Hepatic pseudolymphoma: a surprising finding in a case with suspected generalisation of lung cancer.

INTRODUCTION: Pseudolymphoma is a rare focal lesion which occurs in different locations. Only about 50 cases of liver pseudolymphoma have been reported so far. The diagnostic process is challenging. The lesion can resemble different malignancies using various imaging methods. No typical laboratory markers are available. The right diagnosis is usually made on the basis of histological examination. CASE REPORT: A 67 years old female patient with lung fibrosis was undergoing assessments for a malignant-appearing focal lesion of the left lung and a focal liver lesion of unknown etiology. Upper lobectomy of the left lung proved lung carcinoma. The liver lesion was suspected for being metastatic, therefore a liver resection followed. The biopsy revealed hepatic pseudolymphoma. It took 150 days from the first positive CT scan until the liver resection. Currently, the patient shows no signs of recurrence. CONCLUSION: Hepatic pseudolymphoma is a rare disease and we have only little experience with it so far. The diagnostic process is challenging, which is clear from the presented case. Only histological and immunohistochemical examinations ruled out a malignancy. A long-term observation of the patient is indicated.

[Coincidence of colonic lymphoma and gallstone ileus - case report]. / Koincidence lymfomu tracníku a biliárního ileu - kazuistika.

INTRODUCTION: Primary colonic lymphoma is a very rare malignant disease of the gastrointestinal tract, accounting for 14% of all malignant diseases in this location. It is classified in the group of extranodal lymphomas; its long-term asymptomatic progression makes it different from common colorectal carcinomas making its diagnosis very difficult, more often accidental. Gallstone ileus is quite an uncommon complication of cholecystolithiasis diagnosed with difficulty. Up to 50% of cases are diagnosed during surgery. The obturated location depends on the size of the stone, location of the conjunction between the biliary and gastrointestinal tracts, and also on any preexisting stenosis due to another unknown pathology. CASE REPORT: We present a case of an 86-year-old man treated for acute diverticulitis with typical clinical symptoms. Following further examination (colonoscopy, computed tomography) revealed a tumour-like infiltration in the sigmoid colon wall and a voluminous polyp was suspected according to the colonoscopy. Computed tomography described an obstruction by a biliary stone tumbling through the cholecystocolonic fistula. Subsequent biopsy supported the suspected malignant etiology. The patient underwent resection of the sigmoid colon sec. Hartmann; an infiltration was found in the subhepatic space, which corresponded to the described fistulisation between the biliary tract and the colon. A large 40 mm gallstone was found in the resected sigmoid colon over the stenosis and the bowel wall showed diffuse thickening with several polyps; final histopathological assessment confirmed malignant lymphoma of the plasmocytoma type. No serious complications occurred in the postoperative period; after healing, the patient was transferred to hematooncology care. CONCLUSION: The article describes the presence of two rare diseases - colonic lymphoma and gallstone ileus. Clearly, without the biliary stone obstruction in the preexisting tumorous stenosis in the sigmoid colon, the malignant hematooncology disease would not have been diagnosed. KEY WORDS: primary colonic lymphoma - gallstone ileus - complication of the cholecystolithiasis - extranodal lymphoma - acute diverticulitis.

[The clinical, histopathological and imaging methods characteristics of Non-Hodgkin lymphomas in patients with brain involvement]. / Klinické, histopatologické a zobrazovací charakteristiky nonhodgkinských lymfomu u pacientu s postizením mozku.

