Neuroendocrine adenoma of the middle ear with extension into the external auditory canal.

We report a case of middle ear adenoma (neuroendocrine adenoma of the middle ear) protruding into the external ear canal. The patient was a 65-year-old man with hearing alterations and a headache in whom an otoscopy disclosed a sessile, pea-sized, brown-reddish, focally bleeding mass located in the posterior-superior aspect of the right external auditory canal. Histopathologically, there was a neoplasm composed of closely packed, sometimes back-to-back glandular structures formed by small uniform cuboidal or cylindrical cells. Small solid islands were also present. Following the histopathologic examination, a high resolution computed tomography was performed showing an extensive osteolytic defect mostly involving the mastoid air cells of the mastoid process with a partial destruction of the middle ear cavity. This defect was filled with a mass-like lesion with the density of soft tissue which bulged to the external auditory canal. Histopathologic examination of the mass in the middle ear cavity revealed findings identical to those seen in the original biopsy, confirming diagnosis of middle ear adenoma extending into the external ear canal.

[Portal vein branch embolization in patients with primary inoperable liver tumors]. / Problematika embolizace vetve portální zíly u nemocných s primárne neoperabilními nádory jater.

INTRODUCTION: Portal vein embolization (PVE) is indicated in patients with insufficient liver remnants following liver resections for tumor disorders. Therefore, due to PVE, the number of primary operable patients is higher. Insufficient growth of the liver parenchyma or malignant progression remain the PVE cons. AIM OF THE STUDY: To date outcomes of PVE are assessed based on the authors' own experience and literature data. In particular, the authors focus on difficulties with PVE, i.e. its failures. METHODS: 40 patients (35 with colorectal carcinoma metastases, 2 with breast carcinoma metastases and one with ovarian carcinoma metastases, 2 with hepatocellular carcinoma) were indicated for PVE due to insufficient liver reserve following planned liver resection. RESULTS: Liver resections were completed in 22 subjects, 42.6 days (mean value) after PVE. In 14 (35%) subjects, the liver resection could not be performed (11x tumor progression, 3x insufficient liver tissue growth). In four subjects, only radiofrequency ablation was performed. At year one, two and three after the procedure, the survival rate is 83.7, 69.7 and 52.3% (resp.) of the subjects, while the survival rate following exploration and in unoperated subjects was 22.2% (25 subjects) (p < 0.001). A one-year, resp. two-year relapse--free survival rate was 30.3, resp. 7%. CONCLUSION: PVE has become an established procedure in stage liver procedures, due to its potential to facilitate operability of primary and secondary liver tumors. In order to improve the outcomes, attention must be paid to the post- PVE growth of the liver parenchyma and further assessment of oncological treatment approaches during the pre- and post- PVE period, with the aim to reduce liver and extra-liver malignant progression rates prior to the liver resection procedure.

[Laparoscopic adrenalectomy]. / Laparoskopická adrenalektomie.

OBJECTIVE: Laparoscopy has become the gold standard for the treatment of adrenal tumours in urology. We evaluate our experience with laparoscopic adrenalectomy (LA) in this work. MATERIAL, METHODS: We performed 38 LA between 2003-2008. We use computer tomography (CT) and magnetic resonance imaging (MRI) for the initial evaluation. Indication for procedure is made in cooperation with endocrinologist. We use transperitoneal approach with 3 or 5 ports. RESULTS: Mean age was 57.7 +/- 11.7 year (range 32-74.9 year). Nine LA were made in men (24%), in women 29 (76%). Sixteen tumours (42%) were hormonal active (7 pheochromocytoma, 6 primary hyperaldosteronism, 3 peripheral hypercortisolism). Twenty-two tumours were without hormonal activity. Mean tumour size was 4.1 +/- 2 cm (range 1-10.1 cm), mean operation time was 89 +/- 38 minutes (range 32-220 minutes), mean blood loss was 33 +/- 75 ml (range 0-400 ml), mean hospitalization time was 6.1 days (range 3-12 days). There were histologically 15 cortical adenomas, 5 nodular cortical hyperplasia, 1 calcificated hematoma, 3 cysts, 2 potentional malignant tumours on interface between adenoma and carcinoma, 1 cortical carcinoma and 7 pheochromocytoma. We found 3 metastases of renal carcinoma in adrenal gland and one metastasis mesenchymal chondrosarcoma too. Transperitoneal approach was chosen in 20 patients (53%) after previous abdominal operation (open cholecystectomy, appendectomy, transperitoneal nephrectomy, aortofemoral bypass). Complications were in 3 cases from 38 (8%). It was one perforation of diaphragm, which was resolve with laparoscopic suture, one postoperative delirium with fudge and agitation, one abscess in wound after extraction of specimen. We have got any conversion in our collection. The body mass index was higher than 38 in 3 patients. CONCLUSION: LA is a quick and safe procedure with minimal morbidity and mortality. This procedure requires very experienced surgeon. Patients profit especially from miniinvasivity and short convalescence. Especially benign tumours of smaller size (by 8 cm) are indicated, extensive and especially malignant tumours remain a domain of open approach. Previous operations in abdominal cavity do not have to be a contraindication for LA and operation is possible in patients with monster obesity.

