Papillary Histiocytic and Mesothelial Hyperplasia of the Ovary Related to a Huge Uterus: A Case Report

Nodular histiocytic/mesothelial hyperplasia (NHMH) is a very rare condition. It is generally thought to be associated with repeated mechanical or chemical stimulation. This study reported NHMH with papillary growth pattern in the ovary following high-intensity focused ultrasound (HIFU) treatment for uterine leiomyoma and adenomyosis. A 48-year-old female, who had a history of undergoing HIFU treatment 7 times for adenomyosis and leiomyoma of the uterus 6 months ago, was referred to the hospital. After the hysterectomy and right salpingo-oophorectomy, the patient was confirmedly diagnosed with nodular histiocytic and mesothelial hyperplasia with the papillary growth pattern of the right ovary. This benign reactive inflammatory lesion of the mesothelium mimicking malignancy must be kept in mind to avoid unnecessary treatment.

Human Herpesvirus-8 Positive Multicentric Castleman’s Disease with Complete Response after Rituximab Monotherapy: A Case Report

Castleman’s disease is a rare non-neoplastic lymphoproliferative disorder of unknown origin. It is classified into unicentric or multicentric based on its anatomical distribution. Multicentric Castleman’s disease can be subdivided according to the presence of human herpesvirus-8 (HHV-8) infection. Castleman’s disease has a rare incidence, and HHV-8-positive multicentric Castleman’s disease is even rarer. There are several types of natural course for this disease, and the rapidly progressing type can lead to death within a few weeks, emphasizing the need for prompt diagnosis and treatment. We report a recent case from Korea, presenting with multiple lymphadenopathies, confirmed as HHV-8-positive multicentric Castleman’s disease through biopsy, and achieving complete response with rituximab monotherapy.

Appendiceal Mucocele Developed by Appendiceal Mucosal Prolapse: A Rare Case Report with Review of Literature

Appendiceal mucocele formation can be induced by mechanical obstruction due to various reasons; In very rare cases, mucosal prolapse of the appendix can also cause mucocele. We first present the case of a 38-year-old woman who was diagnosed with appendiceal mucocele associated with appendiceal mucosal prolapse. The lesion was first found by colonoscopy and treated surgically. On examining the specimen, it revealed a dilated lumen containing mucinous fluid at the proximal portion, a polypoid mass at the mid portion, and a slightly dilated distal portion filled with mucoid material. But there was no histologic evidence of polyp. We report the first case of mucosal prolapse of the appendix resulting in mucocele with a review of the concepts of prolapse.

A Case of Solitary Metastatic Deposit in the Orbital Rim from Follicular Thyroid Cancer / 대한두경부종양학회지

Follicular thyroid carcinoma (FTC) is the second most common thyroid cancer, following papillary carcinoma. Metastasis to the orbital rim from FTC is very rare. We recently experienced a case of FTC with metastasis to the orbital rim in a 74-year-old woman, who initially presented with a huge thyroid mass and an asymptomatic solitary orbital rim lesion. The solitary orbital rim lesion was suspected to be a separate disease entity such as lymphoma from the preoperative imaging, but bone metastasis from FTC was finally confirmed after orbital rim resection and total thyroidectomy. During follow-up, the patient presented multiple bone metastasis, so the solitary orbital rim lesion was considered a clinical sign of systemic metastasis from FTC. Therefore, we present this unique case with a review of the literature.

A Case of Chronic Tonsillitis Mimicking a Parapharyngeal Space Tumor / 대한이비인후과학회지

Parapharyngeal space (PPS) tumors are extremely rare, composing only 0.5% of all head and neck tumors. The PPS is shaped like an inverted pyramid bounded by the skull base superiorly and hyoid bone inferiorly. Recently, we experienced a 55-year-old female patient who visited the clinic with a presumed PPS tumor that had been incidentally identified by neck MRI. The patient underwent surgery for the removal of tumor by transoral approach following tonsillectomy. However, contrary to the initial expectations, no tumors were visible at the operative field and only observed were the evidence of chronic inflammatory tissues within the removed tonsil. To date, chronic follicular tonsillitis mimicking a PPS tumor has not been reported in the literature. Therefore, we report this case with a brief discussion of points to consider supported by a review of relevant literature.