BACKGROUND: The Non- Hodgkin-lymphoma (NHL) brain infiltration carries a poor prognosis. Because of relatively rare incidence, we decided to share our experience. PATIENTS AND METHODS: Retrospective analysis of patients with NHL brain infiltration dia-gnosed in 2001- 2011 at our university hospital. RESULTS: Twenty -seven patients with median age of 61 (range 42- 82) years were analyzed. The primary diffuse large cell B cell lymphoma of CNS was defined in 22/ 27 (81%) patients, in the others systemic NHL was present. Median positivity of the proliferative marker Ki 67 was 80%, the number of NHL lesions 1 (1- 8), diameter 28 × 30 × 29 (11 × 16 × 20 to 85 × 76 × 65) mm. The fundamental finding in brain lymphoma MRI imaging was lesion with predominantly homogenous contrast enhancement, diffusion restriction and collateral edema. Thirteen out of 27 (48%) patients underwent lumbar puncture, and lymphoma presence in fluid was detected in only two of them. The most frequent symptoms were limb paresis or hemiparesis (55%), bradypsichysm (22%), expressive aphasia (22%), cephalea (18%). Corticosteroid therapy, as a primary treatment option, was indicated in 15% of patients with a median overall survival of one month, CNS radiotherapy in 37% with a median survival of three months, and chemotherapy in 48% patients with a median overall survival 10 (2- 45) months. CONCLUSION: The brain lymphomas are rare and prognostically very unfavorable affection. When specifying brain focal lesions on MRI, it is necessary to consider this etiology and to elect imaging protocols with contrast agents and diffusion weighted sequence. Biopsy should be performed prior to start of corticosteroid therapy. Intensive chemotherapy or radiotherapy indication must be individually considered, and proposed treatment should be initiated immediately with a potential for somewhat prolonged survival.

Focal histopathological progression of porcine experimental abdominal aortic aneurysm is mitigated by atorvastatin.

AIM: Observational studies in human patients and animal experiments suggested that statins have a potential in slowing the growth of small abdominal aortic aneurysms (AAA). Our aim was to quantify histological postoperative changes of AAA in porcine experimental model of AAA with and without administration of atorvastatin. METHODS: The AAA was induced by intraaortic infusion of porcine pancreatic elastase and subrenal application of plastic cuff. The AAA statin group (N.=14) received atorvastatin 1 mg/kg daily for 28 days, the other AAA group (N.=13) did not. The aortic diameter was measured by ultrasonography. Aortic samples were described using eleven quantitative histological parameters and compared with healthy aortae. RESULTS: There was no difference in aortic diameter between the AAA with statin when compared to AAA without statin. Administration of atorvastatin led to a better postoperative histological condition of the aortic elastin network, preservation of contractile phenotype of vascular smooth muscle, a higher density of vasa vasorum, it prevented thickening of intima and media. The increase in wall thickness in AAA without atorvastatin has not been accompanied by a proportional increase in number of vasa vasorum. CONCLUSION: The effects of atorvastatin seem to prevent the histopathological progression of AAA.

[Angioimmunoblastic T-cell lymphoma as a very poor-prognosis malignancy--a single centre experience]. / Angioimunoblastický T-lymfom (AITL) jako velmi nepríznivá malignita - zkusenost centra.

BACKGROUND: Angioimmunoblastic T-lymphoma (AITL) is a poor prognosis malignancy. Because of relatively rare incidence and lack of publications in Czech, we decided to share our experience. PATIENTS AND METHODS: Retrospective analysis of newly diagnosed AITL patients treated at our institution between 1/2000-12/2010. RESULTS: Twelve patients with median age of 64 (43-82) years were analysed. Two patients over 80 years were treated with corticosteroids. Ten patients were treated with 6 cycles of CHOP-21 chemotherapy resulting in: 2/10 (20%) stable disease, 5/10 (50%) partial remission and 3/10 (30%) complete remission. The median EFS and OS of chemotherapy-treated patients were 8 and 10 months, resp. The EFS and OS were both significantly longer in patients who achieved complete remission within the first line of CHOP or autologous stem cells transplantation therapy: 43 vs 6 (p = 0.0052) and 46 vs 6 months (p = 0.0023), respectively. It was not possible to perform autologous transplantation in 4/7 (57%) patients in need for further reduction of the disease because of poor performance status or early progression of lymphoma and death during salvage chemotherapy. CONCLUSION: AITL is a poor prognosis malignancy with a very high risk of early relapse after CHOP induction chemotherapy. In fit patients, autologous transplantation should be performed immediately after induction chemotherapy; information about availability of stem cells donor, both in the family or any available register, should be found during the induction treatment.

