A Rare Case of Primary Pulmonary Diffuse Large B-Cell Lymphoma Transformed from Marginal Zone Mucosa-Associated Lymphoid Tissue Lymphoma.

Primary pulmonary lymphoma is a rare neoplasm characterized by the proliferation of lymphoid tissue affecting the lungs. The most common subtype is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Rarely, a MALT lymphoma transforms into a diffuse large B-cell lymphoma (DLBCL). Treatment options include chemotherapy, radiotherapy, immunotherapy, and surgery. Here, we describe a patient with a primary pulmonary MALT lymphoma transforming into DLBCL. The purpose of this case report is to raise awareness of the relevant clinical and imaging features and to emphasize the need for a multidisciplinary approach to optimal management. In addition, we screened the PubMed and Embase databases for similar reports with a confirmed presence of transforming lymphoma within the lungs.

Basic Operative Tactics for Pulmonary Echinococcosis in the Era of Endostaplers and Energy Devices.

Human echinococcosis is a zoonotic infection caused by the larvae of the tapeworm species Echinococcus. The liver is the most common location for a primary echinococcosis. However, the parasite may bypass or spread from the liver to the lungs, causing primary or secondary pulmonary echinococcosis, respectively. Pulmonary echinococcosis is a clinically challenging condition in which anthelminthic regiments are important, but surgery has the central role in removing the cysts and preventing recurrences. Surgical treatment may involve cystotomy, enucleation, capitonnage, or atypical resections, which occasionally are in combination with hepatic procedures. The utilization of modern devices is greatly underdescribed in surgery for thoracic infections, even though these facilitate much of the work. Therefore, this article aims to describe pulmonary echinococcosis and the role of modern surgical devices in the treatment process. Furthermore, we report surgical treatment of three different cases of pulmonary echinococcosis. Surgeries of uncomplicated and ruptured hepatic or pulmonary cysts are described. Simple small pulmonary echinococcal lesions can be excised by endostaplers both for diagnostic and curative reasons. Larger cysts can be removed by energy devices unless large bronchial air leaks occur. Complicated cysts require treatment by more extensive techniques. Inexperienced surgeons should not abstain but should carefully decide preoperatively how to proceed.

Surgery quality and tumor status impact on survival and local control of resectable liposarcomas of extremities or the trunk wall.

BACKGROUND: The 5-year survival rates for localized liposarcomas reportedly vary from 75% to 91% with histologic grade as the most important prognostic factor. However, it is unclear which other factors, including the initial surgery quality and recurrent tumors, influence survival in localized liposarcomas (LPS). QUESTIONS/PURPOSES: We analyzed factors (including AJCC staging system) influencing survival and local control of resectable LPS of the extremities/trunk wall and the impact of surgery quality and tumor status and type of disease recurrences according to pathological subtype. METHODS: We retrospectively reviewed 181 patients with localized LPS: 110 were treated for primary tumors, 50 for recurrent tumors, and 21 for wide scar resection after unplanned nonradical resection. We determined survival rates and examined factors influencing survival. The minimum followup was 4 months (median, 52 months; range, 4-168 months). RESULTS: Five-year disease-specific (DSS), disease-free (DFS), and local relapse-free survival (LRFS) rates were: 80%, 58%, and 75%, respectively. Five-year local relapse-free survival rates for primary versus clinically recurrent tumor versus scar after nonradical resection were: 86.1%, 52.1%, and 73.3%, respectively. The following were independent negative prognostic factors for DSS (AJCC Stage ≥ IIb), DFS (Grade 3; clinical recurrence; skin infiltration), and LRFS (clinical recurrence; R1 resection). An unplanned excision, although influencing local relapse-free survival, had no impact on disease-specific survival (calculated from date of first excision 5-year rate of 80%, considering impact of combined treatment of clinical recurrence/scar). CONCLUSIONS: We confirmed the value of AJCC staging for predicting disease-specific survival in extremity/trunk wall LPS. Radical reresection of scar after nonradical primary tumor resection (+ radiotherapy) seems to improve disease-free and local relapse-free survival in liposarcomas. Patients with unplanned excision can be cured when referred to a sarcoma unit. LEVEL OF EVIDENCE: Level IV, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

The role of selected non-coding RNAs in the biology of non-small cell lung cancer.

