Displasia mesenquimal placentaria: caso clínico / Placental mesenchymal dysplasia: a case report

La displasia mesenquimal placentaria es una entidad poco conocida, de etiología incierta y subdiagnosticada. Frecuentemente, es confundida con enfermedad trofoblástica gestacional debido a que se presenta con hallazgos ultrasonográficos caracterizados por una placenta engrosada, con quistes e imágenes hipoecogénicas y niveles de gonadotrofina coriónica humana normales o levemente aumentados. El feto es frecuentemente viable y puede manifestar retraso del crecimiento intrauterino, prematurez o asociarse al síndrome de Beckwith-Wiedemann. Se presenta el caso de una mujer joven con un parto pretérmino con placentomegalia, sospecha de mola hidatidiforme parcial y un recién nacido pequeño para la edad gestacional.

The placental mesenchymal dysplasia is a not well known entity, with an uncertain etiology and under diagnosed. It is frequently confused with gestational trophoblastic disease because of its ultrasonographic features of a thick placenta, cysts and hypoechogenic images, with normal or slightly increased levels of human chorionic gonadotrophic hormone. The fetus is often viable and can manifest intrauterine growth restriction, prematurity or be associated with Beckwith-Wiedemann syndrome. We present a case report of a young woman with a preterm delivery, placentomegaly, suspicious of a partial hydatidiform mole and a low growth newborn.

A biochemical and histopathological evaluation of generalized pulp calcification in young permanent teeth.

Pulp calcifications are a frequent finding on bitewing and periapical radiographs in older age-groups but their occurrence in the entire dentition in young subjects is unusual. We report such an unusual occurrence of generalized pulp calcification in a 13-year-old Indian female. Radiographic examination of the dentition revealed pulp calcifications in all permanent teeth, located mostly in the pulp chamber but with some in the root canals. The patient's dental, medical, and family history was noncontributory. Biochemical analysis of the removed pulp calcification from one of the teeth during endodontic treatment showed large amounts of calcium, phosphorus, and carbonate. However, metabolic evaluation of patient through liver and kidney function tests and other blood investigations did not reveal any metabolic disorder. The patient was also evaluated for any systemic, syndromic, or genetic involvement but this was also noncontributory. Therefore, we propose that this unusual case of generalized pulp calcification is of idiopathic origin. In this work, histopathological and biochemical evaluations of the pulp calcification was done to try and understand the initiation and progress of calcifications in pulpal tissue.

Adult soft-tissue sarcomas of the head and neck.

Background: Head and neck sarcomas are relatively rare tumors, accounting for only about 2% of all head and neck malignancies and for 4 to 10% of all soft tissue sarcomas. The rarity and more over the heterogeneity of these tumors make rigorous study of their clinical behavior difficult. Aims: We analyzed our single center experience of adult soft tissue sarcomas of the head and neck. Settings and Design: Our study included 27 patients of adult soft-tissue sarcomas of the head and neck who presented to our center between January 1996 and December 2005. Materials and Methods: Patient characteristics, clinical features, treatment modalities, clinical course, and long-term outcomes and its determinants were analyzed. Results: Surgery in the form of wide excision aimed at achieving negative margins was the main modality of treatment and adjuvant radiation was used in selected patients. The five-year disease-free survival was 64.5% and overall survival (OS) was 68%. Of the multiple potential factors analyzed, univariate analysis showed grade, margin status, and adjuvant radiation to be the important prognostic factors for the survival. The multivariate analysis however only showed margin status and adjuvant radiation to be prognostic. Conclusions: Surgery with negative margins is a key to long-term survival. Complete resection with adjuvant radiation in selected cases potentially decreases local recurrences and improves OS.

Long-term follow up of mesenchymal hamartoma of liver-single center study

Mesenchymal hamartoma of liver [MHL] is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL. All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically. The total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 +/- 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one [11.11%].: MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory

The Role of Epithelial-mesenchymal Transition in the Gastroenterology / 대한소화기학회지

The epithelial-mesenchymal transition (EMT) plays physiologic roles in the embryogenesis, wound healing, and tissue regeneration. In terms of pathological direction, it causes organ fibrosis, cancer development, progression, metastasis, and chemoresistance. Recently, the underlying mechanism of EMT and many kinds of EMT regulators have been identified. Pharmaceutical treatment strategies which target EMT pathway could be applied for the prevention of tissue fibrosis and cancer progression. In the field of gastroenterology, profuse evidences have been collected about the critical roles of EMT in cancers of the gastrointestinal tract, liver, and pancreas and hepatic fibrosis. However, EMT varies widely among cancer types, and much remains to be identified about the main regulators of EMT in a specific disease. In this review, we present recent research results regarding the roles of EMT in cancers and organic fibrosis, especially in the area of gastroenterology.

