Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver cancer affecting adolescents and young adults without any pre existing liver disease. Hyperammonemic encephalopathy (HAE) is a serious paraneoplastic syndrome, and several cases of HAE have been reported in patients with FLHCC. This condition is rare; hence, there are currently no management guidelines for cancer-related HAE. Herein, we report a case of an 18-year-old man with advanced FLHCC who developed HAE during the first course of chemotherapy consisting of cisplatin, doxorubicin, 5-fluorouracil, and interferon-α. He was successfully treated with continuous venovenous hemofiltration, sodium benzoate, sodium phenylbutyrate, and amino acid supplementation for HAE. After the second course of chemotherapy, he underwent surgery, and thereafter, his ammonia levels were normal without any ammonia scavenger therapy. Treatments for HAE described here will be helpful for this rare, but serious metabolic complication of FLHCC and could partially applied to HAE related to any malignancies.

Cutaneous Müllerian cyst (CMC) is a rare benign cystic lesion that typically occurs on the lower extremity of young females shortly after puberty. They have been widely regarded as Müllerian heterotopias due to the morphological similarities of the cyst lining cells to the epithelium of the fallopian tubes. A 16-year-old female presented with a two-year history of a solitary, subcutaneous mass involving the left ankle. She had no specific symptoms associated with the skin lesion. However, the lesion had been growing in size. Histopathological findings revealed cystic structures lined by pseudostratified ciliated cuboidal to columnar cells, accompanying intraluminal papillary projection. The immunohistochemical staining profiles, including positive staining for estrogen and progesterone receptors, were characteristic of the fallopian tube epithelium. After being surgically removed, the lesion was cured without recurrence. Based on her typical presentation and histopathological findings, we present this patient as a case of CMC.

Background: Various types of miniplates have been developed and used for the reduction of facial bone fractures. We introduced Yang’s Keyhole (YK) plate, and reported on its short-term stability. The purpose of this study was to evaluate the long-term stability of the YK plate, as a follow-up study, by examining the patients who had used the YK plate among the patients with the reduction of mandible fractures and who visited for plate removal. Methods: We reviewed the medical records of 16 patients who underwent mandibular fracture fixation using a YK plate (group I) and 17 patients who underwent mandibular fracture fixation using a conventional plate (group II). Assessment was then made on malunion, occlusal stability, discomfort during the application, and clinical symptoms. Results: From January 2015 to December 2017, a total of 36 patients underwent mandibular fracture surgery using a YK plate. A total of 16 patients received plate removal. Among them, 15 were male and 1 female. The average age was 26 years. The applied surgical sites were the 12 on mandibular angle, 4 on mandibular symphysis, and 2 on subcondyle. The application period of YK plate was an average of 335 days. During the same period, 45 people underwent surgery on the conventional plate. A total of 17 patients received plate removal. Among them, 15 were male and 2 females. The average age was 36 years. The applied surgical sites were the 8 on mandibular angle, 4 on mandibular symphysis, and 2 on subcondyle. The application period of the conventional plate was an average of 349 days. No malocclusion occurred at the time of removal, and occlusion was stable. No patient complained of joint disease or discomfort. Conclusion The YK plate system, in which the screw was first inserted and the plate was applied, for clinical convenience did not cause any particular problem and no significant difference from the conventional plate.

Passive fit of prosthesis is an essential property of implant supported prosthesis for long term success and minimization of complications. And the property is determined mostly by fabrication procedure. There were limitations of extensive implant prosthesis because conventional casting method generate contraction error of long span prosthesis. However, Computer-aided design/Computer-aided manufacturing (CAD/CAM) technology of 3D printing and milling metal framework can overcome those limitations. This case is a full mouth rehabilitation using extensive implant fixed prosthesis. Removable interim prosthesis was made for esthetic, functional evaluation and a guide for implant insertion. After the insertion, implant fixed interim prosthesis was delivered. After additional evaluation and adjustment, final prosthesis was designed with CAD, the fabricated with CAM. Milling technique was used for anterior screw type implant superstructure and 3D printing technique was used for the anterior and posterior implant copings. Fit of the final restoration was favorable. The practitioner and patient were both esthetically and functionally satisfied with the final result.
Dental Caries ; Humans ; Methods ; Mouth Rehabilitation ; Mouth ; Printing, Three-Dimensional ; Prostheses and Implants