[Plasmatic levels of proinflammatory cytokines in abdominal aortic aneurysms]. / Plazmatické hladiny prozánetlivých cytokinu u aneuryzmatu abdominální aorty.

INTRODUCTION: Inflammation within the abdominal aortic wall is generally considered a very significant ethiopathogenic factor in the development of abdominal aortic aneurysms. Proinflammatory cytokines are important mediators of inflammation within the abdominal aortic wall. AIM: The aim of the study was to research, whether plasmatic levels of certain proinflammatory cytokines, which can commonly be evaluated (TNFalpha, IL-1, -2, -6 a -8), play a significant role in the development of AAA. METHOD: The prospective non-randomized study included 345 patients with AAAs. The patients were assigned to 5 subgroups based on their symptoms and AAA diameters. The first subgroup included patients with symptomatic AAAs, including AAA ruptures (N = 69), the second subgroup included subjects with asymptomatic AAAs (N = 276) with AAA diameters up to 5 cm (N = 72), the third subgroup included 5 cm (N = 72), the fourth included 5-8 cm (N = 192) and the fifth subgroup included subjects with AAA diameters of more than 8 cm (N = 81). The mean age of patients was 74.1 +/- 7.8 years (56-84 y.o.a.). The male to female ratio was 5:1. The control group included 30 healthy volunteer subjects of similar age and male to female rates, who had no clinical signs of arterial disorders. Plasmatic levels of cytokines were evaluated from venous blood samples using ELISA (Bender, Austria) testing. Statistical assessment of the results was performed using ANOVA and Wilcoxon tests with Spearman's correlation. P values < 0.05 were considered significant. RESULTS: Plasmatic concentrations of proinflammatory cytokines were found to be statistically significantly higher in patients with AAAs compared to those in healthy volunteers. Plasmatic IL8 levels were significantly decreasing proportionally to decreasing AAA diameters (p < 0.05). TNFalpha levels were found to be significantly low in symptomatic patients with AAA ruptures (p < 0.05). CONCLUSION: The study confirmed the significance of proinflammatory cytokines levels monitoring in AAA patients. The authors showed that, for instance IL8 activity and to a certain extent TNFalpha activity, is the highest in small and developing AAAs. These findings would be significant for customized medication therapy aimed at blocking the effects of these factors on the inflammatory process within the AAA wall.

Histopathologic changes in gastroesophageal reflux disease. A study of 126 bioptic and autoptic cases.

The histologic diagnosis of reflux esophagitis is still complicated by the lack of a consensus opinion on what is the normal mucosa in the area of the gastroesophageal junction (GEJ). Most authors consider GEJ as the junction between the squamous and the cardiac epithelium. The cardiac mucosa is composed of mucinous or mixed mucinous-oxyntic glands. These glands are in fact indistinguishable from metaplastic mucosa that arises in the distal esophagus in consequence of gastroesophageal reflux (GER). The cardiac mucosa shows invariably chronic inflammatory changes referred to as "carditis". The cause of "carditis" is GER and/or Helicobacter pylori (HP) infection. In our series of 120 endoscopic biopsies of the GEJ and distal esophagus the cardia type mucosa (CM) was always present. In 15 cases, it was accompanied by oxyntocardiac mucosa. Both mucosa types showed chronic inflammation that is after exclusion of HP infection regarded as a strong diagnostic sign of the gastroesophageal reflux disease (GERD). In two cases with clinical symptoms of GERD, a few HP were found on the CM. Therefore we diagnosed them as GERD with secondary HP infection. In 17 cases, CM displayed intestinal metaplasia (IM) predominantly of incomplete type and no dysplasia. This IM expressed MUC6 in the glandular zone of the mucosa like it did in the neighboring glands, whereas in the surface and foveolar epithelium the MUC6 was negative or only slightly and focally positive. On the other hand, IM in the surface and foveolar epithelium was reactive for MUC5AC. The positivity and distribution of CK7 and CK20 was very similar in the Barrett's mucosa, cardiac mucosa and antral mucosa. In one specimen of esophagus resected for adenocarcinoma, CM with incomplete IM was found in the vicinity of the tumor. Squamous metaplastic epithelium was often seen near the orifices of submucosal esophageal glands in these areas, indicating the metaplastic nature of the glandular mucosa in the distal esophagus. In the GEJ of 5 autopsy cases of children with spastic quadriplegia (age range 7-10 years) CM in a short segment (0.5-3 mm in length), probably of metaplastic origin was identified, showing chronic inactive inflammation.