Odontoameloblastoma: Report of two cases.

Odontoameloblastoma (OA) is a very rare mixed odontogenic neoplasm, characterized by the simultaneous occurrence of an ameloblastoma and a compound or complex odontoma in the same tumor mass. To date, less than 50 cases of OA and/or ameloblastic odontoma have been reported in the English dental literature. This neoplasm was called ameloblastic odontoma. The term OA was included in the 1971 WHO classification. In this study, we present two cases of OA, which we hope will contribute to the awareness and knowledge of surgeons regarding the existence of this odontogenic tumor so that patients having it may be treated and followed-up properly.

TGF-beta Mediated Epithelial-Mesenchymal Transition in Autosomal Dominant Polycystic Kidney Disease

PURPOSE: Recent studies have showed that epithelial-mesenchymal transition (EMT) is a key process of glomerular and tubulointerstitial pathology in many chronic kidney diseases. However, there are no data of EMT in humane autosomal dominant polycystic kidney disease (ADPKD). PATIENTS AND METHODS: ADPKD kidneys (N = 5) with end stage renal disease (ESRD) and control kidneys (N = 4) were analyzed immnunohistochemically. We evaluated alpha-SMA, E-cadherin, vimentin, TGF-beta1 and Smad 2/3 expression in ADPKD and compared them with those in control kidney. These immunohistochemical findings were quantitatively analyzed by computer-assisted image analyzer and positive tubules (%). RESULTS: There were severe interstitial fibrosis and proliferation of alpha-SMA+ myofibroblasts in ADPKD. Cystic tubular epithelial cells in ADPKD lost epithelial marker (E-cadherin) and expressed mesenchymal markers (alpha-SMA, vimentin). There were significant increases of alpha-SMA (34.3 +/- 11.7% vs 0.9 +/- 1.5%), vimentin (19.9 +/- 3.9% vs 3.3 +/- 1.4%), TGF-beta1 (5.42 +/- 2.83% vs 0%) and Smad 2/3 (3.4 +/- 1.7% vs 0.7 +/- 0.6%) in ADPKD kidneys compared with control kidneys evidenced by computer-assisted image analyzer. When we analyze the positive tubules (%), the results were the same as computer-assisted image analyzer. CONCLUSION: Our results showed that the end stage of ADPKD is associated with TGF-beta, Smad 2/3 and markers of EMT. It suggests that TGF-beta mediated EMT has a role in progression of ADPKD.

Osteomalácia oncogênica: cintilografia com sestamibi-99mTc na localização do tumor / Oncogenic osteomalacia: localization of underlying peripheral tumor with99mTc-sestamibi scintigraphy

A osteomalácia oncogênica hipofosfatêmica (OOH) é uma síndrome paraneoplásica induzida por tumor, de tecidos mole ou ósseo. Apresenta-se com dor e fraturas, acompanhada de hipofosfatemia, hiperfosfatúria e concentrações plasmáticas de 1,25(OH)2D3 inapropriadamente normais/diminuídas. Após a remoção do tumor, a completa resolução das anormalidades clínicas e bioquímicas é sua maior característica. Uma mulher de 44 anos de idade é descrita no caso com dificuldade para caminhar por causa de dores nos membros inferiores, fraqueza muscular generalizada e hipofosfatemia com relativa hiperfosfatúria. A cintilografia de corpo total com sestamibi-99mTc mostrou acúmulo do radiofármaco no terço superior de coxa esquerda onde pequeno tumor foi detectado no exame pelo ultra-som. Com a retirada do tumor, um lipoma, os sintomas melhoraram após um mês, com recuperação completa ao redor do quarto mês. Neste caso, a cintilografia de corpo inteiro com sestamibi-99mTc foi decisiva na localização do tumor causador da osteomalácia oncogênica.

Oncogenic osteomalacia is a paraneoplastic syndrome usually induced by bone or soft tissue tumors. It is presented by the development of pain and fractures with hypophosphatemia, hyperphosphaturia, and inappropriate normal/low plasma 1,25(OH)2D3 concentration. After the removal of the tumor the complete resolution of all biochemical and clinical abnormalities is the main characteristic. A case of a 44-year-old female with difficulty in walking due to leg pain and generalized muscle weakness and hypophosphatemia, with relative hyperphosphaturia, is described. A whole-body 99mTc-sestamibi scintigraphy showed accumulation in the left thigh region, and a small tumor was detected by ultrasound examination. By removal of the tumor, a lipoma, the symptoms improved significantly after a month, with complete recovery by the fourth month. In this case, 99mTc-sestamibi scintigraphy was useful in identifying the location of the tumor, which caused oncogenic osteomalacia.

