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Spindle cell lipoma is a rare benign neoplasm that features a mixture of evenly aligned spindle cells, mature adipocytes, and ropey collagen. Most cases of spindle cell lipoma are found in the subcutaneous tissue, and intradermal spindle cell lipoma is rarely reported. We present a case of intradermal spindle cell lipoma in a 46-year-old female who presented with a 0.7-cm flesh-colored and dome-shaped nodule on the right temple that had developed 6 years ago. This mass was excised, and upon histopathologic examination, an unencapsulated lesion was located in the dermis, which consisted of bland spindle cells, scanty mature adipocytes, rare lipoblasts, and ropey collagen bundles with prominent basophilic myxoid stroma. Immunohistochemical staining showed diffuse positivity for CD34, negativity for the S-100 protein, and loss of retinoblastoma protein expression. Based on these features, intradermal low-fat spindle cell lipoma was diagnosed. No evidence of local recurrence was observed 4 months after excision. Intradermal low-fat spindle cell lipomas are extremely rare and can easily be mistaken for tumors that have similar clinical and histopathological findings. Herein, we report a globally rare case of an intradermal low-fat spindle cell lipoma.
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Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described disease entity characterized by marked nuclear pleomorphism, low mitotic count, and diffuse CD34 positivity. It is a rare, distinctive, low-grade fibroblastic neoplasm. To date, only 44 cases have been reported in the English-language literature. Herein, we report two cases of SCPFT involving a 48-year-old male and a 22-year-old male with superficial tumors on the right and left thighs, respectively. Excision was performed in both cases. Histologically, both tumors showed spindle-to-epithelioid cells arranged in fascicular or sheet-like patterns. Most cells displayed granular or eosinophilic glassy cytoplasm, marked nuclear pleomorphism, and a low mitotic rate. On immunohistochemical staining, tumor cells were diffusely positive for CD34 and negative for S100 protein, smooth muscle actin, and desmin. After wide excision, neither patient experienced recurrence or metastasis after 16 months and 11 months of clinical follow-up, respectively. To the best of our knowledge, these are the first two cases of SCPFT reported in Korea. We believe these case reports would contribute to the clinicopathological understanding of SCPFT and assist clinicians in differentiating this tumor from other superficial soft tissue neoplasms.
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Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histologically, the tumor was composed of small blood vessels and vacuolated stromal cells with clear cytoplasm. On immunohistochemical stain, the stromal cells were positive for S-100 protein, neuron-specific enolase, and synaptophysin. The tumor did not reveal mutation of VHL alleles. We herein present a case of HB of the adrenal gland and review of the literature.
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Background/Aims@#Inflammatory bowel disease (IBD), including Crohn’s disease and ulcerative colitis, is a chronic inflammatory disorder of the gastrointestinal tract that requires lifetime management. Many studies have attempted to establish questionnaires and/or parameters to assess the quality of care for IBD patients. However, no study to date has investigated patients using the Q-methodology, which is subjective and has been studied systematically, to identify and categorize their opinions and subjective thinking about their disease and treatment. We have therefore aimed here to conduct a preliminary study of the Q-methodology to investigate the subjective thinking of IBD patients in Korea. @*Methods@#Q-methodology, a method of analyzing the subjectivity of questionnaire items, was examined in this study. Inputs from 50 IBD patients were classified into 34 normalized statements using a 9-point scale with a normal distribution. The collected data were analyzed using the QUANL PC program. @*Results@#Using the Q-methodology, IBD patients were classified into type I, II, III, and IV treatment needs: medical staff-dependent, relationship-oriented, information-driven, and social awareness, respectively. @*Conclusions@#The subjective needs of IBD patients and their thoughts about the treatment can be classified into four types. Our findings suggest that we can establish a systematic strategy for personalized care according to patient type.