Complete denture rehabilitation of edentulous patient with severe alveolar bone resorption andcondyle fracture using gothic arch tracing and closed mouth impression technique: A case report / 대한치과보철학회지

It is essential to record maxillomandibular relationship accurately for the harmony of esthetic and function in complete denture. Gothic arch tracing visually demonstrates themovement of the mandible, and is useful to establish accurate and reproducible centric relation. Proper retention and stability of complete denture in patients with severe alveolarbone resorption is difficult to attain. In such case, the closed mouth impression technique might be recommended. The denture border and impression are determined bypatient’s physiologic movement in the closed mouth impression technique. And, denture peripheral border is entirely closed with oral mucous membrane. This report presentssatisfactory complete denture restoration using closed mouth impression technique and gothic arch tracing in patients with mandibular condyle fracture and severe absorptionof mandibular alveolar ridge. (J Korean Acad Prosthodont 2020;58:145-52)

Disseminated Peritoneal Leiomyomatosis with Atypical Features and Comorbid Uterine STUMP: a Case Report and Review of the Literature

Disseminated peritoneal leiomyomatosis (DPL) is a very rare benign disease, characterized by multiple solid subperitoneal or peritoneal smooth muscle nodules in abdominopelvic cavity and malignant transformation is extremely rare. Also, uterine smooth muscle tumors of unknown malignant potential (STUMP) is a rare tumor, which is regarded as subclassification in uterine smooth muscle tumors between benign and malignant criteria. Pathogenesis of DPL is uncertain, but increasing evidence of iatrogenic cause including laparoscopic myomectomy has been reported.We report a case of a 28-year-old female with previous history of laparoscopic myomectomy diagnosed with DPL with atypical feature and concurrent uterine STUMP using computed tomography (CT) and magnetic resonance imaging (MRI), as well as present a review of the literature.

Overexpression of Nrf2 promotes colon cancer progression via ERK and AKT signaling pathways

Purpose@#We investigated the expression of Nrf2 in colorectal cancer and its correlation with clinicopathological characteristics as well as mechanisms and roles of Nrf2 expression including cell signaling pathway, survival, proliferation, and migration. @*Methods@#Nrf2 expression was measured in 12 and 30 different colorectal cancer (CRC) tissues by western blot (WB) and immunohistochemistry (IHC), respectively. SW480 cells were used for cell proliferation and cell migration tests. The correlation between the expression of Nrf2 and clinicopathologic parameters were evaluated using the chi-square or Fisher exact test. Data are expressed as the mean ± standard deviation for 3 independent experiments. P < 0.05 was considered statistically significant. @*Results@#Analysis of WB demonstrated that Nrf2 proteins were increased in CRC tissues, and decreased in normal tissues. IHC staining showed that the Nrf2 expression was elevated in CRC tissues, compared to matched normal tissues. When SW480 cells were suppressed with small interfering RNA of Nrf2, cell viability was inhibited, and cell apoptosis was increased. These results were found along with suppression of the phosphorylated form of extracellular signal-regulated kinase 1/2 and AKT. @*Conclusion@#This study suggests that overexpression of Nrf2 may be related to carcinogenesis and progression of CRC.

Functional Outcomes of Hip Arthroscopy for Pediatric and Adolescent Hip Disorders

BACKGROUND: There is a paucity of literature on the use of hip arthroscopy for pathologic conditions in skeletally immature patients. Thus, the indications and safety of the procedure are still unclear. The purpose of this study was to investigate the safety and functional outcomes of hip arthroscopy for pediatric and adolescent hip disorders. We further attempted to characterize arthroscopic findings in each disease.METHODS: We retrospectively reviewed 32 children and adolescents with hip disorders who underwent 34 hip arthroscopic procedures at a tertiary care children's hospital from January 2010 to December 2016. We evaluated functional limitations and improvement after operation by using the modified Harris hip score (HHS), the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), subjective pain assessment with a visual analog scale (VAS), and range of hip motion as well as the complications of hip arthroscopy. Arthroscopic findings in each disease were recorded.RESULTS: Hip arthroscopy was performed for Legg-Calvé-Perthes disease (n = 6), developmental dysplasia of the hip (n = 6), slipped capital femoral epiphysis (n = 5), idiopathic femoroacetabular impingement (n = 6), sequelae of septic arthritis of the hip (n = 3), hereditary multiple exostosis (n = 2), synovial giant cell tumor (n = 3), idiopathic chondrolysis (n = 2), and posttraumatic osteonecrosis of the femoral head (n = 1). Overall, there was a significant improvement in the modified HHS, WOMAC, VAS, and range of hip motion. Symptom improvement was not observed for more than 18 months in four patients who had dysplastic acetabulum with a labral tear (n = 2) or a recurrent femoral head bump (n = 2). There were no complications except transient perineal numbness in five patients.CONCLUSIONS: Our short-term follow-up evaluation shows that hip arthroscopy for pediatric and adolescent hip disorder is a less invasive and safe procedure. It appears to be effective in improving functional impairment caused by femoroacetabular impingement between the deformed femoral head and acetabulum or intra-articular focal problems in pediatric and adolescent hip disorders.

