No abstract available.
Reticulin ; Autoantibodies ; Collagen ; Neoplasms ; Fluorescent Antibody Technique, Indirect ; Neoplasms, Second Primary ; Antibodies, Antinuclear ; Adenocarcinoma ; Carcinoma, Transitional Cell ; Urinary Bladder Neoplasms

MPL mutation is an important molecular marker in myeloproliferative neoplasms (MPN). Although MPL W515 is a hot spot for missense mutations in MPN, MPL S505 mutations have been reported in both familial and non-familial MPN. A 72-year-old male visited the hospital, complaining mainly of dizziness and epistaxis. Leukocytosis, anemia, thrombocytopenia, tear drop cells, nucleated RBCs, and myeloblasts were observed in both complete blood cell counts and peripheral blood smears. Bone marrow aspiration failed due to dilution with peripheral blood. BM biopsy indicated hypercellular marrow, megakaryocytic proliferation with atypia, and grade 3 reticulin fibrosis. Conventional cytogenetics results were as follows: 46,XY,del(13)(q12q22)[19]/46,XY[1]. Molecular studies did not detect JAK2 V617F, BCR/ABL translocation, JAK2 exon 12, and CALR exon 9 mutations. The MPL S505N mutation was verified by colony PCR and Sanger sequencing following gene cloning. Based on the above findings, a diagnosis of overt primary myelofibrosis (PMF) was indicated. Mutation studies of buccal and T cells were not conducted. Further, family members were not subjected to mutation studies. Therefore, we were unable to determine whether this mutation was familial or non-familial. Six months after the first visit to the hospital, the patient died due to pneumonia and sepsis. Thrombotic symptoms or major bleeding events did not develop during the survival period following diagnosis of PMF. To the best of our knowledge, this may be the first reported case of PMF with the MPL S505N mutation in Korea.
Aged ; Anemia ; Biopsy ; Blood Cell Count ; Bone Marrow ; Clone Cells ; Cloning, Organism ; Cytogenetics ; Diagnosis ; Dizziness ; Epistaxis ; Exons ; Fibrosis ; Granulocyte Precursor Cells ; Hemorrhage ; Humans ; Korea* ; Leukocytosis ; Male ; Mutation, Missense ; Pneumonia ; Polymerase Chain Reaction ; Primary Myelofibrosis* ; Reticulin ; Sepsis ; T-Lymphocytes ; Tears ; Thrombocytopenia

The aim of this study is to describe rare variants of adrenocortical carcinoma (ACC) and to compare the prognosis with that of conventional ACC. We retrospectively reviewed 8 cases of myxoid variant, 1 sarcomatoid variant, and 14 cases of conventional ACC, who underwent surgical resection at the Asan Medical Center between 1996 and 2014. An analysis of the clinicopathological characteristics, including the Weiss score, Ki-67 labeling index, and reticulin framework assessment is presented. The mean age of patients with myxoid/sarcomatoid ACC was 45 years; 4 out of 9 patients were women. Mean primary tumor size was 12.9 cm and the mean weight was 702.4 g. Seven patients presented in an advanced stage (stage III/IV); 8 of these eventually developed distant metastasis. The mean Weiss score was 5.0 points and the Ki-67 labeling index was 15.6%. The extent of myxoid or sarcomatoid change on histological examination ranged from 10% to 75% of the examined tumor areas; reticulin framework alteration was observed in all cases. Four patients showed venous tumor thrombus. Most of the clinicopathological parameters were not significantly different from those of conventional ACC. However, myxoid or sarcomatoid variant (hazard ratios [HR], 3.59; 95% confidence intervals [CI], 1.13–11.38; P = 0.030) and Ki-67 labeling index (HR, 3.97; 95% CI, 1.18–13.41; P = 0.030) were independent predictors of overall survival after adjusting for age and sex. Myxoid or sarcomatoid histological features or an increased Ki-67 labeling index may be associated with poor overall survival in patients with ACC.
Adrenocortical Carcinoma* ; Chungcheongnam-do ; Female ; Humans ; Neoplasm Metastasis ; Prognosis ; Reticulin ; Retrospective Studies ; Tertiary Care Centers* ; Tertiary Healthcare* ; Thrombosis

We report a 50-year-old woman with no relevant clinical history who presented with headache and loss of memory. Magnetic resonance imaging showed a left parieto-temporal mass with annular enhancement after contrast media administration, rendering a radiological diagnosis of high-grade astrocytic neoplasm. Tumour sampling was performed but the patient ultimately died as a result of disease. Microscopically, the lesion had areas of glioblastoma mixed with a benign mesenchymal constituent; the former showed hypercellularity, endothelial proliferation, high mitotic activity and necrosis, while the latter showed fascicles of long spindle cells surrounded by collagen and reticulin fibers. With approximately 40 previously reported cases, gliofibroma is a rare neoplasm defined as either glio-desmoplastic or glial/benign mesenchymal. As shown in our case, its prognosis is apparently determined by the degree of anaplasia of the glial component.
Anaplasia ; Collagen ; Contrast Media ; Diagnosis ; Female ; Glioblastoma ; Headache ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Necrosis ; Prognosis ; Reticulin