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Background/Aims@#Steatohepatitis related to metabolic syndrome is a chronic liver disease prevalent in patients not only with non-alcoholic steatohepatitis but also with alcoholic liver disease and chronic viral hepatitis. On the other hand, there is limited data on the effects of hepatotonic agents in these patients. Therefore, this study evaluated the efficacy of a combined hepatotonic agent in this population. @*Methods@#Thirty-three adults with chronic hepatitis and one or more components of metabolic syndrome were assigned randomly to receive biphenyl dimethyl dicarboxylate/ursodeoxycholic acid or a placebo for 24 weeks. The primary outcome was the normalization of ALT (≤40 U/L). The secondary outcomes were the change in controlled attenuation parameter, transient elastography, and Chronic Liver Disease Questionnaire score. @*Results@#The 33 patients were assigned randomly to two groups. Eight (50%) of 16 patients who received the intervention drug showed the normalization of ALT, whereas only one (6%) of 17 patients in the placebo group did so. In contrast, the change in controlled attenuation, transient elastography, and Chronic Liver Disease Questionnaire were similar in the two groups. ALT was changed significantly during the four assessment periods, and this change was affected by the group. The interaction between the group and time was also significant. AST was changed significantly during the same period. This change was not affected by the group. @*Conclusions@#Biphenyl dimethyl dicarboxylate/ursodeoxycholic acid combination reduced ALT in chronic liver disease related to metabolic syndrome. On the other hand, there is no evidence that this leads to improved hepatic steatosis and fibrosis within 6 months.
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Pancreatic metastasis from malignant phyllodes tumor (PT) of the breast is rare, and only a few cases have been reported in the literature. Here, we report a case of pancreatic metastasis from malignant PT of the breast in a 48-year-old woman. She had had three episodes of recurrence of malignant PT in her right breast. She presented with epigastric pain for 2 months. Computed tomography and magnetic resonance imaging revealed a 6 cm-sized, well-defined, heterogeneous mass with peripheral enhancement in the body of the pancreas. Endoscopic ultrasonography-guided fine-needle aspiration was performed, and the pathologic report suggested spindle cell mesenchymal neoplasm. Subsequently, surgical excision was performed, and the mass was confirmed as a metastatic malignant PT. The imaging findings are discussed and the literature is briefly reviewed in this report.
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Ewing sarcomas constitute a group of small, round, blue cell tumors of the bone and soft tissue. Extraskeletal Ewing sarcoma (EES) is a rare malignant neoplasm that arises from soft tissues, and it usually affects children and young adults. EES of the thoracopulmonary region commonly presents with a palpable mass or pain. Although rarely reported, EES affecting the anterior chest wall may present as a breast mass. We report a case of EES arising from the chest wall and manifesting as a palpable breast mass in a 22-year-old woman. The large mass was initially misdiagnosed as a breast origin mass on ultrasonography, but subsequent CT and MRI showed that the mass originated from the chest wall. Radiologists should be aware of the imaging findings of EES, and they should understand that chest wall lesions may be clinically confused as breast lesions.
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Background@#On February 2, 2017, the surgical team of ten board-certified hand specialists of W Hospital in Korea successfully performed the nation's first hand transplantation at Yeungnam University Medical Center (YUMC). This paper reports on the legal, financial, and cultural hurdles that were overcome to open the way for hand transplantation and its functional outcomes at 36 months after the operation. @*Methods@#W Hospital formed a memorandum of understanding with Daegu city and YUMC to comply with government regulations regarding hand transplantation. Campaigns were initiated in the media to increase public awareness and understanding. With the city's financial and legal support and the university's medical cooperation, a surgical team performed a left distal forearm hand transplantation from a brain-dead 48-year-old man to a 35-year-old left-handed man. @*Results@#With this successful allotransplantation, the Korean Act on Organ Transplantation has now been amended to include hand transplantation. Korean national health insurance has also begun covering hand transplantation. Functional outcome at 36 months after the operation showed satisfactory progress in both motor and sensory functions. The disabilities of the arm, shoulder, and hand score were 23. The final Hand Transplantation Score was 90 points. Functional brain magnetic resonance imaging shows significant cortical reorganization of the corticospinal tract, and reinnervation of intrinsic muscle is observed. @*Conclusions@#Hand transplantation at the distal forearm shows very satisfactory outcomes in functional, aesthetical, and psychological aspects. Legal and financial barriers against hand transplantation have long been the most burdensome issues. Despite this momentous success, there have been no other clinical applications of vascularized composite allotransplantation due to the limited acceptance by Korean doctors and people. Further public education campaigns for vascularized composite allotransplantation are needed to increase awareness and acceptance.
