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[Acute appendicitis caused by pregnancy-associated ectopic decidua. Case report and discussion of pathogenesis].

The occurrence of pregnancy-associated ectopic decidua is a well-documented phenomenon. It has been observed most often in the ovaries, uterus, and cervix. An extragenital localization is less frequent and usually an asymptomatic, incidental finding. We report on a 32-year-old woman in her third trimester of pregnancy who developed acute appendicitis caused by ectopic decidua. This case illustrates a rare differential diagnosis of acute appendicitis and discusses the pathogenesis of pregnancy-associated ectopic decidua in such cases.

Acute Disease↗

Ectopic recurrences of craniopharyngioma.

BACKGROUND: Ectopic recurrences of craniopharyngioma after total surgical removal are a rare event. Two possible mechanisms of seeding have been proposed: the dissemination of tumoral cells during the surgical procedure would account for tumor recurrence along the surgical path, whereas the migration of tumoral cells through the subarachnoid or Virchow-Robin spaces would explain ectopic recurrences far from the surgical bed or within the brain parenchyma. CASE REPORT: We report a unique case of metastatic craniopharyngioma recurring in a child after different intervals in which both the mechanisms could have taken place. The first ectopic seeding arose along the operative track of the primary surgical procedure in the frontal region while the second involved the deep cerebral nuclei and the brain stem. OUTCOME: The first recurrence was successfully operated upon. The following recurrences were not operated upon but remained stable for the last 4 years, thus showing dissociation between the capacity of seeding and invasivity.

Brain Neoplasms↗

Retroperitoneal ectopic neural mass: "abdominal brain"--presentation of two cases and proposal of classification of paraneuraxial neural ectopia.

An encapsulated mass of brain tissue was found in the retroperitoneum of a fetus of gestational week 15 and a boy of age 3 years. The masses possessed fibrous tissue that bound them to the spine and intraspinal connective tissue, respectively, but there was no evidence of direct continuity of the ectopic brain tissue with the normal central nervous system. There was no dysraphism. In our fetal case, possible Foix-Alajouanine anomaly was additionally found. The ectopic neural tissue in the retroperitoneal region may be termed "abdominal brain." In the literature, an identical state has been described in the head (paracranial region) but there are no other records of the paraspinal region. Despite the different locations of the masses (head/paracranial or retroperitoneum/paraspinal), these ectopic brain masses should belong to the same disorder spectrum of the paraneuraxial neural ectopia, a new concept.

Cell Differentiation↗

Characterization of nuclear compartments identified by ectopic markers in mammalian cells with distinctly different karyotype.

The functional organization of chromatin in cell nuclei is a fundamental question in modern cell biology. Individual chromosomes occupy distinct chromosome territories in interphase nuclei. Nuclear bodies localize outside the territories and colocalize with ectopically expressed proteins in a nuclear subcompartment, the interchromosomal domain compartment. In order to investigate the structure of this compartment in mammalian cells with distinctly different karyotypes, we analyzed human HeLa cells (3n+ = 71 chromosomes) and cells of two closely related muntjac species, the Chinese muntjac (2n = 46 chromosomes) and the Indian muntjac (2n = 6/7 chromosomes). The distribution of ectopically expressed intermediate filament proteins (vimentin and cytokeratins) engineered to contain a nuclear localization sequence (NLS) and a nuclear particle forming protein (murine Mx1) fused to a yellow fluorescent protein (YFP) was compared. The proteins were predominantly localized in regions with poor DAPI staining independent of the cells' karyotype. In contrast to NLS-vimentin, the NLS-modified cytokeratins were also found close to the nuclear periphery. In Indian muntjac cells, NLS-vimentin colocalized with Mx1-YFP as well as the NLS-cytokeratins. Since the distribution of the ectopically expressed protein markers is similar in cells with distinctly different chromosome numbers, the property of the delineated, limited compartment might indeed depend on chromatin organization.

Animals↗

Alteration of floral organ identity in rice through ectopic expression of OsMADS16.