Sessile serrated adenomas of the large bowel. Clinicopathologic and immunohistochemical study including comparison with common hyperplastic polyps and adenomas.

Sessile serrated adenoma (SSA) is a newly characterized type of the large bowel adenoma. It arises in hyperplastic polyp (HP) and represents a precursor lesion of colorectal carcinoma with microsatellite instability. SSAs differ from common HPs by abnormal proliferation of the crypt epithelium and by nuclear atypia. We examined 15 SSAs from 15 patients. The age range was 25-80 years (average 60 years). Six patients were females and 9 were males. For comparison, we examined 10 conventional tubular adenomas and 10 common HPs with vesicular cells. The sites of SSAs were as follows: 8 in rectum, 4 in rectosigmoid colon, 1 in transverse colon, 1 next to mucinous carcinoma of ascending colon, 1 in anastomosis after resection of the transverse colon adenocarcinoma. The diameter of the lesions ranged from 5 to 12 mm. Histologically, SSAs showed asymmetrical proliferation of the epithelium, irregular shape of the crypts with their branching and some crypt dilatations especially in the basal parts of the crypts. Cellular atypia (dysplasia) was usually low. In 5 cases the nuclei were focally stratified and localized in the lower part of the cells. High-grade dysplasia was found only in SSA adjacent to mucinous adenocarcinoma. Immunohistochemically, SSAs showed secretion of gastrointestinal mucin expressing MUC2 and MUC5A. Both MUC2 and MUC5A were also positive in mucinous carcinoma. In previous studies these expressions were considered specific for serrated type of carcinogenesis. However, our study found positivity of MUC2 and MUC5A also in conventional adenomas. Expression of p53 in SSAs was minimal. SSAs have malignant potential comparable with conventional adenomas and for this reason they must be distinguished from HPs.

[Isolated lymphadenopathy as the first presentation of systemic mastocytosis--description of two cases]. / Izolovaná lymfadenopatie jako první projev systémové mastocytózy--popis dvou prípadu.

Presented are two cases of systemic mastocytosis in 46- and 63-year-old women, where the correct diagnosis was established in randomly disclosed cervical respectively intraabdominal lymphadenopathy. Both cases lacked characteristic skin and systemic mast-cell mediator symptoms at the time of histologic diagnosis. The first case was classified as a indolent systemic mastocytosis without any proven genetic alteration, the second one met the criteria of aggressive systemic mastocytosis with eosinophilia, where the point mutation asp816val in c-kit gene was confirmed and the patient responded unexpectedly well to Gleevec. Discussed are both conventional morphological differential diagnosis of mastocytosis in lymph nodes and recent advances in genetics of these systemic clonal mast cell proliferations. The latter not only outlines the oncopathogenesis but, in particular, also provides important prognostic and biological implications of this peculiar disease.

[Splenosis in the abdominal wall subcutis]. / Splenóza v podkozí brisni steny.

A case of subcutaneous splenosis in the abdominal wall of a 23-year-old oligophrenic man is reported. Further, the most important features of splenosis in various localisations are provided. The patient presented with a well-demarcated 8x7x5 cm subcutaneous tumour in the left inguinal area closely above the scar after a previous operation for hernia. The lesion simulated clinically a hernia or a tumour due to its large size and location. Additionally, no history could be taken from the patient due to his mental handicap. The diagnosis of splenosis was established histologically.

Autoimmune gastritis. A clinicopathologic study of 25 cases.

