Background/Aims: We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). Methods: We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. Results: The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. Conclusions Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

BACKGROUND AND PURPOSE: There are three distinct subtypes of primary progressive aphasia (PPA): the nonfluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). We sought to characterize the pattern of [¹⁸F]-THK5351 retention across all three subtypes and determine the topography of [¹⁸F]-THK5351 retention correlated with each neurolinguistic score. METHODS: We enrolled 50 participants, comprising 13 PPA patients (3 nfvPPA, 5 svPPA, and 5 lvPPA) and 37 subjects with normal cognition (NC) who underwent 3.0-tesla magnetic resonance imaging, [¹⁸F]-THK5351 positron-emission tomography scans, and detailed neuropsychological tests. The PPA patients additionally participated in extensive neurolinguistic tests. Voxel-wise and region-of-interest-based analyses were performed to analyze [¹⁸F]-THK5351 retention. RESULTS: The nfvPPA patients exhibited higher [¹⁸F]-THK5351 retention in the the left inferior frontal and precentral gyri. In svPPA patients, [¹⁸F]-THK5351 retention was elevated in the anteroinferior and lateral temporal cortices compared to the NC group (left>right). The lvPPA patients exhibited predominant [¹⁸F]-THK5351 retention in the inferior parietal, lateral temporal, and dorsolateral prefrontal cortices, and the precuneus (left>right). [¹⁸F]-THK5351 retention in the left inferior frontal area was associated with lower fluency scores. Comprehension was correlated with [¹⁸F]-THK5351 retention in the left temporal cortices. Repetition was associated with [¹⁸F]-THK5351 retention in the left inferior parietal and posterior temporal areas, while naming difficulty was correlated with retention in the left fusiform and temporal cortices. CONCLUSIONS: The pattern of [¹⁸F]-THK5351 retention was well matched with clinical and radiological findings for each PPA subtype, in agreement with the anatomical and functional location of each language domain.
Aphasia, Primary Progressive ; Cognition ; Comprehension ; Humans ; Magnetic Resonance Imaging ; Neurofibrillary Tangles ; Neuropsychological Tests ; Parietal Lobe ; Positron-Emission Tomography ; Prefrontal Cortex ; Rabeprazole ; Semantics ; Temporal Lobe

BACKGROUND AND PURPOSE: To analyze 18F-THK5351 positron emission tomography (PET) scans of patients with clinically diagnosed nonfluent/agrammatic variant primary progressive aphasia (navPPA). METHODS: Thirty-one participants, including those with Alzheimer's disease (AD, n=13), navPPA (n=3), and those with normal control (NC, n=15) who completed 3 Tesla magnetic resonance imaging, 18F-THK5351 PET scans, and detailed neuropsychological tests, were included. Voxel-based and region of interest (ROI)-based analyses were performed to evaluate retention of 18F-THK5351 in navPPA patients. RESULTS: In ROI-based analysis, patients with navPPA had higher levels of THK retention in the Broca's area, bilateral inferior frontal lobes, bilateral precentral gyri, and bilateral basal ganglia. Patients with navPPA showed higher levels of THK retention in bilateral frontal lobes (mainly left side) compared than NC in voxel-wise analysis. CONCLUSIONS: In our study, THK retention in navPPA patients was mainly distributed at the frontal region which was well correlated with functional-radiological distribution of navPPA. Our results suggest that tau PET imaging could be a supportive tool for diagnosis of navPPA in combination with a clinical history.
Alzheimer Disease ; Aphasia, Primary Progressive* ; Basal Ganglia ; Broca Area ; Diagnosis ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Neurofibrillary Tangles ; Neuropsychological Tests ; Positron-Emission Tomography ; Primary Progressive Nonfluent Aphasia ; tau Proteins

Retroperitoneal extraskeletal osteosarcoma is a rare tumor. Typical imaging findings include a soft tissue mass accompanied with internal calcifications. The authors encountered a case involving a 44-year-old woman with a large retroperitoneal extraskeletal osteosarcoma, without calcification, that mimicked a pancreatic tumor. The present report highlights computed tomography features of retroperitoneal extraskeletal osteosarcoma, followed by a brief literature review. It is challenging for radiologists to diagnose retroperitoneal masses. However, in patients who present with large retroperitoneal masses, combined with clinical information including the elevation of serum alkaline phosphatase levels, retroperitoneal extraskeletal osteosarcoma should be considered in the differential diagnosis, even if the mass does not exhibit a gross calcification on the imaging.

Branch duct intraductal papillary mucinous neoplasms of the pancreas (BD-IPMN) without malignant features rarely developed into invasive cancer. However, invasive cancer is aggressive once an invasive change occurs. We report three cases of invasive cancers which developed in patients with BD-IPMN and they showed grave clinical courses. All patients were diagnosed with BD-IPMN < 3 cm without malignant features on imaging. Invasive cancer was detected at 2.5 years, 3.0 years, and 4.0 years after BD-IPMN detection in each patient. The intervals of invasive cancer and the last follow-up were 9 months, 3 years, and 1.5 years in the three patients, respectively. All patients were diagnosed with locally advanced pancreas invasive cancers and were treated with palliative chemotherapy or conservative management. The patients died at 3 months, 9 months, and 10 months after the diagnosis of invasive cancers, respectively. We report three cases of invasive cancer developed in BD-IPMN patients and followed fatal courses.
Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Mucins ; Pancreas ; Pancreatic Neoplasms