Diagnostic problems of abdominal desmoid tumors in various locations.

BACKGROUND: Abdominal desmoid tumor is one of the forms of deep (musculoaponeurotic) fibromatosis. It occurs more often as a desmoid tumor in the abdominal wall, less often in various intra-abdominal locations. In this work, we performed retrospective study concerning diagnostic problems of this disease with the use of imaging techniques. METHOD: Four patients (three females and one male) were examined by postcontrast CT (computed tomography) and one of them also by MR (magnetic resonance). All findings were correlated with the operational findings and histologic examination. RESULTS: The findings were typical only in the case with the lesion located in the abdominal wall, three described cases of the intra-abdominal desmoid provided a broad range of differentially diagnostic possibilities (metastases, GIT tumors, lymphomas, etc.). In particular, the findings in infiltrative processes in intra-abdominal location and retroperitoneal involvement were less typical. Ultimately, the histological findings were decisive. CONCLUSION: The possibility of the occurrence of intra-abdominal desmoid tumor must be considered particularly in younger individuals with rapidly growing tumorous process, which does not immediately arise from the surrounding organs (digestive tract, internal genitalia, etc.), and is located in the abdominal wall or in the abdominal cavity.

[Massive breast angiomatosis with dramatic course leading to acute total mastectomy--a case report]. / Masivní angiomatóza prsu s dramatickým prubehem vedoucím k akutní totální mastektomii--kazuistika.

OBJECTIVE: On the background of clinical, radiological and morphologic settings presented is unusual case of the rare angiomatosis of the female breast. Discussed are both clinicopathological aspects and differential diagnosis from the morphological point of view. DESIGN: Case report. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: As a case report described is a rare case of the angiomatosis of the female breast. Implemented illustrations consist of local findings, CT scan, specimen gross appearance and histological pictures including immunohistochemistry. Applied antibodies and detection system LSAB+ were provided by Dako, visualization was performed by chromogen DAB (diaminobenzidin tetrahydrochlorid). RESULTS: 30-year-old woman suffering of painful, enormous breast asymmetry underwent core needle biopsy of the right breast. The mastogram showed diffuse process of ambiguous significance. During the next few days her condition dramatically changed and under the circumstances of florid apoplexy and suspicion of tumor of her right breast, total mastectomy was carried out. Histological picture revealed widespread, unlimited vascular lesion dissecting throughout the stroma of the entire gland. Bland morphology and the nature of tissue involvement eventually led to diagnosis of benign angiomatosis. The woman underwent several local plastic operations but is out of final reconstruction of her breast so far. Thirteen months after mastectomy there are no signs of recurrence.

[Giant bilateral adrenal myelolipoma associated with congenital adrenal hyperplasia]. / Ein bilaterales Riesenmyelolipom der Nebennieren in Kombination mit kongenitaler adrenaler Hyperplasie.

BACKGROUND: Myelolipoma is a rare benign tumor formed by mature fat tissue with strata of haematopoiesis. It is mainly asymptomatic, only large myelolipomas manifest themselves by abdominal discomfort, bleeding or by symptoms of oppressing adjacent organs. It is usually localized in the region of the adrenal gland. Myelolipomas are mostly clinically inert, only a small number of them are associated with Cushing's type of endocrine disorders, Conn's syndrome, Addison's disease, etc. PATIENT AND METHODS: The authors present a rare case of a giant bilateral myelolipoma emerging out of the adrenal gland cortex in a congenital adrenal hyperplasia, with steroid 21-hydroxylase deficiency, in a woman with pronounced virilism. The principal diagnostic methods include sonography and computer tomography. The therapy available is either a surgical extirpation in the case of large or growing myelolipomas or observation in the case of small ones (< 5 cm). RESULTS: The condition was resolved by bilateral adrenalectomy with extirpation of both myelolipomas and subsequent hormonal substitution treatment during which there was a gradual regression of virilising symptoms. CONCLUSIONS: The coincidence of myelolipoma and congenital disorder with subsequent overproduction of the adrenocorticotropin hormone and androgens, might be explained by the incipient of myelolipoma through chronic hormonal stimulation of the adrenal gland cortex. However, the etiopathogenesis of myelolipoma is still unclear.

