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The differential diagnosis of lesions involving the sella turcica.

The sella turcica and the surrounding area contain several different tissues varying in morphology and cytogenesis. Thus, it is not surprising that a large number of diverse lesions may arise in the sellar region. The most frequent abnormalities are the pituitary adenomas, which based on histology, immunocytochemistry, and transmission electron microscopy can be classified into several distinct entities. Pituitary adenomas originate in and consist of adenohypophyseal cells. They are usually slowly growing benign epithelial tumors, which may be associated with increased hormone secretion or may be endocrinologically nonfunctioning. Pituitary carcinomas also arise in adenohypophyseal cells. They are rare and can be diagnosed only when cerebrospinal and/or systemic metastases are documented. To illustrate the diversity, practical importance, and diagnostic difficulties, four cases were selected for presentation: lymphocytic hypophysitis, thyrotroph hyperplasia, growth hormone-producing pituitary adenoma with neuronal transformation, and composite tumor consisting of adenomatous periodic acid Schiff-positive as well as adrenocorticotropic hormone-immunoreactive adenohypophyseal cells and adrenocortical cells. The first two cases are important from a practical point of view because the proper diagnosis can easily be missed, and appropriate interpretation of the findings is essential to prognosis and treatment. The latter two cases are odd, unusual entities; their histogenesis is unresolved. Study of these and many other cases convinced us that careful and detailed morphologic investigation of lesions involving the sella turcica is of fundamental significance. Histology, immunocytochemistry, transmission electron microscopy, and, in some cases, molecular methods are essential to reach a correct diagnosis and to draw conclusions on histogenesis, growth potential, biologic behavior, prognosis, and therapeutic responsiveness.

Adenoma↗

[Pathological analysis of 1458 cases of tumor in the sella turcica region].

The sella turcica region is an anatomical site preferential for various types of intracranial tumors. Pathological analysis of 1458 cases of tumor in this region is presented. They comprised 20.16% of the intracranial tumors. Among them, 846(59.1%) were pituitary adenomas, 433(29.7%) craniopharyngiomas, 91(6.2%) meningiomas and 42(2.9%) germinomas. In pituitary adenomas, chromophobe adenoma was predominant, mixed cell adenoma next and no basophilic adenoma was observed. Of craniopharyngiomas they were divided into cystic, partly solid and cystic, and calcified types grossly and histologically into adamantinomatous, squamous and calcified types. In this kind of tumor, well differentiated cells were consistent to the clinical benign course. For meningiomas, histologically meningotheliomatous type was most common in this region. In previous reports, germinomas in the sella turcica region were so-called ectopic pinealoma. Now it is well known that this tumor arises from germ cells, hence, the synonym "ectopic pinealoma" is obsolete. The germinomas in the sella turcica region accounted for 44.68% of all intracranial germinomas. Microscopic picture of this tumor was similar to those of seminoma of the testicle and dysgerminoma of the ovary. Although gliomas, teratomas, giant cell tumors and chondromas were uncommon in the sella turcica region, the authors believe that they should be considered in the differential diagnosis of tumors in the sella turcica region.

Adenoma↗

The association between prenatal sella turcica morphology and notochordal remnants in the dorsum sellae.

The purpose of this study was to describe the location and morphology of notochordal remnants in the cranial base in normal and pathological conditions and to relate these findings to the morphological appearance of the sella turcica. Serially cut sagittal sections of paraffin-embedded sella turcica tissue blocks from 88 normal and pathological fetuses, 13 to 24 weeks of gestation, were examined. Twenty-seven specimens out of 88 had visible notochordal remnants in the cranial base, and these constituted the material available for this study. A straight notochordal course is always seen in normal sella turcica morphology, and a non-straight notochordal course is always seen in malformed sella turcica. Among the fetuses diagnosed at autopsy as "normal fetuses," both normal and pathological findings in the sella turcica regions were observed. The pathological findings were always found in the spontaneously aborted fetuses (five cases). Among the fetuses diagnosed at autopsy as "pathological fetuses," both normal and pathological findings were also observed in the sella region. Our conclusion is that the morphological appearance of the notochordal remnants in the dorsum sellae is associated with the morphology of the sella turcica. These structures ought to be analyzed on larger materials of both normal and pathological fetuses. One of the more obvious perspectives opened up by the present study is the probable disclosure of malformations in spontaneously aborted fetuses without external malformations.

