Here, we report a rare case of L3 chordoma progressed to an intradural extramedullary (IDEM) mass and distant metastasis to the fascia lata. A 64-year old female patient presented to a local university hospital due to back pain and received excisional biopsy for a L3 destructive bony lesion. Local radiation therapy was initially administered, assuming a malignancy of unknown origin, but she developed cerebrospinal fluid leakage during adjuvant radiation therapy, which was managed by wound revision and lumbar drainage. As the destructive lesion progressed, she visited our hospital for a second opinion 3 months after the biopsy. After review of outside pathology, we diagnosed the lesion to be a chordoma, and performed a L3 corpectomy with cage and plate fixation. One and a half years later, positron emission tomography and computed tomography (PET-CT) revealed a right tensor fascia lata hypermetabolic lesion. Excisional biopsy confirmed a distant metastasis of the chordoma. One year later, she complained of L2 radiating pain. PET-CT and CT myelogram revealed an IDEM lesion. Surgical excision confirmed the transdural invasion of the chordoma. To our knowledge, this is the first report of an iatrogenic IDEM invasion and distant metastasis to the tensor of the fascia lata by a L3 chordoma.

Somatic mutation is a major cause of cancer progression and varied responses of tumors against anticancer agents. Thus, we must obtain and characterize genome-wide mutational profiles in individual cancer subtypes. The Cancer Genome Atlas database includes large amounts of sequencing and omics data generated from diverse human cancer tissues. In the present study, we integrated and analyzed the exome sequencing data from ~3,000 tissue samples and summarized the major mutant genes in each of the diverse cancer subtypes and stages. Mutations were observed in most human genes (~23,000 genes) with low frequency from an analysis of 11 major cancer subtypes. The majority of tissue samples harbored 20-80 different mutant genes, on average. Lung cancer samples showed a greater number of mutations in diverse genes than other cancer subtypes. Only a few genes were mutated with over 5% frequency in tissue samples. Interestingly, mutation frequency was generally similar between non-metastatic and metastastic samples in most cancer subtypes. Among the 12 major mutations, the TP53, USH2A, TTN, and MUC16 genes were found to be frequent in most cancer types, while BRAF, FRG1B, PBRM1, and VHL showed lineage-specific mutation patterns. The present study provides a useful resource to understand the broad spectrum of mutation frequencies in various cancer types.
Antineoplastic Agents ; Exome ; Genome ; Humans* ; Lung Neoplasms ; Mutation Rate ; Neoplasm Metastasis

Morphea, a subclass of localized scleroderma, is a connective tissue disorder limited to the skin and subcutaneous tissue. We report herein a case of secondary cicatricial alopecia due to morphea, which showed very unusual site and clinical manifestations. A 24-year-old woman presented to the department of dermatology with a solitary skin-colored patch on the frontoparietal area through the eyebrow. The histopathologic findings showed hydrophic changes of the basal cells in the epidermis. In addition, there were broadened, compact, eosinophilic collagen fibers and perifollicular lymphocytic infiltration with loss of elastic fibers and adnexal structures in the entire dermis. On the basis of clinical and pathological findings, she was diagnosed with secondary cicatricial alopecia due to morphea on the frontoparietal area through the eyebrow.
Alopecia ; Collagen ; Connective Tissue ; Dermatology ; Dermis ; Elastic Tissue ; Eosinophils ; Epidermis ; Eyebrows ; Female ; Humans ; Scleroderma, Localized ; Skin ; Subcutaneous Tissue ; Young Adult

OBJECTIVES: The characteristics of depressive symptoms in the family members of home-dwelling patients with dementia have not been clearly reported. This study aimed to investigate the characteristics of depressive symptoms in middle-aged family members living with a patient with dementia. METHODS: This study used the data from the nationwide 2017 Korea Community Health Survey. Among the 228,381 survey participants, 77,276 participants in their 40s and 50s were finally selected for this study. The participants consisted of 760 family members of home-dwelling dementia patients and 76,516 general family members comprising a control group. RESULTS: The positive rate of Patient Health Questionnaire-9 (PHQ-9)-measured depressive symptoms was significantly higher in the family members of home-dwelling dementia patients (4.4%; control group: 1.9%). After adjusting for potential confounders, the prevalence of PHQ-measured depressive symptoms was 1.72 times (95% confidence interval [CI], 1.03 to 2.85) higher in the family members of home-dwelling dementia patients compared to the control group. The positive rate of depressive symptoms was 2.26 times higher (95% CI, 1.26 to 4.05) in the female middle-aged family members of home-dwelling dementia patients compared to the control group. In addition, those who reported having symptoms almost every day in the PHQ-9 questions had significantly higher positive rates on questions about loss of interest, depression, sleep disturbance, fatigue, poor appetite, and suicidal ideation, and not on questions regarding feelings of worthlessness and psychomotor agitation, compared to the control group. CONCLUSIONS Active interventions are needed to relieve depression in the family members of home-dwelling dementia patients.

