinus. CT: a Axial native view showing soft tissue in the inferior left orbit with poor distinction from the lateral rectus muscle. b Paracoronal native view, demonstrating the destruction of the maxillary sinus roof and medial wall leading to tumor invasion into the inferior orbit and nasal cavity (white star). MRI: c Coronal T2-weighted view with demarcation of necrotic tumor parts (high signal). Although destruction of the periorbital area might be expected, no infiltration of the inferior rectus muscle is seen. Note a maxillary sinus cyst (hyperintense) on the right side. d Corresponding T1-weighted native view identifying additional pronounced destruction of the maxillary sinus floor (white arrow) on comparison with c

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Fig. 6.96a–d. A 44-year-old woman with right exophthalmos associated with maxillary sinus carcinoma. Diagnosis: orbital and intracranial infiltration of an adenocarcinoma of the maxillary sinus. MRI: a Coronal T1-weighted native view where the complete extension of the tumor is seen not only in the maxillary, ethmoidal sinus, both nasal cavities and the orbit, but also in the anterior cranial fossa. The transverse hypointensity (arrowheads) demarcates the remaining orbital and ethmoidal floor (see c). Differentiation from the medial and inferior rectus muscles (arrows) is markedly better in the native view than in the corresponding contrast-enhanced view (b) due to normal signal enhancement of the muscles. While the frontobasal dura (arrowheads) appears to be intact, the rectus and medial orbital gyrus are compressed by tumor expansion. CT: c Coronal view (bone window) yielding superior visualization of the full extent of the bony destruction in addition to that of the right maxillary and orbital walls. The tumor crosses the midline, extending into the left ethmoidal cells and the left infundibulum. MR: d Axial, T1-weighted, contrast-enhanced image where the extraconal invasion of the right orbit with dislocation of the medial rectus muscle (arrow) is clearly visualized. Marginal swelling of the mucous membrane of the sphenoid sinus indicates retention of mucus caused by tumor occlusion of the ducts. (With permission of Müller-Forell and Lieb 1995b)

and S. Pitz

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Fig. 6.97a,b. A 68-year-old woman with painful swelling of the left periorbital region. Diagnosis: metastasis of breast carcinoma. CT: a Axial contrast-enhanced view with destruction of the frontal, sphenoid, and temporal bone as a result of intraorbital, intracranial, and infratemporal tumor expansion. b Coronal contrast-enhanced view, the defect of the right frontal bone corresponds to metastasis removal 12 months previously

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Fig. 6.99a–c. A 10-year-old girl with bilateral proptosis and

 

bilateral ecchymotic swelling of the lids. Diagnosis: metastatic

 

neuroblastoma. CT: a Axial native view with bilateral extra-

 

conal tumor mass and osteodestruction of the great sphenoid

 

wing (triangles), dislocating the lateral rectus muscles with

 

emphasis to the left (arrow). b Corresponding bone window

 

yielding better visualization of the osseous destruction (tri-

 

angles). MR: c Corresponding native T1-weighted image. The

 

bilateral extraconal tumor extension with respect to the dis-

 

located rectus muscles and the intracranial extraparenchymal

 

tumor infiltration are more distinctly differentiated than on

 

CT (a). MRI further enables the diagnosis of bony destruction

 

based on the absence of both the characteristic signal loss of

 

the bone cortex and the signal intensity of the bone marrow.

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(With permission of Müller-Forell and Lieb 1995b)

Fig. 6.98a–d. A 59-year-old man with history of carcinoma of the prostate, no subjective symptoms, but nuclide concentration in the left orbital region in bone radionuclide scan. Diagnosis: metastasis of a prostate carcinoma in the left superolateral orbital wall. MR: a Axial T2-weighted image with enhancement of the lateral rectus muscle region (white arrows) and enlargement of the great sphenoid wing (short black arrow). b Corresponding T1-weighted native view with irregular soft-tissue infiltration of the lateral orbital wall (arrowheads) and temporopolar thickening of the dura. c Coronal T1-weighted native view where a soft extraconal tissue mass extending to the intermuscular septum is identified. Note the different signal of both lateral parts of the great wing of the sphenoid (short black arrows), indicating infiltration of the respective bone marrow fat. d Corresponding T1-weighted, contrast-enhanced (FS) view, demonstrating infiltration of the periorbit, the dura, and even the temporal muscle by metastasis

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W. Müller-Forell and S. Pitz

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Fig. 6.100a–d. A 37-year-old woman with previously operated primary melanoma of the maxillary sinus, presenting with swelling of the right eye. Diagnosis: intraorbital expansion of the maxillary sinus melanoma. MR: a Axial T2-weighted view of the inferior orbit, filled with tumor on the right side leading to the suspicion of infiltration of the inferior rectus muscle (white star) and orbital fat. Note additional tumor invasion of the ipsilateral temporal fossa. The bright signal of the ethmoidal cells represents mucous retention. b Corresponding T1-weighted, contrast-enhanced view with slight, homogeneous enhancement of the tumor, but a nearly normal signal intensity for the fat (arrow) medial to the rectus muscle. c Coronal T1-weighted native view showing the entire tumor expansion in the right maxillary sinus, nasal cavity, and ethmoidal sinus, as well as inferior orbital invasion. Note the upward displacement without infiltration of the inferior rectus muscle and globe. d Corresponding T1-weighted, contrast-enhanced view showing nearly homogeneous enhancement

