Current two EGFR mutations in lung adenocarcinoma -  case report.

Nowadays, EGFR TKIs (epidermal growth factor receptor-tyrosine kinase inhibitors) targeted therapy is well established treatment for patients with the so-called EGFR common mutations with advanced or metastatic nonsmall cell lung cancer. The efficacy for the so-called rare and especially for the very rare complex EGFR mutations is not clear. We describe a case of a 63- year-old female with metastatic nonsmall cell lung cancer with complex EGFR mutation (G719X + S768I) who had been treated by gefitinib. She achieved progression free survival within eight months. Then, we discuss our case with other literature case reports. Together, it seems that described complex EGFR mutation has a relatively good sensitivity for EGFR TKIs treatment.Key words: nonsmall cell lung cancer -  EGFR gene -  EGFR protein -  complex mutations -  rare EGFR mutations -  EGFR TKIs.

Typical signs of oncocytic papillary renal cell carcinoma in everyday clinical praxis.

PURPOSE: Types 1 and 2 by Delahunt of papillary renal cell carcinoma (PRCC) are traditionally differentiated. An oncocytic variant of PRCC (O-PRCC) has recently been described. We compare clinical data of O-PRCC with other subtypes of PRCC such as the main tumour size, necrotic changes, presence of pseudocapsula and real extrarenal growth in retrospective study. METHODS: From 1/1992 to 10/2009, 1,398 patients with 1,436 renal tumours were surgically treated in our institution. PRCCs were described in 109 (7.6%). Among PRCC, O-PRCCs were in 12 (11%), PRCC type 1 in 86 (78.9%), PRCC type 2 in 8 (7.3%) and others in 3 (2.8%) cases. RESULTS: The patient's mean age with O-PRCC (M:F ratio = 2:1) was 67.5 +/- 10.9 versus 63.5 +/- 14.1 in type 1 and 57.9 +/- 5.7 in type 2, the mean tumour size was 35 +/- 12 mm versus 47 +/- 22 and 37 +/- 17, respectively. The follow-up of O-PRCC is 35.3 +/- 12.3 months and all cases are without recurrence. We did not find any pseudocapsula in O-PRCC but it was a major sign of PRCC type 1 (32.6%). Huge microscopic necrotic changes were described in 33.3% of O-PRCC, in 33.7% of PRCC type 1 and 62.5% of PRCC type 2. Extrarenal growth was found only in 16.7% O-PRCC versus 40.7% in PRCC type I. CONCLUSIONS: None of the O-PRCC had pseudocapsula and none had massive necroses in comparison of O-PRCC with PRCC types I and II. Extrarenal growth in O-PRCC is relatively rare. The malignant potential of O-PRCC is low.

[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.

[Occult anal sphincter tear--up-to-date knowledge]. / Okultní ruptura análního sfinkteru behem porodu--soucasné znalosti.

OBJECTIVE: Summary of the current knowledge of sonographically and clinically detectable anal sphincter injury. DESIGN: Review. SETTING: Department of Gynaecology and Obstetrics, Charles University and University Hospital Pilsen. SUMMARY: Review of the current international literature covering the given problem. Occult anal sphincter tear is defined as an injury, which is clinically undetectable and recognizable just on endoanal ultrasonography. Total frequence of anal sphincter defects detected by sonography varies between 19-67%. At any rate majority of them likely seem to be injuries which are clinically detectable, however undiagnosed and missed. Isolated defect of internal anal sphincter is an example of real occult anal sphincter injury. This is found in around 2% of all vaginal deliveries. A diligent digital examination of perineum after the delivery and a proper education of perineal anatomy is the cornerstone in improving of the diagnostics. Endoanal ultrasound may serve as a tool in facilitation of the diagnosis.

[The liver splenosis in a patient following a procedure for the malignant seminoma]. / Splenóza jater u nemocného po operaci maligního seminomu.

Splenosis is characterized by a presence of islets of the splenic tissue in the abdominal and, rarely, also in the thoracic cavity, most frequently as a consequence of the splenic injury. The authors describe a case of splenosis in a patient followed-up after surgery and radiotherapy for the testicular seminoma and who, 42 years ago, had splenectomy for polytrauma. With respect to the location of the splenosis in the hepatic region, the differential diagnosis was difficult, bearing in mind a possibility of the malignancy relaps. Although splenosis was highly suspected, based on the results of the examinations conducted, with respect to the preexisting malignancy, the tumor was excised. The histopathologist confirmed the diagnosis of splenosis. This case is rare in the literature, considering the time gap between the diagnosis of splenosis and the trauma.