Lung cancer is the second most frequently diagnosed cancer worldwide and a leading cause of cancer-related deaths. Non-small cell lung carcinoma (NSCLC) represents 85% of all cases. Accumulating evidence highlights the outstanding role of non-coding RNA (ncRNA) in regulating the tumorigenesis process by modulating crucial signaling pathways. Micro RNA (miRNA), long non-coding RNA (lncRNA) and circular RNA (circRNA) are either up- or downregulated in lung cancer patients and can promote or suppress the progression of the disease. These molecules interact with messenger RNA (mRNA) and with each other to regulate gene expression and stimulate proto-oncogenes or silence tumor suppressors. NcRNAs provide a new strategy to diagnose or treat lung cancer patients and multiple molecules have already been identified as potential biomarkers or therapeutic targets. The aim of this review is to summarize the current evidence on the roles of miRNA, lncRNA and circRNA in NSCLC biology and present their clinical potential.

Extremely Rare Pulmonary Metastases of Skin Basal Cell Carcinoma: Report of Two Cases with Clinicopathological Features.

Basal cell carcinoma (BCC) is the most frequent human skin cancer, but metastasizing BCC (MBCC) is extremely rare, developing in approximately 0.0028% to 0.55% of BCC patients. Herein, we report two cases of pulmonary MBCC. The first one developed in a 72-year-old male who underwent surgical resection due to multiple recurrences and adjuvant radiotherapy. Immunohistochemistry showed that neoplastic cells expressed Ber-EP4, CK5/6, p63, EMA (focally), BCL-2, and CD10, but were negative for CK7, CK20, S100, estrogen and progesterone receptors, and TTF-1. The second case is a 64-year-old female treated with vismodegib. Clinicopathological features and differential diagnoses are described.

Intraspinal mesenchymal chondrosarcoma in a 14-year-old patient: diagnostic and therapeutic problems in relation to the review of literature.

Mesenchymal chondrosarcoma (MC) is an infrequent, highly malignant neoplasm of the soft tissues and bone. It is very rare in the pediatric age group, especially in the intraspinal location. Only 24 cases have been reported to date. The authors present a case of a 14-year-old boy with an intraspinal MC who died of the disease 50 months from the initial diagnosis and after the third local recurrence. The patient was treated with a combination of chemotherapy, radiotherapy, and surgery. The authors review the clinical presentation, diagnostics, and the efficacy of treatment of pediatric patients with MC reported in the literature from 1978 to 2010.

Goblet cell carcinoid of the vermiform appendix - a case report.

Goblet cell carcinoid (GCC) is a rare neuroendocrine tumor of the vermiform appendix with uncertain clinical behavior. It was first described by Gagné and Subbuswamy in 1969 and 1974, respectively. The tumor occurs almost exclusively in the vermiform appendix. We present a case of a 60-year-old female, who was referred to the Bielanski Hospital with signs and symptoms of acute appendicitis. Microscopic examination of the appendix showed features of acute appendicitis, however scattered groups of cells with clear cytoplasm as well as strands of single cells with no evidence of atypia were seen. The patient underwent a right hemicolectomy due to the diagnosis of GCC.

Cellular angiofibroma with atypia or sarcomatous transformation - case description with literature review.

Cellular angiofibroma (CAF) is a rare, benign, mesenchymal tumor. It was first described by Nucci et al. in 1997 and then in 1998 by Laskin. The tumor occurs predominantly in the vulvo-vaginal or inguino-scrotal region. We present a 71-year-old male, who was referred to the Bielanski Hospital with a three months' history of a slowly growing nodule in the right groin. Gross examination showed a well-circumscribed tumor attached to the spermatic cord and measuring 6 cm in the greatest dimension. Microscopic examination of the tumor showed a spindle cell lesion with a loose, myxoid, partly collagenized stroma with numerous, prominent thick-walled vessels. Scattered atypical cells were present.

