[Dedifferentiated Liposarcoma with Peculiar Meningothelial-Like Whorling and Metaplastic Bone Formation, Case Report]. / Meningotelyal Benzeri Girdapsi Yapilar ve Metaplastik Kemik Yapimi ile Karakterli Dediferansiye Liposarkom Olgusu.

Dedifferentiated liposarcoma represents a both morphologically and biologically fascinating lesion in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed. A peculiar form of dedifferentiated liposarcoma characterized by a nonlipogenic component with a meningothelial- like whorling pattern of growth and metaplastic bone formation has been described. A 59-year-old man presented with a pelvic retroperitoneal mass measuring 13x10x8 cm. Histopathologically the mass consisted almost entirely of a dedifferentiated nonlipogenic component with meningothelial-like whorling pattern, metaplastic cartilage and bone. Dedifferentiated liposarcoma with meningothelial- like whorling pattern has rarely been encountered. Here, we report this case because of the problems in differential diagnosis especially for cases that have an extensive dedifferentiated component and the uncertainty of the biological behavior of these tumors.

Growth of a solitary pulmonary nodule after 6years diagnosed as oncocytic carcinoid tumour with a high 18-fluorodeoxyglucose (18F-FDG) uptake in positron emission tomography-computed tomography (PET-CT).

INTRODUCTION: Pulmonary carcinoid tumour is low-grade neuroendocrine malignancy that is seen 1%-2% of all lung neoplasms. Oncocytic carcinoid type is a rarely seen variant of pulmonary carcinoids. As carcinoid tumours have hypometabolic activity, they usually have lower 18-fluorodeoxyglucose (18F-FDG) uptake than expected for lung carcinoma on positron emission tomography (PET). CASE REPORT: A 45-year-old non-smoking man had a stable solitary pulmonary nodule followed for 6years; the tumour remained the same size (1.5×2.4cm) during this period. The patient was admitted to the hospital with complaints of repetitive sneezing and rhinorrhoea. He also experienced flushing and bronchospasm. His chest X-ray revealed a minimal increase in the size of the solitary pulmonary nodule (2.0×2.8cm). In PET-computed tomography (CT), the parenchymal nodule in the anterior segment of the right lung had a standard uptake value of 38.0mg/mL, which was interpreted as a malignant nodule. He underwent fibre-optic bronchoscopy, but cytology showed no evidence of malignancy. Right upper and middle bilobectomy was performed, and a pulmonary carcinoid tumour with an oncocytic subgroup was diagnosed. The diagnosis of carcinoid syndrome was further confirmed by an elevated 24-h urinary excretion of 5-hydroxyindoleacetic acid. CONCLUSION: We present a rare case of an oncocytic carcinoid tumour with an increase in the size of a solitary pulmonary nodule after 6years' follow-up. In addition, PET-CT showed a very high 18F-FDG uptake in this patient, which is an unexpected finding with a pulmonary carcinoid tumour.

CD10 expression in epithelial and stromal cells of non-small cell lung carcinoma (NSCLC): a clinic and pathologic correlation.

CD10 is a zinc dependent metallopeptidase, and its expression in stromal and/or epithelial cells of many carcinomas has been suggested to have prognostic value. This study investigates CD10 expression in epithelial and stromal cells of non small cell lung carcinoma (NSCLC), and evaluates its prognostic value for this tumor and its histologic subtypes. Sixty-six cases of NSCLC [35 cases of nonsquamous cell carcinoma (NSCC) and 31 cases of squamous cell carcinoma (SCC)] were analyzed immuno-histochemically for CD10 antibody. Fisher's exact test and univariate survival analyses were performed. Comparison of clinicopathologic characteristics for NSCLC showed that only stromal CD10 expression had worse prognostic impact, associated with the presence of recurrence (p = 0.001), death (p = 0.006) and disease positivity (p = 0.001). For SCC, CD10 was found to be expressed mainly in the stromal cells, and was associated with a decreased survival (p = 0.000) and disease free survival (p = 0.000). CD10 expression was restricted to the epithelial cells in NSCC and associated with an increased disease free survival (p = 0.036). Stromal CD10 expression apppears to be a worse prognostic factor in NSCLCs. CD10 which is expressed in different cell components of SCC and NSCC appears to have opposing effects on the behaviour of these histologic types.

HGF/c-Met overexpressions, but not met mutation, correlates with progression of non-small cell lung cancer.

