Adnexal clear cell carcinoma with comedonecrosis (ACCCC) is a very rare malignancy of the skin with an aggressive clinical course and a predilection for the scalp. This is the first reported case of ACCCC in Korea. A 79-year-old male presented with left abdominal masses that proved to be two subcutaneous nodules. The tumors histologically consisted of epithelial nests that showed a distinctive zonal arrangement. The centrally located clear cell areas with comedonecroses were merged with the peripheral squamoid cells, often exhibiting retraction artifacts and an infiltrating border. Nuclear pleomorphism and frequent mitoses were prominent. The clear cells were immunopositive for carcinoembryonic antigen and epithelial membrane antigen. We report here on a case of ACCCC involving the abdominal skin, and this tumor should be distinguished from the more indolent squamous cell and tricholemmal carcinomas.
Abdomen ; Aged ; Artifacts ; Carcinoembryonic Antigen ; Fluconazole ; Humans ; Korea ; Male ; Mitosis ; Mucin-1 ; Necrosis ; Neoplasms, Adnexal and Skin Appendage ; Scalp ; Skin

Granular cell tumors (GCT) are found in virtually any body site, including the tongue, skin, subcutaneous tissue, breast, rectum and vulva. However, they are rarely seen in the abdominal wall. We report here on a rare case of GCT in the rectus muscle of the abdominal wall. A 44-year-old woman presented with a non-tender, hard mass in the right lower abdominal wall. Upon microscopic examination, the tumor was found to comprise of large polygonal cells with an abundant eosinophilic granular cytoplasm and round to oval nuclei. Upon immunohistochemical staining, the large cells showed S-100 and CD68 positive granular aggregates in the cytoplasm. Many lysosomes of variable size were observed in the cytoplasm.
Abdominal Neoplasms/metabolism/*pathology ; Adult ; Female ; Granular Cell Tumor/metabolism/*pathology ; Humans ; Immunohistochemistry ; Rectus Abdominis/metabolism/*pathology ; S100 Proteins/metabolism

Granular cell tumors (GCT) are found in virtually any body site, including the tongue, skin, subcutaneous tissue, breast, rectum and vulva. However, they are rarely seen in the abdominal wall. We report here on a rare case of GCT in the rectus muscle of the abdominal wall. A 44-year-old woman presented with a non-tender, hard mass in the right lower abdominal wall. Upon microscopic examination, the tumor was found to comprise of large polygonal cells with an abundant eosinophilic granular cytoplasm and round to oval nuclei. Upon immunohistochemical staining, the large cells showed S-100 and CD68 positive granular aggregates in the cytoplasm. Many lysosomes of variable size were observed in the cytoplasm.
Abdominal Neoplasms/metabolism/*pathology ; Adult ; Female ; Granular Cell Tumor/metabolism/*pathology ; Humans ; Immunohistochemistry ; Rectus Abdominis/metabolism/*pathology ; S100 Proteins/metabolism

Juvenile xanthogranuloma (JXG) is a benign and self-limiting non-Langerhans-cell histiocytosis that generally occurs during infancy and childhood. It develops frequently in the head and neck but is very rare in the nasal cavity. To date, only five cases of JXG in the nasal cavity have been reported. Here, we report the second case of JXG in the nasal cavity in Korea. A 19-year-old male patient presented with a protruding 1.1 cm mass in the left nasal vestibule. Histologically, a dense dermal infiltrate of histiocytes with Touton giant cells was observed. Immunohistochemically, the histiocytes tested positive for CD68 and the S-100 protein but negative for CD1a. This shows that a S-100-positive histiocytic lesion dose not exclude a diagnosis of JXG.
Diagnosis ; Giant Cells ; Head ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Korea ; Male ; Nasal Cavity* ; Neck ; S100 Proteins ; Xanthogranuloma, Juvenile* ; Young Adult

Adenomatous polyps of the duodenum is uncommon in general population, but duodenal adenomas are found in the majority of patients with both familial adenomatous polypasis and Gardner's syndrome. These polyps are usually small, multiple and most commonly found in the second portion of the duodenum, and may involve ampulla of Vater. They also have malignant potential so that routine surveillance of the duodenum including ampulla of Vater has been recommended in patients with adenomatous polyposis coli. The adenoma of ampulla of Vater can be found in approximately 50% of patients with familial adenomatous polyposis and usually is asymptomatic, but sometimes, especially villous adenoma, may exhibit clinical problems including partial gastric outlet obstruction, pancreatitis, bleeding, obstructive jaundice, in addition to a high incidence of malignancy. Among the non-malignant complications of the ampullary tumor, there are only a few case reports of acute pancreatitis as the presenting manifestation of an ampullary adenoma or carcinoma in patients with familial adenomatous polyposis in the English literature, but no cases have been reported in Korea. We present here a case of symptomatic adenoma of the ampulla of Vater presenting as acute pancreatitis in a patient with familial adenomatous polyposis.
Adenoma* ; Adenoma, Villous ; Adenomatous Polyposis Coli* ; Adenomatous Polyps ; Ampulla of Vater ; Duodenum ; Gardner Syndrome ; Gastric Outlet Obstruction ; Hemorrhage ; Humans ; Incidence ; Jaundice, Obstructive ; Korea ; Pancreatitis* ; Polyps