Diagnosis and Management of Hip Dislocation in Patients with Kabuki Syndrome

BACKGROUND: Kabuki syndrome is a rare genetic disorder characterized by distinct dysmorphic facial features, growth deficiency, intellectual disabilities, unusual dermatoglyphic patterns, and skeletal abnormalities. The incidence of hip dislocation in Kabuki syndrome ranges from 18% to 62%. We reviewed the outcomes of management of hip dislocations in patients with Kabuki syndrome with special attention to the diagnostic processes for hip dislocation and Kabuki syndrome. METHODS: Among 30 patients with mutation-confirmed Kabuki syndrome, we selected six patients who had hip dislocations and reviewed their medical records and plain radiographs. The modes of presentation and diagnostic processes for both hip dislocations and Kabuki syndrome were investigated. The management and treatment outcomes of hip dislocations in patients with Kabuki syndrome were evaluated. RESULTS: The average age of patients at the time of diagnosis of hip dislocation was 7.7 months (range, 1 week to 22 months). None of the patients were diagnosed as having Kabuki syndrome at that time. Two patients were treated with a Pavlik harness; one, with closed reduction; two, with open reduction and later pelvic and/or femoral osteotomies; and one, with open reduction combined with pelvic osteotomy. The patients were followed up for 5.8 years on average (range, 2.0 to 10.5 years). The radiologic outcome was graded as Severin IA or IB for three patients who were older than 6 years at the latest follow-up (mean age, 9.9 years; range, 7.8 to 12.4 years). In the remaining three patients younger than 6 years (mean age, 3.8 years; range, 2.7 to 5.3 years), the lateral center edge angle was more than 15°. The clinical diagnosis of Kabuki syndrome was made during follow-up after hip dislocation treatment and confirmed by mutational analysis at a mean age of 4.7 years. The mean interval between the diagnosis of hip dislocation and Kabuki syndrome was 4.0 years. CONCLUSIONS: The management of hip dislocation by conservative or surgical method showed successful results. Awareness of Kabuki syndrome could lead to an early diagnosis of this rare disease in patients with hip dislocation and allow for early detection of other underlying conditions and multidisciplinary management.

Concurrent Medullary Thyroid Carcinoma and Primary Thyroid Lymphoma (Diffuse Large B Cell Lymphoma): the First Case Report

Cases of simultaneously occurring medullary thyroid carcinoma (MTC) and lymphoma are extremely rare. An 84-year-old woman visited the hospital due to dyspnea, resulting from rapidly aggravated enlarged neck mass. Ultrasonography revealed two lesions in the thyroid and they were diagnosed as concurrent medullary thyroid carcinoma and diffuse large B cell lymphoma after total thyroidectomy. A few cases simultaneously diagnosed with MTC and systemic lymphoma have been reported. However, the coexistence of MTC and primary thyroid lymphoma is extremely rare.

Differential Diagnosis of Thyroid Follicular Neoplasm from Nodular Hyperplasia by Shear Wave Elastography

OBJECTIVE: More than 50% of diagnostic surgery in cytologically indeterminate thyroid nodules revealed benign nodules of which nodular hyperplasia (NH) accounted for about half. Preoperative exclusion of NH may decrease diagnostic surgery. We aimed to study the diagnostic performance of shear wave elastography (SWE) to differentiate follicular neoplasm (FN) from NH in follicular lesions of thyroid nodules. METHODS: We analyzed the data from 61 patients who underwent SWE before ultrasound-guided core-needle biopsy (CNB) from August 2016 to May 2018 and CNB results were FN (n=19) and NH (n=42). We analyzed the magnitude of elasticity index (EI) and patterns of high EI area (EI >36 kPa). The patterns of high EI area was classified as marginal pattern (high EI areas are restricted in the outer 1/3 of the nodule) and traversing pattern (high EI areas approaching further to the center of the nodule within inner 2/3 of the nodule). RESULTS: The E(Max), E(Mean), E(SD), and percent of high EI area were significantly lower in FN than NH (P<0.001). The diagnostic performance to predict FN showed sensitivity, specificity, and accuracy of 95%, 90%, and 92% by E(Max) <42.1 kPa, and of 95%, 79%, and 84% by E(Mean) <23.5 kPa, and of 89%, 93%, and 92% by marginal pattern of high EI area, respectively. CONCLUSION: Novel diagnostic criteria of E(Max) less than 42.1 kPa and marginal pattern of high EI area on SWE can predict FN with high diagnostic accuracy, waiving diagnostic surgery of NH in indeterminate cytology.