OBJECTIVES: The characteristics of depressive symptoms in the family members of home-dwelling patients with dementia have not been clearly reported. This study aimed to investigate the characteristics of depressive symptoms in middle-aged family members living with a patient with dementia. METHODS: This study used the data from the nationwide 2017 Korea Community Health Survey. Among the 228,381 survey participants, 77,276 participants in their 40s and 50s were finally selected for this study. The participants consisted of 760 family members of home-dwelling dementia patients and 76,516 general family members comprising a control group. RESULTS: The positive rate of Patient Health Questionnaire-9 (PHQ-9)-measured depressive symptoms was significantly higher in the family members of home-dwelling dementia patients (4.4%; control group: 1.9%). After adjusting for potential confounders, the prevalence of PHQ-measured depressive symptoms was 1.72 times (95% confidence interval [CI], 1.03 to 2.85) higher in the family members of home-dwelling dementia patients compared to the control group. The positive rate of depressive symptoms was 2.26 times higher (95% CI, 1.26 to 4.05) in the female middle-aged family members of home-dwelling dementia patients compared to the control group. In addition, those who reported having symptoms almost every day in the PHQ-9 questions had significantly higher positive rates on questions about loss of interest, depression, sleep disturbance, fatigue, poor appetite, and suicidal ideation, and not on questions regarding feelings of worthlessness and psychomotor agitation, compared to the control group. CONCLUSIONS Active interventions are needed to relieve depression in the family members of home-dwelling dementia patients.

BACKGROUNDS/AIMS: Vitamin K may plays a role in controlling hepatocellular carcinoma (HCC) cell growth. In this study, we intended to present 5-year experience of 72 patients receiving oral vitamin K with or without sorafenib. Its end-point was to evaluate the safety of combination therapy using sorafenib and vitamin K. METHODS: An interim analysis was performed as a single-arm cross-sectional study, including 72 HCC patients who underwent liver resection or transplantation and administered oral vitamin K2 alone (n=47) or with sorafenib (n=25). RESULTS: In all patients, administration of vitamin K2 analog 45 mg/day did not show any noticeable adverse side-effect during vitamin K therapy of 23.3+/-10.6 months, except for one patient who experienced skin rash at the third day of vitamin K therapy. In 25 patients receiving sorafenib and vitamin K for 6 months or longer, any noticeable adverse side-effect suspected of vitamin K origin was not identified yet. A small proportion of patients showed unexpectedly favorable anti-tumor effects after use of vitamin K with or without sorafenib. CONCLUSIONS: Because add-on of oral vitamin K did not increase the adverse side-effects of sorafenib, a combination therapy with these two agents appears to be worthy of further clinical trial with an expectation of synergistic therapeutic effects.
Carcinoma, Hepatocellular* ; Cross-Sectional Studies ; Exanthema ; Humans ; Liver ; Neoplasm Metastasis ; Vitamin K 2 ; Vitamin K*

BACKGROUND/AIMS: Long-term use of aspirin can be a risk factor of peptic ulcer diseases. The aim of this study was to evaluate the efficacy of Albis (Daewoong Pharmaceutical Co., Ltd.) for the prevention of gastric mucosal injury caused by aspirin. METHODS: Aspirin users were enrolled and randomized into the Albis or placebo group. Screening and follow-up endoscopy were performed for modified Lanza scores (MLSs). Primary outcome was measured by the incidence rate of peptic ulcer, and secondary outcomes were measured by the incidence rate of gastritis, improvement in MLS and subjective symptoms. RESULTS: In total, 81 aspirin users were randomized, 43 in the Albis group and 38 in the placebo group. There was no incidence of peptic ulcer in both groups. The incidence of gastritis was significantly higher in the placebo group (44.4% vs. 10.0%, p=0.003); however, the scores of mucosal edema, hyperemia and hemorrhage were not statistically different between the two groups (p>0.05). The frequency of subjective symptoms were more improved in the Albis group than in the placebo group (p=0.023). CONCLUSIONS: The incidence of gastritis was lower in the group that received low-dose aspirin and Albis. The development of peptic ulcer due to long-term use of aspirin might be prevented with concomitant use of Albis.
Aspirin* ; Edema ; Endoscopy ; Follow-Up Studies ; Gastritis ; Hemorrhage ; Hyperemia ; Incidence ; Mass Screening ; Peptic Ulcer ; Prospective Studies* ; Risk Factors

