1/16. sweat gland proliferations in scleromyxedema.Eccrine sweat duct proliferations may be found in various inflammatory and neoplastic skin lesions. We report a patient with scleromyxedema with extensive proliferations of intradermal sweat ducts. Three-dimensional reconstruction demonstrated extensive coiling and branching of the sweat ducts leading into cystic lacunae. In contrast to the basal cell carcinoma that had grown within the scleromyxedematous skin, the ducts close to the lumen stained positive for carcinoembryonic antigen and could therefore be differentiated from basal cell carcinoma. In micrographically controlled surgery of cutaneous epithelial tumors that are located in chronically inflamed skin, such sweat gland proliferations have to be considered as differential diagnosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/16. Expression of CD34 in sclerotic ("plywood") fibromas.CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor xiiia or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/16. The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, and SS and a brother with systemic lupus erythematosus.We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53-year-old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical sjogren's syndrome (SS). Case 2 was a 48-year-old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical sjogren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical hla antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common hla antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/16. Sclerosing hemangioma with lymph node metastasis.Sclerosing hemangioma (SH) of the lung is an uncommon type of tumor, which is composed of polygonal and cuboidal cells. This disease is generally regarded as benign but extremely rare cases with lymph node metastasis have been reported. We report a case of SH with a metastasis to the regional lymph nodes. A 19-year-old girl presented with a 2-year history of coughing. A chest X-ray and a CT scan indicated a large mass in the lower lobe. As a result, a left lower lobectomy with a dissection of the hilar and interlobar lymph nodes was performed. The tumor was a well-defined huge mass with partial adhesion to the mediastinal and parietal pleura. The dissected hilar, interlobar, and intrapulmonary lymph nodes demonstrated metastasis. Histologically, the primary and metastatic tumor consisted of polygonal and cuboidal cells. Both types of tumor cells were uniformly immunoreactive to the epithelial membrane antigen (EMA) and the thyroid transcription factor-1 (TTF-1). However, the cuboidal cells tested positive for pancytokeratin, whereas the polygonal cells tested consistently negative. Postoperatively, the patient received chemotherapy and no recurrence or metastasis 2 years after surgery was noted. Although a pulmonary SH is considered to be benign, this case highlights the need for the evaluation of lymph node metastasis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/16. Cutaneous sclerosing perineurioma of the digits: an uncommon soft-tissue neoplasm. Report of two cases with immunohistochemical analysis.BACKGROUND: Cutaneous sclerosing perineurioma is a recently characterized, uncommon tumor composed of perineurial cells, which exhibits immunoreactivity for epithelial membrane antigen (EMA). These lesions occur preferentially in children and young adults and usually develop as dermal or subcutaneous nodules in the hands or palms. methods: We report two cases of cutaneous sclerosing perineurioma in young patients without stigmata of neurofibromatosis. Histologically, these lesions were well-circumscribed masses and were characterized by a variable number of epithelioid and spindle cells with wavy nuclei end elongated cytoplasmic processes embedded in a dense collagen stroma. RESULTS: These cells showed focal whorling formation, demonstrated robust immunoreactivity for EMA and CD99, and were uniformly negative for S-100 protein, actin (HHF-35), CD34, cytokeratin AE1-3, and CD57. CONCLUSION: We comment on the differential diagnosis of fibrous cutaneous lesions based on immunohistochemistry.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/16. Sclerodermatomyositis associated with severe arthritis.Overlap syndromes are a spectrum of diseases with clinical features of two or more classic connective tissue diseases. polymyositis or dermatomyositis that is associated with features of systemic sclerosis, also called sclerodermatomyositis, is an uncommon overlap syndrome, with a rare antibody directed against the nucleolar antigen PM-Scl. This disease generally has good prognosis. We report a case of sclerodermatomyositis with an aggressive pulmonary and articular course.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/16. ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin's lymphoma: report of 10 cases.Anaplastic large cell lymphoma (ALCL) and Hodgkin lymphoma (HL) are recognized as biologically distinct entities. However, occasionally, these two entities may share some morphologic features responsible for diagnostic difficulties. In the last 10 years, we have collected 380 cases of ALK-positive ALCL of which 10 cases were originally diagnosed as nodular sclerosis classic HL (NSHL) on conventional histopathological examination. After immunostaining, these cases proved to be ALK-positive ALCL mimicking HL (so-called Hodgkin-like ALCL). These cases account for 2.6% of our cases of ALK-positive ALCL (10 of 380 cases). Median age was 11 years (3-92 years) with a female predominance (male/female ratio, 3:7). Characteristically, these lesions showed thick nodular fibrosing bands highly suggestive of NSHL. Neoplastic cells were scarce in 6 cases, whereas in the 4 remaining cases, sheets of tumor cells were also present. A perivascular and a sinusoidal growth pattern was observed in various degrees in all cases. Few binucleated Reed-Sternberg-like cells were present in every case in a background of small lymphocytes. Inflammatory cells (ie, granulocytes, eosinophils, and histiocytes) were rare. Neoplastic cells were positive for CD30 (10 of 10 cases), ALK protein (10 of 10 cases), epithelial membrane antigen (EMA) (9 of 9 cases), CD43 (6 of 9 cases), and perforin (8 of 8 cases), but negative for CD15 (10 of 10 cases), CD20 (10 of 10 cases), Pax5/BSAP (6 of 6 cases), and EBV (8 of 8 cases). In addition, in 7 cases, neoplastic cells were of T-phenotype, whereas the 3 remaining cases were considered to be of null/undetermined phenotype. Although rare, Hodgkin-like ALCL may mimic NSHL, and it is advisable to include EMA in the first line panel and to ask for ALK staining in EMA-positive, CD15-negative lesions with morphologic features suggestive of NSHL.