The histopathological diagnosis of autoimmune gastritis (AG) in its early stages can be a diagnostic challenge. Even some advanced cases with complete atrophy of the corpus mucosa may be difficult to recognize. To establish the diagnosis of autoimmune gastritis, several histological features should be assessed and combined with immunostains for enterochromaffin cell-like (ECL) cells and G-cells. The main histological criteria include a mononuclear infiltrate within the lamina propria, foci of destruction of oxyntic glands, intestinal metaplasia (IM), pyloric metaplasia, and parietal cell pseudohypertrophy. These criteria were evaluated in our series of 25 patients with achlorhydria and/or megaloblastic anemia. Some of our patients presented with nonspecific gastrointestinal symptoms. The age ranged between 46 and 79 years; one male patient was only 31 years old. Histologically, the corpus mucosa displayed in all cases chronic inflammation with focal complete IM and advanced pyloric metaplasia. In 4 patients, oxyntic glands were destructed in some sites. There was a pancreatic metaplasia of acinar type in 2 patients and a minimal focal pseudohypertrophy of parietal cells in the 31-year-old man. A tubular adenoma with a low-grade dysplasia was found in one female patient. Immunohistochemically, chromogranin-A highlighted linear or nodular hyperplasia of ECL cells in 19 patients, and adenomatoid ECL hyperplasia in one case (80%). In the remaining cases hyperplasia of ECL cells could not be recognized from their normal count. In 13 cases (52%) a few ECL cells were seen also in IM. Regarding associated pathology, in one woman with nodular ECL cell hyperplasia, a gastric carcinoid was removed endoscopically. The reaction with gastrin antibody revealed in 11 cases (44%) a small number of G cells in IM in the corpus mucosa. In 18 patients, antral mucosa was examined as well. In 8 patients, the mucosa was normal; in 10 cases, a mild chronic inactive gastritis was diagnosed, and in 15 patients G-cell hyperplasia was found. In accordance with other studies, we show that the diagnosis of AG may be established microscopically in endoscopic specimens of the gastric body mucosa when histologic features and immunohistochemical detection of ECL and G cell hyperplasia are combined.

[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.

[Pseudolymphoma of the breast nipple. The problem overview]. / Pseudolymfom prsní bradavky. prehled problematiky.

Clinical, pathological, and molecular-genetic features as well as etiology of cutaneous pseudolymphoma (CPL, cutaneous lymphoid hyperplasia, lymphocytoma cutis) of the breast nipple are summarized. CPL presents as a nipple induration and it is often suspected to be Paget carcinoma pre-operatively. Histologically, atypical microscopic features of a dense lymhoid infiltrate with follicles often mislead to the diagnosis of a malignant lymphoma. However, CPL runs a benign course. Rare cases of CPL contain a clonal lymphoid population. A substantial number of CPL in the breast nipple is caused by antigenic stimulation by Borrelia burgdorferi. In some patients a tick bite is documented. CPL of the breast is commonly treated by excision, but some patients may be cured by antibiotic therapy. The presence of Borrelia burgdorferi should be detected using methods of serology, culture, and molecular biology. Beside CPL, the differential diagnosis of the breast nipple lesions further includes Paget carcinoma, eczema, and florid papillomatosis.

CD44v6 as a prognostic factor in cervical carcinoma FIGO stage IB.

The aim of our study was to clarify whether CD44v6 evaluation can serve as a universally applicable prognostic factor in patients with FIGO stage IB cervical carcinoma. A retrospective study was performed on 178 FIGO stage IB (142 IB N0, 36 IB N1) radically operated cervical carcinoma patients. The expression of CD44v6 was investigated by immunohistochemistry (IHC). The prognostic significance of established prognostic factors and CD44v6 expression was analyzed by univariate and multivariate analyses. To test the reproducibility and to account for interobserver variability, all specimens were evaluated independently at two institutions. Two different IHC scoring systems, several cut-off levels for CD44v6 positivity and several statistical methods for IHC results evaluation were used. In a univariate analysis, the most significant prognostic factor for overall survival (OS) was lymph node status (p<0.001) followed by tumor volume, LVSI, GOG score (p<0.01) and a deep stromal invasion (p = 0.06). We found a strong correlation between CD44v6 expression and squamous cell carcinoma (SCC) (SCC vs. adenocarcinoma - p<0.001) and between CD44v6 expression and deep stromal invasion, LVSI and GOG score (p<0.05). The CD44v6 expression was not a statistically significant prognostic factor for OS in a univariate analysis (p=0.39 Vienna; p=0.54 Freiburg). In a multivariate analysis, the most significant prognostic factor for OS was lymph node status (p =0.002), followed by tumor diameter and LVSI (p<0.05). CD44v6 expression was not a statistically significant prognostic factor for OS or disease-free interval (DFI) independent of the scoring method used. In conclusion, we demonstrated that CD44v6 expression is associated with LVSI, deep stromal invasion and SCC, but has no prognostic influence on OS and DFI in a population of 178 women with FIGO stage IB cervical carcinoma.