Quiz. Correct answer to the quiz. Check your diagnosis. Mesenchymal chondrosarcoma of the orbit: two cases and a brief review of the literature.

Mesenchymal chondrosarcoma (MChS) is a rare, high-grade malignant tumor which occurs both in the bone and soft tissue. The extraskeletal location comprises one third of all MChS and in review of the up-to-date literature, about 30 cases of the orbital involvement were found. The authors present clinical, radiological and pathological findings of two cases of MChS of the orbit occurring in young adult females: primary extraskeletal MChS of the orbit and skeletal MChS of the ethmomaxillary complex with secondary orbit involvement. The histopathological examination revealed a characteristic biphasic pattern composed of small round to spindle-shaped cells, mimicking Ewing sarcoma family of tumors, with areas of a haemangiopericytoma-like pattern and admixed cartilage foci. One of the patients had local recurrence 3 years after initial surgical removal. Subsequently, she underwent enucleation followed by chemotherapy. The other patient had a biopsy and debulking resection of the tumor and started chemotherapy. Ten months follow-up of this patient show no evidence of metastasis.

Benign Metastasizing Leiomyoma of the Lung: Diagnostic Process and Treatment Based on Three Case Reports and a Review of the Literature.

Uterine leiomyomas may occasionally spread to the lungs forming nodular lesions detectable on chest X-ray. This condition known as benign metastasizing leiomyoma (BML) usually occurs in females with a history of hysterectomy or myomectomy. We present three cases of BML demonstrating the diagnostic process and treatment approaches. Two patients presented with the more common multiple-nodule variant while the other had a single mass, but all were symptom-free. The age of presented patients at diagnosis of BML ranged from 46-53. The first patient was diagnosed with BML at the age of 50, and 12 years prior to the diagnosis, underwent a supracervical hysterectomy. The second patient had a myomectomy at 36, and BML was diagnosed 17 years later at the age of 53. The third patient had a hysterectomy with bilateral salpingo-oophorectomy at the age of 46, with lung lesions present before the hysterectomy. Immunohistochemical studies of postoperative materials showed positive staining of spindle cells with antibodies against desmin and smooth muscle actin, as well as estrogen and progesterone receptors. The final histopathological diagnoses were pulmonary BML. All patients are stable and symptom-free: two at two years follow-up and one at six months follow-up.

Clinicopathological Features of Intrathoracic Liposarcoma-A Systematic Review with an Illustrative Case.

BACKGROUND: Liposarcoma (LPS) is one of the most common soft-tissue sarcomas. However, intrathoracic LPS is rare, as only 1% of all LPS cases are found in the thorax. METHODS: A systematic literature review through PubMed and Embase databases was performed. Only eligible case reports and case series reporting intrathoracic LPS in adult patients were included. Kaplan-Meier curves were calculated to evaluate the survival rate of included patients based on the histological subtype of LPS. RESULTS: 123 studies reporting 197 patients were included. We added a case of a 69-year-old female patient with recurrent giant intrathoracic LPS. The primary tumor measured 15.1cm × 22.9 cm × 21.9 cm and weighed 3100 g. Six months later, the patient was admitted to the hospital with another intrathoracic tumor measuring 9.5 cm × 9 cm× 1.4 cm. The immunohistochemical studies showed expression of murine double minute 2 (MDM2) antigen in both primary and recurrent tumor cells. CONCLUSIONS: Dyspnea, chest pain, and cough were the most common symptoms reported in included studies. Overall, the 5-year survival rate was 62%. The highest survival was observed in well-differentiated LPS patients (80%) and the lowest in myxoid LPS (31%).

Clinical utility of the new American Joint Committee on Cancer staging system for gastrointestinal stromal tumors: current overall survival after primary tumor resection.

