Metastatic incidence of (PET)CT positive lung hilar and retroperitoneal lymph nodes in esophageal cancer patients

BackgroundExtra-regional lymph node metastases strongly determine treatment options in patients with esophageal cancer. Staging modalities such as (FDG-PET) CT scanning frequently show activity in retroperitoneal and lung hilar lymph nodes. This study evaluated the incidence of histologically confirmed metastases, treatment approach and recurrence patterns in patients with (FDG-PET) CT positivity in these regions.MethodsAll patients with (FDG-PET-) CT positive hilar and/or retroperitoneal lymph nodes at primary staging or restaging discussed at a multidisciplinary tumor board meeting for staging of esophageal cancer between January 2012–December 2017 were included. Biopsies and follow-up were evaluated to determine the presence of metastases and progression rates.ResultsFrom 2012 to 2017, 65 of 857 patients (7.6%) were selected with positive retroperitoneal and/or hilar lymph nodes. A total of 47/65 (72.3%) patients had positive retroperitoneal lymph nodes, which contained metastases in 19 (29.2%). When no biopsy was performed and curative treatment was given (n = 14), 9 patients had progression or locoregional and distant recurrence. Positive hilar lymph nodes were identified in 21 (32.3%) patients; 4 were biopsied and none contained metastases. In these patients no recurrence of disease was seen during follow-up.ConclusionsThe majority of biopsied (PET)CT-positive retroperitoneal lymph nodes at staging contained metastases, while biopsied (PET)CT-positive hilar nodes did not. Histological evaluation of (PET)CT -positive retroperitoneal lymph nodes at staging imaging is recommended, while based on this small series, (PET)CT-positive hilar lymph nodes most likely represent reactive lymphadenopathy.     

Expression of intercellular adhesion molecule-3 (ICAM-3/CD50) in malignant lymphoproliferative disorders and solid tumors

ICAM-3/CD50 is a recently described LFA-1 counter receptor that seems to play an important role in the initiation of immune responses. In this study we have examined the expression of ICAM-3/CD50 in a large series of human neoplasms including 101 Non-Hodgkin's lymphomas (NHL), 26 Hodgkin's disease, and 38 solid tumors to define the distribution patterns of this molecule in malignant neoplasms and their possible correlation with clinical and pathological characteristics of the patients. In NHL, ICAM-3/CD50 was expressed in almost all the tumors with a tendency to be lost in high grade lymphomas. Reed-Sternberg cells and their variants in Hodgkin's disease were always negative independently of the histological subtype of the disease. No expression was observed in tumor epithelial cells of the 38 solid tumors examined. Strong endothelial cell staining was observed in 31% of the NHL and 31% of Hodgkin's disease. ICAM-3 expression in these cases was restricted to small tumor vessels. ICAM-3 expression in endothelial cells of NHL was significantly more frequent in high grade (40%) than in low grade lymphomas (14%) (p=0.012). In addition, tumor vessels were also positive in 29% of solid rumors independently of the histological type. No correlation was observed between ICAM-3 expression in tumor or endothelial cells and other clinical and pathological characteristics of the patients. These findings indicate that ICAM-3 expression in human tumors is restricted to hematological neoplasms with a tendency to be lost in high grade lymphomas and Hodgkin's disease. ICAM-3 is also expressed by endothelial cells from tumor-associated neovascularization in both lymphoid and solid tumors.     

Henipavirus Encephalitis: Recent Developments and Advances

The genus Henipavirus within the family Paramyxoviridae includes the Hendra virus (HeV) and Nipah virus (NiV) which were discovered in the 1990s in Australia and Malaysia, respectively, after emerging to cause severe and often fatal outbreaks in humans and animals. While HeV is confined to Australia, more recent NiV outbreaks have been reported in Bangladesh, India and the Philippines. The clinical manifestations of both henipaviruses in humans appear similar, with a predominance of an acute encephalitic syndrome. Likewise, the pathological features are similar and characterized by disseminated, multi‐organ vasculopathy comprising endothelial infection/ulceration, vasculitis, vasculitis‐induced thrombosis/occlusion, parenchymal ischemia/microinfarction, and parenchymal cell infection in the central nervous system (CNS), lung, kidney and other major organs. This unique dual pathogenetic mechanism of vasculitis‐induced microinfarction and neuronal infection causes severe tissue damage in the CNS. Both viruses can also cause relapsing encephalitis months and years after the acute infection. Many animal models studied to date have largely confirmed the pathology of henipavirus infection, and provided the means to test new therapeutic agents and vaccines. As the bat is the natural host of henipaviruses and has worldwide distribution, spillover events into human populations are expected to occur in the future.     

