BACKGROUND: Juvenile xanthogranuloma is a benign, self-limited disorder that usually occurs in infants and young children. Xanthogranuloma is rare in adults, and therefore studies reporting adult xanthogranuloma are limited. OBJECTIVE: We investigated the clinical, histopathological, and immunohistochemical characteristics of adult xanthogranuloma. METHODS: In this study, we evaluated 20 lesions in 19 patients with adult xanthogranuloma. RESULTS: A male predominance was observed (male : female ratio 1.4 : 1), and the mean age of patients was 35.1±16.3 years (range 15∼66 years), with the peak incidence observed in patients in their 20s. Notably, 65.0% of the lesions developed on the head and neck. The nodular form was more common than the papular form of this condition. Histopathological examination revealed dense monomorphic histiocytic infiltration without lipidization and scattered eosinophils without multinuclear giant cells in 5 lesions (25.0%), foamy histiocytic infiltration with variations of completely developed Touton giant cells in 10 lesions (50.0%), and fibrohistiocytic proliferation in 3 lesions (15.0%). On immunohistochemical examination, histiocytes including giant cells showed positive test results with Factor XIIIa (90.9%), vimentin (100%), and CD68 (100%) and negative test results with CD1a, smooth muscle actin, and S-100 protein stains. Tumor excision was the treatment for choice. CONCLUSION: Adult xanthogranuloma most commonly manifested as the nodular form of the disease on the head and neck of men in their late 20s. Histopathologically, the classic Touton cell-rich stage was most commonly observed, followed by the stage of early predominantly mononuclear infiltration. This was a single-center, small-sized retrospective study; however, we expect the results of this study to contribute to a better understanding of adult xanthogranuloma.
Actins ; Adult ; Child ; Coloring Agents ; Eosinophils ; Factor XIIIa ; Female ; Giant Cells ; Head ; Histiocytes ; Humans ; Incidence ; Infant ; Male ; Muscle, Smooth ; Neck ; Retrospective Studies ; S100 Proteins ; Vimentin ; Xanthogranuloma, Juvenile

STUDY DESIGN: Case report. OBJECTIVES: We report a case of paraspinal ancient schwannoma located at the upper thoracic level that mimicked an atypical lipoma or complicated epidermoid cyst. SUMMARY OF LITERATURE REVIEW: Few case reports of paraspinal schwannoma have been reported and the incidence of ancient schwannoma in the paraspinal muscle layer is very rare. MATERIALS AND METHODS: A 39-year-old man complained of a growing palpable back mass for 5 years. He experienced aggravated chronic discomfort around the mass while lying down. Both T1- and T2- weighted magnetic resonance imaging (MRI) showed a well-capsuled and heterogeneous high-signal mass in the muscle layer at the level from the T1 to T4 vertebral bodies on the right side of the midline. The tumor was completely removed by en bloc resection. RESULTS: The pathologic examination revealed S-100 protein expression with degenerative changes. The lesion was diagnosed as an ancient schwannoma. CONCLUSIONS: Schwannoma is one among the multiple possible causes of benign back masses. If a mass reveals a well-encapsulated heterogeneous mass on contrast MRI, a schwannoma should be suspected.
Adult ; Back Muscles ; Deception ; Epidermal Cyst ; Humans ; Incidence ; Lipoma ; Magnetic Resonance Imaging ; Neurilemmoma ; Paraspinal Muscles ; S100 Proteins

The aim of this study was to investigate the role of S100 calcium binding protein A16 (S100A16) in lipid metabolism in hepatocytes and its possible biological mechanism. HepG2 cells (human hepatoma cell line) were cultured with fatty acid to establish fatty acid culture model. The control model was cultured without fatty acid. Each model was divided into three groups and transfected with S100a16 over-expression, shRNA and vector plasmids, respectively. The concentration of triglyceride (TG) in the cells was measured by kit, and the lipid droplets was observed by oil red O staining. Immunoprecipitation and mass spectrometry were used to find the interesting proteins interacting with S100A16, and the interaction was verified by immunoprecipitation. The further mechanism was studied by Western blot and qRT-PCR. The results showed that the intracellular lipid droplet and TG concentrations in the fatty acid culture model were significantly higher than those in the control model. The accumulation of intracellular fat in the S100a16 over-expression group was significantly higher than that in the vector plasmid transfection group. There was an interaction between heat shock protein A5 (HSPA5) and S100A16. Over-expression of S100A16 up-regulated protein expression levels of HSPA5, inositol-requiring enzyme 1α (IRE1α) and pIREα1, which belong to endoplasmic reticulum stress HSPA5/IRE1α-XBP1 pathway. Meanwhile, over-expression of S100A16 up-regulated the mRNA expression levels of adipose synthesis-related gene Srebp1c, Acc and Fas. In the S100a16 shRNA plasmid transfection group, the above-mentioned protein and mRNA levels were lower than those of vector plasmid transfection group. These results suggest that S100A16 may promote lipid synthesis in HepG2 cells through endoplasmic reticulum stress HSPA5/IRE1α-XBP1 pathway.
Endoplasmic Reticulum Stress ; Endoribonucleases ; physiology ; Heat-Shock Proteins ; physiology ; Hep G2 Cells ; Humans ; Lipid Metabolism ; Protein-Serine-Threonine Kinases ; physiology ; S100 Proteins ; physiology ; Triglycerides ; biosynthesis ; X-Box Binding Protein 1 ; physiology

