Outcomes of epiretinal proliferation embedding technique in the surgery for full-thickness macular hole.

To compare visual and anatomical outcomes between peeling and embedding of epiretinal proliferation in patients with full-thickness macular holes (FTMH) with epiretinal proliferation (EP), this retrospective cohort study classified patients into two groups based on whether EP was completely peeled (peeling group, n = 25 eyes), or embedded into the hole (embedding group, n = 31 eyes) during surgery. Preoperative characteristics and postoperative outcomes, including best-corrected visual acuity and the length of the disrupted external limiting membrane and ellipsoid zone, were compared. Preoperative features including visual acuity and hole size did not differ between the two groups. All studied eyes achieved closure of the macular hole postoperatively. Visual acuity significantly improved at 3, 6, and 12 months postoperatively in both groups. The visual acuity 1-month after surgery was better in the embedding group than that in the peeling group (0.28 ± 0.29 vs. 0.50 ± 0.42 logarithm of the minimum angle of resolution, P = 0.016), although the difference was not noted after 3 months postoperatively. The embedding group showed shorter disruption of the external limiting membrane than the peeling group postoperatively (62.6 ± 40.2 µm vs. 326.2 ± 463.9 µm at postoperative 12 months, P = 0.045). In conclusion, the embedding technique during surgical repair of a FTMH with EP facilitates recovery of the outer foveal layers and promotes earlier restoration of visual function.

RISK OF EXUDATION IN EYES WITH NONEXUDATIVE POLYPOIDAL CHOROIDAL VASCULOPATHY.

PURPOSE: To investigate the characteristics and natural history of treatment-naive nonexudative polypoidal choroidal vasculopathy (PCV) and to determine biomarkers predicting exudative conversion. METHODS: Patients diagnosed with nonexudative PCV based on indocyanine green angiography and optical coherence tomography were included. Incidence of exudative conversion in nonexudative PCV patients and cumulative estimates for overall risk were assessed. Indocyanine green angiography and optical coherence tomography imaging-based features were analyzed to identify risk factors for exudative conversion. RESULTS: The study included 42 eyes of 40 patients with nonexudative PCV. The mean follow-up duration was 54.3 ± 35.5 months. Of the 42 eyes with nonexudative PCV, exudative conversion developed in 23 eyes (54.8%) after 42.2 ± 28.3 months (range, 8-103 months). Kaplan-Meier analysis showed that the exudation-free survival at 5 years after baseline was estimated to be 53.6%. Multivariate regression analysis showed that sequentially increased protrusion of retinal pigment epithelium in the polyp area was a significant risk factor for exudation in nonexudative PCV (odds ratio = 10.16; 95% CI 1.78-57.81; P = 0.01). CONCLUSION: Exudative conversion has been noted in nearly half of the nonexudative PCV cases in 5 years. The progressive protrusion of polypoidal lesions on optical coherence tomography examination may be a significant biomarker for predicting the near-term onset of exudation.

Biomarkers determining treatment interval of diabetic macular edema after initial resolution by anti-vascular endothelial growth factor.

PURPOSE: To identify predictive factors that help determine the interval of intravitreal anti-vascular endothelial growth factor (anti-VEGF) injection after the initial resolution of diabetic macular edema (DME). METHODS: This retrospective case-control study enrolled treatment-naïve DME patients who had achieved DME resolution after intravitreal anti-VEGF injections. Patients were classified into the recurrence and no-recurrence groups, depending on the development of recurrent DME after deferring intravitreal anti-VEGF injection. The demographics and clinical features, including optical coherence tomography findings, were compared between the two groups. RESULTS: We enrolled 105 eyes. Sixty eyes (57.1%) belonged to the no-recurrence group, and 45 (42.9%), belonged to the recurrence group. The severity of diabetic retinopathy at baseline was related to early DME recurrence (P = 0.009). At the treatment deferring point, the non-recurrence group had both thinner central subfield thickness (289.5 ± 27.2 µm vs. 307.0 ± 38.2 µm, P = 0.011) and thinner central retinal thickness (214.9 ± 41.4 µm vs. 231.8 ± 41.2 µm, P = 0.043) compared to the recurrence group. Intraretinal cyst was observed in 34 eyes (56.7%) in the no-recurrence group and 42 eyes (93.3%) in the recurrence group at the deferring point (P < 0.001). CONCLUSION: A low risk of early DME recurrence is anticipated in the eyes with foveal thinning and no intraretinal cyst when anti-VEGF injection is deferred. These predictive biomarkers can be useful for patient monitoring and determining treatment strategies for DME patients.