Female↗

[A successfully treated case of cerebrospinal fluid fistula caused by fracture of the sella turcica].

Fracture of the sella turcica is rare and is associated with many complications. We successfully treated a cerebrospinal fluid fistula caused by a fracture of the sella turcica. A 66-year-old male in a motor vehicle accident was admitted to an outside hospital with disturbance of consciousness. A computed tomography (CT) scan of the head revealed a subarachnoid hemorrhage and pneumocephalus. Cerebrospinal fluid rhinorrhea developed after admission. Repair of the fistula was attempted without success, and the patient was transferred to our hospital for further examination and treatment. A fracture of the sella turcica was clearly visualized on coronal and sagittal head CT and on a three-dimensional reconstructed CT (3D-CT) image. The source of the CSF fistula was thought to be the anterior wall of the sella turcica. Through a bifrontal interhemispheric approach, the cerebrospinal fluid fistula was repaired microscopically with the assistance of endoscopy. Postoperatively, the fistula stopped completely. Coronal and sagittal head CT and 3D-CT images are useful for making a diagnosis of CSF leakage. Endoscopic images can assist in observation of the dead angle of the microscope.

Aged↗

[Hypophysis volume in computerized tomography and clinical grounds for diagnosing a primary completely empty sella turcica].

Computerized tomography (CT) of the sella turcica was performed in 106 women with hypothalamic-hypophyseal-ovarian hypofunction, aged from 15 to 50 years. In each patient we evaluated tomographically hypophyseal volume and subarachnoid space cistern invaginated into the sella turcica. We analysed statistically the incidence of amenorrhea, infertility, obesity and arterial hypertension in five groups of patients classified according to hypophyseal volume i.e. from below 100 to over 400 mm3. Is was found that hypophyseal volume of women in the so-called empty sella turcica that could correlate significantly with obesity and arterial hypertension was up to about 200 mm3, and in case of amenorrhea and infertility below 100 mm3. In case of empty sella turcica in women with amenorrhea mean hypophyseal volume was significantly lower (about 194 mm3) than in menstruating women (about 248 mm3). Invagination of cisterns of the cerebral basis into the sella turcica equal to or exceeding three time hypophyseal volume in about 82% was associated with its small size i.e. below 150 mm3. Basing on our analysis it seem justified to assume that hypophyseal volume up to 150 mm3 is a CT criterion of diagnosing completely empty sella turcica.

Adolescent↗

A sella turcica bridge in subjects with dental anomalies.

Calcification of the interclinoid ligament (ICL) of the sella turcica, or sella turcica bridging, has been associated with severe craniofacial deviations. Despite no comprehensive study on the sella turcica bridge, a relationship with tooth and eruption disturbances has been reported. In order to investigate whether congenital absence of the second mandibular premolar, or the presence of a palatally displaced canine (PDC), is associated with sella bridging, a retrospective study was performed. Lateral cephalometric radiographs from 20 males and 14 females, aged between 8 and 16 years, with a PDC and second mandibular premolar aplasia were reviewed and compared with a control group. A standardized scoring scale was established to quantify the extent of a sella turcica bridge from each radiograph (no calcification, partially calcified, and completely calcified). The prevalence of complete calcification of the ICL in adolescents with dental anomalies was equal to 17.6 per cent, while an incidence 9.9 per cent was found in the control group. A partially calcified sella turcica was observed in 58.8 per cent of adolescents with dental anomalies compared with 33.7 per cent in the control group. The association between the degree of calcification of the ICL and the presence of dental anomalies in the studied adolescents was statistically significant according to chi-square statistics (P = 0.004). This was confirmed by Fisher's exact test (P = 0.003). According to these findings, the prevalence of a sella turcica bridge in adolescents with dental anomalies is increased, while age and gender do not greatly influence ossification of the ICL. The very early appearance during development of a sella turcica bridge should alert clinicians to possible tooth anomalies in life later.