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Background/Aims@#Inflammatory bowel disease (IBD), including Crohn’s disease and ulcerative colitis, is a chronic inflammatory disorder of the gastrointestinal tract that requires lifetime management. Many studies have attempted to establish questionnaires and/or parameters to assess the quality of care for IBD patients. However, no study to date has investigated patients using the Q-methodology, which is subjective and has been studied systematically, to identify and categorize their opinions and subjective thinking about their disease and treatment. We have therefore aimed here to conduct a preliminary study of the Q-methodology to investigate the subjective thinking of IBD patients in Korea. @*Methods@#Q-methodology, a method of analyzing the subjectivity of questionnaire items, was examined in this study. Inputs from 50 IBD patients were classified into 34 normalized statements using a 9-point scale with a normal distribution. The collected data were analyzed using the QUANL PC program. @*Results@#Using the Q-methodology, IBD patients were classified into type I, II, III, and IV treatment needs: medical staff-dependent, relationship-oriented, information-driven, and social awareness, respectively. @*Conclusions@#The subjective needs of IBD patients and their thoughts about the treatment can be classified into four types. Our findings suggest that we can establish a systematic strategy for personalized care according to patient type.
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Background/Aims@#Steatohepatitis related to metabolic syndrome is a chronic liver disease prevalent in patients not only with non-alcoholic steatohepatitis but also with alcoholic liver disease and chronic viral hepatitis. On the other hand, there is limited data on the effects of hepatotonic agents in these patients. Therefore, this study evaluated the efficacy of a combined hepatotonic agent in this population. @*Methods@#Thirty-three adults with chronic hepatitis and one or more components of metabolic syndrome were assigned randomly to receive biphenyl dimethyl dicarboxylate/ursodeoxycholic acid or a placebo for 24 weeks. The primary outcome was the normalization of ALT (≤40 U/L). The secondary outcomes were the change in controlled attenuation parameter, transient elastography, and Chronic Liver Disease Questionnaire score. @*Results@#The 33 patients were assigned randomly to two groups. Eight (50%) of 16 patients who received the intervention drug showed the normalization of ALT, whereas only one (6%) of 17 patients in the placebo group did so. In contrast, the change in controlled attenuation, transient elastography, and Chronic Liver Disease Questionnaire were similar in the two groups. ALT was changed significantly during the four assessment periods, and this change was affected by the group. The interaction between the group and time was also significant. AST was changed significantly during the same period. This change was not affected by the group. @*Conclusions@#Biphenyl dimethyl dicarboxylate/ursodeoxycholic acid combination reduced ALT in chronic liver disease related to metabolic syndrome. On the other hand, there is no evidence that this leads to improved hepatic steatosis and fibrosis within 6 months.
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Pancreatic collision tumors are rare neoplasm, and cases consisting of ductal adenocarcinoma with a neuroendocrine tumor, intraductal papillary mucinous neoplasm with a neuroendocrine tumor, and solid pseudopapillary neoplasm with a neuroendocrine tumor have been reported. We report a case of a rapidly growing pancreatic collision tumor consisting of desmoidtype fibromatosis and mucinous cystic neoplasm in a 30-year-old pregnant female. To the best of our knowledge, this is the first reported case of a pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm.
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Pancreatic collision tumors are rare neoplasm, and cases consisting of ductal adenocarcinoma with a neuroendocrine tumor, intraductal papillary mucinous neoplasm with a neuroendocrine tumor, and solid pseudopapillary neoplasm with a neuroendocrine tumor have been reported. We report a case of a rapidly growing pancreatic collision tumor consisting of desmoidtype fibromatosis and mucinous cystic neoplasm in a 30-year-old pregnant female. To the best of our knowledge, this is the first reported case of a pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm.
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Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.
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A pyogenic liver abscess (PLA) mostly occurs in association with biliary tract disease, but some PLAs have no apparent underlying cause, i.e., they are cryptogenic. The authors experienced three cases of PLA or liver cyst infection after colon polypectomy without other distinct etiologies. These cases suggest that colonoscopic polypectomy can cause a mucosal defect that provides a route for bacteria to invade the portal system or spread intraperitoneally to the liver. Colonoscopic polypectomy should be considered as a procedure that might cause PLA. Moreover, clinicians should be aware of this possibility if a patient complains of fever or abdominal pain after a colonoscopic polypectomy.