We used a transgenic approach and yeast two-hybrid experiments to study the role of the rice ( Oryza sativa L.) B-function MADS-box gene, OsMADS16. Transgenic rice plants were generated that ectopically expressed OsMADS16 under the control of the maize ( Zea mays L.) ubiquitin1 promoter. Microscopic observations revealed that the innermost-whorl carpels had been replaced by stamen-like organs, which resembled the flowers of the previously described Arabidopsis thaliana (L.) Heynh. mutation superman as well as those ectopically expressing the AP3 gene. These results indicate that expression of OsMADS16 in the innermost whorl induces stamen development. Occasionally, carpels had completely disappeared. In addition, ectopic expression of OsMADS16 enhanced expression of OsMADS4, another B-function gene, causing superman phenotypes. In the yeast two-hybrid system, OsMADS16 did not form a homodimer but, rather, the protein interacted with OsMADS4. OsMADS16 also interacted with OsMADS6 and OSMADS8, both of which are homologous to SEPALLATA proteins required for the proper function of class-B and class-C genes in Arabidopsis. Based on the gene expression pattern and our yeast two-hybrid data, we discuss a quartet model of MADS-domain protein interactions in the lodicule and stamen whorls of rice florets.

Base Sequence↗

Expression of rolB in apomictic Hieracium piloselloides Vill. causes ectopic meristems in planta and changes in ovule formation, where apomixis initiates at higher frequency.

The effects of the Agrobacterium rhizogenes rolB oncogene on apomixis were examined in the facultative apomictic plant Hieracium piloselloides because the oncogene has been shown to alter plant growth, morphogenesis and cellular sensitivity to auxin. Introduction of rolB under the control of either its own promoter or the CaMV35S promoter induced ectopic meristem formation from the inflorescence, confirming in planta a meristem-inducing role for this oncogene previously observed only in tissue culture. These ectopic meristems formed vegetative rosettes and floral plant organs. Upon immersion in water these meristems generated roots, suggesting that meristem commitment towards the generation of a specific organ type is a separate and later event that is dependent upon the developmental context. Ovule identity and form was altered in ectopically induced florets in plants expressing the CaMV35S::rolB construct. In contrast to the ovules of untransformed apomictic plants, the sexual process ceased earlier, prior to meiosis, yet surprisingly, apomixis initiated from a greater number of cells, and embryos and endosperm continued to develop in the structurally altered ovules. The alternative possibilities that the effects on reproduction might result from rolB influencing cellular response to auxin, or from alterations in cell signaling caused by changes in ovule morphology that are induced because of the expression of the oncogene are discussed.

Asteraceae↗

Hepatocellular carcinoma arising from ectopic liver tissue in the pancreas.

Liver tissue ectopia is a well-documented phenomenon. Rarely, hepatocellular carcinoma arises from the ectopic liver tissue. In this paper, we report a case of a primary, well-differentiated hepatocellular carcinoma arising from ectopic liver tissue in the pancreas. The patient is a 58-year-old Hispanic man with no history of underlying liver diseases or chronic pancreatic diseases. Patient presented with a several days history of abdominal pain with radiation to his right upper quadrant. Imaging study revealed a 3.7 x 3.3-cm mass in the distal pancreas. No other lesions were identified. Preoperative fine needle aspiration revealed blood and atypical hepatocytes. The patient underwent distal pancreatectomy and splenectomy for suspected neuroendocrine tumor. Gross examination revealed a well-circumscribed 3.3-cm, beige-tan, pseudolobulated tumor with focal areas of hyperpigmentation. A microscopic examination revealed hepatoid cells arranged in a trabecular pattern with focal bile pigment. Immunohistochemistry study showed that the tumor cells were reactive with hepatocyte antigen (Hep par 1), alpha-1 antitrypsin, but negative for synaptophysin and chromogranin. Immunostain for polyclonal carcinoembryonic antigen showed a typical bile canalicular pattern. These results support that this tumor in the pancreas is hepatocellular carcinoma, most likely arising from ectopic liver tissue within the pancreas.

Carcinoma, Hepatocellular↗

Down-regulation of a gastric transcription factor, Sox2, and ectopic expression of intestinal homeobox genes, Cdx1 and Cdx2: inverse correlation during progression from gastric/intestinal-mixed to complete intestinal metaplasia.