[A postoperative bronchopleural fistule--a success of the conservative treatment (a case review)]. / Pooperacní bronchopleurální pístel--uspech konzervativní lécby (kazuistika).

Disintegration of the bronchial stub following the lung resection procedures together with development of the bronchopleural fistule and the postoperative empyema of the thorax, remain a feared complication following all lung resections, but especially pneumonectomies. In this case review, the authors report on a successful conservative management case, which followed an unsuccessful surgical revision and an attempt for the endobronchial stent introduction. 20 months following the closure of the fistule, the patient shows no signs of a relapse of the disorder.

[Renal angiomyolipoma, histology, diagnostics and therapy]. / Renální angiomyolipom, histologie, diagnostika a terapie.

BACKGROUND: Renal angiomyolipoma is a benign mezenchymal tumour with prevalence of 0.3-3% of all surgically resected renal tumours. Tumour is often associated with tuberous sclerosis complex or with another fakomatosis. Tumour has typical pathological image in computer tomography notation that enables the diagnosis. Symptomatology and possible complications depend on the tumour size. Large or symptomatic tumours are indicated for resection, nephrectomy or local ablation. METHODS AND RESULTS: 612 patients with renal tumour were operated at the Department of Urology faculty hospital in Pilsen. Angiomyolipoma occurred in 7 patients. Average age at the time of operation was 64 years, ratio female and male was 5:2. Clinical symptomatology was expressed in 3 patients. The size of tumour was 2 to 8 cm, in 5 patient the tumors were solitary. Translumbal tumour resection was performed in 3 patients. These tumours were at the same time multifocal. Suspicion from tuberous sclerosis was pronounced in some care. The angiomyolipoma was accidentally found in 2 cases of nephrectomy, which was indicated for other diagnosis. CONCLUSIONS: We define diagnosis by means of CT notation in the most of patients. This notation is not typical by any version of angiomyolipoma and we choose therefore surgical revision.

[Papillary renal cell carcinoma surrounded by unusual fibrotic reaction resembling inflammatory pseudotumour--a case report]. / Papilární renální karcinom lemovaný neobvyklou fibrózní reakcí pripomínající zánetlivý pseudotumor--kazuistika.

Authors report clinicopathological features of an unusual case of composite renal lesion occuring in 32-year-old Caucasian male. The patient was followed for cystic lesion of retroperitoneal-renal region for 5 years. He was indicated for resection of the cystic lesion because of changes of the retroperitoneal mass on CT scan. A cyst was located on upper renal pole. A huge cystic mass filled mainly by necrotic material was resected and submitted for histological examination. The wall of the cyst was composed of fibrous tissue, indistinguishable from inflammatory pseudotumor on histological level. The vital intracystic tissue was formed by well-differentiated papillary renal cell carcinoma. The most important step within differential diagnosis is distinguishing of sarcomatoid differentiation in renal cell carcinoma. This very rare case demonstrates the importance of careful examination of all spindle cell lesions of the kidney.

[Echinococcus cysts (hydatids)]. / Echinokokové (hydatické) cysty.

INTRODUCTION AND MATERIAL: This work is based on rich experience of the first-stated author. During a three-year expertise in Botswana he operated 80 patients with hydatic cysts and he took part in treatment of other patients affected by the Echinococcus granulosus infection. In 59 cases the infectious focus was located in the liver, in 18 cases in the lungs, in 2 cases in the spleen and in one case the foci were multiple peritoneal. METHODOLOGY: During the expertise period, the patients were observed prospectively, throughout the diagnostic decision-making and the treatment. Prior to the surgical procedure, they had been, in a long-run, treated with albendazole. In 18 cases out of the total number of the liver cysts, the experts chose a two-step procedure followed by a secondary plombage of the reduced cavity of the cyst with the omentum. The rest of the cysts were removed. Miniinvasive methods were not applied. However, the authors discuss their risks. RESULTS: None of the patients exited, in 3 cases a peroperational anaphylactic reaction was managed. Throughout the expertise, no cases of the echinococcus dissemination in the operated subjects were recorded. The team even managed to treat the hydatic cysts in patients who were HIV/AIDS positive and who were, originally, in a serious state of immunodeficiency. Five cases are presented in the form of brief case reviews. CONCLUSIONS: Benefits of the long-term preparation with albendazole--plombage operations are beneficial to be conducted in two steps, Betadin is the most suitable lavage solution, miniinvasive surgical procedures (PAIR) may bring on specific complications.

