An IgG4-Related Inflammatory Pseudotumor of the Greater Omentum / 대한내과학회지

IgG4-related sclerosing disease is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tissue infiltration by IgG4-positive plasma cells. Although this disease is a novel entity, it is not rare and is present in a proportion of patients with a wide variety of diseases, including autoimmune pancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, and inflammatory pseudotumor. Despite the effectiveness of steroid therapy, IgG4-related sclerosing disease is often misdiagnosed as a malignant tumor. Here, we report an inflammatory pseudotumor of the great omentum that was misdiagnosed as a malignant tumor and subject to surgical resection.

A Case of Sarcomatoid Carcinoma Arising from Mucinous Cystadenocarcinoma of Appendix

Sarcomatoid carcinoma or carcinosarcoma is a very rare biphasic tumor characterized by a combination of malignant epithelial and mesenchymal cells. The pathogenesis of sarcomatoid carcinoma is not fully elucidated and the guideline of treatment has not been established yet. Although the upper aerodigestive tract, lung and female urogenital system are known to be the most frequently affected, this tumor also can occur in various sites, including the digestive tract. Since sarcomatoid carcinoma in colon was firstly reported in 1986, 24 cases have been reported to date. We report a rare case with sarcomatoid carcinoma of appendix. Interesting histologic feature of our case was the presence of mucinous cystadenocarcinoma with morphological "transition" between carcinomatous and sarcomatous tissue. To our knowledge, this is the first case of sarcomatoid carcinoma arising from mucinous cystadenocarcinoma of the appendix.

A Case of Endoscopic Treatment for Gastrocolocutaneous Fistula as a Complication of Percutaneous Endoscopic Gastrostomy

As a rare complication of percutaneous endoscopic gastroscopy (PEG), a gastrocolocutaneous fistula may occur after PEG placement. This paper reports an interesting case which PEG tube unintentionally penetrated transverse colon during PEG. A 72-year-old female patient who suffered from medullary infarction underwent PEG procedure for enteral nutrition, and fecal materials were observed 6 days after the procedure. Transverse colon located in antero-superior site of stomach was observed through abdominal computed tomography, and also the wrong inserted tube was found through gastroscopy and colonoscopy. Endoscopic treatment for the fistula was performed by the use of hemo-clip and detachable snare, closure of the fistula was finally confirmed 6 days after the endoscopic procedure. Therefore, the gastrocolocutaneous fistula should be considered as one of the complications of PEG when fecal material is observed through PEG tube in a few days after PEG procedure and endoscopic treatment can be feasible in this case.

A Case of Henoch-Schonlein Purpura Associated with Diffuse Large B Cell Lymphoma / 대한혈액학회지

A 69-year-old female was referred to our institution due to abdominal pain and palpable purpura on both buttocks and legs. A skin biopsy of her purpura revealed granulocyte infiltration and leucocytoclasia around the arterioles and venuoles at the dermis, as well as an elevated serum immunoglobulin A level, hematuria and proteinuria. Therefore she was diagnosed with Henoch-Schonlein purpura. She had been diagnosed with diffuse large B cell lymphoma after a biopsy of her left inguinal lymph node 12 years ago and received 6 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, which was followed by a complete remission. Abdominal and chest CT revealed multiple lymph node enlargement and bowel wall thickening at the ileocecal area, and lesions were observed in a whole body PET CT scan. Recurrence of the diffuse large B cell lymphoma was confirmed by a biopsy of the ileocecal area via colonoscopy. The purpura was improved after oral prednisolone therapy and etoposide, oxaliplatin and ifosfamide salvage combination chemotherapy was used to treat the lymphoma.

A Case of Elastofibroma

PURPOSE: Elastofibroma is a rare benign tumor that is characterized histologically by the presence of abnormal elastic fibers within a stroma of fibroadipose tissue. Usually it is slow-growing, solid, ill-defined mass occurring chiefly in elderly woman and arising from the soft tissue around the inferior angle of the scapula. METHODS: We experienced a representative case of elastofibroma. A 73-year-old women complained of a soft-tissue mass, which measured 6x6cm, at the inferior angle of the left scapula, which had been found incidentally 4 months before. When the arm was elevated, the mass projected out beneath the scapula. The entire mass was resected with a tumor-free margin. RESULTS: The resected tumor appeared to be nonencapsulated, fibrous and white mass, and it contained yellow fatty streaks. We confirmed that the histopathologic diagnosis of this tumor was elastofibroma. Follow- up examination revealed no evidence of local recurrence for 10 months. CONCLUSION: This entity is rarely reported in Korea. However, more patients could be diagnosed if physicians pay more careful attention to clinical and radiological features of elastofibroma. Moreover, recognizing the benign nature of this lesion is important to avoid an unnecessary operation.

