Radiological investigations in extrapharyngeal extensions of nasopharyngeal fibromata.
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The spectrum of orbital inflammatory disease (OID) ranges broadly from specific disease diagnoses, for example, Wegener's granulomatosis or sarcoidosis, to nonspecific inflammation which may involve one or multiple structures of the orbit. Mimics of idiopathic OID must be considered in a comprehensive differential diagnosis and include malignancies, congenital mass lesions, infectious diseases, and occult or distant trauma. Idiopathic OID may be secondary to an underlying systemic inflammatory disease, which must be diagnosed in order to develop a comprehensive therapeutic plan, or may represent localized pathologic processes without systemic involvement. Evaluation of the patient with suspected OID must include a careful history, physical examination, directed laboratory, and radiologic studies, and may sometimes require tissue for diagnostic studies. Therapeutic options for inflammatory diseases are expanding as biologically targeted agents become available that act on specific segments of the inflammatory cascades. The purpose of this paper is to provide a framework for the evaluation and management of patients with the spectrum of diseases known as OID and to discuss some of the new advances in immunologic monitoring and targeted immune therapies that will likely play an increasingly important role in the care of these patients.
Adenoid cystic carcinoma is a rare neoplasm arising from the exocrine glands such as salivary glands, lachrymal glands, upper respiratory tract, breast or uterine cervix. Intracranial involvement is commonly from direct skull base involvement, although metastasis may rarely be seen. The predisposition of the adenoid cystic carcinoma for perineural and perivascular invasion is the prime reason for the locally invasive character of the tumour, often extending into the cranium via foramina at the skull base. The authors report a case of primary orbito-cranial extradural adenoid cystic carcinoma and cranial metastasis away from the primary site. This patient initially had a local excision, and later an exenteration of the right eye followed by with radiotherapy. Within months she presented with an extradural cranial recurrence, distant torcular metastasis without any neurological deficit. Craniotomy and radical excision was undertaken as these tumours have slow growth rates, and long term survival of the se patients even in the presence of local recurrence and metastasis has been well documented.
PURPOSE: Over the last decade, MRI has become the standard procedure for the diagnosis of orbital masses. Purpose of the study was to evaluate criteria for differential diagnosis for intraconal masses on MRI in a large patient group. MATERIAL AND METHODS: In a retrospective study, MR examinations of 78 patients with intraconal masses were evaluated. All examinations were performed using surface coils for high spatial resolution. Signal behavior on T2-WI and T1-WI before and after contrast application as well as further tumor characteristics (i. e., shape, size, position, delineation and infiltration) were evaluated. Every diagnosis was proven by histopathological results from extirpation (n = 18) or biopsy (n = 22), or confirmed by other imaging modalities (n = 13) or clinical course (n = 25). RESULTS: Different tumor characteristics are helpful in the differentiation between cavernous hemangioma, lymphangioma, varices, AV malformations and solid tumors. Due to overlapping in the imaging appearance, the differentiation between inflammatory pseudotumor, lymphoma and orbital metastasis was more difficult. CONCLUSION: While high resolution MRI using surface coils allows differentiation between the typical vascular tumors, the differentiation between solid tumors is more difficult. Thus, a histopathologic verification of the diagnosis is necessary in solid tumors.
The aesthesioneuroblastoma (olfactory neuroma) is a rare neuroepithelial tumour of the nasal cavity, the clinical symptoms of which become manifest very late in most patients. In general, with the light microscope used routinely (fixation of the specimens with formaldehyde, staining with haematoxylin-eosine) a malignant round cell neoplasia can be recognised without further differentiation. To ensure the diagnosis of an aesthesioneuroblastoma, immunohistological techniques (vimentin, S-100 protein, neurofilaments, neuron-specific enolase) are undoubtedly necessary. In some cases of unclear findings the electron microscope might be used to prove an aesthesioneuroblastoma. The immunohistological and electron microscopic features of aesthesioneuroblastoma are demonstrated and problems of histological differential diagnosis are discussed.
