IgG4 Related Disease Misdiagnosed to Carotid Body Tumor / 대한두경부종양학회지

Neck mass has various etiologies, including inflammatory, congenital, neoplastic causes. The IgG4-related disease can cause symptoms in the head and neck areas with an inflammatory neck mass. It also shows clinical and pathological findings from inflammation caused by immune reactions, such as lymphocyte and plasma cell infiltration, storiform fibrosis, obliteration phlebitis, and invigorated serum IgG4 levels. The treatment guideline has not been established and still under debate, but systemic glucocorticoid seems to be effective in the most cases. In this brief report, a 48-year-old male patient presented with voice change for 3 weeks. Left side paramedian vocal fold palsy was observed in the flexible laryngoscopy. About 2.5X2.0X1.2cm size, heterogeneously enhanced neck mass with irregular margin encasing left carotid artery was noted on preoperative contrast enhanced neck CT scan, and it was suspicious of left carotid body tumor. The pathology shows IgG4-related disease rather than carotid body tumors. We report this case of IgG4-related disease, which can be misdiagnosed to carotid body tumors.

Appendiceal Mucinous Neoplasm Detected due to the Protrusion of Mucin, in the Absence of Appendiceal Distension: A Case Report / 대한소화기암학회지

A mucocele is a cystic dilatation of the vermiform appendix that contains mucous material. The symptoms associated with it are not specific and the diagnosis is seldom made prior to surgery. The reported prevalence in appendectomy specimens procured during surgery is 0.2-0.3%. Recently, we experienced a case of patient with appendiceal mucocele detected by colonoscopic examination. This case did not show typical colonoscopic features of a mucocele, demonstrating protrusion of mucin, in the absence of a smooth mound with normal overlying mucosa surrounding the appendiceal orifice. The case involved a 64-year-old woman who underwent a colonoscopy. An abdominopelvic computed tomography scan suggested a normal appendix. Subsequently, we performed an appendectomy. The pathologic finding was a low-grade appendiceal mucinous neoplasm.

IgG4 Related Disease Misdiagnosed to Carotid Body Tumor / 대한두경부종양학회지

Neck mass has various etiologies, including inflammatory, congenital, neoplastic causes. The IgG4-related disease can cause symptoms in the head and neck areas with an inflammatory neck mass. It also shows clinical and pathological findings from inflammation caused by immune reactions, such as lymphocyte and plasma cell infiltration, storiform fibrosis, obliteration phlebitis, and invigorated serum IgG4 levels. The treatment guideline has not been established and still under debate, but systemic glucocorticoid seems to be effective in the most cases. In this brief report, a 48-year-old male patient presented with voice change for 3 weeks. Left side paramedian vocal fold palsy was observed in the flexible laryngoscopy. About 2.5X2.0X1.2cm size, heterogeneously enhanced neck mass with irregular margin encasing left carotid artery was noted on preoperative contrast enhanced neck CT scan, and it was suspicious of left carotid body tumor. The pathology shows IgG4-related disease rather than carotid body tumors. We report this case of IgG4-related disease, which can be misdiagnosed to carotid body tumors.

Appendiceal Mucinous Neoplasm Detected due to the Protrusion of Mucin, in the Absence of Appendiceal Distension: A Case Report / 대한소화기암학회지

A mucocele is a cystic dilatation of the vermiform appendix that contains mucous material. The symptoms associated with it are not specific and the diagnosis is seldom made prior to surgery. The reported prevalence in appendectomy specimens procured during surgery is 0.2-0.3%. Recently, we experienced a case of patient with appendiceal mucocele detected by colonoscopic examination. This case did not show typical colonoscopic features of a mucocele, demonstrating protrusion of mucin, in the absence of a smooth mound with normal overlying mucosa surrounding the appendiceal orifice. The case involved a 64-year-old woman who underwent a colonoscopy. An abdominopelvic computed tomography scan suggested a normal appendix. Subsequently, we performed an appendectomy. The pathologic finding was a low-grade appendiceal mucinous neoplasm.

Bisifusarium Delphinoides, an Emerging Opportunistic Pathogen in a Burn Patient with Diabetes Mellitus

An 82-year-old man with diabetes was admitted to the emergency department with a third-degree burn on his left leg. The deep swab specimen from his left leg was cultured on Sabouraud dextrose agar without cycloheximide and incubated at 25 °C for 5 days. On the basis of morphological characteristics and multigene phylogenetic analyses of the internal transcribed spacer region of ribosomal DNA and partial fragments of beta-tubulin and translation elongation factor 1-alpha, the causal agent of fungal skin infection was identified as Bisifusarium delphinoides, which was newly introduced by accommodating a Fusarium dimerum species complex. Thus, we describe here the first case of skin infection caused by B. delphinoides on a burn patient with diabetes mellitus based on morphological observation and molecular analysis.