Hepatocyte Growth Factor (HGF) and its receptor c-Met are suggested to play an important role in progression of solid organ tumors by mediating cell motility, invasion and metastasis. Overexpression of HGF and c-Met have been shown in non-small-cell lung cancer (NSCLC). However, their role in tumor progression is not clearly defined. The aim of this study is to determine the role of HGF/c-Met pathway and its association with invasion related markers and clinicopathologic parameters in NSCLC. Immunohistochemical analysis was performed on 63 paraffin-embedded NSCLC tumor sections. The expressions of invasion related markers such as Matrix Metalloproteinases (MMPs) 2 and 9, Tissue Inhibitor Metalloproteinase (TIMP) 1 and 3 and RhoA were also examined. Co-expression of HGF/c-Met was significantly associated with lymph node invasion and TIMP-3 and RhoA overexpressions. There were positive correlation between TIMP-3 overexpression and advanced stage and negative correlation between RhoA overexpression and survival. DNA sequencing for Met mutations in both nonkinase and tyrosine kinase (TK) domain was established. A single nucleotide polymorphism (SNP) in sema domain and two SNPs in TK domain of c-Met were found. There was no statistically significant correlation between the presence of c-Met alterations and clinicopathologic parameters except shorter survival time in cases with two SNPs in TK domain. These results suggest that HGF/c-Met might exert their effects in tumor progression in association with RhoA and probably with TIMP-3. The blockade of the HGF/c-Met pathway with RhoA and/or TIMP-3 inhibitors may be an effective therapeutic target for NSCLC treatment.

[Aspergillus colonization of an echinococcal cyst cavity: case report]. / Ekinokok Kisti Kavitesinde Aspergillus Kolonizasyonu: Olgu Sunumu.

Although lungs are common sites for the occurrence of saprophytic or invasive mycosis as well as hydatid cyst, aspergilloma within a hydatid cyst is a rare occurrence. A 36-year-old immunocompetent male presented with hemoptysis episodes of about 1 year duration. Chest X-ray revealed a smooth countered mass lesion located in the upper part of lower lobe of left lung. Surgical enucleation of the cyst wall was performed. Microscopic examination showed that the laminated membranes of hydatid cyst were invaded by septated fungal hyphea oriented with acute angles consistent with aspergillus. Herein, we report such a unique coexistence of hydatid cyst and aspergilloma, and emphasize possible complications depending on the immunostatus of the patient, if the aspergillus colonization is unnoticed in resected cystectomy material.

Prevention of pleural adhesions using a membrane containing polyethylene glycol in rats.

BACKGROUND: Recurrent thoracotomies regardless of the cause are not a rare occurrence. However, each thoracotomy results in adhesion to some extent. This adhesions increase morbidity and mortality presents a significant inconvenience for surgeons and prolongs the length of operations. OBJECTIVE: We investigated the efficacy of Prevadh®, an anti-adhesion agent to prevent intrapleural adesions following thoracotomy in a rat model. METHODS: Twenty male adult Wistar Albino rats were divided into a sham group (Group A, n = 4), a control group (Group B, n = 8), and a study group (Group C, n = 8). Only left thoracotomy was performed in Group A. Group B underwent left thoracotomy, induction of adhesion, and 1 ml saline solution was administered to the thoracic cavity. However, in Group C underwent left thoracotomy, induction of adhesion, and Prevadh® was placed between the pleura and the lung. The rats were sacrificed on day 21, and adhesions were analyzed using both macroscopic and histopathological methods. The results were statistically analyzed. A value of P<0.05 was considered statistically significant. RESULTS: Mean lengths of adhesion differed statistically significantly among all three groups, while mean intensity of adhesion differed between Group A and Group B, and between Group B and Group C (P>0.05). There was also a statistically significant difference between Group A and Group C in mesothelium proliferation score (P>0.05). No statistically significant differences were found among the groups in terms of pleural thickness, macrophage and mononuclear cell infiltration (P>0.05). CONCLUSIONS: Prevadh® was shown in a rat model to effectively prevent post-thoracotomy adhesions.

A case of fever of unknown origin: co-existence of Kikuchi-Fujimoto disease and acute disseminated encephalomyelitis (ADEM).

