Atypical Dysphagia in an Anti-Hu Positive Non-Small-Cell Lung Cancer Patient / 대한연하장애학회지

Anti-Hu antibodies usually present in small cell lung cancer are associated with encephalomyelitis, encephalitis, cerebellitis, and neuropathies. However, to our knowledge, non-small-cell lung cancer (NSCLC) related anti-Hu syndrome presenting acute exacerbation of dysphagia has not been reported. Herein, we report a rare case of atypical dysphagia in an anti-Hu positive NSCLC patient resulting in a fatal outcome. A 73-year-old Korean male went through surgical interventions for recurrent tongue cancer and NSCLC. After the surgery, he resumed full oral feeding with a regular diet. However, four weeks later, he developed a sudden difficulty in swallowing. Neck computed tomography angiography, brain magnetic resonance imaging, brain positron emission tomography, computed tomography and electromyography all showed nonspecific findings. A paraneoplastic antibody test was positive for anti-Hu antibodies. His dysphagia worsened and eventually, he expired due to malignant pleural effusion. Our case shows that anti-Hu antibodies can be expressed in NSCLC and when associated with dysphagia, the outcome can be fatal.

CD40 Agonists Alter the Pancreatic Cancer Microenvironment by Shifting the Macrophage Phenotype toward M1 and Suppress Human Pancreatic Cancer in Organotypic Slice Cultures

Background/Aims@#CD40 agonists are thought to generate antitumor effects on pancreatic cancer via macrophages and T cells. We aimed to investigate the role of CD40 agonists in the differentiation of macrophages and treatment of human pancreatic adenocarcinoma. @*Methods@#Immunohistochemistry was performed on paraffin-embedded surgical blocks from patients with pancreatic cancers to evaluate macrophage phenotypes and their relationship with survival. The effects of CD40 agonists on macrophage phenotypes and human pancreatic cancer were evaluated utilizing cell cocultures and organotypic slice cultures. @*Results@#CD163 + (predominant in M2 macrophages) and FOXP3 + (predominant in regulatory T cells) expression levels in the tumors were significantly lower in patients with stage IB pancreatic cancer than in those with stage II or III disease (p=0.002 and p=0.003, respectively). Patients with high CD163 + expression had shorter overall survival than those with low CD163 + expression (p=0.002). In vitro treatment of THP-1 macrophages with a CD40 agonist led to an increase in HLA-DR + (predominant in M1 macrophages) and a decrease in CD163 + expression in THP-1 cells. Cell cocultures showed that CD40 agonists facilitate the suppression of PANC-1 human pancreatic cancer cells by THP-1 macrophages. Organotypic slice cultures showed that CD40 agonists alter the pancreatic cancer microenvironment by shifting the macrophage phenotype toward M1 (increase HLA-DR + and decrease CD163 + expression), decreasing the abundance of regulatory T cells, and increasing tumor cell apoptosis. @*Conclusions@#CD163 is related to advanced human pancreatic cancer stages and shorter overall survival. CD40 agonists alter macrophage phenotype polarization to favor the M1 phenotype and suppress human pancreatic cancer.

Metachronous Development of Peritoneal Carcinomatosis in a Patient with Autoimmune Pancreatitis / 대한소화기학회지

Autoimmune pancreatitis (AIP) is a rare and unique type of chronic pancreatitis. The prognosis of AIP, particularly when associated with pancreatic cancer or a related malignancy, is not known. Only a few cases, where metachronous pancreas-related cancer developed during follow-up, have been reported. Most of these patients either underwent surgery or steroid therapy. This paper reports a case of a 66-year-old woman with untreated type I AIP who developed peritoneal carcinomatosis more than 2 years later. Initially, the patient had a markedly elevated serum IgG4 level and a diffuse, infiltrative mass-like lesion in the pancreatic head, in which the biopsy results were consistent with type I AIP. The patient was not treated with steroids because of a cerebellar infarction. Twenty-eight months after the diagnosis of AIP, peritoneal carcinomatosis developed without noticeable changes in the pancreas from the initial findings.

A Rare Case of Overlap Syndrome with Diffuse Systemic Sclerosis, Rheumatoid Arthritis, and Cutaneous Sarcoidosis

Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we report a rare case of a 59-year-old woman with diffuse systemic sclerosis with lung involvement-rheumatoid arthritis overlap syndrome accompanied by cutaneous sarcoidosis. Although there is no consensus for the optimal treatment of overlap syndrome to date, this case of co-existing rheumatoid arthritis and systemic sclerosis with interstitial lung disease successfully responded to abatacept.