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Background/Aims@#With the improvement of hygiene, the incidence of amebic liver abscess is decreasing in South Korea. On the other hand, there is little data on the status of amebic liver abscess compared to pyogenic liver abscess. @*Methods@#Patients with an amebic liver abscess, in whom Entamoeba histolytica (E. histolytica) IgG was positive, were identified retrospectively in a university hospital. The clinical, laboratory, and radiological characteristics of amebic liver abscess were compared with those of pyogenic liver abscess in the same period. @*Results@#Between March 2010 and October 2016, 413 patients with a liver abscess were identified. Among them, the serologic test for E. histolytica was performed in 209 patients. Fifteen (7.2%) were classified as an amebic liver abscess, and the remainder were diagnosed with a pyogenic liver abscess. The age, gender, white blood cell, and CRP was comparable between the two groups. Procalcitonin was lower in amebic liver abscess than the pyogenic one. On CT, peripheral rim enhancement was more frequent, but cluster signs were not observed in amebic liver abscess compared to pyogenic liver abscess. None of the patients with amebic liver abscess died. In contrast, the mortality of pyogenic liver abscess was 4.7%. @*Conclusions@#Amebic liver abscess should still be considered as one of the causes of liver abscess in Korea. It is difficult to discriminate an amebic liver abscess from a pyogenic liver abscess only according to the clinical, laboratory, and radiologic findings. Therefore, it is necessary to perform a serologic test for E. histolytica for a precise evaluation of liver abscess in a high-risk group.
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Synovial sarcoma is a malignant soft tissue tumor that usually involves the extremities, particularly near the knees; a synovial sarcoma originating in the chest wall is extremely rare. We describe a 26-year-old woman diagnosed with a synovial sarcoma originating in the chest wall, based on CT and MRI findings. Contrast-enhanced CT images revealed a small, well-defined enhancing mass with calcification, in the subpleural area. This lesion was initially diagnosed as a benign tumor; however, the patient developed sudden severe pain with spontaneous bleeding and hemothorax, suggesting the possibility of malignancy. MRI revealed a multilobulated mass with a fluid-fluid level, which characterizes a synovial sarcoma.
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Background/Aims@#Data on the trends in the prevalence of chronic liver disease (CLD) in Korea are scarce. This study aimed to evaluate whether the CLD prevalence changed between 1998–2001 and 2016–2017. @*Methods@#Data were extracted from the Korea National Health and Nutrition Examination Survey (1998–2001 to 2016– 2017; n=25,893). Non-alcoholic fatty liver disease (NAFLD) was defined as a hepatic steatosis index >36 in the absence of any other evidence of CLD. The definition of alcohol-related liver disease (ALD) was excessive alcohol consumption (≥210 g/week for men and ≥140 g/week for women) and an ALD/NAFLD index >0. @*Results@#The prevalence of NAFLD increased from 18.6% (95% confidence interval [CI], 17.8–19.5%) in 1998–2001 to 21.5% (95% CI, 20.6–22.6%) in 2016–2017. During the same time period, increases were observed in the prevalence of obesity (27.0 vs. 35.1%), central obesity (29.4 vs. 36.0%), diabetes (7.5 vs. 10.6%), and excessive drinking (7.3 vs. 10.5%). ALD prevalence also increased from 3.8% (95% CI, 3.4–4.2%) to 7.0% (95% CI, 6.4–7.6%). In contrast, chronic hepatitis B decreased from 5.1% (95% CI, 4.6–5.5%) to 3.4% (95% CI, 3.0–3.8%). The prevalence of chronic hepatitis C was approximately 0.3% in 2016–2017. @*Conclusions@#The prevalence of NAFLD and ALD increase among Korean adults. Our results suggest potential targets for interventions to reduce the future burden of CLD.
