The simultaneous appearance of a nasal natural killer-cell lymphoma and acute myelogenous leukemia.

INTRODUCTION AND AIM: Sinonasal malignant neoplasms are uncommon, with an annual incidence of less than 1/100,000. About 80% of these are squamous cell carcinoma. Adenocarcinoma and adenoid cystic carcinoma are next in frequency. Lymphoma of the nasal cavity, paranasal sinuses and nasopharynx are rare, constituting less than 5% of all extranodal lymphomas. CASE REPORT: A 47-year-old man was referred to our hospital because of severe headache and progressive facial pain. He also complained of right-sided visual acuity. He had a manifest exopthalmia with disturbed eye movements. Nasoscopy showed a large mass with atypical appearance. CT and MRI showed a bilateral ethmoid mass invading the frontal sinuses, the right orbit, the lamina cribrosa and the right frontal cerebral region, and growing posteriorly through the choana. The first biopsies were inconclusive, showing only necrotic cells and purulent inflammation with epithelial elements. A larger biopsy demonstrated a high-grade malignant tumour with necrosis. The differential diagnosis of undifferentiated sinonasal carcinoma, undifferentiated neuro-endocrine tumour or T-cell lymphoma was suggested. In the meantime our patient developed high fever and sudden-onset pancytopenia. Bone marrow punction showed 65% blasts, leading to the diagnosis of AML type M2. He was immediately referred for chemotherapy, but died in intensive care before his first session. The biopsy of the sinonasal mass was diagnosed surprisingly as a natural killer cell lymphoma stage IVB. CONCLUSIONS: Natural killer cell lymphoma is rare in Europe. The simultaneous appearance of a NK-cell lymphoma and acute myelogenous leukemia has, as far as we know, never been described in the English literature before.

Imaging of the opacified middle ear.

Middle ear opacification on imaging studies performed in a non-traumatic setting mostly reflects chronic inflammatory/infectious disease. In some of these patients an underlying cholesteatoma will be found. High-resolution computed tomography examinations and magnetic resonance imaging are often used in the work-out of the disease. High-resolution computed tomography of the opacified middle ear serves to describe the status of the ossicular chain, and its suspensory apparatus, as well as the status of the tympanic and mastoid wall. When ossicular erosions are visualized, the probability of a present cholesteatoma is about 90%. Whereas high-resolution computed tomography is not able to differentiate cholesteatoma from other types of opacification, magnetic resonance imaging is. The combined use of delayed post-Gd T1-weighted images and non-EPI based DWI seems to be the actual best option on this matter.

Arachnoid granulations of the posterior temporal bone wall: imaging appearance and differential diagnosis.

Arachnoid granulations are rarely seen on high-resolution CT (HRCT) at the posterior temporal bone wall, where they appear as erosions, without bone spicules and often with a lobulated surface. Differential diagnosis includes endolymphatic sac tumor, paraganglioma, chordoma, and chondromatous and metastatic tumors. MR imaging can confirm the diagnosis because arachnoid granulations behave like CSF without gadolinium enhancement. This report aims to illustrate the appearance and differentiation of temporal bone arachnoid granulations on HRCT and MR imaging.

Imaging Features of Hypertrophic Olivary Degeneration.

Hypertrophic olivary degeneration (HOD) is a unique form of transneuronal degeneration caused by a disruption of the dentato-rubro-olivary pathway, also known as the triangle of Guillain-Mollaret. The triangle of Guillain-Mollaret is involved in fine voluntary motor control and consists of both the inferior olivary nucleus and the red nucleus on one side and the contralateral dentate nucleus. Clinically, patients classically present with symptomatic palatal myoclonus. Typical magnetic resonance imaging findings include T2-hyperintensity and enlargement of the inferior olivary nucleus evolving over time to atrophy with residual T2-hyperintensity. In this article, we provide a case-based illustration of the anatomy of the Guillain-Mollaret-triangle and the typical imaging findings of hypertrophic olivary degeneration.

Otologic disease in turner syndrome.

OBJECTIVE: This study involved the assessment of the otologic and audiologic characteristics of a group of patients with Turner syndrome. STUDY DESIGN: Prospective study consisting of a questionnaire (77 of 123 responders) and an otologic and audiologic evaluation in patients with Turner syndrome (41 participants). SETTING: Tertiary academic medical setting. PATIENTS: Children, adolescents, and adults with Turner syndrome (median age, 24 yr). INTERVENTIONS: Otomicroscopy, audiometry, immittance measurements, and diagnostic imaging. RESULTS: Otologic disease is an important characteristic in Turner syndrome. Sixty-six percent of the patients studied via the questionnaire reported a history of chronic or recurrent middle ear disease. Analysis of audiometric data in 40 patients tested reveals an equal amount of normal ears (38.8%) and pure sensorineural ears (38.8%), each constituting approximately one-third of the patient population. Pure conductive losses represent only one-fifth (21.3%) of auditory abnormality encountered. CONCLUSIONS: Careful follow-up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent sequelae. However, long-term periodic review is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.