A Ruptured Cystic Artery Pseudoaneurysm with Concurrent Cholecystoduodenal Fistula: A Case Report and Literature Review

Pseudoaneurysms of the cystic artery and cholecystoduodenal fistula formation are rare complications of cholecystitis and either may result from an inflammatory process in the abdomen. A 68-year-old man admitted with acute cholecystitis subsequently developed massive upper gastrointestinal (GI) bleeding. Abdominal computed tomography showed acute calculous cholecystitis and hemobilia secondary to bleeding from the cystic artery. Angiography suggested a ruptured pseudoaneurysm of the cystic artery. Upper GI endoscopy showed a deep active ulcer with an opening that was suspected to be that of a fistula at the duodenal bulb. The patient was managed successfully with multimodality treatment that included embolization followed by elective laparoscopic cholecystectomy. Presently, there is no clear consensus regarding the clinical management of this disease. We have been able to confirm various clinical features, diagnoses, and treatments of this disease through a literature review. A multidisciplinary approach through interagency/interdepartmental collaboration is necessary for better management of this disease.

Continuous Low-Dose Temozolomide Chemotherapy and Microvessel Density in Recurrent Glioblastoma

OBJECTIVE: The purpose of this study was to evaluate the clinical efficacy of continuous low-dose temozolomide (TMZ) chemotherapy for recurrent and TMZ-refractory glioblastoma multiforme (GBM) and to study the relationship between its efficacy and microvessel density within the tumor. METHODS: Thirty patients who had recurrent GBM following Stupp's regimen received TMZ daily at 50 mg/m2/day until tumor progression between 2007 and 2013. The median duration of continuous low-dose TMZ administration was 8 weeks (range, 2-64). RESULTS: The median progression-free survival (PFS) of continuous low-dose TMZ therapy was 2 months (range, 0.5-16). At 6 months, PFS was 20%. The median overall survival (OS) from the start of this therapy to death was 6 months (95% CI : 5.1-6.9). Microvessel density of recurrent tumor tissues obtained by reoperation of 17 patients was 22.7+/-24.1/mm2 (mean+/-standard deviation), and this was lower than that of the initial tumor (61.4+/-32.7/mm2) (p-value=0.001). It suggests that standard TMZ-chemoradiotherapy reduces the microvessel density within GBM and that recurrences develop in tumor cells with low metabolic burden. The efficacy of continuous low-dose TMZ could not be expected in recurrent GBM cells in poor angiogenic environments. CONCLUSION: The efficacy of continuous low-dose TMZ chemotherapy is marginal. This study suggests the need to develop further treatment strategies for recurrent and TMZ-refractory GBM.

Metastatic Brain Neuroendocrine Tumor Originating from the Liver

A 67-year-old male presented with left temporal hemianopsia and left hemiparesis. A contrast-enhanced magnetic resonance image revealed a 4.5x3.5x5.0 cm rim-enhancing mass with central necrosis and associated edema located in the left occipital lobe. Of positron emission tomography and abdominal computed tomography, a 9-cm mass with poor enhancement was found in the right hepatic lobe. Craniotomy and right hemihepatectomy was performed. The resected specimen showed histological features and immunochemical staining consistent with a metastatic neuroendocrine tumor (NET). Four months later, the tumors recurred in the brain, liverand spinal cord. Palliative chemotherapy with etoposide and cisplatin led to complete remission of recurred lesions, but the patient died for pneumonia. This is the first case of a metastatic brain NET originating from the liver. If the metastatic NET of brain is suspicious, investigation for primary lesion should be considered including liver.

HPV-Related Retroperitoneal Squamous Cell Carcinoma of Unknown Primary: A Case Report / Journal of the Korean Cancer Association, 대한암학회지

A 56-year-old female was referred to our hospital due to a mass measuring 5 cm in size in the left pelvic cavity, which was found incidentally during a health examination by ultrasonography. Exploratory laparotomy was performed and the mass was located at the left retroperitoneal parametrium without invasion of the uterus and ovary. The pathology report confirmed squamous cell carcinoma. Even after further studies, we did not find any other primary lesion. Human papillomavirus (HPV) DNA chip test (HPV 9G DNA Membrane Kit, Biometrixtechnology Inc.) showed that the surgical specimen was positive for HPV 18. She received adjuvant chemotherapy and would receive radiation therapy for the possibility of occult gynecologic cancer. Retroperitoneal squamous cell carcinoma of unknown primary is extremely rare and little is known about it. It is reported that HPV may be associated with the disease. Hence, the result of HPV test could have an impact on finding a suspicious primary lesion and treatment modality in this case.