Local ocular factors associated with the development of diabetic macular edema: an inter-eye study.

To investigate local ocular factors associated with the development of diabetic macular edema (DME), we classified each eye of patients with unilateral DME as the DME eyes or the fellow eyes (without DME). We compared the clinical characteristics, optical coherence tomography (OCT), and OCT angiography (OCTA), ultra-wide field fundus photography, and angiography features of each eye. As a result, fifty-five patients with unilateral DME were enrolled. Although the diabetic retinopathy stage was not different between each group of eyes, DME eyes showed a higher prevalence of venous beading and a larger area of nonperfusion region than did fellow eyes (all P < 0.05). OCTA features of DME eyes also showed a larger foveal avascular zone in the deep capillary plexus and a lower vascular density in both the superficial and deep capillary plexuses (all P < 0.05). This study highlighted ocular features reflecting retinal ischemia, such as venous beading, area of nonperfusion region, and vascular density in the central retinal area, are associated with the development of DME. OCTA and ultra-wide field fluorescein angiography may be useful for evaluating the parameters of retinal ischemia and the risk of DME development.

Detecting Donor-Derived DNA by Real-Time PCR in Recipients Suspected of Graft-Versus-Host-Diseases After Liver Transplantation: A Case Series and Literature Review.

BACKGROUND Graft-versus-host disease (GVHD) after liver transplantation (LT) is a rare but fatal complication. GVHD diagnosis is usually based on clinical symptoms and pathologic confirmation. However, it is often misdiagnosed due to its non-specific symptoms. Here, we report the detection of donor-cell chimerism using peripheral blood (PB) donor-derived deoxyribonucleic acid (ddDNA) for 3 cases with suspected GVHD after LT (GVHD-LT) through real-time quantitative polymerase chain reaction (qPCR) assay targeting 39 insertions and/or deletions of chromosomes. MATERIAL AND METHODS The qPCR assay for detecting donor-cell chimerism was performed for 3 post-LT patients with suspected GVHD using KMRtype® and KMRtrack® assays (GenDx, Netherlands). The mean recipient/donor-cell fraction of informative markers unique to each recipient or donor was calculated. RESULTS In Case 1, who received living donor LT (LDLT) from his daughter, initial sign was diarrhea at post-operative day (POD) #23. Case 2 received unrelated deceased donor LT and initial sign was cytopenia at POD #29. Case 3 received LDLT from her son and GVHD associated cytopenia was developed at POD #80. Average PB ddDNA fractions in post-transplant samples of cases 1, 2, and 3 were 39.68%, 78.38%, and 4.76%, respectively. Despite an active treatment including steroid and tumor necrosis factor-alpha inhibitor, 2 patients (cases 1 and 2) died due to multiple organ failures. CONCLUSIONS Early detection of donor-cell chimerism may help halt fatal progression of GVHD-LT. A qPCR test targeting INDEL of chromosomes would be a helpful procedure for timely diagnosis of GVHD.

Microscopy and Immunocytochemistry-Based Methods to Study Cell Wall Biosynthetic Enzymes in the Golgi.

The Golgi apparatus has essential roles in all eukaryotic cells, and its importance in plants is further exemplified by a critical role in building a cellulosic cell wall. The Golgi apparatus houses numerous cell wall-synthesizing or cell wall-modifying enzymes to generate the complex cell wall structure. However, several putative cell wall biosynthetic candidates await characterization, which requires verification of the subcellular localization and enzymatic products. Here, we describe detailed methods to analyze the localization of proteins that are transiently produced in tobacco leaves or stably produced in transgenic plants, by confocal microscopy using fluorescent-tagged proteins along with known Golgi markers or the trafficking inhibitor brefeldin A. We also detail a procedure to analyze the enzymatic products through antibody-based immunoblotting after cell wall enrichment.