PURPOSE: The molecular mechanisms underlying the development of intestinal metaplasia (IM) of the human stomach have yet to be clarified in detail. Besides ectopic expression of intestinal transcription factors, Cdx1 and Cdx2, little information is available regarding other regulatory factors. Hence, we here analyzed Sox2, a human homolog of a chicken gastric transcription factor, with reference to our new classification for gastric/intestinal (GI)-mixed type IM. METHODS: Twenty specimens of surgically resected antral mucosa were subjected to a gland isolation technique. Isolated glands were classified into gastric (G), GI-mixed, and solely intestinal (I) types according to Alcian blue and paradoxical concanavalin A staining and were quantified for mRNA levels of gastrointestinal markers. RESULTS: MUC5AC and MUC6 transcripts decreased with the progression of IM, while MUC2 and villin-1 were inversely correlated. Sox2 showed a gradual decrease from G, through GI, to the I type (G vs GI and GI vs I, P<0.01 and P<0.005, respectively). On the other hand, Cdx1 (G vs GI and GI vs I, P<0.0001 and P=0.337, respectively) and Cdx2 (G vs GI and GI vs I, P<0.0001 and P<0.05, respectively) appeared in IM. Immunohistochemical study confirmed decreased expression of Sox2 and ectopic emergence of Cdx2 protein in IM glands. CONCLUSION: Down-regulation of Sox2, besides ectopic expression of Cdx genes, may be important factors for the development of IM.

Avian Proteins↗

The Drosophila dominant wing mutation Dichaete results from ectopic expression of a Sox-domain gene.

The dominant Drosophila wing mutation Dichaete is characterised by the deletion of proximal wing structures. By analysing a number of new Dichaete alleles, phenotypic revertants and enhancer piracy lines, we show that the wing phenotype results from ectopic expression of the Sox-domain gene Dichaete. Ectopic expression of the Sox gene results in an increase in cell death in the proximal region of the wing imaginal disc and leads to alterations in the normal expression of wingless. Since ectopic expression of wingless in the proximal region of the wing disc can rescue aspects of the Dichaete phenotype, it is likely that Dichaete specifically interferes with the establishment or maintenance of a critical domain of wingless expression in the wing disc.

Alleles↗

Ectopic lingual thyroid as unusual cause of severe dysphagia.

Ectopic lingual thyroid is an uncommon embryological aberration characterized by the presence of thyroid tissue located in a site different from the pretracheal region as in the normal. Lingual thyroid is the most frequent ectopic location of the thyroid gland, although its clinical incidence varies between 1:3000 and 1:10,000. We present the case of a 26-year-old woman who presented severe dysphagia caused by a mass located on the base of the tongue in the midline. An endoscopic partial removal of the ectopic tissue allowed her to rapidly regain her swallowing capacity. The literature regarding the incidence and diagnosis of lingual thyroid is reviewed and the possibilities of treatment discussed.

Adult↗

Laparoscopically guided transperitoneal percutaneous nephrolithotomy for calculi in pelvic ectopic kidneys.

Although percutaneous nephrolithotomy is a well-established endourological modality for the management of calculi in the normally placed kidney, it is not easy to apply in the management of calculi in pelvic ectopic kidneys. We report the cases of three patients who were found to have large calculi in pelvic ectopic kidneys and subsequently underwent laparoscopically guided transperitoneal percutaneous nephrolithotomy, all with successful outcome. In all patients, complete stone clearance was achieved in a single operation with no intraoperative or postoperative morbidity. They remain asymptomatic and recurrence-free at a follow-up ranging from 2 to 38 months. Laparoscopic guidance allows the transperitoneal route to be used safely for percutaneous nephrolithotomy in patients with calculi in pelvic ectopic kidneys. We believe it to be a feasible, safe, and valid minimally invasive management option for this uncommon but challenging urological condition.

Abdominal Pain↗

Nephron-sparing surgery in a duplex system associated with a vaginal ectopic ureter.

Continuous dribbling of urine is the major symptom in girls with an ectopic vaginal ureter. However, the diagnosis is somewhat difficult in girls with such an entity before toilet-training has been achieved. The current concept in the management of these entities is to remove the renal moieties with the vaginal ectopic ureter drain. We report on a case of an obstructed vaginal ectopic ureter associated with a duplicated collecting system which was managed successfully by renal-sparing surgery.

Child, Preschool↗

Ectopic thyroid in the gallbladder: report of a case.

Ectopic thyroid is a rare developmental anomaly. We describe an unusual case of ectopic thyroid tissue found in the gallbladder wall of a 68-year-old man who underwent cholecystectomy for acute cholecystitis. Pathological findings were compatible with ectopic thyroid tissue in the gallbladder wall. This very rare pathology is discussed with a review of the relevant literature.

Aged↗

Ectopic clival craniopharyngioma.

