Management of hepatic adenoma in glycogen storage disease Ia.
Explore the source record for details and available documents.
Adenoma↗
Biomedical subjects
G M Groisman
Publications and source records attributed to G M Groisman.
Early prenatal diagnosis of Larsen's syndrome by transvaginal sonography.
Explore the source record for details and available documents.
Abnormalities, Multiple↗
Anal malignant melanoma and soft-tissue malignant fibrous histiocytoma in neurofibromatosis type 1.
A 48-year-old man with the nonfamilial form of neurofibromatosis type 1 (NF-1) developed a malignant fibrous histiocytoma in the deep soft tissue of the calf. Following excision and local radiotherapy, the tumor did not recur. At age 59, due to rectal bleeding, a polypoid malignant melanoma of the anal canal was detected and resected. One year later a local recurrence was removed, a pelvic mass was seen on computed tomography, and the patient died. The malignant melanoma was amelanotic and negative for HMB-45 (a melanoma-specific antibody), and the recurrent tumor showed areas of desmoplasia and prominent perineural and intraneural spread. The latter properties underscore the similarities of this melanoma to malignant peripheral nerve sheath tumor. In addition to neurofibroma and malignant schwannoma, NF-1 is associated with other neuroectodermal tumors, including brain tumors and pheochromocytoma. However, it is still controversial whether cutaneous malignant melanoma is more prevalent in NF-1 patients. Anorectal melanoma and malignant fibrous histiocytoma are exceedingly rare in NF-1. Second primary cancers are probably more frequent in NF-1 patients compared to the general population and may be related to alterations in the NF-1 gene.
Anus Neoplasms↗
Microsatellite instability in ulcerative colitis-associated colorectal dysplasias and cancers.
Microsatellites are short nucleotide repeat sequences present throughout the human genome. Alterations of microsatellites, comprising extra or missing copies of these sequences, have been termed microsatellite instability. This abnormality occurs in sporadic and hereditary adenocarcinomas of the proximal colon, as well as in many other tumor types. We determined whether microsatellite instability occurred in ulcerative colitis-associated cancers or precancerous dysplasias. Sixty-three patients were evaluated, consisting of 188 samples of genomic DNA (63 normal controls, 68 cancers, 52 dysplasias, and 5 adjacent tissues) at loci D2S119, D2S123, D2S147, D10S197, and D11S904. Multiplex polymerase chain reaction was performed using one radiolabeled nucleotide, and the products were electrophoresed on denaturing polyacrylamide gels. Seventeen of the 63 patients (27%) possessed lesions showing instability at 1 or more loci. Fourteen of 68 tumor samples (21%) and ten of 52 dysplasias (19%) displayed instability. There was no tendency for a greater number of loci to manifest instability in more advanced lesions. Neither anatomic location nor loss of heterozygosity at the p53 locus were associated with microsatellite instability by 2-way table analysis. These data support a role for defective DNA repair in the generation of a subset of both early and advanced ulcerative colitis-associated colorectal neoplastic lesions.
Colitis, Ulcerative↗
Langerhans cell histiocytosis of the stomach. A cause of granulomatous gastritis and gastric polyposis.
Langerhans cell histiocytosis (LCH) of the alimentary tract is a rare condition, and LCH of the stomach especially so. We report a histologically and endoscopically unusual case of gastric LCH, the diagnosis of which was based on gastric mucosal biopsy specimens from an adolescent with multi-organ LCH and striking gastric polyposis. Microscopically, the gastric mucosa was expanded by discrete granulomatous microaggregates of Langerhans cells, resulting in a close resemblance to other, more common nonnecrotizing granulomatous gastritides. The diagnosis of LCH was confirmed immunohistochemically and ultrastructurally. To our knowledge, this is the first report of gastric LCH with a granulomatous histologic pattern, and the third to cause diffuse gastric polyposis. Langerhans cell histiocytosis should be included in the histologic differential diagnosis of granulomatous gastritis, as well as in the endoscopic differential diagnosis of gastric polyposis.
Adolescent↗
Inflammatory pseudotumor of the maxillary sinus in a 15-year-old boy.
Inflammatory pseudotumor is a benign neoplasm composed of fibroblasts, histiocytes, and inflammatory cells. Its occurrence in the paranasal sinuses is rare. We report a case of a 15-year-old boy with a maxillary sinus inflammatory pseudotumor that simulated an aggressive neoplasm on CT scan.
Adolescent↗
Ulcerative appendicitis in universal and nonuniversal ulcerative colitis.
Ulcerative appendicitis (UA), the appendiceal counterpart of ulcerative colitis (UC), is frequently present in colectomy specimens from patients with universal UC and is regarded as part of the continuous inflammatory process that is a hallmark of this disease. It has been reported, although not universally accepted, that UA may also occur in UC that spares the proximal colon, constituting a skip lesion. Colectomies, which included appendices, from 160 consecutive adult and pediatric patients with UC were reviewed histologically and separated into two groups, universal and nonuniversal UC, on the basis of a stringent histopathological definition of normal mucosa. Forty-five cases with obliterated appendiceal lumens were excluded. UA was present in 82 of the 94 cases of universal UC (87%), including 75 of the 83 adult cases (90%) and 7 of the 11 pediatric cases (64%). UA was present in 18 of the 21 cases of nonuniversal UC (86%), all of which were adults, representing skip lesions. UA was found in 12 of the 14 cases in which colitis commenced distal to the hepatic flexure, including 2 of the 3 cases of left-sided colitis and 2 of the 2 cases of proctosigmoiditis. Among patients whose clinical indication for colectomy was the presence of dysplasia or carcinoma and who had patent appendices, UA was present in 6 of the 9 cases with universal UC (68%) and in each of the 3 cases with nonuniversal UC (100%). We conclude that in colectomy specimens discontinuous appendiceal involvement in nonuniversal UC is as prevalent as continuous involvement in universal UC.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Resolution of protein-losing hypertrophic lymphocytic gastritis with therapeutic eradication of Helicobacter pylori.