The clinical impact of donor against recipient HLA one way mismatch on the occurrence of graft versus host disease in liver transplantation.

Graft versus host disease (GVHD) after liver transplantation (LT) is a rare, fatal disease. This study aimed to evaluate the risk factors of GVHD after LT including the human leukocyte antigen (HLA) donor-recipient relationship after LT. LT recipients, who underwent HLA typing together with donors, were included in the study. The donor against recipient (D → R) one-way mismatch of HLA loci was evaluated. HLA relationships, along with basic characteristics, were analyzed as variable factors of GVHD, graft survival, and patient survival. A total of 994 living donor LT (LDLT) and 393 deceased donor LT (DDLT) patients were included. Nine patients had suffered GVHD, four LDLT with D → R one-way at three loci, one LDLT without D → R one-way at three loci, and four DDLT without D → R one-way at three loci. Four (57.1%) of seven LDLT patients, with D → R one-way mismatch at three loci, developed GVHD. D → R one-way mismatch at three loci was related to high GVHD incidence (HR 787, p < 0.001, multivariate). D → R one-way mismatch at three loci was related to graft failure and patient death (HR 9.90, p = 0.020 and HR 12.8, p < 0.001, respectively, multivariate). Only one GVHD without D → R one-way mismatch at three loci, survived despite receiving multiple modalities including tumor necrosis factor-alpha inhibitors. D → R one-way mismatch at three loci was significantly related to GVHD incidence after LT.

PARAFOVEAL INNER RETINAL THINNING AS THE BIOMARKER PREDICTING LESS RECURRENCE OF MACULAR EDEMA IN CENTRAL RETINAL VEIN OCCLUSION AFTER DISCONTINUING ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR.

PURPOSE: To identify the predictive factors for the recurrence of macular edema after the cessation of antivascular endothelial growth factor treatment in eyes with central retinal vein occlusion (CRVO). METHODS: This retrospective study included participants who had discontinued intravitreal bevacizumab injections for complete resolution of macular edema related to CRVO at 3 months after the last injection. Fifty-two eyes were enrolled in this study and classified into two groups based on the recurrence of macular edema within 1 year after the stopping point, when the decision to discontinue injections was made. Clinical characteristics and optical coherence tomographic parameters at baseline and at the stopping point were investigated. RESULTS: Multivariate logistic regression analysis demonstrated that, at baseline, old age was associated with a significantly higher risk of macular edema recurrence (odds ratio, 1.092; P = 0.022). At the stopping point, parafoveal inner retinal thickness (odds ratio: 1.043, P = 0.014) and the presence of ellipsoid zone disruption (odds ratio: 5.922, P = 0.032) were predictive factors for recurrence. The receiver operating characteristic curve showed that parafoveal inner retinal thinning of >7 µm compared with that in the fellow eye was significantly associated with decreased recurrence of macular edema. CONCLUSION: Parafoveal inner retinal thinning and intact ellipsoid zone after resolution of macular edema by antivascular endothelial growth factor treatment were predictive of a lower risk of recurrence of macular edema in CRVO. These intuitive biomarkers may help predict future disease courses and design optimal treatment strategies.

Association Between Retinopathy of Prematurity in Very-Low-Birth-Weight Infants and Neurodevelopmental Impairment.

