A durable complete response to immunotherapy in a patient with metastatic alveolar soft part sarcoma.

BACKGROUND: Recent years have brought the dynamic development of a new method of cancer treatment: immunotherapy. Monoclonal antibodies blocking the programmed death receptor 1 (PD-1) are now widely used in the treatment of several malignancies: melanoma, lung, head and neck cancer, among others. The therapeutic benefit of immunotherapy in soft tissue sarcoma (STS) has not yet been proven. The exception is results obtained in the treatment of a rare STS subtype alveolar soft part sarcoma (ASPS). CASE REPORT: We describe a case of a man with a diagnosis of metastatic ASPS in whom the use of immunotherapy with nivolumab resulted in excellent long-term clinical benefit and a pathologically confirmed complete response. CONCLUSION: There are strong indications that immunotherapy may become the next important treatment method of ASPS.

Primary mediastinal neoplasms: a report of 1,005 cases from a single institution.

BACKGROUND: This study was an epidemiological analysis of all primary mediastinal neoplasms (PMNs) diagnosed between 2000 and 2016 at the National Tuberculosis and Lung Diseases Research Institute, Poland. METHODS: All patients with any mediastinal abnormality were included in the analysis. The patients' age and gender were obtained from the institutional database. RESULTS: From a cohort of 5,108 patients, 3,691 primary mediastinal lesions were found, including 1,005 (19%) PMNs: lymphomas (533, 53% of PMNs), thymomas (215, 21%), neurogenic tumors (NTs) (100, 10%), germ cell tumors (GCTs) (62, 6%), soft tissue tumors (STTs) (47, 5%) and thymic carcinomas/thymic neuroendocrine tumors (TCs/TNETs) (37 in total, 4%). The most frequent lymphomas were classical Hodgkin lymphomas [256] and primary mediastinal large B-cell lymphomas [163]. Type AB [73] predominated in thymomas and squamous cell carcinomas [9] and carcinoids [10] in TCs/TNETs. NTs encompassed mainly schwannomas [49], ganglioneuromas [21] and neurofibromas [10]. The most frequent STTs were hemangiomas [13] and lymphangiomas [11]. Lymphomas, thymomas and NT were more often in women, TCs/TNETs in men (P<0.001). Lymphomas predominated between the 2nd and 4th decade of life, NTs under the 3rd decade and thymic epithelial tumors between the 6th and 8th decade (P<0.001). There was no correlation between the subtypes of thymomas and the patients' gender (P=0.389) but it was found between histology and patients' age: in patients <30 years of age type B2 and B3 thymomas and >70 years of age AB type and micronodular thymomas with lymphoid stroma (P<0.001) predominated. In the group of GCTs half of them were malignant and these were noted exclusively in men. No correlation between subtypes of NTs or TCs/TNETs and patients' age and gender was found (P>0.05). CONCLUSIONS: PMNs are rare conditions thus awareness of basic epidemiology may be very helpful for final diagnosis.

D2-40 Antibody is a Specific Marker for B2 Thymomas.

The correlation between the worse outcome of thymomas and expression of podoplanin (D2-40 antibody) in neoplastic cells has been proved in recent studies. We investigated the expression of podoplanin in thymic epithelial tumors of different histologic types and stages resected in our institution. The presence and type of reaction (membranous or cytoplasmic) with D2-40 antibody were assessed. Analyzed group consisted of 72 tumors: 3 type A, 19 type AB, 5 type B1, 24 type B2, 4 type B3, 2 micronodular, 1 metaplastic, and 9 combined B2B3 thymomas and 5 thymic carcinomas. Positive reaction with D2-40 was detected in 7 cases (37%) of AB type, 2 (40%) of B1, 28 (85%) of B2, 3 (23%) of B3, and 1 case (100%) of metaplastic thymoma. All positive B2 and 2 cases of B3 thymomas revealed membranous type of reaction, whereas other subtypes showed less conspicuous cytoplasmic reactivity. A correlation between B2 thymoma and membranous type of reaction was statistically significant (P<0.0001). There was also a slight relationship between cytoplasmic type of reaction and AB or B1 thymomas (P=0.0256). No correlation was detected between D2-40-reactivity and stage (P=0.4) or myasthenic symptoms (P=0.21). In conclusion, membranous type of reaction with D2-40 antibody in thymomas is highly specific for B2 subtype, but antipodoplanin immunoreactivity has no relationship with the tumor stage.