[The peroperative biopsy--an overview of its problematical aspects from the pathologist's viewpoint]. / Peroperacní biopsie-prehled problematiky z pohledu patologa.

A summary work presenting technically difficult to process lesion samples and those which cannot be diagnosed on peroperative biopsy. The authors specify arguments against examinations of small mammary gland tumors (measuring less than 1 cm in diameter), post-bioptic tumors and sentinel lymph nodes. Furthermore, the authors describe difficulties in processing the adipose tissue resulting in complicated evaluation of the mammary gland tumors in the resection line. Obstacles in the peroperative examination of the thyroid gland lesions are also listed here. In this case, the freezing technique results in smearing specific cytological featuries of the examined tissue. Diagnostic criteria for all non-papillary tumors of the thyroid gland are explained in this article. Application of the above mentioned criteria is, however, worthless in the peroperative biopsy, which is also explained in this work. In the last part of this study, the authors describe macroscopic featuries of the mucinous ovarial tumors and their differentiation from the gastrointestinal tract adenocarcinoma metastases, using macroscopy and histological techniques.

[Prognostic factors of the cervical carcinoma]. / Prognostické faktory karcinomu delozního hrdla.

OBJECTIVE: Review of prognostic factors of the cervical carcinoma, new perspective methods. SETTING: Department of Obstetrics and Gynecology, Medical Faculty Hospital, Plzen. DESIGN: Review article. RESULTS: The most important prognostic factors of the early cervical carcinoma are the stage and regional lymph node status followed by the depth of stromal invasion, tumor volume, and lymphovascular invasion. Other factors are still only subjects of research. CONCLUSION: A detailed knowledge of particular prognostic factors of the cervical carcinoma is necessary for optimal treatment and flow-up strategy.

[Dexrazoxane in patients with B-lymphomas or acute leukemias in the 2nd complete remission enables further therapy with cardiotoxic anthracyclines over recommended cumulative doses]. / Dexrazoxan u nemocných s B-lymfomy nebo akutními leukémiemi ve 2. kompletní remisi umoznuje dalsí lécbu kardiotoxickými antracykliny nad doporucené kumulativní dávky.

UNLABELLED: Daunorubicin (DNR) and doxorubicin (DOX) have significant antitumor activity in acute myeloid leukemias (AML) and non-Hodgkin's lymphomas (NHL) but their use is limited by their life-threatening cumulative dose related cardiotoxicity. It is generally recommended not to administer DOX or DNR to patients in doses greater than 500 mg/sqm or 700 mg/sqm, respectively. the aim of the study was to follow up cardiotoxicity and efficacy of DNR or DOX above these limits in the 2nd complete remission (CR) patients pretreated with anthracyclines when they were given 30 minutes after cardioprotective agent dexrazoxane (DRZ) in the ratio 1:10 of DZR. RESULTS: Two patients (54 and 53 years old) with mantle cell or diffuse large cell B-NHL, stage IV, who had relapsed after 6-8 cycles of classical CHOP therapy, reached their 2nd CR after 2-3 cycles of IDEA therapy (ifosfamide 1000 mg/sqm/day x 4, dexamethasone 30 mg/sqm/day x 4, etoposide 75 mg/sqm/day x 4, DOX 30 mg/sqm/day on days 1 and 3). Then they received further 3 cycles IDEA with DRZ 300 mg/sqm before every dose of DOX. After cumulative doses of DOX 600 mg/sqm and 700 mg/sqm these patients survived 12 months in their 2nd CR without significant signs of cardiotoxicity, even after their successful autologous peripheral stem cells transplantation. Their left ventricular ejection fraction (LVEF) remained above 60%. Six patients with AML in their 2nd CR were treated with consolidation cycles consisting of 10 high doses of cytosine arabinoside (2000 mg/sqm/12 hr) plus 2 doses of DNR 45 mg/sqm on the day 4 and 5. Two patients received cumulative doses corresponding to 1300 mg/sqm and 1000 mg/sqm of DNR, the other received DNR doses 550-850 mg/sqm. No signs of significant cardiotoxicity were observed in all 6 patients and their LVEF remained over 50%. One of two patients, transplanted with HLA-identical sibling bone marrow in her 2nd complete remission (CR), is still 8 years in her 2nd CR. Dexrazoxane enables to administer anthracyclines in doses over the recommended cumulative ones in pretreated patients with B-NHL or AML in their 2nd CR with the follow-up of their LVEF.