An implementation strategy for IMRT of ethmoid sinus cancer with bilateral sparing of the optic pathways.

PURPOSE: To develop a protocol for the irradiation of ethmoid sinus cancer, with the aim of sparing binocular vision; of developing a strategy of intensity-modulated radiation therapy (IMRT) planning that produces dose distributions that (1) are consistent with the protocol prescriptions and (2) are deliverable by static segmental IMRT techniques within a 15-minute time slot; of fine tuning the implementation strategy to a class solution approach that is sufficiently automated and efficient, allowing routine clinical application; of reporting on the early clinical implementation involving 11 patients between February 1999 and July 2000. patients and methods: Eleven consecutive T1-4N0M0 ethmoid sinus cancer patients were enrolled in the study. For Patients 1-8, a first protocol was implemented, defining a planning target volume prescription dose of 60 to 66 Gy in 30-33 fractions and a maximum dose (Dmax) of 50 Gy to optic pathway structures and spinal cord and limit of 60 Gy to brainstem. For Patients 9-11, an adapted (now considered mature) protocol was implemented, defining a (planning target volume) prescription dose of 70 Gy in 35 fractions and a Dmax to optic pathway structures and brainstem of 60 Gy and to spinal cord of 50 Gy. RESULTS: The class solution-directed strategy developed during this study reduced the protocol translation process from a few days to about 2 hours of planner time. The mature class solution involved the use of 7 beam incidences (20-37 segments), which could be delivered within a 15-minute time slot. Acute side effects were limited and mild. None of the patients developed dry eye syndrome or other visual disturbances. The follow-up period is too short for detection of retinopathy or optic nerve and chiasm toxicity. CONCLUSION: Conventional radiotherapy of ethmoid sinus tumors is associated with serious morbidity, including blindness. We hypothesize that IMRT has the potential to save binocular vision. The dose to the optic pathway structures can be reduced selectively by IMRT. Further enrollment of patients and longer follow-up will show whether the level of reduction tested by the clinical protocol is sufficient to save binocular vision. An adaptive strategy of IMRT planning was too inefficient for routine clinical practice. A class solution-directed strategy improved efficiency by eliminating human trial and error during the IMRT planning process.

The Meier-Gorlin syndrome, or ear-patella-short stature syndrome, in sibs.

The Meier-Gorlin syndrome, first described by Meier and Rothschild [1959: Helv Paediatr Acta 14:213-216] and further delineated by Gorlin et al. [1975: A Selected Miscellany, p 39-50], is characterized by short stature, slender body build, craniofacial anomalies, microtia, delayed skeletal development, hypogonadism, and absence of the patellae. It has also been called the ear-patella-short stature syndrome [Boles et al., 1994: Clin Dysmorphol 3:207-214]. We report on two brothers with Meier-Gorlin syndrome, the younger of whom was more severely affected. Both patients had severe deafness and congenital labyrinthine anomalies, which have not previously been described as features of this syndrome. The neuromotor and mental development of these patients was adversely affected by late diagnosis, deafness, and their sociocultural environment, but their cognitive ability fell within the range observed in other Meier-Gorlin patients. Neuroradiographic imaging and functional inner ear investigations are recommended in the diagnostic workup of this rather specific, probably autosomal recessive mental retardation syndrome with multiple congenital anomalies.

Early predictors of mortality and morbidity after severe closed head injury.

Mortality and morbidity of 158 patients with severe head injury were studied in relation to age, and early (24-h) clinical and computed tomography data. For comparison of outcome data in survivors, a group of 32 patients with traumatic injuries to parts of the body other than the head was used as controls. Within the head-injured group, the mortality rate was 51%. Logistic regression analyses combined 13 out of 16 predictors into a model with an accuracy of 93%, a sensitivity of 90%, and a specificity of 95%. These include age, Glasgow Coma Scale (GCS) score, pupillary reactivity, blood pressure, intracranial pressure, blood glucose, platelet count, body temperature, cerebral lactate, and subdural, intracranial, subarachnoid, and ventricular hemorrhage. At 6 months postinjury, head-injury survivors and trauma controls were evaluated with the Glasgow Outcome Scale (GOS), a neuropsychological test battery and the Sickness Impact Profile (SIP). Head-injury survivors had a higher proportion of disabilities and neuropsychological dysfunctions than trauma controls. They also report more quality of life-related functional limitations on the SIP scales for mobility, intellectual behavior, communication, home management, eating, and work. Linear regression analysis resulted in age being the only important predictor of outcome on the GOS, the GCS score being the best predictor of neuropsychological functioning, and pupillary reactivity being the most predictive for self-reported quality of life as measured by SIP. Those factors important for predicting mortality (clinical variables such as ICP or blood glucose level, and CT observations) failed to show any significant relationship with morbidity.