Damage-associated molecular patterns (DAMPs) are endogenous danger molecules that are released from damaged or dying cells and activate the innate immune system by interacting with pattern recognition receptors (PRRs). Although DAMPs contribute to the host's defense, they promote pathological inflammatory responses. Recent studies have suggested that various DAMPs, such as high-mobility group box 1 (HMGB1), S100 proteins, and heat shock proteins (HSPs), are increased and considered to have a pathogenic role in inflammatory diseases. Here, we review current research on the role of DAMPs in inflammatory diseases, including rheumatoid arthritis, systemic lupus erythematosus, osteoarthritis, atherosclerosis, Alzheimer's disease, Parkinson's disease, and cancer. We also discuss the possibility of DAMPs as biomarkers and therapeutic targets for these diseases.
Alzheimer Disease ; Arthritis, Rheumatoid ; Atherosclerosis ; Biomarkers ; Heat-Shock Proteins ; Immune System ; Inflammation ; Lupus Erythematosus, Systemic ; Osteoarthritis ; Parkinson Disease ; Receptors, Pattern Recognition ; S100 Proteins

A primary benign schwannoma of the liver is extremely rare. Only 30 cases have been reported in the medical literature worldwide, and only one case has been reported in Korea previously. A 56-year-old man was admitted to Gil Medical Center with incidental findings of a hepatic mass by abdominal computed tomography. The computed tomography and magnetic resonance image revealed a 3×2 cm-sized solid mass in the left lobe of the liver. Histological examination confirmed the diagnosis of a benign schwannoma, proven by positive immunoreaction with the neurogenic marker S-100 protein and a negative response to CD34, CD117, and smooth muscle actin. We report a primary benign schwannoma of the liver and review the literature.
Actins ; Diagnosis ; Humans ; Incidental Findings ; Korea ; Liver ; Middle Aged ; Muscle, Smooth ; Neurilemmoma* ; Peripheral Nervous System Neoplasms ; S100 Proteins

OBJECTIVE: This study aimed to determine whether simultaneous decreases in the serum levels of cell adhesion molecules (intracellular cell adhesion molecule-1 [ICAM-1], vascular cell adhesion molecule-1 [VCAM-1], and E-selectin) and S100 proteins within the first 24 hours after the return of spontaneous circulation were associated with good neurological outcomes in cardiac arrest survivors. METHODS: This retrospective observational study was based on prospectively collected data from a single emergency intensive care unit (ICU). Twenty-nine out-of-hospital cardiac arrest survivors who were admitted to the ICU for post-resuscitation care were enrolled. Blood samples were collected at 0 and 24 hours after ICU admission. According to the 6-month cerebral performance category (CPC) scale, the patients were divided into good (CPC 1 and 2, n=12) and poor (CPC 3 to 5, n=17) outcome groups. RESULTS: No difference was observed between the two groups in terms of the serum levels of ICAM-1, VCAM-1, E-selectin, and S100 at 0 and 24 hours. A simultaneous decrease in the serum levels of VCAM-1 and S100 as well as E-selectin and S100 was associated with good neurological outcomes. When other variables were adjusted, a simultaneous decrease in the serum levels of VCAM-1 and S100 was independently associated with good neurological outcomes (odds ratio, 9.285; 95% confidence interval, 1.073 to 80.318; P=0.043). CONCLUSION: A simultaneous decrease in the serum levels of soluble VCAM-1 and S100 within the first 24 hours after the return of spontaneous circulation was associated with a good neurological outcome in out-of-hospital cardiac arrest survivors.
Blood-Brain Barrier ; Cardiopulmonary Resuscitation ; Cell Adhesion ; Cell Adhesion Molecules ; E-Selectin ; Emergencies ; Endothelium ; Heart Arrest* ; Humans ; Intensive Care Units ; Intercellular Adhesion Molecule-1 ; Observational Study ; Out-of-Hospital Cardiac Arrest ; Prospective Studies ; Retrospective Studies ; S100 Proteins ; Survivors* ; Vascular Cell Adhesion Molecule-1*