Lymphocytic gastritis (LG) is a recently described histological entity characterized by increased lymphocytes in the superficial gastric epithelium and foveolae. It includes a subgroup of patients with giant gastric folds and, often, a protein-losing state, a condition termed hypertrophic lymphocytic gastritis (HLG). Despite close endoscopic and clinical similarities to classical Menetrier's disease, the histopathological features of these two diseases are sufficiently distinct that they are regarded as separate entities. The etiology and pathogenesis of HLG are unknown, and the possible etiological role of Helicobacter pylori in particular is controversial. For this reason we report the case of a 48-yr-old female with HLG, hypoproteinemia, and H. pylori infection whose disease resolved clinically, endoscopically, and pathologically with therapeutic eradication of the H. pylori. H. pylori infection may be a treatable cause of at least some cases of HLG and should therefore be carefully sought in any patient with this condition.
Amoxicillin↗
Thymoma with foci of medullary differentiation in an 11-year-old-boy.
We report a benign encapsulated thymoma in an 11-year-old boy. Histologically, it showed foci of medullary differentiation mimicking the normal corticomedullary thymic architecture. We emphasize the rarity of thymomas in children and the possibility of mistaking this uncommon pattern of thymoma for other mediastinal tumors.
Child↗
Osseous metaplasia in benign colorectal polyps.
We report two unique cases of metaplastic ossification occurring within a tubulovillous adenoma and a juvenile polyp. In both lesions, well-formed bony spicules were present that were adjacent to living epithelial cells. The metaplastic bone revealed vimentin positivity and cytokeratin negativity. The pathogenesis of osseous metaplasia in colorectal tumors remains unclear, but the process seems to have no clinical significance.
Adenoma, Villous↗
The value of polyclonal carcinoembryonic antigen immunostaining in the diagnosis of microvillous inclusion disease.
Microvillous inclusion disease is a specific disorder recognized as a cause of intractable diarrhea of infancy. We studied three cases by light microscopy, electron microscopy, and immunostaining for polyclonal carcinoembryonic antigen (CEA). Histologically, all cases had villous atrophy and abnormal accumulation of periodic acid-Schiff-positive material in surface enterocytes. Ultrastructurally, poorly developed brush-border and intracytoplasmic inclusions lined by intact microvilli were present in surface enterocytes. Crypt cells showed well-preserved surface microvilli. Carcinoembryonic antigen immunostaining showed prominent intracytoplasmic reactivity in surface enterocytes and linear brush-border reactivity in crypt cells. Normal and diseased small bowel biopsy specimens used as controls revealed linear brush-border reactivity without intracytoplasmic staining. Intracytoplasmic positivity for carcinoembryonic antigen in microvillous inclusion disease is explained by its presence in the glycocalyx within the microvillous inclusions. The demonstration of a distinct staining pattern for polyclonal carcinoembryonic antigen in routinely processed small bowel biopsy specimens provides a new useful criterion that complements other established techniques for accurate diagnosis of microvillous inclusion disease.
Antibodies↗
Choledochal cyst. Preoperative sonographic and scintigraphic assessment.
Explore the source record for details and available documents.
Child↗
Multicystic mesothelioma with endometriosis.
A multicystic mesothelioma of the omentum in a 36 year old woman consisted of a multicystic mass with foci of typical endometriosis and 'necrotic pseudoxanthomatous nodules'. The presence of endometriosis within multicystic mesothelioma has never been reported. Our findings support the hypothesis that endometriosis plays a rôle in the pathogenesis of multicystic mesothelioma and that this is a reactive rather than a neoplastic lesion.
Adult↗
Juvenile granulosa cell tumor of the testis: a comparative immunohistochemical study with normal infantile gonads.
This study concerns the nature of two different cell populations in a juvenile granulosa cell tumor (GCT) of the infantile testis. Immunohistochemical features of the tumor were compared with those of normal infantile testes (six cases) and ovaries (six cases). The testicular neoplasm showed follicles, cysts and solid nodules composed of an internal layer of polyhedral cells that expressed cytokeratin and vimentin. Most of the follicles and nodules were surrounded by an external layer of spindle cells that reacted to muscle-specific actin, vimentin, and focally to desmin. A neoplastic rather than reactive origin of the spindle cell population is favored by their concentric arrangement in a peritubular-like or theca-like fashion and by their immunohistochemical correlation with normal peritubular-myoid and theca external cells. Sertoli and granulosa cells of normal infantile gonads were positive for cytokeratin and vimentin; peritubular myoid and theca externa cells expressed muscle-specific actin, vimentin, and focally desmin. The occurrence of two well-differentiated components in the tumor favors its origin from the primitive specialized gonadal stromal cell that during neoplastic transformation develops bidirectional differentiation toward epithelial-like and smooth muscle-like lineages. The possibility that this tumor is composed of immature Sertoli and peritubular myoid cells is discussed.
Cytoskeletal Proteins↗