We present a case of 58-year-old female with dilated cardiomyopathy(DCMP) in whom we performed left ventricular(LV) remodeling surgery(Batista operation) to reduce the left ventricle diameter and improve left ventricular function. The patient was admitted September 1996 with heart failure NYHA class IV. There was severe orthopnea and peripheral edema. 2-D echocardiography(Echo) showed DCMP with the ejection fraction(EF) 15%, LV end diastolic dimension(LVEDD) 80mm, mitral regurgitation(MR) grade IV, tricuspid regurgitation (TR) grade II. Preoperative cardiac output(CO) was 1.5L/min and cardiac index(CI) was 1.0 L/min/m2. We proceeded with LV remodeling surgery by resection a part of LV lateral wall between both papillary muscle, from the mitral annulus to the LV apex. Size of resected LV wall was 90 x 100 x 15 mm. At the mean time, mitral valve and tricuspid valve were repaired. Postoperative 2-D Echo showed the EF 37%, LVEDD 50 mm, trivial MR, no TR. CO was 3.5L/min and CI was 2.3 L/min/m2. Her fuctional NYHA class was I.
Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Edema ; Female ; Heart Failure ; Heart Ventricles ; Humans ; Middle Aged ; Mitral Valve ; Papillary Muscles ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Ventricular Function, Left ; Ventricular Remodeling*

Bednar tumor was described by Bednar in 1957. The histologic pattern of this tumor shows similar to dermatofibrosarcoma protuberans (DFSP) but melanin pigments are scattered within the tumor, It's rare neoplasm accounting for approximately 1-5% of all case of DFSP. Clinically, this tumor is considered to be intermediated malignancy, because of slow growth and frequent local recurrence and lack of distant metastasis. The majority are located on the trunk and the upper and lower extremities, but extremely rate in the head and neck area. Microscopically, this tumor is characterized by tight storiform spindle cells and long slender cells that admixed with a small population of melanin containing dendritic cells. This dendritic cells are the primary features distinguising this lesion from conventional DFSP. Complete surgical excision and close follow-up case are necessany for this neoplasm because of probable intermediate malignancy. A patient was admitted to Our Department of Oral and Maxillofacial Surgery due to swelling on right parotid area and numbness of the right lower lip on September, 1994, By clinical examinations and C-T finding, we dignosed tentatively as myxoma or pleomorphic adenoma. Surgical excision of this tumor was performed with parotidectomy and mandibular osteotomy under the frozen biopsy. By final microscopic and electromicroscopic examination and immunohistochemical study, this tumor was diagnosed as Bednar tumor. So, we report a case of pigmented DFSP with review of literatures.
Adenoma, Pleomorphic ; Biopsy ; Dendritic Cells ; Dermatofibrosarcoma* ; Follow-Up Studies ; Head ; Humans ; Hypesthesia ; Lip ; Lower Extremity ; Mandibular Osteotomy ; Melanins ; Myxoma ; Neck ; Neoplasm Metastasis ; Recurrence ; Surgery, Oral

Benign laryngeal injuries have been usually differentiated by the clinical finding. Traditionally the pathologic findings between the polyps and nodules by hematoxylin and eosin(H and E) staining looks alike. So we have used immunohistochemical technique to differentiate the laryngeal response to injury. We have studied 25 paraffin embedded tissues(20 cases: vocal polyps, 5 cases: vocal nodules) to compare their distribution of fibronectin and type IV collagen in vocal fold lamina propria by using their monoclonal antibodies. We have chosen fibronectin, a critical structural glycoprotein in the lamina propria, because of its deposition as a result of tissue injury and type IV collagen because of its location only in the basement membrane zone. The results were as follows: 1) In vocal polyps there were sparse fibronectin(less than normal) staining in superficial layer of lamina propria and the type IV collagen staining in basement membrane zone shows thin, normal thickness band form. These suggests that the injury often seems to be confined to the lamina propria only without basement membrane zone disruption. 2) In voal nodules there were intense fibronectin staining(more than normal) in the superficial layer of the lamina propria and the type IV collagen band in basement membrane shows thick and often coupled with disruption injury. The above results represent only two structural proteins, but it may be possible to suggest that the pathogenesis and the development origin of these laryngeal injuries are different. The deposition of fibronectin may explain why some nodules do not respond to voice therapy and suggest the following better treatment.
Antibodies, Monoclonal ; Basement Membrane* ; Collagen Type IV* ; Fibronectins* ; Glycoproteins ; Hematoxylin ; Mucous Membrane* ; Paraffin ; Polyps ; Vocal Cords ; Voice

Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm the diagnosis. We report a typical case of inclusion body myositis with histopathologic and ultrastructural study. The patient was a 31 year old male who presented with progressive weakness of both forearms, hands and lower extremities for 10 years.
Adult ; Case Report ; Human ; Male ; Muscles/pathology ; Myositis, Inclusion Body/*pathology/physiopathology

Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.
Adult ; Carcinoma, Papillary/*pathology/surgery ; Case Report ; Diagnosis, Differential ; Ependymoma/*pathology/surgery ; Fatal Outcome ; Female ; Follow-Up Studies ; Glioma/*pathology ; Human ; Magnetic Resonance Imaging ; Middle Age