Asthma: where is it going?: [review]

Asthma is characterized by reversible airway obstruction, airway hyperresponsiveness, and airway inflammation. Although our understanding of its pathophysiological mechanisms continues to evolve, the relative contributions of airway hyperresponsiveness and inflammation are still debated. The first mechanism identified as important for asthma was bronchial hyperresponsiveness. In a second step, asthma was recognized also as an inflammatory disease, with chronic inflammation inducing structural changes or remodeling. However, persistence of airway dysfunction despite inflammatory control is observed in chronic severe asthma of both adults and children. More recently, a potential role for epithelial-mesenchymal communication or transition is emerging, with epithelial injury often resulting in a self-sustaining phenotype of wound repair modulation by activation/reactivation of the epithelial-mesenchymal trophic unit, suggesting that chronic asthma can be more than an inflammatory disease. It is noteworthy that the gene-environmental interactions critical for the development of a full asthma phenotype involve processes similar to those occurring in branching morphogenesis. In addition, a central role for airway smooth muscle in the pathogenesis of the disease has been explored, highlighting its secretory function as well as different intrinsic properties compared to normal subjects. These new concepts can potentially shed light on the mechanisms underlying some asthma phenotypes and improve our understanding of the disease in terms of the therapeutic strategies to be applied. How we understand asthma and its mechanisms along time will be the focus of this overview.

Fatal bilateral congenital mesenchymal hamartoma of the chest wall.

The authors reported a newborn female baby, 36 weeks gestational age, 2680 gms who developed respiratory distress at 2 hours after birth. Her chest roentgenograms showed a normal-sized heart, sparse lung markings and bilateral masses involving the posterior 6th to 8th ribs. Thoracotomy with partial removal of the left chest mass was performed when she was 12 hours age. Postoperatively, the baby developed progressive respiratory distress and expired at 29 hours-age. Autopsy revealed bilateral nodular masses arising from the inner side of the posterior aspect of the 6th to 8th ribs, measuring 6 x 5 x 4 cm (right) and 7 x 6 x 4 cm (left). The cut surfaces showed multicystic spaces containing blood. Histologically, many blood filled spaces with walls of fibroblasts, cartilage and bone tissue were noted. The diagnosis was mesenchymal hamartoma of the chest wall. Mesenchymal hamartoma of the chest wall usually arises from the posterior or lateral portions of the rib and usually involves many ribs. Multi focal lesions and bilaterality are rare. The clinical presentation can be asymptomatic, mild or severe respiratory distress. Surgical resection is the treatment of choice.

Solitary fibrous tumour: a diagnostic dilemma.

Solitary fibrous tumour (SFT) is a rare spindle cell neoplasm arising at pleural and extrapleural sites. Five cases of SFT diagnosed at our institution over a five year period were reviewed. Haematoxylin and eosin stained histological sections, immuno-histochemical markers including CD34 and electron microscopy were the different methods used to study these tumours. Three histological features were consistently observed in all the tumours: the tumours were composed of short spindle cells separated by dense collagen bands and arranged in alternate hypocellular and hypercellular areas. CD34 positivity was seen in all the cases. SFT's have been reported to behave in an unpredictable fashion and hence prolonged follow up is essential. Histology, CD34 positivity and electron microscopy are useful tools in diagnosing SFT. While the pleural tumours can be diagnosed based on histology, this must be substantiated by ancillary techniques in case of extrapleural tumours.

Leiomyoma of the prostate--a rare mesenchymal tumor: a case report.

Prostatic enlargement due to benign adenomatous hyperplasia is very common in elderly males. However, benign mesenchymal tumors, especially true leiomyoma, are rare in prostate. True prostatic leiomyoma has been defined by Kaufman and Berneike as a smooth muscle tumor within the prostate or juxta-prostatic in position, devoid of glandular elements. The recognition of leiomyoma is important because of the potential of malignancy in such cases, and histopathology is the only tool to do so. We describe the case of a 65-year-old male presenting with urinary obstruction for eight months. Per rectal examination revealed an enlarged firm prostate, a trucut biopsy from which showed only stromal tissue. A suprapubic prostatectomy was performed, and histopathological examination revealed a benign smooth muscle tumor (confirmed by immunohistochemistry), in absence of glandular hyperplasia. Thus, a diagnosis of true leiomyoma of the prostate was made. True leiomyoma is a rare tumor in prostate, which can be diagnosed only on histopathological examination. In addition, careful intra-operative and extensive pathologic assessment is mandatory for predicting the potential behaviour.

Clinico-pathological profile of 22 cases of cystic renal dysplasia.