Ectopic craniopharyngioma is a rare entity. The authors present a very rare case of an ectopic clival craniopharyngioma completely separate from the sella turcica.A 44-year old woman presented with abducens palsy. A MR imaging study and a CT scan revealed a cystic clival lesion separate from the sella turcica. Surgical resection was performed successfully with flexible endoscope-assisted procedure using an endonasal transsphenoidal approach. No evidence of involvement of the sellar region was found according to radiological, intra-operative, and clinical findings.A review of the literature revealed no other such cases. The discussion includes the formation of craniopharyngioma from the ectopic Rathke's pouch remnants and the surgical approach for clival lesions. We believe that our approach provides good results with minimal invasiveness for some clival lesions.

Adult↗

Ectopic prostatic tissue of the anal canal presenting with rectal bleeding: report of a case.

PURPOSE: Ectopic prostatic tissue at various sites within and outside the genitourinary system has been reported previously. A case of ectopic prostatic tissue located in the anal canal causing rectal bleeding is presented. METHOD: The patient was referred to our clinic with rectal bleeding. At rectal examination a bleeding sessile polypoid mass 2.5 cm in size was found in anal canal and removed surgically. RESULTS: Histopathologic and immunohistochemical staining of the specimen confirmed the prostatic nature of the tissue. CONCLUSION: Prostatic heterotopia is significant in several respects. Either it may be an important cause of hematuria or unusually, as in our case, it may cause rectal bleeding. In addition, ectopic tissue may be endoscopically confused with malignancy in either urinary or lower gastrointestinal system. This and other reports may disclose the genesis and significance of this peculiar tissue remnant.

Anus Diseases↗

Ectopic recurrence of craniopharyngioma: a case report and review of the literature.

The ectopic recurrence of craniopharyngioma is a rare postoperative complication, for which there are fifteen reported cases that have been surgically verified. The authors present a rare case of ectopic recurrence along the tract of the surgical route. A 12-year-old girl, who had undergone total removal of a suprasellar craniopharyngioma 4 years previously, presented with a generalized tonic-clonic seizure. Magnetic resonance imaging revealed a strongly enhancing cystic tumor in the right frontal lobe, under the bone flap used for the previous craniotomy. Gross total resection was achieved by revision of previous craniotomy. The intraoperative findings revealed that the recurrent tumor was anatomically related to the previous surgical tract, suggesting dissemination along the operative tract. The histopathological features of the mass were the same as those of the previous suprasellar craniopharyngioma. The case presented here and a review of the reports on the previous instances of ectopic recurrence of craniopharyngioma suggest that meticulous protection of the surgical field and careful handling of the tumor during the operation are required. It should be emphasized that long-term follow-up is mandatory, even in patients who underwent a gross total removal.

Brain Neoplasms↗

Activation of the WUS gene induces ectopic initiation of floral meristems on mature stem surface in Arabidopsis thaliana.

A gain-of-function Arabidopsis mutant was identified via activation tagging genetic screening. The mutant exhibited clustered ectopic floral buds on the surface of inflorescence stems. The mutant was designated as sef for stem ectopic flowers. Our detailed studies indicate that the ectopic flower meristems are initiated from the differentiated cortex cells. Inverse PCR and sequence analysis indicated that the enhancer-containing T-DNA from the activation tagging construct, SKI015, was inserted upstream of the previously cloned WUS gene encoding a homeodomain protein. Studies from RT-PCR, RNA in situ hybridization and transgenic plant analysis further confirmed that the phenotypes of sef are caused by the overexpression of WUS. Our results suggest that overexpression of WUS could trigger the cell pluripotence and reestablish a new meristem in cortex. The type of new meristems caused by WUS overexpression was dependent upon the developmental and physiological stages of a plant. With the help of some undefined factors in the reproductive organs the new meristems differentiated into floral buds. In a vegetative growth plant, however, only the new vegetative buds can be initiated upon the overexpression of WUS. These studies provide new insights of WUS on flower development.

Arabidopsis↗

Radiofrequency catheter ablation of atrial fibrillation initiated by spontaneous ectopic beats.

Several reports have demonstrated that most paroxysmal atrial fibrillation is initiated by ectopic beats from a focal area, and radiofrequency catheter ablation can effectively cure atrial fibrillation. Although most of the ectopic beats originate from the orifices of the pulmonary veins or from the myocardial sleeves in the pulmonary veins, ectopic beats can also originate from superior vena cava, crista terminalis, coronary sinus, ligament of Marshall, or left atrial posterior free wall. Owing to the potential risk and complexity of catheter ablation, the ideal candidates should have frequent episodes and drug refractory paroxysmal atrial fibrillation.

Action Potentials↗