BACKGROUND: The objectives of the current study were to assess the reliability of the new revision of the American Joint Committee on Cancer (AJCC) staging system for gastrointestinal stromal tumors (GISTs) based on the National Comprehensive Cancer Network-Armed Forces Institute of Pathology risk classification and to analyze the factors that influence after resection for primary GISTs in 2 AJCC groups: patients with GISTs originating from the stomach and omentum (G-GISTs) and patients with other primary GISTs located mainly in the small bowel (nongastric GISTs [NG-GISTs]). METHODS: The authors prospectively analyzed a group of 640 patients with primary, CD117-positive GISTs who underwent surgery with curative intention (R0/R1 resection), including 340 G-GISTs (55.5%) and 300 NG-GISTs (44.5%). Factors were explored that had an effect on disease-free survival time (DFS), which was calculated from the date of radical operation to the date of recurrence or last follow-up. The median follow-up was 39 months. RESULTS: Compared with NG-GISTs, G-GISTs were characterized by a significantly lower median size (5.3 cm and 8.5 cm, respectively; P < .0001) and lower mitotic activity (median, 3 in 50 high-power fields [HPF] vs 5 in 50 HPF; P < .0001), and they were diagnosed in older patients (median age, 62 years vs 57 years; P = .002). The most commonly detected mutations in G-GIST were those located in KIT exon 11 (60.5%) and platelet-derived growth factor receptor alpha (PDGFRA) exon 18 (19%) versus KIT exons 11 and 9 in NG-GISTs (72% and 17.4%, respectively). The prognosis of patients who had G-GISTs was significantly better compared that of patients who had NG-GISTs, with 5-year DFS rates of 69% (median, 83 months) versus 43% (median, 33 months), respectively (P < .00001). The most significant prognostic factors that correlated with shorter DFS in both G-GISTs and NG-GISTs were primary tumor size >5 cm and >10 cm (P < .0001) and mitotic index >5 in 50 HPF and >10 in 50 HPF (P < .0001). The 5-year DFS rates in G-GISTs according to AJCC stage categories were as follows: 96% for stage IA tumors, 92% for stage IB tumors, 51% for II tumors, 22% for stage IIIA tumors, and 22% for stage IIIB tumors (P < .0001). The 5-year DFS rates in NG-GISTs according to AJCC categories were as follows: 92% for stage I tumors, 66% for stage II tumors, 28% for IIIA tumors, and 16% for IIIB tumors (P < .0001). The high prognostic significance of the AJCC classification also was confirmed for overall survival data, including the impact of therapy with tyrosine kinase inhibitors. CONCLUSIONS: The reliability of AJCC risk classification after resection of primary GIST was confirmed for DFS and overall survival. Patients with primary G-GISTs had a better prognosis than patients with NG-GISTs. In both groups, primary tumor size and mitotic activity were the most important prognostic factors in terms of DFS.

Benign fibrous histiocytoma of the skin metastasizing to the inguinal lymph node.

Benign fibrous histiocytoma (FH, dermatofibroma) is a common skin lesion but its metastasizing variant is extremely rare and only a few cases have been reported to date. The usual sites of metastases include locoregional lymph nodes and lung. In the majority of cases, the clinical course is indolent. At present, there are no reliable clinical or histological features of the primary tumour that could predict the risk of locoregional or distant metastases. Authors describe a case of metastasizing FH and briefly review available data.

Squamous cell carcinoma antigen 1 and 2 expression in cultured normal peripheral blood mononuclear cells and in vulvar squamous cell carcinoma.