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Fig. 6.101a,b. A 10-year-old girl with persistent swelling of the left cheek. Diagnosis: neuroblastoma. CT: a axial image, showing tumor invasion of the left maxillary sinus, ipsilateral retromaxillary (arrow), and nasal space with bone destruction. b Coronal view with upper globe displacement due to intraorbital tumor expansion with suspected infiltration of the external inferior muscle. Tumor growth probably started in the left upper maxillary sinus roof, destroying the maxillary and nasal bone and also invading the ipsilateral ethmoid

6.3.1.4.2

Olfactory Neuroblastoma (Syn. Esthesioneuroblastoma)

The relatively uncommon olfactory neuroblastoma represents a malignant neuroectodermal tumor originating from bipolar olfactory receptor cells high in the mucosa of the nasal cavity, affecting both sexes with an approximately equal frequency (Finkelstein et al. 2000). This slowly growing tumor may occur at any age, with a cluster around 20 and 50 years, and is typically associated with longstanding symptoms of nasal obstruction, anosmia, and epistaxis. The involvement of the orbit and/or endocranium already represents an advanced stage (Kadish et al. 1976). Imaging discloses an upper nasal vault mass with focal bony destruction,presenting with mixed signal intensity on MRI and a moderate but inhomogeneous enhancement after contrast administration (Osborn 1994) (Figs. 6.102–6.104). In cases of difficult differential diagnosis concerning other tumors of the paranasal sinuses, the performance of 123I-MIBG SPECT may be justified (Sasjima et al. 2000).

6.3.1.4.3

Langerhans-Cell Histiocytosis (LCH)

Histiocytosis X was first defined by Lichtenstein (1953) to include an inflammatory eosinophilic granuloma of the bone and the adjacent soft tissue. Involvement of a dendritic cell of bone marrow origin (Langerhans-type histiocyte) has been demonstrated as the common pathologic element and unique identifier, described as Langerhans-cell histiocytosis (Neselof et al. 1973; Chu et al. 1987; Tien et al. 1991). Langerhans-cell histiocytosis is a disease of unknown origin with variable clinical manifestations ranging from nonprogressive solitary eosinophilic granuloma of the bone to progressive, more aggressive, and often fatal multisystemic involvement (Howarth et al. 1999; Poe et al. 1994) (see also Sect. 7.2.1.2.3).A single system disease, found in about 70% of the patients, is most frequently seen as an isolated bone lesion, with preference for the skull and femur. Solitary or monostotic eosinophilic granuloma is the most common presentation of LCH in children, typically affecting the skull vault and presenting as a welldefined circumscribed mass (Fig. 6.105). On CT, the lesion is typically of similar density as the cortical gray matter, while on MRI a marked hypointensity on T2-weighted images appears to be characteristic and may be related to a severe fibrotic reaction of the involved meningeal tissue. After administration of contrast medium, a marked contrast enhancement is seen (Fig. 6.105c) (Barkovich 2000).

(Text continues on p. 248)

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W. Müller-Forell and S. Pitz

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Fig. 6.102a–e. A 38-year-old man, presenting with persistent pressure on both eyes in addition to persistent nasal obstruction. Diagnosis: esthesioneuroblastoma. MRI: a Axial T1weighted image, demonstrating complete tumor-like obstruction of the ethmoid and sphenoid sinuses with marked bilateral intraorbital extension in the region of the lamina papyracea. b Corresponding T1-weighted, contrast-enhanced (FS) image showing intraorbital tumor growth with slight impression of both medial rectus muscles but the presence of an apparently intact periorbit. Note the hypointensity at the center of the tumor, corresponding to necrotic areas. c Coronal T1-weighted native view demonstrating the intraorbital growth. Widening of the frontal groove may be responsible for the destruction of the frontal base and intracranial expansion. Note the olfactory bulbs (small arrows), located below the rectus gyri. d Corresponding T1-weighted, contrast-enhanced (FS) image with tumor invasion of the base of both supra-

e orbital recesses and mucous retention, identified by homogeneous signal intensity enhancement (arrow), no conclusive dural enhancement. e Sagittal paramedian, T1-weighted, con- trast-enhanced view showing dural enhancement along the entire sphenoid plane (small arrows). Note the AP and craniocaudal dimension of the tumor growth with destruction of the sphenoid bone and the bony palate

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Fig. 6.103a–h. A 65-year-old woman, presenting with slight proptosis of the right eye, diffuse pressure in the orbital region, and difficulty in nasal breathing for a period of several months. Diagnosis: esthesioneuroblastoma. a Axial contrast-enhanced CT demonstrating a very large tumor occupying predominantly the right ethmoid and sphenoid sinuses and crossing the midline. Although the medial orbital wall is destroyed and intraorbital extraconal tumor expansion with medial dislocation of the medial rectus muscle is visualized, the presence of a small fat border (arrow) indicates sparing of the periorbit. b Corresponding T1-weighted native MRI, showing intraorbital expansion less conclusively, but providing a superior view of the periorbital growth. Note the expansion to the bony optic canal. c Coronal CT in the bone window, demonstrating additional destruction of the skull base and craniocaudal expansion of the tumor into the entire right nasal cavity. d Corresponding T2-weighted MRI with excellent differentiation between the mixed solid and necrotic tumor with slight high intensity and the bright signal of congestion due to maxillary mucus, but with only poor differentiation between tumor and brain parenchyma/gray matter. e–h see next page

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