It is believed that viral infections and the hyperimmune reaction due to these infections are involved in the etiology of Kikuchi-Fujimoto Disease (KFD), a rare cause of fever of unknown origin. Axillary lymphadenopathy and neurologic involvement are rare in KFD. We present a patient diagnosed with KFD histopathologically during an investigation of the origin of fever and axillary lymphadenopathy. Subsequently, incidental sinus aspergilloma was diagnosed radiologically in the patient and acute disseminated encephalitis developed during follow-up. This report aims to draw attention to the co-existence of KFD and Acute Disseminated Encephalomyelitis, two diseases of which the origins are not clear.

Primary pulmonary extranodal marginal zone lymphoma/low grade B-cell lymphoma of MALT type combined with well-differentiated adenocarcinoma.

We describe a rare case of extranodal marginal zone/low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) combined with areas of well-differentiated adenocarcinoma. In addition, the MALT lymphoma was synchronously systemic, with involvement of the lung, stomach and duodenum.

Pedunculated cutaneous spindle cell/pleomorphic lipoma.

Spindle cell/pleomorphic lipoma is an infrequently seen benign adipose tissue tumor. This tumor, mostly arising from the subcutaneous tissue, usually affects male patients and occurs in back, shoulders, head and neck area. It is rarely localized to dermis. Cutaneous spindle cell/pleomorphic lipoma differs from its subcutaneous counterpart by wider anatomical distribution, female predilection and infiltrative margins. We here present a pedunculated type, cutaneous pleomorphic lipoma case localized to the nasolabial region of 59-year-old man.

Expression and methylation pattern of p16 in neuroblastoma tumorigenesis.

Understanding migration, population and differentiation of primordial neural crest cells will help in evolving biology of neuroblastoma. P16 is a tumour suppressor gene contributing in cell cycle arrest as cyclin dependent kinase inhibitor. Methylation is an important mechanism for silencing tumor suppressor genes. The aim of this study was to evaluate the role of p16 and its methylation pattern in neuroblastoma tumorigenesis. This study included 23 cases (11 male; 12 female) and 31 samples from archival paraffin embedded tissues. P16 was studied in 5 samples of normal adrenal medullar tissue, 5 samples of adrenal tissue including blastic rests, 5 samples of neuroblastoma in situ tissue and in 8 samples of neuroblastoma tissues primary and after chemotherapy in each group. The adrenal gland tissues were obtained from paediatric autopsy cases. Expression of p16 was searched by immunohistochemistry. Methylation specific PCR was used to detect the methylation rate of p16. The age range of autopsy cases was between 20 weeks of foetal age and 36 months of infant age. The mean age of neuroblastoma cases was 45 months. P16 expression was positive in normal adrenal tissues, in one of 5 samples of adrenal blastic rest tissue and in all of samples of after chemotherapy; while no expression was observed in neuroblastoma and neuroblastoma in situ tissues. P16 methylation was observed in samples of neuroblastoma in situ and primary neuroblastoma tissues. Our results suggest that p16 and its methylation seems to play role in neuroblastoma tumorigenesis and in the migration, population and differentiation of primordial neural crest cells. Inhibitors of DNA methylation may provide a useful tool for restoring p16 activity in neuroblastoma treatment.

Nodular lymphoid hyperplasia of the lung: the role of positron emission tomography in diagnosis.

Pulmonary nodular lymphoid hyperplasia (NLH) is defined as reactive lymphoid proliferation forming solitary or multiple nodules or localized infiltrates localized in the lungs. Radiological presentations are generally solitary or multiple nodules, but air bronchograms and ground glass attenuation may be present. Patients mostly asymptomatic and the lesions were detected coincidentally on routine chest X-rays. We present a case of NLH with cavitary lesion arising in the lung of a 61 year-old man who admitted with cough and massive hemoptysis. The lesion had positive fluorodeoxyglucose (FDG) uptake. To our knowledge, this is the only patient reported in the literature presenting with massive hemoptysis and a cavitary lesion with positive FDG uptake.

Focal adhesion kinase (FAK) expression in normal and neoplastic lymphoid tissues.