[Injuries to the biliary tract during cholecystectomy]. / Poranení zlucových cest pri cholecystektomii.

Injuries to the biliary tract during both the laparoscopic or the open cholecystectomic procedures, remain among the most serious iatrogenic injuries with high morbidity and mortality rates. The higher the number of the laparoscopic cholecystectomies, the higher the number of the injuries to the biliary tract. Early peroperative recognition of these injuries is a prerequisite for successful biliary tract reconstructions. Mucosal hepaticojejunoanastomosis according to Roux is the golden standard of the reconstrucion treatment. Stenoses in anastomoses followed by development of cholangitides are considered serious postoperative complications. In these cases, endoscopic and transparietal dilation with plastic stents implantation is the method of choice. Technically exacting reoperations then follow. The authors present a trial group of 11 patients, who were treated in the Surgical Clinic of the Faculty Hospital in Plzen for biliary tract injuries during cholecyctomic procedures (8 were laparoscopic and 3 open), from 01-01-2000 to 01-09-2004. Severe inflammatory changes in the region of the Calot triangle, were the commonest cause of the biliary tract injuries during primary operations. In most cases (N = 8) the injury was diagnosed and managed immediately during the primary procedure. Hepaticojejunoanastomosis according to Roux was the principal procedure used to repair the biliary tract (N = 8). Postoperative morbidity reached 36.4%, 2 elderly patients exited (18.2%) due to septic multiorgan failure on the 15th day and the 7th month after the surgical procedure. Multidisciplinary approach of a team of experienced surgeons, endoscopists and radiologists in the hepatobiliary region is a fundamental prerequisite for long-term successful outcomes of technically exacting reconstructive procedures of the hepatobiliry tract.

The contribution of MRI to the diagnosis of diffuse meningeal lesions.

We analysed MRI findings in patients in whom a diffuse abnormality of the meninges was revealed by MRI. We looked at T1 and T2-weighted spin-echo or fast spin-echo images and contrast-enhanced T1-weighted images. There were 15 patients with abnormalities on MRI, clinically suspected in ten. Four had meningoencephalitis, one meningeal and subcortical sarcoidosis nodules, four meningeal malignancies--one disseminated oligodendroglioma, one with meningeal infiltration around an adenocarcinoma, three meningeal infiltration by a haematological malignancy, and one a chronic subdural haematoma without a history of injury. We excluded patients with primary meningeal tumours and typical injury-related meningeal bleeding. The relatively small number of patients is due to both the infrequency of diffuse meningeal disease and to the low frequency of suspected meningeal pathology as an indication for MRI. The latter's diagnostic contribution is greatest in infectious disease and neoplastic infiltration, and less obvious in haematological malignancies. Contrast-enhanced T1-weighted images are most useful.

[Primary hyperparathyroidism (osteitis fibrosa cystica generalisata Recklinghausen)]. / Primární hyperparatyreóza (osteitis fibrosa cystica generalisata Recklinghausen).

The case of a 67-year-old man presenting with parathyroid gland adenoma associated with multiple organ manifestations of a rare normocalcemic form of primary hyperparathyroidism is described. The authors draw attention to the importance of correlating clinical and laboratory examinations with histological, radiological and scintigraphic findings for the establishment of diagnosis. They emphasize the role of two-phase 99mTC-MIBI (methoxyisobutyl-isonitril) scintigraphy not only for diagnostic purposes but also for exact localization of the adenoma by means of a gamma probe during surgery. They also point out the possibility of accompanying pathological conditions, in this case, the development of a tumor described as "brown tumor". This was shown by angiography as a hypervascularized and strongly stained expanding lesion. The authors conclude that the early surgical removal of this parathyroid gland adenoma was important for the patient's successful recovery.

[Periosteal desmoid]. / Periostální desmoid.