Three Cases of Malignant Pleural Mesothelioma Misdiagnosed as Tuberculous Pleurisy / 결핵및호흡기질환

Malignant pleural mesothelioma(MPM) is an uncommon neoplasm which is originated from pleural mesothelial cells. The majority of MPM is associated with prior asbestos exposure. Patients often present with chest pain and dyspnea due to pleural effusion, which might be diagnosed with tuberculous pleurisy especially in Korea. MPM is well known for its poor prognosis with a median survival time of less than 12 months after diagnosis and no established standard treatment modality. We report 3 cases of MPM confirmed by video-assisted thoracoscopic biopsy first misdiagnosed as tuberculous pleurisy.

A Large Malignant Peripheral Nerve Sheath Tumor in the Neurofibromatosis Patient: A Case Report

PURPOSE: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. METHODS: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. RESULTS: There was no evidence of recurrence of tumor for 19 months of follow-up examination. CONCLUSION: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.

Experience of Single Stage Treatment of Caniosynostosis, Hypertelorism, Exophthalmos Patient

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

Case Study of Secondary Operation in Blowout Fracture for Correction of Implant Displacement

The operative treatment of orbital blowout fracture involves restoration of intra-orbital soft tissue and bony structural integrity. In extensive blowout fracture cases, inadequate dissection inappropriate implant insertion, postoperative edema and subsequent increase of intraorbital pressure may sometimes lead to displace the implant. This case study was performed on 6 patients who underwent secondary blowout fracture operation. After primary blowout fracture reduction operation, we found severely displaced implant in the postoperative computed tomogram study. The most prevalent sites were the posterior portion of the inferior and medial wall. We performed further dissection of the fracture sites and inserted more larger sized implant than before. Then we fixated the implant to prevent postoperative displacement. Among these patients, 3 patients who operated more than 3 months after the first operation, complained residual diplopia and eyeball movement disorder. These clinical cases have troublesome and unique features, so we report our experiences with review of the literatures.

The Prognostic Role of B-type Natriuretic Peptide in Acute Pulmonary Thromboembolism / 결핵및호흡기질환

BACKGROUND: Vital stability and right side heart failure are major prognostic factors of acute pulmonary thromboembolism. While it is important to recognize right side heart failure, it is often difficult in real practice. Recently, several studies have described early diagnostic tools for detecting right side heart failure including echocardiography and biochemical markers. This study, we evaluated the prognostic role of the B-type natriuretic peptide (BNP) in an acute pulmonary thromboembolism. METHODS: Thirty-four patients with a diagnosis of acute pulmonary thromboembolism were enrolled in the study. The BNP levels were measured and echocardiography was performed at the Emergency Department. Data on the prognostic factors including ventilatory support, vital stability, pulmonary artery pressure, degree of tricuspid valve regurgitation, complications and death was collected from the patients' medical records. The patients with an acute pulmonary thromboembolism were divided into two groups based on the vital stability and the BNP level and the cutoff values and prognostic factors of the two groups were compared. RESULTS: The predictors of the vital stability that influence the prognosis of patients with acute pulmonary thromboembolism were the BNP level, ventilatory support and death. The plasma BNP levels showed a strong correlation with the vital stability, ventilatory support, thrombolytic therapy and death. When the BNP cutoff level was set to 377.5 pg/dl in a ROC curve, the sensitivity and the specificity for differentiating between the groups with stable or unstable vital signs was 100% and 90%, respectively. CONCLUSION: This study indicates that a measurement of the plasma BNP levels may be a useful prognostic marker in patients with an acute pulmonary thrombo-embolism.

Rib/costal Cartilage Combination Graft in Rhinoplasty

Augmenatation rhinoplasty may be effectively accomplished with alloplastic materials. However, certain circumstances mandate the use of autologous grafts(e.g., dorsal augmentation that exceeds 8 mm and patient intolerance of alloplastic implants). Rib or costal cartilage graft have gained wide-spread use in rhinoplasty. Rib cartilages are pliable and soft but they easily undergo warpage, whereas solid bone graft frequently lead to an unnatural hardness of the nasal tip and dorsal resorption. The rib/costal cartilage combination graft(=autogenous chondro-osseous rib grafting) are excellent alternatives. In this study 12 patients received 14 rib grafts harvested in the 7th or 8th rib. Materials were used as cantilevered dorsal onlay grafts, columellar struts, and tip grafts. During 8 months of follow up periods, most patients have firm and stabilized nasal dorsum with soft and mobilized tip without prominent complications such as infection, protrusion, mobilization, etc. In conclusion, this autogenous chondro-osseous rib grafting technique can be an effective and safe method in the primary or secondary rhinoplasty.