The radial forearm flap is a versatile tissue transfer in the head and neck. It can be raised either from the distal or the proximal aspect of the volar forearm, and can also include the whole volar forearm skin. The flap is ideal for reconstruction of superficial defects in the head and neck, for the pharynx and oral cavity, as well as for skin defects of the face and the neck. The distal flap is thin and pliant due to the small amount of subcutaneous tissue and the fairly long vascular pedicle. A proximal flap is more bulky due to subcutaneous fat. Since the vascular pedicle of the distal flap is very long, it can also be anastomosed in the contralateral neck. Different applications and indications for the tissue transfer of the radial forearm flap are presented.
MR and CT examinations of 16 patients with lymphoma, pseudolymphoma and inflammatory pseudotumours were analysed to describe morphologic features of lymphocytic infiltration. Density and signal did not allow for differentiation, but localisation was the most important criterion: lymphoma and pseudolymphoma were located in the anterior superior orbit, inflammatory pseudotumours being retrobulbar lesions. Differentiation is of clinical importance, since both lymphoma and pseudolymphoma are accompanied by generalised malignant lymphoma while inflammatory pseudotumours are localised. Imaging of topographic relations of lens, optic nerve and lesion on sagittal MR images was found helpful for radiation therapy planning.
PURPOSE: Retinal ischemia secondary to hypoperfusion of the central retinal artery is recognized as one factor that may contribute to the development of loss of vision in eyes with intraorbital tumors. We study intraorbital tumors which produce motility disturbances and visual problems by color Doppler imaging to evaluate this factor. METHODS: We examined the central retinal artery velocities of 3 patients with disc edema caused by intraconal masses (2 cavernous hemangiomas and 1 presumed optic nerve glioma) via color Doppler imaging. RESULTS: The time-velocity waveform demonstrated abnormally high vascular resistance in the central retinal artery of all affected eyes in the primary position compared with the normal waveform seen in the other eyes. We compared the pulsatility index of eyes with an intraconal mass and contralateral, control eyes using Student's t test for paired samples and significant differences were noted between both groups (p< 0.01). CONCLUSIONS: Intraconal tumors could produce increased pressure in the optic nerve sheath and the optic nerve tissue which could be associated with impaired retinal and optic nerve blood flow and the subsequent amaurosis encountered with intraorbital tumors.
PURPOSE: To analyze the clinical, morphologic, immunophenotypic, and molecular genetic differences between idiopathic inflammatory pseudotumor and lymphoid tumors of the orbit. METHODS: 209 patients with IOIP and 110 patients with lymphoid tumors seen between January 1, 1978 and December 31, 1999 in Zhongshan Ophthalmic Center, Sun Yat-sen University were evaluated retrospectively. RESULTS: More patients with lymphoid tumors had palpable mass than patients with idiopathic orbital inflammatory pseudotumor (IOIP) (P < 0.0001), with the percentage of 90% and 65%, respectively, whereas more patients with IOIP had swollen eyelid, eyelid or conjunctival congestion, pain, retinal folds or hamorrhage, and optic nerve atrophy than patients with lymphoid tumors, with the percentage of 55% and 40% (P = 0.014), 42% and 24% (P = 0.001), 24% and 1% (P < 0.0001), 14% and 4% (P = 0.004), 7% and 2% (P = 0.043), respectively. Ultrasound and computed tomography/magnetic resonance image (CT/MRI) scan usually could not differentiate IOIP from lymphoma. Nearly one third of patients with IOIP could not be easily differentiated pathologically from lymphoid tumor. Immunophenotypic, and molecular genetic analyses can differentiate IOIP from lymphoid tumor based on polyclonal or monoclonal proliferation of lymphocytes with the IOIP being polyclonal and lymphoma monoclonal. CONCLUSIONS: Idiopathic orbital inflammatory pseudotumor and lymphoid tumor showed distinguishing clinical, morphologic, immunophenotypic, and molecular genetic characteristics.