Von Willebrand's Syndrome Presenting as an Acquired Bleeding Disorder in Association With a Monoclonal Gammopathy

The clinical and laboratory findings of apatient with a bleeding disorder, laboratoryfeatures of von Willebrand's disease, and amonoclonal gammopathy are described.There was no evidence of von Willebrand'sdisease in his five children. Laboratory features of von Willebrand's disease in thepatient included a long bleeding time, lowfactor VIII level by one- and two-stageclotting assays and by immunoassay, decreased platelet glass adhesiveness usingnative and heparinized blood, and correction of platelet adhesiveness by additionof cryoprecipitate in vitro. Attempts to implicate the monoclonal IgG in the pathogenesis of the von Willebrand's syndromewere unsuccessful using the followingtests: immunoprecipitation with normalplasma, antibody activity identified bypassive cutaneous anaphylaxis, inhibitionof biological factor VIII activity, and binding to factor VIII. Despite these negativefindings, it is suggested that the clinicalpattern of this and previously describedcases of "acquired" von Willebrand's disease has an immunologic basis that maybe mediated by either humoral or cellularmechanisms.

Submitted on September 26, 1972 Revised on February 8, 1973 Accepted on February 9, 1973     

Pilocytic astrocytomas of the posterior fossa a follow-up study in 33 patients

Summary The extent of resection in pilocytic astrocytoma of the posterior fossa often remains undefined and the indications for further treatment in incompletely resected tumours are a matter of debate. It has been also realized that the problem of hydrocephalus in patients with pilocytic astrocytoma of the posterior fossa has not yet been solved and the diagnostic impact of postoperative CT findings remains questionable.We retrospectively reviewed the data from 33 patients harbouring a pilocytic astrocytoma of the posterior fossa to evaluate the impact of surgical technique in terms of radicality and of postoperative imaging results upon prognosis and adjunctive treatment. In addition, the issue of hydrocephalus was considered and related to different treatment modalities.Thirty patients underwent surgical treatment whereas 3 had open biopsy of the tumour. Macroscopically gross total resection of the tumour was performed in 20 patients, whereas resection was partial in 10.Follow-up was obtained in 29 patients for a period which ranged between 2 and 184 months (85 months ±56 months). Outcome was good in 24 patients who had only slight neurological deficit and poor in 3 patients, who were severly disabled. Two patients died during the follow-up period. Recurrent tumour growth occurred in 2 cases with incompletely resected tumours.From the series presented, it was concluded that long-term follow-up with CT seems mandatory in cases with contrast-enhancing residual tumour. Recurrent tumour growth should be assumed in postsurgical patients with an enlarging area of enhancement shown in follow-up CT studies. Permanent ventriculoperitoneal shunting is required in certain patients with pre- or postoperative hydrocephalus. While percutaneous irradiation treatment is not curative, the role of radiosurgery has to be defined in the future.     

Lymphocytic vasculitis in X-linked lymphoproliferative disease

Systemic vasculitis is an uncommon manifestation of X-linkedlymphoproliferative disease (XLP), a disorder in which there is aselective immune deficiency to Epstein-Barr virus (EBV). The molecularbasis for XLP has recently been ascribed to mutations withinSLAM-associated protein (SAP), an SH2 domain-containing proteinexpressed primarily in T cells. The authors describe a patient who diedas a result of chronic systemic vasculitis and fulfilled clinicalcriteria for the diagnosis of XLP. Sequencing of this patient'sSAP gene uncovered a novel point mutation affecting the SH2domain. The patient presented with virus-associated hemophagocytic syndrome (VAHS) and later had chorioretinitis, bronchiectasis, andhypogammaglobulinemia develop. He further developed mononeuritis andfatal respiratory failure. Evidence of widespread small and mediumvessel vasculitis was noted at autopsy with involvement of retinal,cerebral, and coronary arteries as well as the segmental vessels of thekidneys, testes, and pancreas. Immunohistochemical analysis usingantibodies to CD20, CD45RO, and CD8 revealed that the vessel wallinfiltrates consisted primarily of CD8⁺ T cells,implying a cytotoxic T-lymphocyte response to antigen. EBV DNA wasdetected by polymerase chain reaction (PCR) in arterial wall tissuemicrodissected from infiltrated vessels further suggesting that theCD8⁺ T cells were targeting EBV antigens within theendothelium. The authors propose that functional inactivation of theSAP protein can impair the immunologic response to EBV, resulting insystemic vasculitis.     

Malignant Rhabdoid Tumor Presenting as a Hemangioma

Abstract: Malignant rhabdoid tumor is a rare and highly aggressive malignancy of unknown etiology. We report a primary cutaneous rhabdoid tumor on the upper back of a newborn. It was initially diagnosed as a hemangioma clinically, and that diagnosis was supported by radiologic studies, including magnetic resonance imaging. However, detailed investigation with light microscopy, immunohistochemistry, and electron microscopy enabled us to make a diagnosis of rhabdoid tumor. This is the seventh reported case of rhabdoid tumor with primary cutaneous involvement.