Neurocutaneous melanosis presenting with intracranial amelanotic melanoma.

We describe imaging findings in a 2-year-old girl with neurocutaneous melanosis and malignant cerebral melanoma. Because the cerebral melanoma in this child was of the amelanotic type, high-signal intensity on unenhanced T1-weighted images was not present. The cutaneous lesions played a crucial role in establishing a correct (presumed) histopathologic diagnosis on the basis of the imaging findings. To our knowledge this is the first report describing an intracranial amelanotic malignant melanoma in association with neurocutaneous melanosis.

CT of the normal suspensory ligaments of the ossicles in the middle ear.

PURPOSE: To establish the range of normal variation in the CT appearance of the middle ear ligaments and the stapedius tendon as an aid in detecting abnormal changes in these structures. METHODS: CT scans of the temporal bone in 75 normal middle ears, obtained with 1-mm-thick sections, were reviewed by two observers, who rated the visibility of the structures of interest on a scale of 1 to 5. RESULTS: The anterior, superior, and lateral malleal ligaments and the medial and lateral parts of the posterior incudal ligament were seen in 68%, 46%, 95%, 26%, and 34% of the ears, respectively. The stapedius tendon was seen in 27% of the cases. When visible, the ligaments were judged to be complete in 90% to 100% of the ears and the stapedius tendon was complete in 65% of cases. Their width varied considerably. Interobserver variability was high for most observations. CONCLUSION: CT scans are more likely to show the malleal than the incudal ligaments. Although the interobserver agreement was statistically significant for most study parameters, the percentage of agreement above that expected by chance was low. When seen, the ligaments usually appeared complete. Understanding the normal range of appearance may help identify abnormalities of the ligaments and tendons of the middle ear.

Unilateral semicircular canal aplasia in Goldenhar's syndrome.

A patient with Goldenhar's syndrome (oculoauriculovertebral dysplasia) and unilateral aplasia of all semicircular canals is presented. This is the first report of such a finding and may support the hypothesis that Goldenhar's syndrome and the CHARGE association have a common pathogenetic mechanism.

MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac.

BACKGROUND AND PURPOSE: Large endolymphatic duct and sac (LEDS) is one of the most common anomalies seen in patients with congenital sensorineural hearing loss (SNHL), and is known to occur with other inner ear findings. Our purpose was to use high-resolution T2-weighted fast spin-echo (FSE) MR imaging to describe the features and prevalence of specific anomalies that occur in association with LEDS. METHODS: We retrospectively reviewed MR images of the inner ear obtained in 63 patients with LEDS and in 60 control subjects. We evaluated each image for features of cochlear and vestibular dysplasia, including deficiency of the cochlear modiolus, gross cochlear dysmorphism, asymmetry of the cochlear scalar chambers, enlargement of the membranous vestibule, gross vestibular dysmorphism, and abnormality of the semicircular canals (SCC). RESULTS: Cochlear anomalies were present in 76% of ears with LEDS. Modiolar deficiency, gross dysmorphism, and scalar asymmetry were seen in 94%, 71%, and 65% of abnormal cochleas, respectively. Vestibular abnormalities were present in 40% of ears with LEDS. Simple enlargement, gross dysmorphism, and distortion of the lateral SCC were seen in 84%, 16%, and 32% of abnormal vestibules, respectively. CONCLUSION: Coexistent cochlear anomalies, vestibular anomalies, or both are present in most ears with LEDS, and appear as a spectrum of lesions, ranging from subtle dymorphism to overt dysplasia. The presence of coexistent anomalies in LEDS affects treatment decisions and prognosis. Newer techniques of high-resolution FSE MR imaging provide a means of exquisite characterization of LEDS, as well as more sensitive detection of associated vestibulocochlear anomalies.

55Cobalt (Co) as a PET-tracer in stroke, compared with blood flow, oxygen metabolism, blood volume and gadolinium-MRI.