Hepatic sinusoidal dilatation is a rare benign vascular disorder characterized by focal dilatation of the sinusoidal spaces. In most cases, the underlying etiology is unclear but it may be related to the impairment of venous outflow or sinusoidal infiltration by diverse causes. Diagnosing hepatic sinusoidal dilatation based soley on imaging study is not easy since there are no pathognomonic radiologic findings indicative of this condition. Recently, the authors experience two cases of hepatic sinusoidal infiltration. The first patient was a 53-year-old man detected to have multiple hepatic nodules on ultrasonography (US) during a routine medical check-up. The second patient was an 82-year-old woman with abdominal discomfort who was referred from local clinic with high suspicion of hepatic metastases on US. In both cases, CT scan demonstrated multiple nodules with rim enhancement on arterial phase that became iso-dense to adjacent liver parenchyma on delayed phase. On MRI, these nodules showed rim enhancement on arterial phase, had high signal intensity on T2 weighted images, and became iso-intense with partial defect on hepatobiliary phase. Because imaging studies could not exclude the presence of hepatic metastases, liver biopsy was performed and it demonstrated hepatic sinusoidal dilatation with well preserved reticulin fiber without any evidence of malignancy. Herein, we report two cases of idiopathic hepatic sinusoidal dilatation mimicking hepatic metastases.
Aged, 80 and over ; Biopsy ; Dilatation* ; Female ; Humans ; Liver ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis* ; Reticulin ; Tomography, X-Ray Computed ; Ultrasonography

This study was purposed to explore the correlation of chromosome karyotype with dyshaematopoiesis and reticulin in myelodysplastic syndrome (MDS). The data of 202 MDS patients diagnosed and treated in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed in term of chromosome karyotype, dyshaematopoiesis and reticulin detection results. The chromosome karyotypes were categorized according to the International Prognostic Scoring System (IPSS). The results showed that there was a positive correlation between chromosome karyotype grading and number of lineages with dyshaematopoiesis (r = 0.443, P < 0.05). The detected rates of multilineage dyshaematopoiesis in patients with good, intermediate and poor chromosome karyotypes were 44.4%, 71.4% and 96.3% respectively. There was a positive correlation between chromosome karyotype grading and reticulin grading (r = 0.451, P < 0.05). The positive rates of reticulin in patients with good grading, intermediate and poor chromosome karyotypes were 36.8%, 64.3% and 92.6% respectively. The detected rate of multilineage dyshaematopoiesis, number of lineages with dyshaematopoiesis, the positive rate of reticulin and reticulin grade in patients with poor karyotypes were higher than those in patients with intermediate or good chromosome karyotypes (separately P < 0.01). The above data in patients with intermediate chromosome karyotypes were higher than those in patients with good chromosome karyotypes (separately P < 0.01). It is concluded that the chromosome karyotype grading positively correlates with the number of lineages with dyshaematopoiesis and reticulin grading. When the chromosome karyotype changed from good to poor, the detected rate of multilineage dyshaematopoiesis, number of lineages with dyshaematopoiesis, positive rate of reticulin and reticulin grading became higher and higher.
Adolescent ; Adult ; Aged ; Bone Marrow Examination ; Female ; Humans ; Karyotype ; Karyotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; diagnosis ; genetics ; pathology ; Reticulin ; analysis ; Retrospective Studies ; Young Adult

No abstract available.
Antineoplastic Agents/therapeutic use ; Bone Marrow Cells/pathology ; Cladribine/therapeutic use ; DNA Mutational Analysis ; Female ; Humans ; Immunophenotyping ; Leukemia, Hairy Cell/*diagnosis/drug therapy/*genetics ; Middle Aged ; *Mutation ; Proto-Oncogene Proteins B-raf/*genetics ; Reticulin/metabolism

Myelofibrosis is a myeloproliferative neoplasm characterized by abnormal bone marrow megakaryocyte proliferation with reticulin and collagen fibrosis, leukoerythroblastosis, anemia, increased level of serum lactate dehydrogenase and splenomegaly. Myelofibrosis associated with malignant lymphoma is rare and survival rates appear to have been poor. Herein, we describe our experience in a patient who remained in complete remission with high-dose therapy (HDT) with autologous peripheral blood stem cell transplantation (PBSCT) for ALK-negative ALCL presenting with rapidly progressing myelofibrosis.
Anemia ; Bone Marrow ; Collagen ; Fibrosis ; Humans ; L-Lactate Dehydrogenase ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Megakaryocytes ; Peripheral Blood Stem Cell Transplantation ; Primary Myelofibrosis ; Reticulin ; Splenomegaly ; Stem Cell Transplantation ; Survival Rate

Actins ; analysis ; Animals ; Dimethylnitrosamine ; Immunohistochemistry ; Liver ; metabolism ; pathology ; Male ; Necrosis ; chemically induced ; pathology ; Rats ; Rats, Wistar ; Reticulin ; analysis ; Silver Staining

A solitary cutaneous myxoma, referred to as superficial angiomyxomas, is relatively uncommon benign myxoid soft tissue tumors. The lesion usually present on the head, neck, and trunk of adults as a slowly growing asymptomatic nodule. Herein, we describe a case of a pedunculated solitary cutaneous myxoma arising on the vertex of a 48-year-old man, but with no evidence of Carney syndrome. Histologically, the tumor was composed of vascular, mucinous matrix with a network of fine collagen and reticulin fibers, containing stellate and spindle-shaped fibroblasts. There was no evidence of malignancy
Adult ; Carney Complex ; Collagen ; Fibroblasts ; Head ; Humans ; Middle Aged ; Mucins ; Myxoma* ; Neck ; Reticulin