Comparison of Radiological and Histological Findings of Lung Parenchyma in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

PURPOSE: The present study investigated chest computed tomography (CT) patterns and lung histological features, as well as the consistency between radiological and histological features among patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA (EGPA). MATERIALS AND METHODS: The medical records of 74 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with radiological lung parenchymal lesions were reviewed along with the histological results for 28 of them. Chest CT patterns were divided according 12 items mostly suggested by radiologists and histological features were divided according to necrotising granuloma, necrotising vasculitis, eosinophilic infiltration, and hemosiderin laden macrophages as defined by a pathologist. RESULTS: The mean age was 57.1 years (22 men). The most common clinical manifestation other than lung manifestation was renal manifestation (62.2%), and the most common chest CT pattern was lung involvement of vasculitis (35.1%). In MPA patients, the major histological features were hemosiderin-laden macrophages in the alveolar space and vasculitis. In GPA patients, the major histological features were necrotizing vasculitis and necrotizing granuloma, while in EGPA patients, the major histological feature was only necrotising vasculitis. The consistency rate in GPA patients was the highest (100%), followed by that in MPA patients (66.7%) and EGPA patients (50.0%). CONCLUSION: When lung involvement of AAV is suspected on chest CT, lung biopsy should be recommended for the proper classification of AAV, due to the discordance rate between radiological and histological findings in MPA and EGPA patients, but not GPA patients.

A Case of Idiopathic Massive Rice Bodies in the Knee Joint without Rheumatoid Arthritis or Tuberculosis and a Literature Review

Rice bodies are materials with an amorphous nucleus and a fibrin layer found floating in the synovial space and bursa. These bodies have often been detected in patients with rheumatoid arthritis, tuberculous arthritis, and bursitis. Although the etiology and pathogenesis of rice bodies are not yet fully understood, it has been hypothesized that they might be caused by chronic inflammation originating from the synovium. However, we report on a case of idiopathic massive rice bodies in the knee joint without evidence of inflammatory articular disease or infection including rheumatoid arthritis, seronegative spondyloarthritides, tuberculosis, or bacterial or fungal infection.

An Unusual Cause of Gastrointestinal Hemorrhage: Gastrocolic Fistula Caused by Colon Cancer Invasion / 영남의대학술지

Gastrocolic fistula is a fistulous communication between the stomach and the colon. It is a passage between the gastric epithelium and the colonic epithelium. This uncommon complication is caused by benign and malignant diseases of the stomach or the colon. Its clinical manifestations include weight loss, diarrhea and fecal vomiting; occasionally, anemia, poor oral intake, fatigue and dizziness; and very rarely, gastrointestinal bleeding. In this paper, an unusual case of gastrocolic fistula accompanied by hematochezia, which was revealed to have been caused by colon cancer invasion, is described.

Human Cutaneous Protothecosis: Report of a Case and Literature Review

The Prototheca species is achlorophyllic algae and rarely causes human infection. Human protothecosis presents clinically as a cutaneous infection, olecranon bursitis, and disseminated systemic disease. We report a case of human cutaneous protothecosis involving the left wrist. A 68-year-old man presented with an ill-defined erythematous lesion with crust at the dorsal aspect of his left wrist. A punch biopsy was performed to reveal the histologic features of granulomatous inflammation with necrosis at the upper dermis, containing Prototheca organisms, of which, the characteristic features were highlighted by special staining. Through a molecular study, the Prototheca zopfii species was identified.

Recurrent Cerebral Infarction due to Rhino-Orbito-Cerebral Mucormycosis / 대한의진균학회지

Rhino-orbito-cerebral mucormycosis (ROCM) is caused by invasion of orbital and intracranial structures directly or through the blood vessels of fungi of the Order Mucorales. It is the most fulminant form of mucormycosis and can manifest brain abscess, cranial nerve palsies, thrombosis or aneurysm, as well as sinusitis, facial or nasal deformity. We report one ROCM case complicated by recurrent cerebral infarctions after involvement of cavernous sinus and meningeal inflammation, despite treatment with surgical debridement and high dose intravenous amphotericin B.