Several studies have shown the feasibility of divalent cobalt (Co)-isotopes (55Co and 57Co) in imaging of neuronal damage in stroke, multiple sclerosis, cerebral tumors and traumatic brain injury. Little is known how regional Co uptake relates to other pathophysiological changes after stroke. Therefore, we compared 55Co-PET with functional parameters such as regional cerebral blood flow (rCBF) using C(15)O(2), regional oxygen metabolism (rCMRO(2)) using 15O(2), regional cerebral blood volume (rCBV) and post-gadolinium (Gd) T(1)w-MRI to assess the permeability of the blood-brain-barrier (BBB). Sixteen patients (10 female; six male) aged 43 to 84 (mean 69) years with first ever stroke, as shown by CT or MRI, were examined with 55Co-PET and C(15)O(2)-, 15O(2)- and C(15)O-PET in one single session, in a period varying from 0 to 30 days after stroke-onset. Regions of infarction on C(15)O(2)- and 15O(2)-PET (defined by rCMRO(2)<65% or rCBF<45% of the contralateral value) were subsequently superimposed on the 55Co-PET scan. Clinical status was established using the Orgogozo stroke scale, which was assessed both at day 1 and at discharge (at least 6 weeks after day 1). Accumulation of 55Co was seen in eight out of 16 patients, occurring in areas showing a diminished oxygen metabolism, was only partially related to blood flow, and was located mainly outside the extent of the infarction or luxury perfusion as seen on post-Gd T(1)w-MRI. Statistical analysis showed a negative correlation between the Orgogozo score at discharge and the uptake of radioactive cobalt.

Primary sinonasal mucosal melanoma: three different therapeutic approaches to inoperable local disease or recurrence and a review of the literature.

Sinonasal melanoma is an uncommon disease with a bad prognosis and a high local recurrence rate. The use of radiotherapy in such conditions remains controversial. A review of the literature is presented in an attempt to answer some of the questions regarding therapeutic options. Data on incidence, aetiology, pathology and prognosis are also provided. Case studies are reported of three patients with sinonasal melanoma referred to our department in a relatively short time period and needing radiotherapy as a single treatment modality, using a conformal three-dimensional treatment technique. One patient was also treated with temporary 1251 seeds, while another was treated using intensity modulation. Both intensity modulation and temporary 1251 seeds are feasible techniques, providing satisfactory dose distributions encompassing the tumour volume while sparing critical structures. Surgery remains the treatment of choice for sinonasal melanoma. Radiotherapy should be used postoperatively and is a good alternative in cases of inoperable disease. Adjacent critical structures limit the radiation dose to the tumour area, especially when high fraction doses are used. Therefore, beam intensity modulation and 1251 seeds can be used to increase the tumour dose without exceeding the radiation tolerance of the surrounding structures.

Non-invasive diagnostic assessment of extensive vertebral artery tortuosity with enlargement of the intervertebral foramen.

Two cases with an incidental finding of a widened intervertebral foramen due to extensive tortuosity of the vertebral artery are presented. Computed tomography angiography and magnetic resonance imaging/magnetic resonance angiography (MRA) both proved to be useful as non-invasive techniques to provide the diagnosis. In-plane saturation is a disadvantage seen with the two-dimensional time-of-flight MRA technique.

Traumatic dissection of the internal carotid artery as unusual cause of hypoglossal nerve dysfunction.

A dissection of the internal carotid artery (ICA) in a 50-year-old man with hypoglossal nerve dysfunction is reported. This case illustrates how non-invasive imaging techniques, such as computed tomography and magnetic resonance angiography, can demonstrate this unusual etiology.

99Tc(m) labelled HL91 versus computed tomography and biopsy for the visualization of tumour recurrence of squamous head and neck carcinoma.