PURPOSE: To evaluate the impact of retinopathy of prematurity (ROP) severity and the treatment of very-low-birth-weight infants (VLBWIs) on neurodevelopmental impairment in early childhood. DESIGN: Prospective cohort study. METHOD: This was a prospective cohort study. The data were obtained from the Korean Neonatal Network (KNN), a nationwide registry for VLBWIs. Infants who were born from 2013 to 2015 and underwent ROP evaluation at birth and neurodevelopmental examinations at corrected ages of 18 to 24 months were included in the study. Infants with a history of meningitis or severe congenital anomalies were excluded. The VLBWI patients were grouped into no ROP, no treatment-requiring ROP (non-TR-ROP), and treatment-requiring ROP (TR-ROP) groups. Neurodevelopmental impairment was defined as participants who had at least 1 developmental problem according to the Bayley Scales of Infant and Toddler Development-2nd Edition (Bayley-II; <70), Bayley Scales of Infant and Toddler Development-3rd Edition (Bayley-III; <70), and Korean Developmental Screening Test (K-DST) tests (below -1 SD), and the Korean Ages and Stages Questionnaire (K-ASQ) (below the threshold) and Gross Motor Function Classification System (GMFCS; at level 2 or above). Multivariable logistic regression analysis was performed to evaluate the association between ROP and neurodevelopmental impairment. RESULT: Among 3132 infants, 1093 (34.9%) had ROP. Among the ROP infants, 644 were not treated for ROP (non-TR-ROP group) and 449 received ROP treatments (TR-ROP group). The patients in the TR-ROP group had an increased risk of developing neurodevelopmental problems compared to those in the no ROP group (odds ratio [OR] = 1.72, 95% CI = 1.33-2.21). The TR-ROP group had a higher risk of all 3 types of neurodevelopmental problems: mental (OR = 1.62, 95% CI = 1.25-2.09), social (OR = 1.62, 95% CI = 1.12-2.09), and motor (OR = 1.69, 95% CI = 1.31-2.18). The risk of neurodevelopmental problems in patients treated with laser therapy did not differ from that in patients treated with anti-vascular endothelial growth factor (anti-VEGF) therapy (OR = 1.17, 95% CI = 0.73-1.88). CONCLUSION: ROP was independently associated with neurodevelopmental impairment in early childhood. The type of ROP treatment (anti-VEGF or laser treatment) did not affect neurodevelopmental impairment in patients in the TR-ROP group.

Tumor regionalization after surgery: Roles of the tumor microenvironment and neutrophil extracellular traps.

Surgery is unanimously regarded as the primary strategy to cure solid tumors in the early stages but is not always used in advanced cases. However, tumor surgery must be carefully considered because the risk of metastasis could be increased by the surgical procedure. Tumor surgery may result in a deep wound, which induces many biological responses favoring tumor metastasis. In particular, NETosis, which is the process of forming neutrophil extracellular traps (NETs), has received attention as a risk factor for surgery-induced metastasis. To reduce cancer mortality, researchers have made efforts to prevent secondary metastasis after resection of the primary tumor. From this point of view, a better understanding of surgery-induced metastasis might provide new strategies for more effective and safer surgical approaches. In this paper, recent insights into the surgical effects on metastasis will be reviewed. Moreover, in-depth opinions about the effects of NETs on metastasis will be discussed.

Prediction models of hepatocellular carcinoma recurrence after liver transplantation: A comprehensive review.

Liver transplantation (LT) is one of the most effective treatments for hepatocellular carcinoma (HCC). Although LT eliminates HCC and greatly reduces recurrence, some patients experience recurrence after LT. Criteria and models for screening patients with a high probability of HCC recurrence after LT, starting with the Milan criteria, have been published. These models have changed over time, but a standard has not been established. We summarized HCC prediction models after LT by focusing on the application of radiologic, serologic, and pathologic factors and recent trends. This review will look at studies that are based on living donor LT and deceased donor LT, as well as studies that downstaging procedures have been performed preoperatively. This ultimately aims to help make decisions for evaluating the HCC state and selecting candidates for LT according to the circumstances of each transplantation center.

Early menopause is associated with increased risk of retinal vascular occlusions: a nationwide cohort study.

This nationwide population-based cohort study evaluated the association between female reproductive factors and the incidence of retinal vein occlusion (RVO) and retinal artery occlusion (RAO) using data provided by the Korea National Health Insurance Service. A total of 2,289,347 postmenopausal women over 50 years of age who participated in both national health screening and cancer screening in 2013 or 2014 were included. Data on female reproductive factors, including age at menarche, age at menopause, parity, history of hormone replacement therapy, and oral contraceptive pill usage, were collected. Patients were followed up until December 2018, and incident cases of RVO and RAO were identified using registered diagnostic codes from claim data. During an average follow-up period of 4.90 years, 7461 and 1603 patients were newly diagnosed with RVO and RAO, respectively. In the multivariable-adjusted Cox proportional hazard model, patients who experienced menopause after 55 years of age had a lower risk of RVO and RAO development compared to those who had menopause before 45 years of age, with a hazard ratio (95% confidence interval) of 0.83 (0.76-0.95) for RVO and 0.80 (0.66‒0.98) for RAO. In conclusion, early menopause was an independent risk factor for future development of RVO and RAO.