Focal adhesion kinase (FAK) is a protein tyrosine kinase essential for intracellular regulatory events, such as cell growth, differentiation, migration and tumor metastasis. The aim of this study was to analyze the expression of FAK protein in a series of normal and neoplastic lymphoid tissues. An anti-FAK antibody was used to study the protein expression in paraffin-embedded samples of normal and neoplastic, hematolymphoid and non-hematolymphoid tissues by immunohistochemistry. In normal hematolymphoid tissue, the strongest expression of FAK was detected in germinal center and marginal-zone B cells; positive staining was also found in mantle zone B cells. In human lymphomas, FAK was expressed mostly in B-cell lymphomas and was predominantly negative in T-cell lymphoma. In Hodgkin lymphomas, FAK was found only in the neoplastic cells of lymphocyte predominant type, whereas the tumor cells of the classical form were FAK-negative. We demonstrate for the first time the expression of FAK in paraffin-embedded hematolymphoid tissue samples. Its differential expression in lymphomas may be of relevance for some B-cell neoplasms by using it as an additional marker to distinguish B- from T-lymphoblastic leukemia/lymphoma to further differentiate lymphocyte predominant from classical Hodgkin lymphoma.

Fatal disseminated mucormycosis in a patient with mantle cell non-hodgkin's lymphoma: an autopsy case

A patient with mantle cell non-Hodgkin's lymphoma presented herself with fever, nausea, right upper quadrant pain on the 7th day of R-CHOP chemotherapy. After hospitalization with the suspicion of acute cholecystitis, she received antibiotherapy with G-CSF because of emerging neutropenia at the 10th day of chemotherapy. Abdominal computed tomography revealed small infarcts in the spleen and kidneys. The echymotic lesion which developed on her right lateral malleolus, became bullous in the following days and treated as ecthyma gangrenosum. Altough the patient was afebrile with a normal neutrophil count on the third day of antibiotherapy, she developed acute renal failure and deteriorated rapidly. The patient underwent hemodialysis but expired on the 10th day of hospitalization. Post mortem autopsy findings showed ischemic infarction and necrosis of parenchyma due to mycotic thrombosis of arteries and veins of many organs (heart, lung, diaphgram, kidneys, spleen, gut mucosa) as well as invasion of vessel walls and parenchyma by mucor. We reviewed mucormycosis in the light of this case.

Prognostic significance of immunohistochemical classification of diffuse large B-cell lymphoma.

AIM: To evaluate the clinical significance of immunoperoxidase staining for CD10, bcl-6, mum-1 and bcl-2 to subdivide DLBCL into prognostic subgroups, we analysed 50 DLBCL cases using immunohistochemical methods. METHODS AND RESULTS: Fifty DLBCL patients were evaluated retrospectively. The expression of CD10 was associated with better OS (p=0.04) whereas expression of mum-1 was associated with worse OS (p=0.009). There were no significance of OS in case of expression of bcl-6 (p=0.05) and bcl-2 (p=0.3). They were subclassified using CD10, mum-1, bcl-6 as germinal center B-cell like (GCB) lymphoma (30%) and non-GCB lymphoma (70%). The OS and EFS (event free survival) were longer in GCB group (p=0.002) and 5-year OS for GCB group was 92% compared with only 44% for the non-GCB group (p=0.02). The OS of the GCB group also was longer compared to that of the non-GCB group in low IPI subgroup (p=0.01). CONCLUSION: The existence of survival differences between GCB a non-GCB group also in the patients with low IPI score, showed the importance of prognostic classification in the risk-adaptive treatment approaches. The classification as GCB and non-GCB based immunostains may enable to define more accurate prognostic groups in DLBCL.

Atypical infectious mononucleosis in a patient receiving tumor necrosis factor alpha inhibitory treatment.

The objective is to report a case of atypical acute infectious mononucleosis in a juvenile ankylosing spondylitis patient who was treated with infliximab. A 20-year-old man was hospitalized for the evaluation of lymphadenopathy and systemic symptoms. His symptoms developed at the eighth week of the infliximab treatment and he required hospitalization. Lymph node biopsy was performed and he was diagnosed as atypical infectious mononucleosis (absence of fever, pharyngitis, lymphocytosis and negative atypical lymphocytosis on blood smear). Infections have become major concerns in patients treated with TNF-blocking agents. In theoretical base, it is not surprising as TNF-alpha has a crucial role in the body's defense against both bacterial and viral invasion. Blocking the action of TNF may also change the course of the disease and could lead to a delay in the diagnosis. TNF-alpha-blocking treatment may mask the typical symptoms of infectious mononucleosis and atypical cases should be included in the differential diagnosis of lymphadenopathy in patients receiving anti-TNF-alpha agents.