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BACKGROUND/AIMS: The serum aminotransferase level is usually elevated in rhabdomyolysis, and these enzymes originate from the skeletal muscle. On the other hand, there is limited data showing whether the degree of elevation of these enzymes differs according to the concurrent liver disease. METHODS: Patients with rhabdomyolysis were selected when their serum creatinine kinase level was >1,000 U/L. They were categorized as the group with and without concurrent liver disease. The AST and ALT levels in both groups were compared. In addition, the aminotransferase level was compared between those with rhabdomyolysis and those with alcoholic liver disease. RESULTS: Among the 165 patients with rhabdomyolysis, 19 had concurrent liver disease. The median peak AST was higher in the group with concurrent liver disease (332 U/L [interquartile range (IQR), 127–1,604] vs. 219 U/L [IQR, 115–504]). In addition, the median peak ALT was higher in the group with concurrent liver disease (107 U/L [IQR, 74–418] vs. 101 U/L [IQR, 56–218]). On the other hand, there was no significant difference in both enzymes between the two groups. The median peak AST level was significantly higher in those with rhabdomyolysis than in those with alcoholic liver disease (221 U/L [IQR, 118–553] vs. 103 U/L [IQR, 59–206]), but the median peak ALT was not significantly different (102 U/L [IQR, 58–222] vs. 51 U/L [IQR, 26–117]). CONCLUSIONS: Rhabdomyolysis showed an elevated AST-dominant aminotransferase level, which is not different according to concurrent liver disease. Therefore, it is recommended that rhabdomyolysis be considered first in cases of elevated aminotransferase levels in patients with a suspicious skeletal muscle injury.
Assuntos
Humanos , Alanina Transaminase , Aspartato Aminotransferases , Creatinina , Mãos , Hepatopatias , Hepatopatias Alcoólicas , Fígado , Músculo Esquelético , Fosfotransferases , RabdomióliseRESUMO
BACKGROUND/AIMS: The serum aminotransferase level is usually elevated in rhabdomyolysis, and these enzymes originate from the skeletal muscle. On the other hand, there is limited data showing whether the degree of elevation of these enzymes differs according to the concurrent liver disease.METHODS: Patients with rhabdomyolysis were selected when their serum creatinine kinase level was >1,000 U/L. They were categorized as the group with and without concurrent liver disease. The AST and ALT levels in both groups were compared. In addition, the aminotransferase level was compared between those with rhabdomyolysis and those with alcoholic liver disease.RESULTS: Among the 165 patients with rhabdomyolysis, 19 had concurrent liver disease. The median peak AST was higher in the group with concurrent liver disease (332 U/L [interquartile range (IQR), 127–1,604] vs. 219 U/L [IQR, 115–504]). In addition, the median peak ALT was higher in the group with concurrent liver disease (107 U/L [IQR, 74–418] vs. 101 U/L [IQR, 56–218]). On the other hand, there was no significant difference in both enzymes between the two groups. The median peak AST level was significantly higher in those with rhabdomyolysis than in those with alcoholic liver disease (221 U/L [IQR, 118–553] vs. 103 U/L [IQR, 59–206]), but the median peak ALT was not significantly different (102 U/L [IQR, 58–222] vs. 51 U/L [IQR, 26–117]).CONCLUSIONS: Rhabdomyolysis showed an elevated AST-dominant aminotransferase level, which is not different according to concurrent liver disease. Therefore, it is recommended that rhabdomyolysis be considered first in cases of elevated aminotransferase levels in patients with a suspicious skeletal muscle injury.
Assuntos
Humanos , Alanina Transaminase , Aspartato Aminotransferases , Creatinina , Mãos , Hepatopatias , Hepatopatias Alcoólicas , Fígado , Músculo Esquelético , Fosfotransferases , RabdomióliseRESUMO
Imatinib mesylate is currently used as the first-line treatment for metastatic gastrointestinal stromal tumors (GISTs). Imatinib-induced hepatotoxicity in patients with GIST is very rare. Its features vary from subclinical elevation of serum aminotransferase to clinically apparent acute hepatitis, which is associated with immunologic reactions. Imatinib-induced hepatotoxicity with autoimmune-like features can be treated by the discontinuation of imatinib mesylate and the administration of oral steroids. Here, we report a case of late-onset imatinib-induced hepatitis with autoimmune-like features in a patient with metastatic GIST, which was improved by oral corticosteroids.