Adolescent↗

Pituitary function in patients with enlarged sella turcica and primary empty sella syndrome.

The pituitary function in 20 patients with primary empty sella syndrome has been evaluated. The only endocrine symptom was secondary amenorrhoea in four patients. In half of the patients, pituitary function tests showed minor abnormalities, the most common being impaired growth hormone response to glucagon. In addition to the empty sella, other intracranial abnormalities such as pituitary tumour and hydrocephalus were found in four patients. Although commonly a benign condition, the empty sella may occasionally be associated with clinically important endocrine dysfunction, pituitary tumours and other intracranial abnormalities.

Adult↗

The association of Cushing's disease and primary empty sella turcica.

The empty sella turcica is defined as the extension of the subarachnoid space toward the intrasellar region with displacement of the pituitary towards the posteroinferior wall. By autopsy studies, the incidence in the general population is around 20%. The association of Cushing's disease (CD) and empty sella has been infrequently reported. In our group, from a total of 68 patients with CD studied by magnetic resonance imaging (MRI), we found the presence of a primary empty sella syndrome (ESS) in 11 (16%). Of these, 9 had partial and 2 total ESS, and in four of them a microadenoma could be identified. Remission, ascertained by subnormal postoperative cortisol levels in blood and/or urine was obtained in 5 of 6 patients operated on by the transphenoidal route. Following surgery, 2 patients presented cerebrospinal fluid (CSF) leakage, 2 diabetes insipidus, and 2 some form of hypopituitarism, figures apparently higher than in non-ESS patients with CD. Ketoconazole was indicated as second line treatment in 2 patients and as primary therapy in 4, resulting in normal urinary free cortisol (UFC) values, with no complications. The relationship of ESS and CD is probably fortuitous given that the frequency of ESS in the general population is similar. Although in empty sella both surgery and radiotherapy seem to have greater risk of complications, surgery remains the first line treatment. Nevertheless, chronic treatment with ketoconazole could be a useful first choice, particularly when no adenoma is seen, or in those who have contraindications for surgery.

Adult↗

Bridging and dimensions of sella turcica in subjects treated by surgical-orthodontic means or orthodontics only.

The aim of the study was to compare the incidence of sella turcica bridging and sella turcica dimensions in 150 Caucasian subjects who had combined surgical-orthodontic correction of their malocclusion with a randomly selected group of 150 Caucasian subjects who were treated contemporaneously by orthodontic means only. Pretreatment lateral cephalometric radiographs were scanned and analyzed. A sella turcica bridge was identified as a continuous band of bony tissue extending from the anterior cranial fossa to the posterior cranial fossa. The dimensions of the sella turcica were measured. In the group treated by combined surgical-orthodontic means, the incidence of bridging was 16.7%, whereas it was 7.3% in the orthodontics-only group (P = .012). Significant increases in the mean surface area (P = .02) and mean perimeter of the sella turcica (P = .01) were found for the combined surgical-orthodontic group compared with the orthodontics-only group. The mean interclinoid distance was significantly smaller in the surgical-orthodontic group (P = .02). These findings appear to indicate the greater likelihood of sella turcica bridging and abnormal sella turcica dimensions in subjects treated by combined surgical-orthodontic means rather than by orthodontics only.

Cephalometry↗

A sella turcica bridge in subjects with severe craniofacial deviations.