Utility of shear wave elastography to detect papillary thyroid carcinoma in thyroid nodules: efficacy of the standard deviation elasticity

BACKGROUND/AIMS@#The aim of this study was to address the role of the elasticity index as a possible predictive marker for detecting papillary thyroid carcinoma (PTC) and quantitatively assess shear wave elastography (SWE) as a tool for differentiating PTC from benign thyroid nodules.@*METHODS@#One hundred and nineteen patients with thyroid nodules undergoing SWE before ultrasound-guided fine needle aspiration and core needle biopsy were analyzed. The mean (E(Mean)), minimum (E(Min)), maximum (E(Max)), and standard deviation (E(SD)) of SWE elasticity indices were measured.@*RESULTS@#Among 105 nodules, 14 were PTC and 91 were benign. The E(Mean), E(Min), and E(Max) values were significantly higher in PTCs than benign nodules (E(Mean) 37.4 in PTC vs. 23.7 in benign nodules, p = 0.005; E(Min) 27.9 vs. 17.8, p = 0.034; E(Max) 46.7 vs. 31.5, p < 0.001). The E(Mean), E(Min), and E(Max) were significantly associated with PTC with diagnostic odds ratios varying from 6.74 to 9.91, high specificities (86.4%, 86.4%, and 88.1%, respectively), and positive likelihood ratios (4.21, 3.69, and 4.82, respectively). The E(SD) values were significantly higher in PTC than in benign nodules (6.3 vs. 2.6, p < 0.001). E(SD) had the highest specificity (96.6%) when applied with a cut-off value of 6.5 kPa. It had a positive likelihood ratio of 14.75 and a diagnostic odds ratio of 28.50.@*CONCLUSIONS@#The shear elasticity index of E(SD), with higher likelihood ratios for PTC, will probably identify nodules that have a high potential for malignancy. It may help to identify and select malignant nodules, while reducing unnecessary fine needle aspiration and core needle biopsies of benign nodules.

Mucinous Adenocarcinoma Arising within a Colonic Diverticulum Mimicking a Diverticular Abscess: A Case Report

Colon cancer arising in a colonic diverticulum is very rare. There are only a few reported cases of colon cancer associated with a diverticulum. Of these reported cases, only a few are those of a mucinous adenocarcinoma. Here, we report a case of an 82-year-old female with a mucinous adenocarcinoma arising in the ascending colonic diverticulum, which clinically and radiologically mimicked perforated diverticulitis with abscess formation. Although such cases are rare, our findings suggest that malignant tumors may be misdiagnosed as diverticular diseases and should be considered during work-up.

Regarding Camurati-Engelmann Disease: In Reply

No abstract available.

Orthopedic Manifestations of Type I Camurati-Engelmann Disease

BACKGROUND: Camurati-Engelmann disease (CED) is a rare genetic skeletal disorder characterized by limb pain, muscle emaciation and weakness, and cortical thickening of the diaphysis of long bones. It is caused by mutations in the transforming growth factor beta 1 (TGFB1) (type I) or other unknown gene(s) (type II). We present 8 consecutive patients with type I CED. METHODS: We retrospectively reviewed medical records and radiographs of type I CED patients with special reference to the mode of presentation, process of diagnostic work-up, and disease course. They were 4 sporadic patients, and two pairs of mother and son. RESULTS: We categorized the mode of presentation into three groups. Group I had 4 patients who mainly presented with motor disturbances in young age. They drew medical attention for waddling gait, awkward ambulation or running, difficulty in going upstairs, or a positive Gower's sign at age 4 to 6 years. Subsequent development of limb pain and radiographic abnormality led to the diagnosis of CED at age 6 to 29 years. Group II had 3 patients who mainly presented with limb pain at age 15, 20, and 54 years, respectively. Radiographic evaluation and molecular genetic test led to the diagnosis of CED. The remaining 1 patient (group III) was asymptomatic until age 9 years when bony lesions at the tibiae were found incidentally. For the last 10 years, he intermittently complained of leg pain in the morning or after sports activities, which did not interfere with daily life. All the patients in group I showed a body mass index in the underweight range (< 18.4 kg/m²). At the latest follow-up, 4 patients in groups I and II required medication for the limb pain. CONCLUSIONS: CED presents with a wide range of severity. Awareness of this rare disease entity may be the key to timely correct diagnosis. This disease entity should be considered in the differential diagnosis of limb pain or motor disturbance in children to avoid unnecessary diagnostic work-up.