Treatment of Recurrent Conjunctival Papilloma with Topical Mitomycin C

PURPOSE: To provide a case report of 1 patient (1 eye) who experienced recurrences of conjunctival papilloma and was treated with complete resection, electrocauterization, cryotherapy, intraoperative application of mitomycin C and postoperative topical mitomycin C. CASE SUMMARY: We evaluated the clinical course of a 42-year-old male with recurrent conjunctival papillomas on the left upper and lower tarsal conjunctiva who was treated 10 times with a simple resection over the past 8 years. The patient was treated with complete resection, electrocauterization, intraoperative application of mitomycin C and cryotherapy. Topical mitomycin C was applied for 1 month. There was no recurrence or complications for 12 months postoperatively. CONCLUSIONS: Complete resection, electrocauterization, intraoperative application of mitomycin C, cryotherapy and topical mitomycin C in patients with recurrent conjunctival papilloma was shown to be an effective treatment without recurrence and complications.

Treatment of Recurrent Conjunctival Papilloma with Topical Mitomycin C

PURPOSE: To provide a case report of 1 patient (1 eye) who experienced recurrences of conjunctival papilloma and was treated with complete resection, electrocauterization, cryotherapy, intraoperative application of mitomycin C and postoperative topical mitomycin C. CASE SUMMARY: We evaluated the clinical course of a 42-year-old male with recurrent conjunctival papillomas on the left upper and lower tarsal conjunctiva who was treated 10 times with a simple resection over the past 8 years. The patient was treated with complete resection, electrocauterization, intraoperative application of mitomycin C and cryotherapy. Topical mitomycin C was applied for 1 month. There was no recurrence or complications for 12 months postoperatively. CONCLUSIONS: Complete resection, electrocauterization, intraoperative application of mitomycin C, cryotherapy and topical mitomycin C in patients with recurrent conjunctival papilloma was shown to be an effective treatment without recurrence and complications.

Chest Wall Fibromatosis in the Axilla

No abstract available.

Telomerase Activity in Urethane-Induced Mouse Lung Tumorigenesis

BACKGROUND: Telomerase activity in precancerous conditions of lung adenocarcinomas has not been well studied. This study is designed to investigate the role of telomerase in premalignant lesions of urethane-induced mouse lung adenocarcinoma. METHODS: We harvested A/J mouse lung tissues at 3, 6, 9, 12, 28, 41, and 48 weeks after intraperitoneal urethane treatment, and classified each lesion in terms of histologic findings. We examined telomerase activity using a modified version of the telomeric repeat amplification protocol assay using both gel-based and enzyme linked immunosorbent assay methods. An immunohistochemical analysis of proliferating cell nuclear antigen (PCNA) was performed. RESULTS: In urethane-induced mouse lung tissues, it was sequentially developed from hyperplasia, adenoma, and eventually to adenocarcinoma. Telomerase activity began to show a positive level in tissues with no histologically visible nodule after urethane administration. It revealed a statistically significant increase in hyperplasia compared to the "control" lung tissue (p<0.05), which was proportionally elevated relative to adenoma and adenocarcinoma. There was a direct correlation between telomerase activity and the PCNA labeling index (p<0.05). CONCLUSIONS: The elevation of telomerase activity in normal-appearing lung lesions is thought to be a possible marker of early detection of pulmonary adenocarcinoma.

Removal of Odontogenic Keratinocyst using Versatile Maxillary Window in BCNS

PURPOSE: Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder. It is characterized by complex neoplastic syndrome with multisystemic manifestations, involving six major features. This article presents a rare genetic disorder and usage of the author's methods for odontogenic keratocyst, developed in the maxillary sinus. METHODS: A 67-year-old man was presented with large calcified maxillary mass and multisystemic manifestations and findings that matched with basal cell nevus syndrome. The calcified maxillary mass was removed via the versatile maxillary window and maxillary bone segment was repositioned. RESULTS: Histopathologic findings revealed that maxillary and mandibular lesions were odontogenic keratocysts and the skin lesions were basal cell carcinoma. CONCLUSION: Basal cell nevus syndrome is a rare genetic disease that requires surveillance and care for basal cell carcinoma and multisystemic problems. The author's method was satisfactory for maxillary odontogenic keratocyst in the aspect of the approach and reconstruction.

Clinicopathologic Changes of IgA Nephropathy in Children During Long-term (average 10.8 yrs) Follow-up