In earlier studies, a sella turcica bridge was stated to occur in 1.75 to 6 per cent of the population. The occurrence of a sella turcica bridge has not previously been studied in a group of patients with craniofacial deviations treated by surgery. Profile radiographs from 177 individuals who had undergone combined orthodontic and surgical treatment at the Copenhagen School of Dentistry were studied. A sella turcica bridge was registered in those subjects where the radiograph revealed a continuous band of bony tissue from the anterior cranial fossa to the posterior cranial fossa across the sella turcica. Two types of sella turcica bridge were identified. A sella turcica bridge occurred in 18.6 per cent of the subjects.

Calcinosis↗

Fracture of the sella turcica.

Fracture involving the sella turcica is a rare complication of head trauma, but extremely important due to its strategic location adjacent to vital vascular and nervous structures. Of 14 cases reviewed, nine had at least one cranial nerve paralysis, with endocrine abnormalities and cerebrospinal fluid otorrhea seen to a lesser degree. The radiographic findings of sphenoid sinus fluid (13 of 14 cases), pneumocephalus, and unusual vascular occlusions are discussed.

Adolescent↗

[Endocrine disorders in "empty" sella turcica].

The results of endocrine examination of 37 patients with an "empty" sella turcica (22 with primary and 15 with secondary) are discussed. The diagnosis in all cases was verified by the findings of pneumocisternotomography and computer-aided tomography. The trophic function of the hypophysis was mainly studied. It was found that primary "empty" sella turcica mostly occurs in females with a history of many pregnancies. Obesity and disorders of menstrual function were the principal clinical manifestations of endocrine disorders. The inconstantly encountered moderate hyperprolactinemia disappeared after parlodel medication. In secondary "empty" sella turcica hyperprolactinemia was of a more stable character. The trophic function of the hypophysis was reduced in primary "empty" sella turcica as a result of which lesser doses of substitutive hormonal preparations were needed than in secondary "empty" sella turcica. The endocrine disorders in primary "empty" sella turcica were probably of hypothalamic origin, those in a secondary condition were evidently associated with a tumor of the adenohypophysis.

Acromegaly↗

Post-natal size and morphology of the sella turcica in Williams syndrome.

Williams syndrome (WS) is a rare congenital disorder with distinctive craniofacial features, cardiovascular abnormalities, mental retardation, and behaviour characteristics. The purpose of this study was to investigate the size and morphology of the sella turcica on profile cephalograms in a group of individuals with WS. The material consisted of radiographic cephalograms of 62 Norwegian children, adolescents, and adults with an age range of 4.7-44.4 years. The length, depth, and diameter of the sella turcica were measured and the mean values were compared with normal reference material from the Oslo University Craniofacial Growth Archive. In total, the two-dimensional size of the sella turcica in the WS group was smaller in length, depth, and diameter compared with the control group, but only occasionally reached a significance level of 5 per cent (Student's t-test). The morphology of the sella turcica was assessed and five different morphological types were identified; oblique anterior wall, extremely low sella turcica, sella turcica bridging, irregularity (notching) in the posterior part of the dorsum sellae, and pyramidal shape of the dorsum sellae. The occurrence of these morphological types was more frequent in the WS subjects compared with the reference material, except for sella turcica bridging, which was equally frequent. The females with WS had more dysmorphic sella turcicas than males. This study has demonstrated morphological aberrations in the sella turcica in Norwegian individuals with WS, which should be further elucidated in future research and combined with neurological andendocrinological investigations.

Adolescent↗

Diplopia and diabetes insipidus secondary to type II fracture of the sella turcica: case report.

Fractures of the sella turcica are rare and are often difficult to diagnose radiologically. They can produce a wide variety of complications, including septic, neuroendocrine, neurovascular and neuroophthalmologic problems. The authors describe the case of a 17-year-old boy with diabetes insipidus and diplopia secondary to a type II fracture of the sella turcica. They emphasize the clinical importance of fractures in the sellar region in view of their proximity to vital structures and they discuss possible mechanisms of causation.

Adolescent↗