Eleven patients with solitary plasmacytoma of bone (SPB) and six patients with extramedullary plasmacytoma (EMP) were treated at the UCLA Center for the Health Sciences. Primary treatment in 14 of 17 patients was with radiation, while three patients were irradiated for recurrent disease. Eleven patients with SPB were irradiated with dose of 32-55 Gy, with 10 of 11 patients receiving doses of 40-55 Gy. Local control was achieved in 10 of 11 patients with SPB. One patient died with metastatic disease with unknown local status. Six patients with EMP were irradiated with doses of 38-56 Gy. Of these patients, two were locally controlled; one patient failed locally; one patient died during treatment; one patient died with local disease at 85 months after multiple resections, chemotherapy, and two courses of irradiation; and one patient was lost to follow-up. Progression to multiple myeloma was seen in 5 of 11 patients with SPB and in none of six patients with EMP. For patients with SPB, we recommend treating the entire bone to 40 Gy, with a boost when feasible. Patients with EMP receive the same dose, including the lymph nodes in tumors at high risk for spread. Radical surgical resections appear to be unwarranted.
Complex midface defects following trauma and tumor resection present a challenging problem for maxillofacial surgeons. Regardless of the cause of these defects, restoration of accurate contour is essential to achieve both proper function and cosmetically acceptable appearance. Traditionally, bone grafts have been the preferred method of reconstruction. We present the first report of the use of Vitallium micromesh in reconstruction of midface bone and cartilaginous defects in 3 patients. In 1 patient, bone loss resulted from trauma; in the other 2, loss was due to tumor resection. In all 3 patients, Vitallium micromesh was used successfully as a replacement for the bony skeleton without morbidity or complications. Use of this material is contingent on its rigid fixation. We do not recommend use of this technique in the absence of rigid fixation.
The use of three-dimensional, computer-generated anatomic models can be used in the diagnosis and reconstruction of a variety of craniofacial problems. They are readily manufactured from computed tomography scans at a reasonable cost with only several weeks of preparation and delivery time. Their contemporary value is in the preoperative treatment planning, intraoperative implant fashioning, and preoperative implant fabrication in appropriately selected patients.
The purpose of the present study was to determine whether proliferating cell nuclear antigen (PCNA), an immunohistochemical marker for a nuclear protein abundant in actively proliferating (dividing) cells, is useful as an aid in differentiating idiopathic orbital inflammatory syndrome (IOIS) from lymphoproliferative lesions (LLs). Records of all patients with IOIS and LLs were studied retrospectively. Tissue biopsy specimens from four patients with IOIS and nine patients with LLs were examined. The diagnosis in each case was based on presenting signs and symptoms, orbital computed tomography (CT) and/or magnetic resonance (MR) scans, histopathologic criteria, and follow-up data consistent with the entity. These findings were correlated with the percentage of B- and T-cells in the lesions as well as with the number of cells that demonstrated staining for PCNA in formalin-fixed tissue. PCNA activity was markedly increased in the higher grade (HG) lymphoma group as compared to that in the low grade (LG) lymphoma and idiopathic inflammatory group. Lymphoma cases showed a significantly increased B-/T-cell ratio compared to IOIS lesions. PCNA activity in conjunction with the ratio of B-/T-cells may be a helpful immunohistologic adjunct for differentiating purely inflammatory lesions of the orbit from lymphoid tumors. Further studies are necessary to compare PCNA activity in fresh frozen tissue with that in formalin-fixed tissue.