PURPOSE: We know little about the natural course of IgA nephropathy (IgAN) in association with histologic changes especially in children. We investigated clinicopathologic features with long-term follow-up biopsy to clarify the outcomes and prognostic indicators for childhood IgAN. METHODS: From our patients' medical records, we retrieved 20 patients with IgAN, to whom renal biopsies had been performed for the initial diagnosis and follow-up to find out any histologic changes. Initial and follow-up biopsies were classified by Haas classification. The changes of these parameters were compared with the evolution of clinical features. RESULTS: Patients were treated with angiotensin-converting enzyme inhibitors in combination with angiotensin receptor blockers (in subclass II or above) and short-term cyclosporine A(in patients showing nephrotic syndrome). Histologic improvement in 7 cases and deterioration in 3 cases were observed. At the time of last biopsy, 10 cases (50%) showed clinical remission and the others showed improved clinical features. These clinical outcomes did not correlate with initial Haas classifications. Hypertension at onset observed in 5 cases (25%) revealed significant correlation with clinical outcome (P=0.01) and last Haas classification (P=0.007). None of the cases showed progression to CRF or ESRD. CONCLUSION: During a mean follow-up of 10.8+/-3.4 years, childhood IgAN showed good clinicopathologic outcome. Hypertension at onset was only a strong predictor of clinicopathologic outcomes, but initial Haas classification cannot predict outcomes in children. Histologic change of IgAN in long term follow-up period cannot be completely predicted by clinical data and vice versa. Therefore, a renal biopsy should be considered as a part of follow-up plan.

A Case of a Duodenal Gastrointestinal Stromal Tumor with a Bleeding Ulcer / 대한소화기내시경학회지

Duodenal gastrointestinal stromal tumors (GISTs) are relatively rare tumors that commonly present with gastrointestinal bleeding. Evaluation with endoscopic ultrasonography (EUS) is a useful technique for the differential diagnosis of GISTs from other submucosal tumors. A 58-year-old man presented with melena and anemia. An esophago-gastroduodenoscopy showed the presence of a submucosal tumor in the second portion of the duodenum with central ulceration and a flat spot. EUS showed the presence of a suspicious malignant GIST that originated from the proper muscle layer, which was successfully resected. We report a case of a duodenal GIST with a bleeding ulcer. Positive reactivity for CD117 and S-100 was demonstrated by immunohistochemical staining.

A Case of Membranoproliferative Glomerulonephritis with False-Positive Reaction for Anti-GBM Antibody / 대한신장학회지

Anti-glomerular basement membrane (Anti-GBM) nephritis is an autoimmune disorder characterized by rapidly progressive crescentic glomerulonephritis (RPGN). The treatment of anti-GBM nephritis with plasmapheresis, steroids and immunosuppressant has improved outcomes. An early diagnosis is essential for the survival of patients and a recovery of renal function. The diagnosis of anti-GBM disease has been traditionally based on the demonstration of linear deposits of immunoglobulins along the glomerular basement membrane by immunofluorescence (IF) microscopy. However, a kidney biopsy cannot always be easily performed in such ill patients. Recent development of specific enzyme immunoassays for anti-GBM antibody in the serum has made possible a provisional diagnosis without a kidney biopsy. A 46-year-old male patient with hypertension and hepatitis B presented with generalized edema and general weakness. Laboratory findings were compatible with acute renal failure and nephrotic syndrome with positive serum anti-GBM antibodies. After plasmapheresis with steroid pulse therapy, renal biopsy was performed and diagnosed as membranoproliferative glomerulonephritis (MPGN) with granular deposit of Ig G and C3. Follow-up antibody titers were negative. This case demonstrates the possibility of false-positive anti-GBM antibody in the serum. Therefore, enzyme immunoassay for anti-GBM antibody should be used only as a screening or follow-up test in patients that have been confirmed positive by IF microscopy.

Early Onset Renal Failure in Congenital Nephrotic Syndrome associated with Congenital Diaphragmatic Hernia by WT1 Gene Mutation

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene andG the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

A Case of IgA Nephropathy with a Membranoproliferative Glomerulonephritis-like Pattern Presenting as Massive Ascites in a Patient with Alcoholic Liver Disease / 대한신장학회지

IgA nephropathy can occur commonly in alcoholic liver cirrhosis and is the most common form of secondary IgA nephropathy. Defective clearance of IgA-containing complexes by liver is thought to contribute to the development of IgA nephropathy in alcoholic cirrhosis. Although IgA deposition is found up to 64% in autopsy cases of alcoholic cirrhosis, most patients have mild clinical symptoms, and nephrotic range proteinuria occurs rarely in these patients. We report a case of IgA nephropathy with a membranoproliferative pattern that is detected with unusual massive ascites in a patient with alcoholic liver disease. A 60-year-old male patient was referred to our hospital for evaluation and management of abdominal distension. Abdominal ultrasonographic findings were compatible with diffuse liver cirrhosis with splenomegaly and large amount of ascites. He had nephrotic range proteinuria, azotemia, hyperlipidemia, and hematuria in dipstick. Renal biopsy performed under the impression of acute nephritis revealed mesangial and endocapillary proliferative glomerulonephritis with double contour of capillary loop. Immunofluorescence findings showed mesangial IgA and C3 deposit, compatible with IgA nephropathy. He was treated with high dose steroid, and steroid was tapered during 2 months. Steroid treatment induced complete remission state, and ascites was resolved.