Organizing pneumonia appearing in B-cell chronic leukemia malignancy progression - a case report.

Patients with chronic lymphocytic leukemia or non-Hodgkin's lymphoma are at risk of infectious diseases of respiratory system because of immunodeficiency. Occurrence of organizing pneumonia in leukemic patients is most commonly correlated with bone marrow transplant or treatment with antimitotic agents. There have been only four reported cases of organizing pneumonia related solitarily to leukemia or lymphoma. We present a case of 65-year old gentlemen, diagnosed 8 months earlier with B-cell chronic lymphocytic leukemia with no previous hematologic treatment, who presented symptoms of persistent pneumonia with no significant reaction to antibiotics. Chest computed tomography scans showed well-localized consolidation with ground glass opacities and some air bronchogram, suggesting infectious disease. All results of microbiological examinations were negative. Due to radiological progression of parenchymal consolidation despite two intravenous courses of antibiotics open lung biopsy was performed. The histologic examination of lung specimen revealed structures typical for organizing pneumonia pattern. There was no evidence for leukemic involvement in lung tissue, as no sign for infectious factors from histological staining was observed. In the inferior mediastinal lymph node sample progression of chronic lymphatic leukemia to mixed cell lymphoma was diagnosed. Patient was commenced on prednisone 60 mg/daily with fast improvement. We believe that this is the first case of organizing pneumonia as a reaction to the conversion of B-cell chronic lymphocytic leukemia progression to more malignant stage.

[Expression of the selected adhesive molecules (cadherin E, CD44, LGAL3 and CA50) in papillary thyroid carcinoma]. / Ekspresja wybranych molekul adhezyjnych (kadheryny E, CD44, LGAL3 i CA50) w raku brodawkowatym tarczycy.

INTRODUCTION: The aim of the study was to determine the expression of selected adhesive molecules in papillary thyroid carcinoma. MATERIAL AND METHODS: 47 papillary thyroid carcinoma cases and 11 nonmalignant goiter cases were analyzed by immunohistochemistry. RESULTS: Galectin-3 (LGAL3) was a sensitive and specific marker, present in 91% of analyzed tumors and only in 5% of tumor margin. The presence of CA50 was 86% and 3% respectively with only 3% positive non-malignant cases. Cadherin E expression was noted in 91% of primary tumors, in 84% of the surrounding tissue and in 63% of non-malignant goiter. CD44 (DF1485) was observed in 89% of primary tumors and 48% of surrounding tissue; the reaction with BBA10 was more characteristic for metastases. CONCLUSIONS: Our study confirms the high diagnostic value of galectin-3 in papillary thyroid carcinoma and reveals the similar efficiency of CA50. CD44 (DF1485) expression in primary tumor is more intensive than in surrounding tissue, but the diagnostical inportance is not high because it is often observed in benign lesions. Using of BBA10 is more sensitive, but less specific. High expression of cadherin E in benign lesions impairs its diagnostical application in papillary thyroid cancer.

Diagnostic problems concerning epithelioid sarcoma--case analysis.

We discuss here five cases of epithelioid sarcoma (ES) with final diagnosis established after reexamination of initial findings. Problems with differential diagnosis of these neoplasms arise since their microscopic picture may simulate several other pathological conditions such as non-neoplastic granulomatous reactions, squamous cell carcinomas and adenocarcinomas, melanomas and soft tissue sarcomas with epithelioid component. Final ES diagnosis requires presence of cytokeratin, EMA and vimentin in neoplastic cells, as confirmed by immunohistochemical reactions. Differential diagnosis is also helped by concurrent cytology assessment that allows recognizing more easily such characteristic features as presence of plasmacytoid or spindle-shaped cells.

Primary undifferentiated small cell carcinoma (Merkel cell carcinoma) in a patient after heart transplantation--case report.

Here we report a case of a 48-year-old man after heart transplantation in whom two years after the procedure an appendage germ tumour was diagnosed and a year after its removal in the same area a primary undifferentiated small cell carcinoma (Merkel cell carcinoma) with metastases to the axillary lymph nodes was detected. The diagnosis of Merkel cell carcinoma was confirmed by immunohistochemical staining showing a typical perinuclear expression of cytokeratin 20 with the absence of reactivity with wide spectrum cytokeratin and the presence of neuroendocrine markers of neurone-specific enolase and chromogranin. Primary undifferentiated small cell carcinoma occurs more frequently in patients receiving immunosuppressive treatment as compared with a general population and occurs much more frequently in those below 50 years of age.