Healthy Spouses can be Considered as Living Liver Donors.

BACKGROUND: Spousal donors have gradually been accepted as an alternative living liver donors to alleviate the organ shortage and prevent donations from children. No information is available regarding the effects of spousal donation on donor safety and recipient outcomes. Our purpose in this study was to determine how spousal liver grafts in living donor liver transplantation (LDLT) affect donor safety and recipient outcomes compared with those of LDLT from children. METHODS: We retrospectively analyzed 656 patients, including spouses and children, who underwent a right or extended right hepatectomy for living liver donation between January 2009 and December 2018. RESULTS: Spouses represented 18.8% (n = 123) of living liver donors. Female donors comprised 78.9% (n = 97) of spousal donors, and the proportion of male donors in the children group was 72.6% (n = 387). The mean donor operation time of the spousal group was shorter than that of the children group (330 min vs. 358 min; P = 0.011), and the complication rate in the spousal group was lower than that in the children group (12.2% vs. 22.9%; P = 0.006). However, there were no differences in severe complication rates, hospitalization, or liver function tests between the 2 groups at 3 months after donor surgery. The overall survival of recipients in the spousal group was not reduced compared to that of recipients in the children group. CONCLUSION: The present study suggests that, with careful selection, spousal donation is feasible and safe in LDLT.

Long-term outcomes of polypoidal choroidal vasculopathy in comparison with typical exudative age-related macular degeneration.

PURPOSE: To compare long-term outcomes between typical exudative age-related macular degeneration (TexAMD) and polypoidal choroidal vasculopathy (PCV), and to investigate factors related to the outcomes. METHODS: This retrospective study included 319 eyes (164 with TexAMD and 155 with PCV) treated with anti-vascular endothelial growth factor and followed more than 5 years. The primary outcome was visual acuity (VA) change from baseline to final visit. Linear regression analyses were used to determine factors associated with final VA. RESULTS: Baseline logMAR VA was 0.7 ± 0.5 in the TexAMD group and 0.5 ± 0.4 in the PCV group (p < 0.001). After a mean follow-up of 9 years, final VA was also significantly worse in the TexAMD group than in the PCV group (0.9 ± 0.6 vs. 0.6 ± 0.5; p < 0.001). The PCV group showed longer maintenance of improved vision and later onset of significant visual decline than the TexAMD group. In multivariate analysis, loss to follow-up, worse baseline VA, macular atrophy, and subretinal fibrosis were significantly associated with poor final VA in both groups. CONCLUSION: PCV eyes showed relatively favorable long-term visual outcome than TexAMD eyes. The results of this study emphasized the importance of compliance with treatment, along with other well-known prognostic factors.

OCULAR MANIFESTATIONS OF ASP38ALA AND THR59LYS FAMILIAL TRANSTHYRETIN AMYLOIDOSIS.

PURPOSE: To describe the ophthalmic manifestations of familial transthyretin amyloidosis (FTA) mutations, including Asp38Ala and Thr59Lys, which have not been previously reported to have ocular involvement. METHODS: This is an observational case series of prospectively collected data of 16 patients with FTA who were taking tafamidis for mild peripheral neuropathy and underwent a comprehensive ophthalmic examination at a single tertiary center, between January 2013 and March 2020. The ocular involvement of each FTA mutation type and the specific manifestations were the main outcome measures. RESULTS: Six of 16 patients with FTA manifested ocular involvement. Ocular involvement was noted in two of three patients with Glu89Lys mutations having retinal deposits, retinal hemorrhages, and corneal opacity. Three of nine patients with Asp38Ala mutations and one of two patients with Thr59Lys mutations showed ocular involvement that had not been previously described. The ophthalmic findings included glaucoma, anterior lens capsule opacity, vitreous opacity, and retinal deposits. The decrease in vascular flow due to perivascular cuffing of the amyloid deposits was detected by optical coherence tomography angiography. CONCLUSION: The current study newly described that two transthyretin mutation types of FTA, Asp38Ala and Thr59Lys, may manifest with ocular findings such as anterior lens capsule opacity and retinal deposits.