Renal dysplasia is one of the major renal developmental anomaly characterized by abnormal structural organization and development of metanephric elements. It is usually detected antenatally or in early childhood. The kidney may be multicystic, aplastic, hypoplastic or duplex. We studied 22 cases of cystic renal dysplasia diagnosed over a period often years to identify the spectrum of morphological changes in dysplastic kidney, with special emphasis on mesenchymal changes. Clinical, radiological and gross morphologicalfeatures were noted. Microscopic features were studied in detail, including the epithelial and mesenchymal changes. Twenty-one of the 22 cases studied were children. One case was a 21-year-old adult, which is a rare age at presentation. Male to female ratio was 1.1:1. One of our patients had contra-lateral ureteric stenosis, a rare anomaly reported with renal dysplasia. Ten patients, all autopsy cases, had multi-system congenital anomalies. As cystic renal dysplasia is not a hereditary disease, it must be differentiated from polycystic kidney disease. Other differential diagnoses are cystic nephroma and cystic partially differentiated nephroblastoma. Histopathological examination is the final diagnostic tool since radiological features alone may not be sufficient to exclude other cystic renal lesions. Cartilage may not be seen in all cases of renal dysplasia. Once diagnosed, other associated anomalies should also be looked for.

Mechanisms of Epithelial-Mesenchymal Transition of Peritoneal Mesothelial Cells During Peritoneal Dialysis

A growing body of evidence indicates that epithelial-mesenchymal transition (EMT) of human peritoneal mesothelial cells (HPMC) may play an important role in the development and progression of peritoneal fibrosis during long-term peritoneal dialysis (PD) leading to failure of peritoneal membrane function. Here, we review our own observations and those of others on the mechanisms of EMT of HPMC and suggest potential therapeutic strategies to prevent EMT and peritoneal fibrosis during long-term PD. We found that high glucose and H2O2 as well as transforming growth factor-beta1 (TGF-beta1) induced EMT in HPMC and that high glucoseinduced EMT was blocked not only by inhibition of TGF-beta1 but also by antioxidants or inhibitors of mitogen-activated protein kinases (MAPK). Since MAPKs are downstream target molecules of reactive oxygen species (ROS), these data suggest that high glucose-induced generation of ROS and subsequent MAPK activation mediate high glucose-induced EMT in HPMC. We and others also observed that bone morphogenetic protein-7 (BMP-7) prevented EMT in HPMC. Glucose degradation products (GDP) were shown to play a role in inducing EMT. Involvement of a mammalian target of rapamycin (mTOR) in TGF-beta1-induced EMT has also been proposed in cultured HPMC. A better understanding of the precise mechanisms involved in EMT of HPMC may provide new therapeutic strategies for inhibiting peritoneal fibrosis in long-term PD patients.

Paraurethral leiomyoma: a case report.

A paraurethral leiomyoma is a rare benign hormone-dependent tumor of mesenchymal origin. A 43-year-old woman presented with a palpable mass near the urethral opening with no abnormal urinary symptom. Simple excision was performed Histopathological diagnosis was paraurethral leiomyoma with confirmation by immunohistochemical study. Further hormonal study of the tumor yielded positive estrogen and progesterone receptor expression, suggesting conservative treatment with gonadotropin-releasing hormone (GnRH) agonist or antagonist may have a role as an alternative treatment in an inoperable case.

Macrodystrophia Lipomatosa: CT and MR findings

Macrodystrophia lipomatosa is a rare congenital form of localized gigantism characterized by an increase in all mesenchymal elements, particularly fibroadipose tissue. MR imaging can be used to examine the soft tissue and detect the fibrous and fatty hypertrophy that characterizes this condition and also diagnose an associated fibrolipomatous hamartoma of the median or plantar nerve. We describe the clinical and radiological findings [including CT scan, MRI and bone scan] and also progression of this rare entity during a 10 year period

Mixoma de los maxilares: ¿tumor de alta agresividad? / Maxillary myxoma: a highly aggressive tumor?

Los tumores de los maxilares de origen odontogénico son aquellos que se originan a partir de la células epiteliales y mesenquimales especializadas que forman los tejidos del diente. En la patología oral y máxilo facial tenemos tumores de alta agresividad, tales como el Mixoma, el Tumor Odontogénico Epitelial Calcificante y el Ameloblastoma, importantes a tener en cuenta para el diagnóstico diferencial. Los autores reportan cuatro casos de mixoma en los maxilares, de los cuales dos fueron ubicados en la mandíbula y los restantes en el maxilar superior. El uso del colgajo de Weber-Ferguson para la resección de los Mixomas del maxilar superior fueron los elegidos debido al comportamiento y tamaño de dicho tumor. Mientras que los Mixomas mandibulares fueron resecados marginalmente debido al menor compromiso de estructuras mandibulares afectadas