Solitary immunoglobulin G4-related inflammatory pseudotumor in the abdomen wall

No abstract available.

A Case Report of Central Retinal Artery Occlusion Caused by Cardiac Myxoma

PURPOSE: We report the case of a patient diagnosed with central retinal artery occlusion caused by cardiac myxoma who underwent surgery to remove the myxoma. CASE SUMMARY: A 47-year-old woman came to our clinic presenting with a sudden decrease of visual acuity in the left eye. At the first visit, left eye visual acuity was hand motion, and intraocular pressure was 15.4 mmHg. A relative afferent pupillary defect was observed in the left eye. On fundus examination, a pale retina and cherry-red spot were observed at the posterior pole. On optical coherence tomography, macular edema was found. On fluorescein angiography and indocyanine green angiography, delayed blood circulation of the retina and choroid was found at early and late stages. Cerebral angiography was performed in the neurosurgery department and showed no occlusion of the ophthalmic artery. Cardiac ultrasonography and brain magnetic resonance imaging were performed. On cardiac ultrasonography, 4.46 × 2.09 cm cardiac myxoma was found. Resection of the cardiac myxoma was conducted in the thoracic and cardiovascular surgery department. Multiple cerebral infarcts were detected by brain imaging, and antithrombotic treatment was administered. After one month, blood circulation in the retina and choroid was observed in fluorescence angiography, but there was no improvement of visual acuity. At the 3-month follow-up visit, macular edema was decreased, but retinal atrophy and epiretinal membrane were observed on optical coherence tomography. CONCLUSIONS: Central retinal artery occlusion is a disease related to one's general condition. We experienced this case of central retinal artery occlusion caused by cardiac myxoma.

Sjogren's Syndrome Presenting as a Large Pericardial Effusion / 대한내과학회지

Primary Sjogren's syndrome (pSS) is characterized by lymphocytic infiltration to the lacrimal and salivary glands. However, it also includes a wide spectrum of clinical manifestations, including musculoskeletal, lung, kidney, neurological, and cardiac manifestations. Cardiac involvement in pSS leads to various conditions including pericardial effusion (PE), left heart failure, pulmonary hypertension, and left atrial hypertrophy. A large PE without pulmonary arterial hypertension is extremely rare and there has been no case in Korea, to date. We report a case of a 45-year-old woman with newly-diagnosed pSS who presented with a large PE and pleural effusion. The patient experienced a significant improvement of the PE following treatment with steroids.

Analysis of Histologic Features Suspecting Anaplastic Lymphoma Kinase (ALK)-Expressing Pulmonary Adenocarcinoma

BACKGROUND: Since 2007 when anaplastic lymphoma kinase (ALK) rearrangements were discovered in non-small cell lung cancer, the ALK gene has received attention due to ALK-targeted therapy, and a notable treatment advantage has been observed in patients harboring the EML4/ALK translocation. However, using ALK-fluorescence in situ hybridization (FISH) as the standard method has demerits such as high cost, a time-consuming process, dependency on interpretation skill, and tissue preparation. We analyzed the histologic findings which could complement the limitation of ALK-FISH test for pulmonary adenocarcinoma. METHODS: Two hundred five cases of ALK-positive and 101 of ALK-negative pulmonary adenocarcinoma from January 2007 to May 2013 were enrolled in this study. The histologic findings and ALK immunohistochemistry results were reviewed and compared with the results of ALK-FISH and EGFR/KRAS mutation status. RESULTS: Acinar, cribriform, and solid growth patterns, extracellular and intracellular mucin production, and presence of signet-ring-cell element, and psammoma body were significantly more often present in ALK-positive cancer. In addition, the presence of goblet cell-like cells and presence of nuclear inclusion and groove resembling papillary thyroid carcinoma were common in the ALK-positive group. CONCLUSIONS: The above histologic parameters can be helpful in predicting ALK rearranged pulmonary adenocarcinoma, leading to rapid FISH analysis and timely treatment.