Most hamartomatous polyps in the stomach occur in patients with adenomatous polyposis coli and dysplasia. The authors report a case of a 57-year-old man without prior history of polyposis coli who presented with adenocarcinomas derived from hamartomatous polyps in the stomach. The patient underwent a radical subtotal gastrectomy with Billroth I anastomosis. Pathology revealed adenocarcinomas with moderate differentiation without evidence of lymph node metastasis in 60 nodes. We report a case of gastric cancers arising from de novo gastric hamartomatous polyps.
Adenocarcinoma ; Adenomatous Polyposis Coli ; Gastrectomy ; Gastroenterostomy ; Hamartoma ; Humans ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis ; Polyps ; Stomach ; Stomach Neoplasms

The incidence of heterotopic gastric mucosa located in the submucosa in resected stomach specimens has been reported to be 3.0 to 20.1%. Heterotopic gastric mucosa is thought to be a benign disease, which rarely becomes malignant. Heterotopic gastric mucosa exists in the gastric submucosa, and gastric cancer rarely occurs in heterotopic gastric mucosa. Since tumors are located in the normal submucosa, they appear as submucosal tumors during endoscopy, and are diagnosed through endoscopic biopsies with some difficulty. For such reasons, heterotopic gastric mucosa is mistaken as gastric submucosal tumor. Recently, two cases of early gastric cancer arising from heterotopic gastric mucosa in the gastric submucosa were treated. Both cases were diagnosed as submucosal tumors based on upper gastrointestinal endoscopy, endoscopic ultrasound, and computed tomography findings, and in both cases, laparoscopic wedge resections were performed, the surgical findings of which also suggested submucosal tumors. However, pathologic assessment of the surgical specimens led to the diagnosis of well-differentiated intramucosal adenocarcinoma arising from heterotopic gastric mucosa in the gastric submucosa.
Adenocarcinoma ; Biopsy ; Endoscopy ; Endoscopy, Gastrointestinal ; Gastric Mucosa ; Incidence ; Stomach ; Stomach Neoplasms

Mesenteric fibromatosis is a monoclonal, fibroblastic proliferation arising from musculoaponeurotic structure, and it is distinctive lesions defined as a group of non-metastasizing fibroblastic tumors which has local invasion and has a high recurrence rate after the surgical excision. The main treatment modality is the surgical excision. Radiation therapy, chemotherapy, and hormone therapy are also known as useful treatments. We report our experience of a recent case of Mesenteric fibromatosis. A 62-year old female patient had undergone gastrectomy due to gastric cancer. 18 months after gastrectomy, we detected an abdominal mass. The preoperative radiologic findings were suggestive of recurrence. Exploratory laparotomy was performed and post-operative pathologic diagnosis was confirmed as fibromatosis. We report a patient with mesenteric fibromatosis that mimic recurrence after gastrectomy for gastric cancer.
Female ; Fibroblasts ; Fibroma ; Fibromatosis, Abdominal ; Gastrectomy ; Humans ; Hydrazines ; Laparotomy ; Recurrence ; Stomach Neoplasms

This study investigated freshwater fish for their current infection status with metacercariae of Clonorchis sinensis in Korea. Twenty-one species of freshwater fish (n = 677) were collected from 34 regions nationwidely from February 2007 to June 2008. They were individually examined by digestion technique. Eight species of freshwater fish from 17 different regions were recognized positive for the metacercariae of C. sinensis. The positive rates (range of metacercariae number per fish) of fish by the species were as follows: 48% (1-1,142) in Pseudorasbora parva, 60% (1-412) in Pungtungia herzi, 15.7% (1-23) in Pseudogobio esocinus, 29% (1-7) in Acheilognathus intermedia, 21% (1-4) in Odontobutis interrupta, 33% (1-6) in Zacco temmincki, 3.6% (1-4) in Zacco platypus, and 26.3% (1) in Hemibarbus labeo. The two species, P. parva and P. herzi, are able to be the index fish for estimation of C. sinensis transmission in a certain locality. Still several species of freshwater fish are briskly transmitting C. sinensis infection in many riverside areas of southern Korea.
Animals ; Clonorchiasis/epidemiology/parasitology/*veterinary ; Clonorchis sinensis/*isolation & purification ; Fish Diseases/epidemiology/*parasitology ; Fishes ; Fresh Water ; Korea/epidemiology

Subperiosteal hematoma of the orbit is a rare disease and most of the cases occur in young adult males as a result of direct facial or orbital trauma. In the absence of direct facial or orbital trauma, nontranmatic subperiosteal orbital hematoma has rarely been reported in association with a sudden elevation of cranial venous pressure or venous congestion, systemic diseases associated with a bleeding diathesis and paranasal sinusitis. We report here on a rare case of subperiosteal orbital hematoma associated with ethmoid sinusitis, as was seen on CT imaging.
Disease Susceptibility ; Ethmoid Sinus* ; Ethmoid Sinusitis* ; Hematoma* ; Hemorrhage ; Humans ; Hyperemia ; Male ; Orbit* ; Rare Diseases ; Sinusitis ; Venous Pressure ; Young Adult