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/16. Cutaneous sclerosing perineurioma of the digit.An 11-year-old Japanese girl noticed a small nodule, with mild tenderness, on the right index finger 5 years before visiting our outpatient clinic. She had no familial history of neurofibromatosis or past history of traumatic injury at the site of the tumor. physical examination revealed a slightly elevated, subcutaneous, nodular tumor in the volar aspect between the proximal and distal interphalangeal joints of the digit (Fig. 1A). By magnetic resonance imaging examination, the tumor showed low density on both T1- and T2-weighted images, and was located just adjacent to the tendon with no invasive signs. The tumor was extirpated; at operation, it was well circumscribed and mobile without adhesion to adjacent tendon or nerve, and was easily removed. Grossly, the tumor was a well-circumscribed, firm nodule (10 mm x 8 mm x 5 mm in size) (Fig. 1B). The cut surface was whitish, homogeneous, and solid without cystic lesions. Histologically, it was an unencapsulated, paucicellular dense, fibrous nodule with a concentric circular arrangement of collagen bundles (Fig. 2A). Amongst the fibrous bundles, a small number of ovoid/epithelioid or plump spindle cells were arranged in a corded, trabecular, or whorled (onion bulb-like) pattern (Fig. 2B); a storiform pattern was not noted. These cells were relatively uniform and had a somewhat elongated, slightly hyperchromatic nucleus with fine granular chromatin. Neither nuclear pleomorphism nor multinucleated cells were evident, and necrosis and mitotic figures were not observed. periodic acid-Schiff (PAS) stain after diastase digestion highlighted the corded or whorled pattern of the tumor cells by encasing them. For immunohistochemical examination, formalin-fixed, paraffin-embedded serial tissue sections were stained by a labeled streptavidin-biotin method. The tumor cells were positive for vimentin and epithelial membrane antigen (EMA) (Fig. 3A), and negative for pan-cytokeratin, carcinoembryonic antigen (CEA), CD34, alpha-smooth muscle actin, desmin, and CD68. Type IV collagen and laminin (Fig. 3B) were detected along the cords or whorls of the tumor cells, similar to the staining pattern of the diastase-PAS reaction. schwann cells and axonal components, immunoreactive for S100 protein and neurofilament, respectively, were focally detected just adjacent to the cords or whorls, although the tumor cells per se did not express these proteins. Consequently, the tumor was found to be perineurial in origin and was diagnosed as cutaneous sclerosing perineurioma.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/16. Osteomyelosclerosis with granulocytic sarcoma of chest wall. Morphological, ultrastructural, immunologic, and cytogenetic study.A case of granulocytic sarcoma presenting as a soft-tissue tumor in the chest wall in a patient with osteomyelosclerosis is reported. The tumor mass was detected by a computed tomographic scan during an investigation of the cause of chest pain in a 58-year-old man. biopsy of the mass showed findings compatible with either a large-cell lymphoma or a granulocytic sarcoma. The latter was confirmed by naphthol-ASD-chloracetate esterase stain and electron microscopic examination. Immunologic study of the tumor mass showed expressions of membrane/cytoplasmic CD 13 and CD 15 antigens. In addition, the tumor cells coexpressed CD 19, although all other T- and B-cell-associated antigens were absent. Cytogenetic study showed translocation t(1;7)(q11;q11) with a net deletion of the entire long arm of chromosome 7 and duplication of the long arm of chromosome 1. Peripheral blood examination showed typical leukoerythroblastosis with teardrop poikilocytosis, large hypogranular platelets, and 0.11 myeloblasts. A bilateral iliac bone marrow biopsy at this time showed osteomyelosclerosis. The patient was treated with hydroxyurea followed by local irradiation, resulting in marked reduction in the size of the tumor and in the pain. He was asymptomatic without any progression in hematologic parameters 10 months after the initial diagnosis.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/16. A case of sclerosing lymphangitis of the lip.A 62-year-old woman noted a cord-like swelling on the inner surface of her upper lip. A cross-section of the lesion showed a radial-shaped lumen surrounded by a thickened fibrous wall. Immunohistochemical staining with the anti-factor viii-related antigen (FVIII-RAg) antibody was negative on the luminal surface and swollen wall, although the vasa vasorum in the swollen wall were positive. The lesion was thus considered to be of lymphatic origin; that is, it derived from a lymphatic collecting vessel. For the present case, the term 'lymphangiopathia obliterans' is considered appropriate. This is the second report of such a lesion appearing on the lip in the literature.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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