[Splenic peliosis associated with adrenal adenoma]. / Pelióza sleziny v kombinaci s adenomem nadledviny.

PURPOSE: Peliosis of the spleen is very rare disease diagnosed mainly histologically. The ethiopathogenesis is still unknown and various factors can play the important role (steroids, chemotherapy, chronic infection, hematology disease etc.). Authors present the case of peliosis of the spleen which was combined with suprarenal adenoma. METHOD: The splenectomy for the large spleen with suspicion from lymphoma was done in hypertonic patient with normocytic normochromic anaemia. During operation the left suprarenal adenoma was revealed and epinephrectomy was performed. The diagnosis of peliosis of spleen was done by histological examination. The hypothesis of the connection between hormonal activity of the suprarenal adenoma and development of the peliosis was expressed. Unfortunately the suprarenal adenoma was not diagnosed before operation and so plasma hormone levels were not evaluated. RESULTS: The postoperative course of patient was without complications and patient was discharged 8th postoperative day. One year after operation the patient is in the good health condition. The hypertension is corrigated on the lower dosage of antihypertensive medicaments. CONCLUSIONS: It is probable, that the suprarenal adenoma could play the important role in the ethiopathogenesis of peliosis of the spleen in our case.

[Spindle cell and cuboidal renal cell carcinoma (loopoma). 10 case reports]. / Vretenobunecný a kuboidální renální karcinom (loopom). Popis 13 prípadu.

BACKGROUND: Current classification systems of neoplasms arising from renal parenchyma distinguish 5 categories of renal cell carcinoma (RCC), i.e. conventional RCC, papillary RCC, chromophobe RCC, collecting duct/medullary RCC and unclassified RCC. We present 13 cases of unusual and unclassified spindle and cuboidal renal cell carcinomas. METHODS AND RESULTS: The studied group consisted of 13 patients (7 men and 6 women). They ranged in age from 22 to 65 years (mean 57.3). Generally, the tumours were well circumscribed and confined to the kidney, whitish to grey on section with a diameter 4.5-13 cm (mean 8.6 cm). One patient was investigated for loin pain and nocturia. Three patients had staghorn nephrolithiasis and vague sonographic findings in renal parenchyma. In one patient the renal tumour was found when examined on follow-up examination for prostatic adenocarcinoma. None of our patients was known to have elevated levels of parathyroid hormone due to hyperplasia, adenoma or carcinoma of the parathyroid gland. Clinical follow-up of the patients ranged from 9 months to 8 years (mean 2.3 years). Microscopically, the tumours were composed of two main populations of cells: flattened, spindle cells with sparse cytoplasm and small cuboidal cells with clear to light eosinophilic cytoplasm. Eight patients are currently well without signs of recurrence or metastasis, one had metastasis in the regional lymph node at the time of nephrectomy, one died of unrelated cause, and three were lost to follow-up. CONCLUSIONS: We present 13 cases of unclassified RCC. Our cases were histologically, immunohistochemically and ultrastructurally similar to the hitherto reported case reports of this variant of RCC. It is obvious, that that variant of RCC should be recognised as a new subtype of RCC.

[Mesenteric venous thrombosis--a shift in therapeutic strategy?]. / Mezenteriální zilní trombózy--posun v lécebné strategii?

The authors present a group of nine patients suffering from mesenteric venous thrombosis, which were treated in three hospitals with participation of the first author. Basic principles of diagnostic and therapeutic procedure are summarized and a shift in the treatment strategy over the last 10 years is pointed out. The patient after a very radical intestinal resection may expect to survive and entertain an acceptable quality of life, provided the surgery is followed by an extensive team care in the post-operation period, later accompanied by the care of specialists in modern ways of nutrition.