Squamous cell carcinoma antigen (SCCA) is expressed in normal squamous cell epithelia and in squamous cell carcinomas (SCC). Two nearly identical genes encode the inhibitory serpins SCCA1 (SERPINB3) and SCCA2 (SERPINB4). Serum levels of SCCA are elevated in patients with benign skin diseases and in patients with SCC. SCCA, used for the monitoring of SCC patients, presents no satisfactory diagnostic specificity. As we have shown previously, the reverse transcription polymerase chain reaction (RT-PCR)-based SCCA messenger RNA (mRNA) testing aimed at detecting disseminated cancer cells may be hampered by the false-positive results due to SCCA expression in activated peripheral blood mononuclear cells (PBMC). The aim of this study was to assess the expression of SCCA at mRNA and protein levels in cultured normal PBMC, compared to that in vulvar SCC (VSCC) samples. High SCCA concentrations were found in vulvar tumours and in metastatic lymph nodes, while negative inguinal lymph nodes from the same patients often presented significantly less SCCA. In normal activated PBMC, the level of SCCA protein was the lowest. At the mRNA level SCCA was detectable in normal PBMC even in cultures with no mitogen stimulation, but only by the nested RT-PCR, contrary to VSCC samples found to be SCCA positive already in one-step PCR. Both SCCA1 and SCCA2 transcripts were present in cultured PBMC; SCCA1 was expressed at a higher level than SCCA2. In conclusion, both SCCA forms are detectable in normal PBMC cultured in vitro. SCCA expression level in normal PBMC is much lower than in the squamous epithelium-derived cells. In VSCC, in addition to tumour itself, metastatic lymph nodes seem also to be a potential source of serum SCCA.

HAX-1 overexpression, splicing and cellular localization in tumors.

BACKGROUND: HAX-1 has been described as a protein potentially involved in carcinogenesis and especially metastasis. Its involvement in regulation of apoptosis and cell migration along with some data indicating its overexpression in cancer cell lines and tumors suggests that HAX-1 may play a role in neoplastic transformation. Here we present the first systematic analysis of HAX-1 expression in several solid tumors. METHODS: Using quantitative RT-PCR, we have determined the mRNA levels of HAX1 splice variant I in several solid tumors. We have also analyzed by semiquantitative and quantitative RT-PCR the expression of five HAX-1 splice variants in breast cancer samples and in normal tissue from the same individuals. Quantitative PCR was also employed to analyze the effect of estrogen on HAX1 expression in breast cancer cell line. Immunohistochemical analysis of HAX-1 was performed on normal and breast cancer samples. RESULTS: The results reveal statistically important HAX1 up-regulation in breast cancer, lung cancer and melanoma, along with some minor variations in the splicing pattern. HAX-1 up-regulation in breast cancer samples was confirmed by immunohistochemical analysis, which also revealed an intriguing HAX-1 localization in the nuclei of the tumor cells, associated with strong ER status. CONCLUSION: HAX-1 elevated levels in cancer tissues point to its involvement in neoplastic transformation, especially in breast cancer. The connection between HAX-1 nuclear location and ER status in breast cancer samples remains to be clarified.

The frequency of human papillomavirus infection in polish patients with vulvar squamous cell carcinoma.

INTRODUCTION: Vulvar cancer is a rare condition representing about 4% of all female genital tract tumors. In contrast to the established relationship of virtually all cervical cancer cases with the human papillomavirus (HPV) infection, the reported HPV positivity in vulvar carcinoma ranges widely. METHODS: Using the Linear Array HPV Genotyping Test, we investigated the HPV incidence in a group of 46 Polish patients with vulvar squamous cell carcinoma (age range, 37-93 years; median age, 70.2 years) in clinical stages T1-2, N0-2, and M0. RESULTS: The presence of HPV DNA was confirmed in 7 of 46 (15%) primary tumor samples. HPV 16 was found in 5 tumors (71%). HPVs 6 and 58 were detected in the remaining 2 cases of virus-associated tumors. CONCLUSIONS: We conclude that a fraction of cancers of vulva associated with HPV is insignificant, given the HPV prevalence of 8.6% in the Polish population aged 55 to 59 years (the oldest cohort of Polish women studied to date).

Differential diagnosis of small round cell tumours (SRCT), fluorescence in situ hybridization (FISH) and immunohistochemical (IHC) study.