[Matrix-producing breast carcinoma with myoepithelial differentiation--description of 11 cases and review of literature aimed at histogenesis and differential diagnosis]. / Matrix produkující karcinom prsu s myoepiteliální diferenciací--popis 11 prípadu a prehled literatury se zamerením na histogenezi a diferenciální diagnostiku.

OBJECTIVE: Presentation of variable morphology of the so-called matrix-producing carcinoma, the rare variant of metaplastic breast carcinoma. Establish the extent of myoepithelial phenotype by immunohistochemistry and assess behaviour of the lesion. DESIGN: Review of clinicopathologic data of 11 cases. SETTING: Private Biopsy Lab s.r.o., Pilsen and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Included are eleven cases from Breast Registry, Biopsy Lab s.r.o., Pilsen and Pathology Department, Faculty Hospital in Hradec Králové and Martin and from Biopsy Lab in Horovice. Analyzed were morphology, stage, grade and follow up. Myoepithelial phenotype was demonstrated immunohistochemically by LSAB+ system (DAKO) and seven conventional myoepithelial markers (GFAP, aktin S, calponin, cytokeratin 14, p63, CD10 and P-cadherin) were used. Oncogene p53 and Her2/neu were also detected. RESULTS: Matrix-producing carcinoma is an extremely rare type of metaplastic carcinoma of the breast, where high-grade epithelial component continually merges with heterologous mesenchymal chondroid component without overt spindle cell sarcomatoid pattern in between. There were all women at the age of 32 to 86-years (average 54 years, median 52 years) and the maximum tumor diameter ranged from 18 to 60 mm (average 32 mm). The axillary LN was positive in six cases in the time of diagnosis. The follow up available in 9 women ranged from three months to ten years (average 24 months); dissemination of the disease was observed in three cases; 2 of these patients died of disease after three and ten years, respectively. The heterologous mesenchymal chondroid component was produced in two different ways; one displayed typical structure of low-grade hyaline cartilage, in the second one the epithelial tumorous cells were embedded in the homogenous eosinophilic extracellular matrix giving appearance of chondroid aura. Metaplastic component constituted 5-75% of the tumor volume. Immunohistochemical evidence of myoepithelial differentiation in all neoplasms was demonstrated, with at least two conventional myoepithelial markers (actin S, calponin, GFAP, CK14, CD10, p63 and P-cadherin) being positive in every case. Expression of p53 was identified in six cases, all tumors were Her2/neu negative. Histologically the metastases were formed either by carcinoma cells only, or more frequently, they replicated the structure of primary lesion. CONCLUSION: As a rare entity, matrix-producing carcinoma of breast displaying myoepithelial phenotype, deserves separate position in tumor classification. It differs from conventional myoepithelial carcinoma and from heterologous metaplastic carcinoma, where the matrix emanate from undifferentiated sarcomatous tissue, but precise histogenesis in not clear yet. This is a very aggressive lesion and our findings show that previous reports of indolent behaviour were preliminary. Spectrum of differential diagnosis, namely benign mixed tumor, phylloid tumor, primary breast sarcoma and colloid carcinoma warrant clear knowledge about this unique entity.