Notalgia paresthetica (NP) is a chronic localized itch, affecting mainly the inter-scapular area particularly between the T2-T6 dermatomes. Occasionally it has a more widespread distribution and involves the shoulders, back, and upper chest. There are no specific cutaneous signs, apart from those attributed to scratching and rubbing. Various etiologies have been reported, but the cause of NP is not established. The current hypothesis regarding its etiology postulates that a neuropathic itch develops due to nerve entrapment of the posterior rami of spinal nerve arising at T2-T6. Another recent documented case showed an increase in the number of intradermal nerves by neural immunochistochemistry staining of S-100 protein, protein gene product 9.5 (PGP 9.5). Herein, we experienced an uncommon case of NP of the back and tried to clarify pathogenesis by using quantitative sensory testing, such as neurometer and Von-Frey filaments. Also, we performed neural immunochemistry to confirm an increase in nerve fibers at the site of the lesion.
Immunochemistry ; Nerve Compression Syndromes ; Nerve Fibers ; Pregabalin* ; S100 Proteins ; Shoulder ; Spinal Nerves ; Thorax

Hyalinizing Spitz nevus was first introduced in 1994 by Suster. It is considered a rare variant in the spectrum of Spitz nevus, with only a dozen cases having been reported to date. Herein we report a case of a 28-year-old Korean woman who had a brown papular lesion on the left palm, which was diagnosed as hyalinizing Spitz nevus. Upon histopathological investigation, discohesive growth patterns of nevus cells in a hyalinized stroma were noted. Immunohistochemical stains showed positive reactivity of nevus cells with S-100 protein and vimentin, but negative stains with HMB-45 and CD68. This case demonstrates a rare variant of Spitz nevus located on an unusual site, and calls for awareness in the diagnosis of cutaneous lesions that histologically exhibit a prominent hyalinized stroma.
Adult ; Biopsy ; Coloring Agents ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Hyalin* ; Nevus ; Nevus, Epithelioid and Spindle Cell* ; S100 Proteins ; Vimentin

A 39-year-old man visited Soonchunhyang University Gumi Hospital with abdominal pain. Abdominal computed tomography showed a large heterogeneously enhancing mass abutting the stomach body and a large amount of fluid collected in the peritoneal cavity. An emergency laparotomy revealed a ruptured large tumor arising from the anterior wall of the middle section of the stomach with bloody fluid in the peritoneal cavity. The patient underwent wedge resection of the tumor. The tumor was 6.5×5.5×4.0 cm in size and 49 g in weight. Immunohistochemical staining demonstrated positivity to C-kit protein, CD34, and S-100 protein. The final diagnosis was gastric gastrointestinal stromal tumor (GIST). Hemoperitoneum caused by a spontaneously ruptured GIST with cystic degeneration is extremely rare.
Abdominal Pain ; Adult ; Diagnosis ; Emergencies ; Gastrointestinal Stromal Tumors* ; Gyeongsangbuk-do ; Hemoperitoneum* ; Humans ; Laparotomy ; Peritoneal Cavity ; Proto-Oncogene Proteins c-kit ; Rupture ; S100 Proteins ; Stomach

PURPOSE: Solitary plexiform neurofibroma of the eyelid without neurofibromatosis is a rare disease. We report a case of solitary plexiform pigmented neurofibroma of the eyelid without neurofibromatosis. CASE SUMMARY: A 12-year-old male visited our clinic with a painless palpable subcutaneous mass on the right lower eyelid. He had a history of Batter syndrome and attention deficit hyperactivity disorder. On initial presentation, clinical features regarding neurofibromatosis such as Lisch nodule, optic nerve glioma, or high myopia were not observed. We performed excision and biopsy of the lower lid mass under general anesthesia. Macroscopically, the tumor was 4.0 × 1.5 × 1.5 cm in size with irregular nodules. Microscopically, the tumor consisted of multiple, variably sized tortous enlarged nerve fascicles with clusters of pigmented cells. Immunohistochemical results revealed expression of S-100 protein. Pigmented cells express both S-100 and melan-A proteins, while nonpigmented cells express S-100 protein only. The tumor was finally diagnosed as plexiform pigmented neurofibroma. Dermatological evaluation revealed no evidence of systemic neurofibromatosis. CONCLUSIONS: Plexiform neurofibroma should be considered in the differential diagnosis of an eyelid mass, even if the patient does not have a history or clinical features of neurofibromatosis. Plexiform neurofibroma can be successfully managed with surgical excision.
Anesthesia, General ; Attention Deficit Disorder with Hyperactivity ; Biopsy ; Child ; Diagnosis, Differential ; Eyelids* ; Humans ; Male ; MART-1 Antigen ; Myopia ; Neurofibroma ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Optic Nerve Glioma ; Rare Diseases ; S100 Proteins