PURPOSE: Severe cutaneous adverse reactions (SCARs), including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) to antiepileptic drug (AED), are rare, but result in significant morbidity and mortality. We investigated the major culprit drugs, clinical characteristics, and clinical course and outcomes of AED-induced SCARs using a nationwide registry in Korea. METHODS: A total of 161 patients with AED-induced SCARs from 28 referral hospitals were analyzed. The causative AEDs, clinical characteristics, organ involvements, details of treatment, and outcomes were evaluated. We compared the clinical and laboratory parameters between SJS/TEN and DRESS according to the leading causative drugs. We further determined risk factors for prolonged hospitalization in AED-induced SCARs. RESULTS: Carbamazepine and lamotrigine were the most common culprit drugs causing SCARs. Valproic acid and levetiracetam also emerged as the major causative agents. The disease duration and hospital stay in carbamazepine-induced SJS/TEN were shorter than those in other AEDs (P< 0.05, respectively). In younger patients, lamotrigine caused higher incidences of DRESS than other drugs (P= 0.045). Carbamazepine, the most common culprit drug for SCARs, was associated with a favorable outcome related with prolonged hospitalization in SJS (odds ratio, 0.12; 95% confidence interval, 0.02-0.63, P= 0.12), and thrombocytopenia was found to be a risk factor for prolonged hospitalization in DRESS. CONCLUSION: This was the first large-scale epidemiological study of AED-induced SCARs in Korea. Valproic acid and levetiracetam were the significant emerging AEDs causing SCARs in addition to the well-known offending AEDs such as carbamazepine and lamotrigine. Carbamazepine was associated with reduced hospitalization, but thrombocytopenia was a risk factor for prolonged hospitalization. Our results suggest that the clinical characteristics and clinical courses of AED-induced SCARs might vary according to the individual AEDs.
Anticonvulsants ; Carbamazepine ; Cicatrix ; Drug Hypersensitivity Syndrome ; Epidemiologic Studies ; Hospitalization ; Humans ; Incidence ; Korea ; Length of Stay ; Mortality ; Referral and Consultation ; Risk Factors ; Stevens-Johnson Syndrome ; Thrombocytopenia ; Valproic Acid

PURPOSE: A hybrid-dynamic conformal arc therapy (HDCAT) technique consisting of a single half-rotated dynamic conformal arc beam and static field-in-field beams in two directions was designed and evaluated in terms of dosimetric benefits for radiotherapy of lung cancer. MATERIALS AND METHODS: This planning study was performed in 20 lung cancer cases treated with the VERO system (BrainLAB AG, Feldkirchen, Germany). Dosimetric parameters of HDCAT plans were compared with those of three-dimensional conformal radiotherapy (3D-CRT) plans in terms of target volume coverage, dose conformity, and sparing of organs at risk. RESULTS: HDCAT showed better dose conformity compared with 3D-CRT (conformity index: 0.74 ± 0.06 vs. 0.62 ± 0.06, p < 0.001). HDCAT significantly reduced the lung volume receiving more than 20 Gy (V20: 21.4% ± 8.2% vs. 24.5% ± 8.8%, p < 0.001; V30: 14.2% ± 6.1% vs. 15.1% ± 6.4%, p = 0.02; V40: 8.8% ± 3.9% vs. 10.3% ± 4.5%, p < 0.001; and V50: 5.7% ± 2.7% vs. 7.1% ± 3.2%, p < 0.001), V40 and V50 of the heart (V40: 5.2 ± 3.9 Gy vs. 7.6 ± 5.5 Gy, p < 0.001; V50: 1.8 ± 1.6 Gy vs. 3.1 ± 2.8 Gy, p = 0.001), and the maximum spinal cord dose (34.8 ± 9.4 Gy vs. 42.5 ± 7.8 Gy, p < 0.001) compared with 3D-CRT. CONCLUSIONS: HDCAT could achieve highly conformal target coverage and reduce the doses to critical organs such as the lung, heart, and spinal cord compared to 3D-CRT for the treatment of lung cancer patients.
Heart ; Humans ; Lung Neoplasms ; Lung ; Organs at Risk ; Radiotherapy ; Radiotherapy, Conformal ; Spinal Cord

Verruca plana is a subtype of warts, which are one of the most common dermatological diseases. A 37-year-old man presented with multiple asymptomatic skin-colored, 1∼2-mm, flat-topped papules on both arms, neck, and face. The patient had ulcerative colitis, which had been treated with immunosuppressants for 15 years. After skin biopsy, verruca plana was confirmed. The patient was treated with 5% imiquimod cream for 6 months. However, only mild improvement was observed. Acitretin was then added to the treatment regimen. After 44 weeks of treatment, acitretin was stopped. Dramatic and rapid clinical improvement was achieved after 3 weeks of treatment, and no sign of recurrence after treatment cessation has been reported for 60 weeks. Consequently, the combination of oral acitretin and topical 5% imiquimod cream should be recommended for the effective and safe treatment of recalcitrant verruca plana in immunosuppressed patients.
Acitretin* ; Adult ; Arm ; Biopsy ; Colitis, Ulcerative ; Humans ; Immunosuppressive Agents ; Neck ; Recurrence ; Skin ; Warts* ; Withholding Treatment

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.
Biopsy ; Diagnosis ; Eyelids* ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Intention* ; Methods ; Mitosis ; Muscle, Skeletal ; Necrosis ; Recurrence ; Sarcoma*

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.
Biopsy ; Diagnosis ; Eyelids* ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Intention* ; Methods ; Mitosis ; Muscle, Skeletal ; Necrosis ; Recurrence ; Sarcoma*