The authors describe a rare benign disease occurring in children and adolescents with an increased physical activity at the age of 8-20 years. It is a tumour-like lesion developed by a repeated micro-trauma of the origin of distal fibres of adductor magnus and aponeurotic origin of the medial head of the gastrocnemius. This disease must be paid increased attention as in the region of the knee joint in children it is a location of a frequent development of sarcoma and osteomyelitis. With the knowledge of this lesion such cases may be diagnosed merely by a classic radiograph, preferably in external rotation and CAT and MRI examinations. Of the highest value for the examination is the CAT examination. Radiograph changes are located in the region of the distal part of the medial crest of linea aspera, tuberculum adductorium and posteromedial part of the medial epicondyle of femur.

Gastric lipoma presenting as upper gastrointestinal obstruction.

A 61-year-old man presented with an upper gastrointestinal obstruction caused by a submucosal gastric lipoma in the prepyloric area. The diagnosis was made coincidentally during his admission for another disease. Gastric resection was performed because of a large lipoma combined with florid gastric ulcers. The frequency of gastric lipoma, its differential diagnosis, means of diagnosis, and treatment are discussed.

[Rheumatoid arthritis--a diffuse connective tissue disease]. / Revmatoidní artritida--difuzní choroba pojiva.

The authors describe in a group of 100 patients, mostly with a severe form of rheumatoid arthritis, subjects with so-called "severe" extraarticular affections which can threaten the patients' lives or cause substantial deterioration of its quality, and those subjects where rheumatoid arthritis was the cause of death. These affections were associated--directly or indirectly--with the basic disease. From the absolute number of revealed affections the authors do not want to draw any conclusions as they are aware of various factors which could influence them. They assume, however, that the assembled data provide evidence that the American Rheumatological Association was right to include rheumatoid arthritis among diffuse connective tissue diseases and that it must be considered not only a disease of the locomotor apparatus but a potential disease of the organism as a whole.

[Hereditary pancreatitis]. / Hereditární pankreatitida.

The authors describe a family with hereditary pancreatitis where in three generations in five subjects in the same line complete penetrance of the disease was found. It is the first report on this disease in Czech republic.

[Incidence of aneurysms of the abdominal aorta in the Czechoslovak population and possibilities of its detection]. / Výskyt aneurysmatu abdominální aorty v nasí populaci a moznosti jeho depistáze.

A significant increase not only in the number of operations for AAA but also in the incidence of autopsy findings of AAA from 1.5 per cent to 4.5 per cent has been observed during the last 20 years. Ultrasound screening of the abdominal aorta in patients examined for other reasons revealed asymptomatic dilatation of the infrarenal aorta to more than 4 cm in 3.8 per cent of men older than 60 years. AAA should be actively searched for at every physical and ultrasound examination of the abdomen in men over 60 years of age.

[Nonspecific spondylodiscitis in childhood. New findings about the disease]. / Nespecifická spondylodiscitis v detském veku. Nekteré nové poznatky o onemocnení.

Nonspecific spondylodiscitis at the child age represents a distinctive disease of intervertebral disc and adjacent subchondral parts of vertebral bodies. Their early diagnostics may be difficult due to latency of radiographic manifestation of the finding and possible mistaking for other pathological conditions, especially tuberculosis spondylitis. The paper demonstrates several cases of the disease which we encountered recently. The authors discuss clinical and X-ray symptoms of the disease with a particular attention to modern methods of visualization, especially computer tomography. Etiopathogenesis of the disease is analyzed and some new knowledge of the disease is presented, differential diagnosis is discussed. In the conclusion attention is paid to early diagnosis of the disease and introduction of proper therapy.

[Bone changes in tuberous sclerosis (Bourneville-Pringle disease)]. / Pozorování kostních zmen pri tuberózní skleróze (m. Bourneville-Pringle).

The tuberous sclerosis is a rare hereditary disease which mostly becomes manifest in a triad of symptoms: epilepsy, mental defects and skin changes of the adenoma sebaceum Pringle type. There may be an atypical clinical picture without neurological or psychiatric signs, in 50% of cases also without skin changes. Bone changes in tuberous sclerosis are asymptomatic and present in up to 50% of patients. Their finding in patients without basic known symptoms may be the cause of mistaking it for osteoplastic metastases. A case like that was described in the case history part of the communication. The knowledge of the possibilities of these changes in tuberous sclerosis may considerably ease differential diagnosis by suitable indication of computer tomography of the brain with the finding of typical calcifications.