EN-FACE IMAGING OF ATYPICAL EPIRETINAL TISSUE IN LAMELLAR MACULAR HOLE.

PURPOSE: To elucidate the significance of en-face optical coherence tomography imaging of atypical epiretinal tissue (AET) in the lamellar macular hole (LMH). METHODS: This study involved 29 eyes of 29 patients who underwent surgical treatment for LMH with AET. Best-corrected visual acuity, metamorphopsia assessment (M-score), and optical coherence tomography were evaluated before and 6 months after surgery. The novel en-face optical coherence tomography parameters, such as the area of AET and hyperreflective fringe, were correlated with clinical factors before and after LMH surgery. RESULTS: Preoperatively, hyperreflective fringe was noted in 25 (86.2%) patients. The splitting of the inner retina, disruption of the ellipsoid zone, the extent of foveal cavitation, symptom duration, and change in best-corrected visual acuity were correlated with the area of AET (all P < 0.05). Multivariate regression analysis revealed that a larger area of AET was associated with longer symptom duration and less improvement in postoperative vision (all P < 0.05). CONCLUSION: The area of AET may represent the chronicity of LMH and is significantly associated with visual outcomes after LMH surgery. This novel en-face optical coherence tomography parameter can be used as a predictive factor for surgical outcomes in LMH with AET.

A structured exercise to relieve musculoskeletal pain caused by face-down posture after retinal surgery: a randomized controlled trial.

Face-down posture after vitrectomy physically burdens patients. Despite being of significant concern for patients, the intraoperative pain and discomfort has not been of great interest to retinal surgeons or researchers. This randomized controlled trial evaluated the effect of a 3-day novel structured exercise on reducing musculoskeletal pain from the face-down posture in 61 participants (31 in the exercise group) who underwent vitrectomy. Among the subjects, the median age was 62 years, 42 were female, 42 had macular holes, and 19 had retinal detachments. Participants in the exercise group received initial education on the exercise and performed three daily active exercise sessions. After the sessions, the exercise group had median numeric pain scores of 2, 1, and 1 at the back neck, shoulder, and lower back, respectively, while the control group had corresponding scores of 5, 3, and 4, respectively. The exercise group reported significantly lower pain scores (P = .003, .039, and .006 for the back neck, shoulder, and lower back, respectively). Application of the structured exercise would alleviate the patients' position-induced postoperative physical burden, by reducing pain and discomfort.

ASSOCIATIONS WITH RECURRENCE OF MACULAR EDEMA IN BRANCH RETINAL VEIN OCCLUSION AFTER THE DISCONTINUATION OF ANTI VASCULAR ENDOTHELIAL GROWTH FACTOR.

PURPOSE: To identify factors predicting the recurrence of macular edema after the discontinuation of intravitreal antivascular endothelial growth factor injection in patients with branch retinal vein occlusion. METHODS: This retrospective study included only subjects who had discontinued injections at 3 months after the final bevacizumab injection due to fully resolved macular edema. Fifty-two eyes meeting the criteria were included in the study and divided into two groups (recurrence and no recurrence). Clinical features and measurements of retinal thickness at the time of the diagnosis and when the decision to stop injections was made (stopping point) were analyzed. RESULTS: At the stopping point, the no recurrence group showed a thinner parafoveal inner retina, better best-corrected visual acuity, and lower incidence of ellipsoid zone disruption in multivariate logistic regression analysis (all P < 0.05). Similarly, parafoveal inner retinal thinning of more than 30 µm, when compared with the corresponding region of the fellow eye or the unaffected region of the affected eye, was significantly related to less recurrence of macular edema. CONCLUSION: Thinning of the parafoveal inner retina as well as better vision and intact outer retinal layers are associated with a lack of recurrence of macular edema. These findings suggest that inner retinal atrophy after branch retinal vein occlusion may result in a reduction in oxygen demand in the affected retinal tissue and less production of vascular endothelial growth factor.

Surgical treatment outcomes of primary hepatic sarcomas: A single-center experience.

BACKGROUND: Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it's rarity. However, with development of medical technology, surgical treatment may lead to better survival. AIM: To investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute. METHODS: From August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach. RESULTS: The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain (P = 0.002). CONCLUSION: Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.