Twenty-two patients with clinical presentations of either orbital lymphoma or orbital inflammatory syndrome (OIS) involving 25 orbits were examined by magnetic resonance imaging (MRI) before biopsy. To determine whether MRI adds specificity to radiographic diagnosis, the MRI signal characteristics of all tumors were examined. Attention was focused on the signal intensity of each tumor on T1- and T2-weighted images. In nine of 12 (75%) orbital lymphomas, the tumors appeared hyperintense to fat on T2-weighted images and became brighter relative to their appearance on T1-weighted images. In 11 of 13 (85%) orbital infiltrates with OIS, the tumors appeared isointense to fat on T2-weighted images and became slightly darker or unchanged relative to their appearance on T1-weighted images. Tumor density and homogeneity were fairly similar in all 25 lesions and were therefore not useful for further differentiation. Similarly, the presence of moderate enhancement with gadopentetate dimeglumine was seen in all but one tumor, and it was therefore not useful for further differentiation.
The benign anterior visual pathway glioma undoubtedly is the most controversial subject in neuro-ophthalmology. Although we are persuaded by the evidence that they are true neoplasms rather than hamartomas, the controversy over their biological nature seems moot, as they have a low growth potential. That is not to say that serious visual loss is not common or that, in some cases (especially among those lesions arising in the chiasmalhypothalamic region), tumor-related death and disability cannot occur. These posteriorly located lesions may cause hydrocephalus, and shunting is required if that occurs. The need for shunting in such cases may be the only point on which experts agree. What has yet to be established is whether any of the forms of therapy now available for anterior visual pathway gliomas without hydrocephalus is better than no treatment. Surgery, radiation, and chemotherapy all carry risks. A randomized, controlled study, if feasible, would be apt to settle the issue. The low incidence of these tumors might frustrate attempts to complete such an investigation in a reasonable period. A team of physicians should manage patients with benign anterior visual pathway gliomas. Radiologists, neurologists, neurosurgeons, radiation oncologists, pediatricians, endocrinologists, and oncologists all have a role, but the neuro-ophthalmologist should be the leader. Neuro-ophthalmologists are in the unique position of being able to recognize and document changes in the patient's visual function. Furthermore, neuro-ophthalmologists, being disinterested, are free to choose without bias from among the various management options. The malignant anterior visual pathway glioma is not a subject of controversy. The clinical features at presentation are apt to be misinterpreted as evidence of an inflammatory or ischemic neuropathy rather than the announcement of a fatal brain tumor. Though these patients receive radiotherapy and chemotherapy, these measures have not improved survival significantly, and the outcome is uniformly fatal. The only positive thing that can be said about the malignant gliomas of the anterior visual pathway is that they are so rare.
OBJECTIVE: The differentiation between orbital lymphoma and subacute or chronic inflammatory pseudotumor (SCIPT) may be difficult clinically and radiologically. The aim of this study was to evaluate the enhancement characteristics of orbital lymphoma and SCIPT with two-phase helical computed tomography (CT) and delayed coronal CT and to determine whether attenuation measurements on CT can be used to differentiate between the 2 diseases. METHODS: Nineteen histopathologically proven orbital lymphomas and 9 SCIPTs were examined with two-phase helical CT. After injecting 90 mL contrast material at a rate of 3 mL/s, early- and late-phase axial CT scans were obtained with scanning delays of 30 and 90 seconds, respectively. Delayed coronal scans were obtained with delays of 4-9 minutes. Attenuation of the lesions at each phase was measured quantitatively. Relative percentages of contrast enhancement (CE) were calculated to determine the cutoff value for differentiating SCIPT from lymphoma. RESULTS: The CT attenuation change over time was significantly different between orbital lymphoma and SCIPT (P < 0.05). Increased CT attenuation between early- and late-phase axial scans was seen in 42% (n = 8) of lymphoma cases, and decreased CT attenuation was seen in 58% (n = 11). In 17 lymphomas (90%), the CT attenuation decreased between late-phase axial and delayed coronal scans. Conversely, in 7 SCIPTs (78%), the CT attenuation increased gradually over time from early-phase axial to delayed coronal scans. The relative percentage of CE at the delayed coronal scan had a cutoff value of -6.97%, a sensitivity of 84%, and a specificity of 100%. CONCLUSION: Different characteristics of attenuation change on two-phase helical CT and delayed coronal CT can be helpful in differentiating between orbital lymphoma and SCIPT.
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