[Intralobar pulmonary sequestration]. / Intralobäre Lungensequestration.

Sequestration is defined as an area of abnormal pulmonary tissue not connected with the bronchial tree, supplied by an aberrant systemic artery and without a normal pulmonary function. Extralobar (ELS) and intralobar (ILS) forms are distinguished. During the year 2002 the authors diagnosed and operated upon two cases of the intralobar form of pulmonary sequestration, and in last 25 years five cases - 4 x ILS and 1 x ELS. Reported are a 35 year old man with relapsing infections of the sequester and a 21 year old woman where the sequestration was accidentally found without clinical symptoms. The focus was localized in both cases in the left lower lobe of the lungs, anomalous supply arteries derived from the thoracic aorta. Venous drainage of the sequester was different - in the man a systemic drainage via the v. azygos, in the woman via the pulmonary veins was found. In one case the diagnosis was made on the basis of angiography and computer tomography, in the other case it was made on the basis of multidetector CT angiography (MDCTA). Both findings were treated by primary surgical intervention lobectomy. The postoperative course was uneventful.

[Urologic complications in pelvic injuries]. / Urologické komplikace úrazu pánve.

OBJECTIVE: Urological complications of pelvic fractures include in particular rupture of the urinary bladder (RUB), injury of the posterior urethra (IPU) and erectile dysfunction (ED). The authors present their own group of patients and in particular the diagnostic and therapeutic algorithm in IPU. MATERIAL AND METHODS: In the Plzen Faculty Hospital in 1/1998 to 8/2002 a total of 19 patients were treated with serious urological complications of pelvic fractures--9x RUB, 11x IPU (once with RUB). RUB was in one instance intraperitoneal, in the remainder extraperitoneal. RESULTS: IPU was without dislocation 6x, with dislocation 5x. Primary "realignment" of the urethra was made in 6 patients (in dislocations and in concurrent rupture of the bladder). In the remaining 5 an epicystostomy was established. In 4 after an interval of 3 months a posterior resection urethroplasty was made because of a distraction defect. One patient with a distraction defect was referred to the urological department of the catchment area and in another patient after-treatment is planned. Severe ED developed in 6 IPU of 10, always in dislocations of the urethra. In one patient we lack information on erections. CONCLUSION: When IPU is suspected (urethrorhagia), dislocation of the prostate on examination p.r.) ascendent urethrography and IVU are essential. Do not catheterize before completed examination. Then needle epicystostomy is performed, in major dislocations of the urinary bladder or in associated injuries primary "realignment" of the urethra open on a catheter or endoscopically. In distraction defects after 12 weeks a posterior resection plastic operation follows.

[An AV aneurysm of a branch of the pulmonary artery associated with a pulmonary tumorlet]. / A-V aneuryzma vetve plicní tepny s plicním tumorletem.

The authors present a rare case of a relatively large A-V aneurysm of the branch of the pulmonary artery in the right upper pulmonary lobe. Successful and technically easy surgical treatment was preceded by a serious dilemma whether to select in a high risk patient another (less invasive) therapeutic method or even conservative treatment. The surgical finding and smooth postoperative course confirmed that the indication of surgery was correct. As a further pathological finding in the resected portion a pulmonary tumourlet was detected. The combination of the two rare findings is an extreme rarity.

Successful surgical treatment of the brachial plexus paresis in leiomyosarcoma of the subclavian artery.

Described here is a unique case of surgical treatment of brachial plexus paresis in a 63-year-old female patient. The paretic condition was considerably improved by excision of a tumor in the upper mediastinum, growing from the left subclavian artery, and classified as leiomyosarcoma.

[Severe injury of the tracheal bifurcation]. / Závazné poranení bifurkace trachey.

The authors demonstrate a rare case of serious rupture in the area of the tracheal bifurcation with incomplete rupture of both bronchi which occurred after a severe multiple injury. The authors describe the difficult surgical solution, using temporary selective intubation of both main bronchi across the surgical field. The imperfect success of jet ventilation is ascribed to severe pulmonary contusion and major haemorrhage from the trachea which increased the risk of barotrauma. The authors emphasize the importance of peroperative and postoperative bronchoscopy.

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.