This phase I pilot study reports on (1) the safety and feasibility of 99Tc(m)-HL91, an amine oxime core radioligand that has shown oxygen dependent binding, and imaging; and (2) its usefulness for the visualization of local tumour recurrence of a biopsy proven squamous cell carcinoma of the head and neck (SCCHN) as compared to spiral computed tomogaphy (CT) and biopsy. Nine men (mean age 33 years, range 34-74 years) were prospectively included. For safety measurements, vital signs were recorded and serum chemical analysis carried out, with a complete blood cell count and urine analysis, and an ECG was performed prior to injection of 99Tc(m)-HL91 and repeated during the investigation. Single photon emission computed tomography (SPECT) scans of the head and neck, and of a standard, were performed at 2 h and 4 h post-injection of 740 MBq 99Tc(m)-HL91. Tumour-to-normal tissue background (T/N) ratios and percentage uptake were measured for all 99Tc(m)-HL91 scans. Spiral CT scans were obtained using a Somaton 4+ Siemens scanner within 1 week from the 99Tc(m)-HL91 scans. Based on CT and the 99Tc(m)-HL91 scan findings guided biopsies were performed. No adverse or subjective side effects were noticed. Vital signs, ECG findings, clinical laboratory, blood and urine assays remained stable in all patients. Spiral CT suggested local recurrence in 5/9 patients accompanied by nodal involvement in three, all of which proved positive on biopsy. 99Tc(m)-HL91 scintigraphy was false positive in one patient and true positive (TP) in 3/5 local recurrences and two out of three sites of lymph node involvement depicted by spiral CT. The mean T/N ratios at 2 h and 4 h in TPs were 1.28 (range 1.1-1.66) and 1.40 (range 1.0-1.6), respectively. The corresponding absolute percentages of 99Tc(m)-HL91 lesional uptake at 2 h and 4 h were mu = 0.05% (SD = 0.03%) and mu = 0.048% (SD = 0.035%). The findings suggest 99Tc(m)-HL91 is a safe radioligand and that metabolic binding in a large fraction but not all of local SCCHN recurrences may be expected. The inference that tumour 99Tc(m)-HL91 avidity could be a non-invasive measure of tumour hypoxia deserves however independent confirmation with needle oximetry.

Hearing loss as a presenting symptom of cleidocranial dysplasia.

OBJECTIVES: To report two cases of cleidocranial dysplasia in which hearing loss was the first presenting symptom. STUDY DESIGN: Retrospective case review. PATIENTS: Two cases of cleidocranial dysplasia, a rare autosomal dominant skeletal dysplasia affecting both membranous and enchondral bone formation. SETTING: Tertiary referral center. INTERVENTIONS: Clinical, audiometric, and imaging diagnostic procedures. CONCLUSION: With this report, we want to illustrate the possibility of a rare genetic disorder as the underlying cause of hearing loss. We also want to emphasize the need for a multidisciplinary approach and evaluation of unexplained hearing loss to obtain a correct diagnosis, which is important for genetic counseling and management of the patient and his or her family.

Otological manifestations of CHARGE association.

The combined findings of abnormalities of the incus and stapes, absence of the oval window, absence of the stapedius muscle (with absent pyramidal eminence and tympanic sinus in most cases), and abnormalities of different parts of the inner ear (dysplastic cochlea, hypoplastic or dysplastic vestibule, and absent semicircular canals) are characteristic of the CHARGE association. Computed tomography and magnetic resonance imaging using 1-mm-thick contiguous slices proved useful in demonstrating the broad spectrum of temporal bone abnormalities in patients with the CHARGE association.

55Co-PET in stroke: relation to bloodflow, oxygen metabolism and gadolinium-MRI.

BACKGROUND: Several studies have shown the feasibility of Co-isotopes (55Co and 57Co) in imaging of neuronal damage in stroke, multiple sclerosis, cerebral tumors and trauma. These studies indicate that Co-isotopes allow visualization of brain pathology related to inflammatory processes, reactive gliosis and cell death. Until now, it is not clear if 55Co accumulation occurs in the core of infarction or in the penumbra. Therefore, in the present study, we compared 55Co-PET with functional parameters such as cerebral bloodflow (rCBF) using C15O2, oxygen metabolism (rCMRO2) using 15O2 and cerebral bloodvolume (CBV) using C15O in PET and with the anatomical parameter Gd-MRI. PATIENTS AND METHODS: Seventeen patients (11 male; 6 female) age 43 to 84 (mean 69) with middle cerebral artery (mca) stroke, as proven by CT or MRI, were examined with 55Co-PET (0.5-1.0 mCi 55CoCl2), C15O2-, 15O2- and C15O-PET in one session 0-30 days after stroke-onset. Regions of infarction were defined by rCMRO2 being smaller than 65% or rCBF below 45% of the contralateral value and were subsequently superimposed on the cobalt scan. To compare the Cobalt uptake with the Gd-MRI, a realignment program was used that matches the MRI with the bloodflow images. Clinical status was established using the Orgogozo stroke scale at admission and at discharge (at least 6 weeks after admission) and the Barthel index. RESULTS: Eight patients showed a positive Co-PET scan and were used for further analysis. It appeared that Co accumulates in areas with a diminished oxygen metabolism and with a preserved bloodflow. We found Co-uptake in only a part of the Gd enhanced brain tissue with a tendency to be located peripherally or outside the Gd demarcated brain tissue. CONCLUSION: The results of the present study suggest that Co accumulates into infarcted brain tissue with a rather preserved flow independently of blood-brain barrier breakdown.