Thymic enlargement in a patient with juvenile idiopathic arthritis during etanercept therapy.

Tumor necrosis factor (TNF) alpha inhibitors are effective in the treatment of inflammatory rheumatic diseases. Despite their effectiveness, anti-TNF drugs have some drawbacks such as severe adverse effects including infections and possibly lymphoproliferative disorders. In this report we described a case of juvenile idiopathic arthritis patient who developed thymic enlargement (true thymic hyperplasia), mediastinal lymphadenopathy and pleurisy associated with systemic symptoms under Etanercept treatment. The clinical presentation was highly suggestive of malignancy and the patient underwent diagnostic mediastinoscopy with biopsy.

Prognostic significance of cell proliferation and apoptosis-regulating proteins in Epstein-Barr Virus positive and negative pediatric non-Hodgkin's lymphoma.

Apoptosis-related proteins and proliferation activity and their relationship with Epstein-Barr Virus (EBV) are contemporary issues in pediatric non-Hodgkin's lymphoma (pNHL). In this study prognostic or pathogenetic role of EBV latent infection, proliferating activity, and apoptosis-regulating proteins in pNHL were explored. EBV-EBER, lmp-1, ki-67, bcl-2, survivin, bax, fas, c-myc, p53 and apoptotic index by TUNEL method were explored in 70 pNHL cases and evaluated statistically. Of the 70 cases evaluated, 24 were female and 46 were male. Seven cases were stage I/II and 63 cases were stage III/IV. The mean age was 7.16 + or - 3.72(1-15). EBV was positive in (25.7%) cases. Overall survival was 82%, while event free survival was 75%. Bax was expressed in 40% of the cases, while the expression of bcl-2,was 50%, survivin 42.9%, p53 8.6%, fas 18.6% and c-myc in 45.7%. Mean apoptotic index was 131.29 + or - 96.69 per 5,000 cells. Mean proliferation index was 55.97% (12-92%). Fas positivity was high in EBV positive cases (p = 0.0001). EBV positivity was not related with prognosis. Apoptotic index was found to be an independent prognostic factor (p = 0.017). Our results suggest that apoptosis-regulating proteins have a role in the pathogenesis of pNHL. EBV was correlated with apoptotic index and fas, bcl-2. No correlation was observed with proliferation index and studied factors. High apoptotic index was related with good prognosis.

Fatal disseminated mucormycosis in a patient with mantle cell non-Hodgkin's lymphoma: an autopsy case.

A patient with mantle cell non-Hodgkin's lymphoma presented herself with fever, nausea, right upper quadrant pain on the 7th day of R-CHOP chemotherapy. After hospitalization with the suspicion of acute cholecystitis, she received antibiotherapy with G-CSF because of emerging neutropenia at the 10th day of chemotherapy. Abdominal computed tomography revealed small infarcts in the spleen and kidneys. The ecchymotic lesion which developed on her right lateral malleolus, became bullous in the following days and treated as ecthyma gangrenosum. Although the patient was afebrile with a normal neutrophil count on the third day of antibiotherapy, she developed acute renal failure and deteriorated rapidly. The patient underwent hemodialysis but expired on the 10th day of hospitalization. Post mortem autopsy findings showed ischemic infarction and necrosis of parenchyma due to mycotic thrombosis of arteries and veins of many organs (heart, lung, diaphragm, kidneys, spleen, gut mucosa) as well as invasion of vessel walls and parenchyma by mucor. We reviewed mucormycosis in the light of this case.

Diffuse gastrointestinal involvement of mantle cell lymphoma.

The gastrointestinal tract is the predominant site of appearance of extranodal non-Hodgkin lymphomas. The most frequent endoscopic finding of mantle cell lymphoma is multiple lymphomatous polyposis, which is a very rare entity. Multiple lymphomatous polyposis is characterized by multiple polypoid lesions involving long segments of the gastrointestinal tract and it accounts for 2% of primary gastrointestinal tract lymphomas. A 68-year-old patient was admitted to our clinic with intermittent diarrhea, weight loss, hematochezia and fatigue. Multiple lymphomatous polyposis was detected on the endoscopic evaluations. Gastrointestinal mantle cell lymphoma was confirmed with histopathological and immunohistochemical studies on biopsy specimens from colon, small intestine and stomach. The patient was successfully treated by combination chemotherapy.