Nodular Fasciitis of the Periorbital Area

Nodular fasciitis is a reactive, non-neoplastic lesion that is most commonly found in the subcutaneous or superficial fascia of the extremities and trunk. Head and neck lesions are relatively uncommon and reports vary from 7% to 15% depending on the authors. Nodular fasciitis grows quickly, and shows a pleomorphic spindle cell pattern with increased mitotic activity. Such factors lead to cases where the lesion is mistaken for a malignancy such as fibrosarcoma and the case may end up with unnecessarily aggressive treatments. The intent of this paper is to report a relatively rare case of nodular fasciitis occurring in the periorbital area and also to highlight the importance of accurate diagnosis and non-aggressive management of this benign lesion.

Recurrence of Multiple Focal Nodular Hyperplasia in a Young Male Patient / 대한소화기학회지

Focal nodular hyperplasia (FNH) is the second most common benign hepatic tumor that is usually found in women. Diagnosis of FNH mainly depends on imaging studies such as color Doppler flow imaging, computed tomography, and magnetic resonance imaging. It is characterized by the presence of stellate central scar and is nowadays incidentally diagnosed with increasing frequency due to advances in radiologic imaging technique. FNH typically presents as a single lesion in 70% of cases and generally does not progress to malignancy or recur after resection. Herein, we report a case of a young male patient with recurrent multiple FNH who underwent surgical resection for presumed hepatic adenoma on computed tomography.

A Case of Pulmonary MALT Lymphoma Arising from Lymphocytic Interstitial Pneumonitis / 결핵및호흡기질환

Pulmonary mucosa-associated lymphoid tissue-derived (MALT) lymphoma is a rare disease. This disorder is considered to be a model of antigen-driven lymphoma, which is driven either by autoantigens or by chronic inflammatory conditions. Low-grade B-cell MALT lymphoma may develop from a nonneoplastic pulmonary lymphoproliferative disorder, such as lymphocytic interstitial pneumonitis (LIP). A recent estimate predicts that less than 5% of LIP patients acquire malignant, low-grade, B-cell lymphoma. In Korea, there has been no previous report of malignant low-grade, B-cell lymphoma, acquired from LIP. Here, we present the case of a patient with LIP that developed into pulmonary MALT lymphoma, six years after diagnosis.

Apocrine Carcinoma of the Axilla with Predominant Signet Ring Cell Features A Case Report

Apocrine carcinoma arising from the apocrine sweat glands is a rare cutaneous malignant tumor which occurs predominantly in the axilla of elderly individuals. The typical histologic features of apocrine carcinoma is within a well developed glandular lumina with abundant eosinophilic cytoplasm and evidence of decapitation secretion. In rare instances, predominant signet ring cell features in apocrine carcinoma has been reported. We experienced a case that occured in the right axilla of a 59-year-old. Histopathologic examination showed a solid tumor that extended from the upper dermis into the subcutis, with a delicate infiltrate of epithelial cells. The cells had granular amphophilic cytoplasm, predominantly showed distinct signet ring cell morphology, and were strongly positive for epithelial mucin. Both lysozyme and gross cystic disease fluid protein-15 were identified in the tumor cells. We diagnosed this to be a case of primary signet ring cell apocrine carcinoma of the axilla after several immunohistochemical and clinical evaluations.

A Case of Hypertrophic Cranial Pachymeningitis Presenting with Scleritis in a Patient with Undifferentiated Connective Tissue Disease

Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been reported to be infrequently associated with systemic autoimmune disorders such as Wegener's granulomatosis, rheumatoid arthritis, sarcoidosis, Behcet's disease, Sjogren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferenciated connective tissue disease (UCTD). HCP was initially suspected on brain magnetic resonance imaging and defined pathologically on meningial biopsy. Immunologic studies showed the presence of anti-RNP antibody. After high dose corticosteroid therapy, the patient's symptoms and radiologic abnormalities of brain were improved. Our case suggested that HCP should be considered in the differential diagnosis of headache in a patient with UCTD presenting with scleritis.

A Case of Pseudomalignant Erosion in an Inflammatory Polyp at the Esophagogastric Junction / 대한소화기내시경학회지

Some cases of polypoid lesions arisen from the gastric mucosa just below a lower esophageal erosion. This lesion can have various sizes and shapes. It is known as a hyperplastic lesion associated with esophagitis or an inflammatory lesion. Sometimes only a polyp is found without an erosion. This polypoid lesion is often called as sentinel polyp and is usually considered to be a benign lesion. Here we report a case of a pseudomalignant erosion in which an inflammatory polyp at the esophagogastric junction was successfully removed by endoscopic submucosal dissection even though the polyp was initially mistaken for a squamous cell carcinoma.

Intratendinous Ganglion in the Extensor Digitorum Communis of the Hand: A Case Report

Ganglions are most common benign lesion of the hand and wrist. Although they usually arise from tendon sheaths, those originating within a tendon substance itself are rare. The etiology, pathogenesis and treatment of the intratendonous ganglion are not completely understood. We report a case of an intratendinous ganglion that developed in the extensor digitorum communis tendon of the hand and caused the extension limitation of the wrist. Treatments consisted of the intratendinous ganglion excision and meticulous tendon repair followed by tenosynovectomy.