INTRODUCTION: Small round cell tumours (SRCT) of bone and soft tissue constitute a heterogeneous group of neoplasms with similar histological and cytological features. Immunohistochemical studies with panels of antibodies are necessary in order to make the diagnosis. A molecular testing is helpful in many cases. AIM OF THE STUDY: To assess the value of IHC and FISH tests in the differential diagnosis of SRCT. MATERIAL AND METHODS: The material was obtained from patients diagnosed and treated at the Maria Sklodowska-Curie Memorial Cancer Center-Institute in Warsaw between February 2003 and March 2009. One hundred and thirty one patients with the initial diagnosis of SRCT of bone or soft tissue were qualified to the investigation. The material from the primary tumour was obtained by an open or core biopsy in all the patients. During the treatment the patients were monitored, the local recurrence and the distant metastases were reported. The IHC study was performed routinely using wide panels of antibodies. FISH tests: EWSR1, SS18 (SYT), FKHR (F0X01A) and FUS were carried out using dual colour, break-apart probes. RESULTS: IHC tests for CD99 and FLI-1 showed low specificity, had low sensitivity, myogenin staining revealed high specificity and sensitivity. A "lymphoma" panel with LCA, CD20, CD79a, TdT, CD3 showed acceptable specificity and sensitivity. There were 28 (21.37%) uninformative FISH results showing no acceptable signals. CONCLUSIONS: Diagnostic assessment of SRCT requires IHC studies as an introductory method. FISH is necessary in many cases of SRCT for the final diagnosis but it requires well-fixed and processed tissue, otherwise there is a high percentage of uninformative results. A diagnostic algorithm including IHC and FISH tests has been proposed.

Oncogenic osteomalacia associated with phosphaturic mesenchymal tumour, mixed connective tissue type of the knee.

One of the most unusual and uncommon types of osteomalacia is the oncogenic osteomalacia that is predominantly caused by a soft tissue or bone tumour, mostly by a phosphaturic mesenchymal tumour, mixed connective tissue type (PMTMCT). We report a case of a 27-year-old male presented with complaints of progressive and generalized muscle weakness, bone pains and multiple fractures. Intra-articular PMTMCT of the knee was diagnosed and surgically removed. We describe histopathological features of PMTMCT and review the most recent studies concerning this diagnostic problem.

Her2, EGFR and TOPIIA gene amplification and protein expression in synovial sarcoma before and after combined treatment.

Synovial sarcoma (SyS) occurs mostly in young adults and is characterized by an aggressive course. Combined treatment including chemotherapy, radiotherapy and surgical excision of the tumour is still not satisfactory, with mean 5-year survival of 30-50%. New targeted treatment options have appeared recently, e.g. HER2 and EGFR antagonists. Initial studies have revealed immunohistochemical overexpression of the EGFR in SyS; therefore trials with EGFR antagonist therapy have commenced. The aim of our study was to evaluate the status of HER2, EGFR and TOPIIA in SyS before and after combined therapy. Immunohistochemistry and FISH tests were performed. Significant discrepancies between protein expression and gene status were found. The authors discuss the potential reasons for that phenomenon.

Epidermal growth factor receptor (EGFR) status in chordoma.

Chordoma is a rare tumour arising from the embryonal remnants of a notochord occurring most commonly in the sacrococcygeal as well as head and neck locations. Current treatment includes surgery and/or proton beam radiotherapy. In several cases especially in the head and neck location, surgery is not advised. Proton beam therapy is not always effective enough to eradicate the tumour. Additional modes of therapy are needed. One of the current therapeutic approaches in various tumours is targeted therapy and one of the targets is EGFR. The aim of this study was to evaluate EGFR expression and EGFR gene status of chordoma. Twenty-one cases of chordoma were retrieved from the in-house and consultation files of the Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology in Warsaw. Immunohistochemistry with an anti-EGFR antibody and FISH was performed on slides obtained from representative archival paraffin blocks. In our study 81% of cases of chordoma showed low to high EGFR expression in immunohistochemistry. In six cases (26.6%) the FISH results for EGFR were classified as positive (an average EGFR copy number > or = 4 per cell). There was one case of chromosome 7 aneuploidy reported.