BACKGROUND: The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. METHODS: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. RESULTS: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. CONCLUSIONS: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases.
Central Nervous System ; Diagnosis ; Follow-Up Studies ; Gene Fusion ; Hemangiopericytoma ; Humans ; Liver ; Lung ; Meningeal Neoplasms ; Necrosis ; Neoplasm Metastasis* ; Prognosis ; Solitary Fibrous Tumors* ; Survival Rate

BACKGROUND: Multidisciplinary team care (MTC) is a collaborative approach to treatment plan and ongoing care. We aimed to evaluate the clinical effect of MTC on the regulation of chronic kidney disease-mineral and bone disorder (CKD-MBD) complications in dialysis patients. METHODS: This retrospective observational study was approved by the institutional review board. Among patients who have undergone dialysis at admission, the patients admitted to the nephrology ward were allocated to MTC group, and the others to usual care (UC) group. The MTC group had collaborative care by nephrologists, nurses, pharmacists, and nutritionists. The endpoints were the regulation of corrected calcium (cCa) and phosphate (P), the percent of patients in target level of cCa-P product (cCa×P), and the prescription rate of non-calcium based P-binders. RESULTS: A total of 163 patients were included from January to December 2009. A significant difference was shown in the percentage of patients in target cCa×P level at admission (MTC vs. UC, 81.40% vs. 91.67%; P = 0.038), but there was no significant difference at discharge. During admission, the cCa and P levels of patients in only UC group were significantly changed. In addition, compared with UC group, patients in MTC group were more likely prescribed appropriate P-binders, when they had higher cCa×P levels than 55 mg²/dL² (P <0.001). CONCLUSION: It was found that MTC had beneficial effect on improving the regulation of CKD-MBD and the appropriate phosphate binder uses. Therefore, application of the MTC is anticipated to enhance quality of clinical care in chronic diseases.
Calcium ; Chronic Disease ; Dialysis* ; Ethics Committees, Research ; Humans ; Kidney ; Miners* ; Nephrology ; Nutritionists ; Observational Study ; Pharmacists ; Prescriptions ; Renal Insufficiency, Chronic* ; Retrospective Studies

Background/Aims: Combination therapy with immunomodulators (IMMs) was proposed as a strategy to prevent the development of loss of response (LOR) to anti-tumor necrosis factor (TNF) for patients with inflammatory bowel disease (IBD). However, the effect is unclear in patients already exposed to IMMs. The aim of this study was to evaluate whether combination therapy with IMMs is superior to monotherapy for prevention of LOR to anti-TNF. Methods: This was a retrospective study of patients in Seoul National University Bundang Hospital with IBD between January 2009 and October 2018. LOR was defined as clinical deterioration after maintenance of anti-TNF for at least 6 months. We investigated the difference in incidence of LOR to anti-TNF between the monotherapy and combination groups. We additionally assessed factors affecting LOR development to anti-TNF. Results: A total of 116 patients with IBD were included in this study (monotherapy 61 patients; combination 55 patients). Overall, LOR to anti-TNF occurred in 31 patients during the follow-up period. The combination of an anti-TNF agent and IMM showed no significant difference in the incidence of LOR compared to anti-TNF agent monotherapy (hazard ratio [HR], 1.64; 95% confidence interval [CI], 0.786 to 3.148; p = 0.182). Female sex was significantly associated with the development of LOR to anti-TNF (HR, 3.032; 95% CI, 1.467 to 6.268; p = 0.003). Conclusions Anti-TNF and IMM combination therapy did not prove efficacious in preventing the development of LOR in IBD patients. Female sex was associated with the development of LOR to anti-TNF; further studies are required to confirm these results.