Loss of Heterozygosity on Chromosome 15q15 Near Thrombospondin-1 Gene in Breast Carcinomas

BACKGROUND: Chromosome 15q15 near the thrombospondin-1 (THBS-1) gene may be associated with tumor progression and metastasis. To clarify the potential role of the15q15 region in progression of breast carcinoma, we investigated the loss of heterozygosity (LOH) and the microsatellite instability (MSI) status of chromosome 15q15. Methods : LOH and MSI were detected in 84 breast carcinoma specimens using PCR-based microsatellite analysis with three microsatellite markers. METHODS: LOH and MSI were detected in 84 breast carcinoma specimens using PCR-based microsatellite analysis with three microsatellite markers. RESULTS: Of 77 breast carcinomas containing the heterozygous alleles, 25 (32%) showed LOH in at least one microsatellite marker. Partial LOH and total LOH were detected in 14 (18.27%) and 11 (14.3%) cases. The total LOH were inversely correlated with node metastasis. A single LOH at D15S514 was inversely correlated with nuclear grade and a single LOH at the D15S129 allele was associated with increased expression of the THBS-1 gene. MSI-positive breast carcinomas detected in 14 (17%) cases showed no correlation with any clinicopathologic feature. CONCLUSIONS: These results indicate that loss of the chromosome 15q15 region delays the progression of breast carcinoma because the magnitude of LOH is large and involves the THBS-1 gene and additional genetic elements. The genes located on chromosome 15q15 probably play a tissue-type-dependent role in malignant growth of the tumor.

A Case of a Pigmented Neurofibroma Resembling a Giant Congenital Melanocytic Nevus / 대한피부과학회지

In the spectrum of neurofibroma, pigmented neurofibroma is a rare variant observed in patients with or without neurofibromatosis. It is histologically characterized by the presence of scattered melanin-laden pigmented cells within the neurofibromatous lesion. Herein, we describe a case of a 30-year-old man who presented with a large pigmented patch showing focal hypertrichosis on the upper back. The lesion had been present at birth and there was no other cutaneous features to support a diagnosis of neurofibromatosis. Histologically, a proliferation of melanocytes was evident in the epidermis and upper dermis and within underlying neurofibromatous tissue. This abnormality resulted in diagnostic difficulty in the differentiation between pigmented neurofibroma and large congenital melanocytic nevus with complete neurotization because of the clinical and histological similarities. In support of the use of immunohistochemical markers, the diagnosis of pigmented neurofibroma could be made.

A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndrome with Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells / 대한진단검사의학회지

We report a case of IgA kappa light chain deposition disease and combined adult Fanconi syndrome with Auer rod-like intracytoplasmic inclusions in plasma cells and proximal renal tubular cells in a 54-yr-old female. Cytochemical stainings revealed a strong acid phosphatase activity of the inclusions and weak periodic acid-Schiff positivity, whereas the reactions for peroxidase and alpha-naphthyl acetate esterase were negative. An immunostaining verified IgA-kappa inside the plasma cells. Kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity, and Congo red staining was negative. Electron microscopy showed needle-shaped crystals located in tubular epithelial cells.

Borderline Clear Cell Adenofibromatous Tumors of the Ovary: Two Case Reports

Borderline clear cell adenofibromatous tumors are rare with only 26 cases reported in the English literature. Five of these cases exhibited microinvasion and 4 demonstrated intraepithelial carcinoma. We report 2 cases, one typical case and the other with microinvasion. The histological findings revealed widely spaced and focally crowded, variably-sized atypical glands or tubules lined by clear, eosinophilic or hobnail cells set in a dense fibrous stroma. One of the two cases had small solid nests or single cells in the stroma around the proliferative glands less than 1 mm in length that was considered to be a microinvasion.

A Case of Fibroma of Tendon Sheath on the Big Toe / 대한피부과학회지

Fibroma of tendon sheath presents as a small subcutaneous nodule that slowly increases in size, and it is most frequently found on the hands, with the thumb being the most common site of involvement. A 60-year-old woman presented with an asymptomatic, solitary, 1.5 x 2.0 cm-sized, firm, subcutaneous nodule on the left big toe. On magnetic resonance imaging view, the tumor was adherent to tendon sheath of the volar aspect of the left big toe. The skin biopsy specimen showed that the well-circumscribed tumor contained hypocellular, hyalinized, collagenous area and hypercellular densely-arranged collagen area with spindle-shaped fibroblasts and slit-like vascular channels. We herein report a rare case of fibroma of tendon sheath on the left big toe.