Pancreatic metastasis of extra-skeletal mesenchymal chondrosarcoma diagnosed by ultrasound endoscopy-guided fine-needle aspiration

A 47-year-old man with a history of ESMC resection of the left chest wall seven years ago was admitted to our hospital due to mid-upper abdominal pain and jaundice for more than 10 days. Laboratory tests showed elevated direct bilirubin, alanine aminotransferase, gamma-glutamyltranspeptidase, and alkaline phosphatase. Computed tomography (CT) of the abdomen revealed soft tissue mass in the head and body of the pancreas with irregularly shaped calcifications, and an enhanced scan showed heterogeneous enhancement. Combined with the patient's past medical history, the possibility of pancreatic metastasis of ESMC was considered. After anti-inflammatory, hepatoprotective, and cholagogical treatment jaundice improved, and ultrasound endoscopy-guided fine-needle aspiration (EUS-FNA) was performed to clarify the nature of the mass, which showed a 4.1*4.2 cm mixed echogenic area with internal calcification in the head of the pancreas. Aspiration pathology showed proliferation of short spindle and round cells into nests, the immunohistochemistry stain showed CD99 (+); CD34, CD117, Dog-1, and S-100 were negative. Pancreatic metastasis of ESMC was diagnosed. Four months later, endoscopic biliary metal stent drainage (EMBD) was performed when the patient developed obstructive jaundice again due to lesion progression. PET/CT at a 2-year follow-up showed multiple high-density calcifications and abnormally increased FDG metabolism throughout the body. (AU)

A Huge Mass in a Boy's Chest: An Unusual Case of Mesenchymal Chondrosarcoma / Enorme masa en el tórax de un niño: caso inusual de condrosarcoma mesenquimal

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Inhibitory effect and mechanism of mesenchymal stem cells on melanoma cells

Purpose. To explore the inhibitory effect and mechanism of MSCs on melanoma proliferation. Methods. The inhibitory effect of MSCs on melanoma A375 cells was detected by co-culture and conditioned medium (CM) experiments using MTT method. The cell cycle was analyzed by flow cytometry. Then, Western Blot experiment detected the expression of proteins related to NF-κB signaling in A375 cells. The expression of IL-1Ra in MSCs was proved by RT-PCR. The over-expression and silencing vector pcDNA3.1-EGFP-IL-1Ra and pGPH1-IL-1R were constructed and transfected into MSCs cells. After that, the changes of inhibitory effect and cell cycle from MSCs-S and MSCs-O CM on A375 cells were explored. The expression of proteins related to NF-κB signaling in A375 cells after MSCs-S or MSCs-O CM treatment was detected by Western Blot. MSCs, MSCs-S, or MSCs-O and A375 cells were co-injected into nude mice under the arms, the growth of tumor was observed, the frozen sections were made, and H&E staining of tumor tissue was performed. Results. The proliferation of A375 cells was inhibited and the cell cycle of A375 was arrested by MSCs. The expressions of cytokines related to NF-κB signaling were down-regulated. Over-expression and silence of Interleukin 1 receptor antagonist (IL-1Ra), specifically blocking activation of NF-κB signaling, indicated that inhibitory effect from MSCs was enhanced or weakened respectively, which suggested that IL-1Ra was involved in the inhibitory effect. In vivo, tumor initiation and growth were significantly inhibited when A375 cells were co-injected with MSCs into nude mice, which were related to the expression level of IL-1Ra. Conclusion. MSCs could inhibit the proliferation and tumor initiation of melanoma A375 cells through NF-κB signaling. MSCs could secret IL-1Ra and inhibit expressions of NF-κB signaling-related factors of tumor cells, and cause cell cycle arrest in G1 phase (AU)

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Lesiones lipomatosas uterinas: presentación de un caso y revisión de la literatura / Uterine fatty lesions: case report and a review of the literature

Las lesiones lipomatosas uterinas son una variante poco frecuente de los tumores mesenquimales benignos. Se dividen en lipomas puros y lesiones lipomatosas (difusas o focales) en el seno de un leiomioma. Presentamos el caso de una mujer de 70 años con histerectomía simple tras el diagnóstico ecográfico de «mioma gigante». Macroscópicamente la lesión correspondía a una formación lipomatosa de 13,8cm de eje máximo e histológicamente estaba constituida por células adiposas maduras, sin septos, y con cambios atróficos y traumáticos, positivas para S100 y vimentina y negativas para MDM2 y CDK4 por inmunohistoquímica e hibridación fluorescente in situ. Focalmente se identificaban áreas de degeneración mixoide y no se observaba necrosis ni hemorragia. El estudio ultraestructural fue congruente con una proliferación celular con diferenciación adiposa (AU)

Uterine fatty lesions are a rare variant of benign mesenchymal tumours. They are divided into pure and lipomatous lesions (diffuse or focal) within a leiomyoma. We report the case of a 70 year old woman who underwent a simple hysterectomy after an ultrasound diagnosis of «giant leyomioma». Macroscopically the lesion corresponded to a lipomatous formation of 13.8cm maximum diametre. Histologically the lesion consisted of mature adipose cells without septa and traumatic and atrophic changes. There was focal myxoid degeneration, but necrosis and hemorrhage were not observed. The cells were S100 and vimentin positive on immunochemistry. MDM2 and CDK4 were negative by immunochemistry and fluorescence in situ hybridization. The ultrastructural study was consistent with a cell proliferation with adipose differentiation (AU)

Schwannoma gástrico como hallazgo ecográfico casual / Gastric schwannoma presenting as a casual ultrasonographic findings

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Angiomixoma agresivo. Un raro tumor mesenquimal pélvico / Aggressive angiomyxoma. A rare mesenchymal pelvic tumor

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Tumores mesenquimales de intestino delgado: descripción de dos casos inusuales / Small bowel mesenchymal tumors: description of two unusual cases

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Osteomalacia en un adulto joven / Osteomalacia in a young adult

Los cuadros de osteomalacia hipofosfatémica responden a diversas causas genéticas y adquiridas. Algunas variantes de tumores mesenquimales producen cantidades inapropiadas de factor de crecimiento fibroblástico 23 (FGF-23), un mediador que induce una pérdida renal de fosfatos. El cuadro bioquímico se caracteriza por hipofosfatemia, disminución de la reabsorción tubular de fosfatos, niveles bajos o inapropiadamente normales de calcitriol sérico y niveles altos o inapropiadamente normales de FGF-23 plasmático. Este síndrome paraneoplásico es denominado osteomalacia tumoral u oncogénica. Existen limitadas series de casos publicadas, pero su reconocimiento es creciente en los últimos años. El diagnóstico puede ser complejo por su baja incidencia, la dificultosa localización de los tumores y la heterogeneidad en la interpretación histopatológica. La exéresis quirúrgica completa es curativa, pero puede haber recidivas y los suplementos orales de fósforo y calcitriol son alternativas de tratamiento médico (AU)

Cases of hypophosphatemic osteomalacia respond to various causes, both genetic and acquired. Some variants of mesenchymal tumors produce inappropriate amounts of fibroblast growth factor 23 (FGF-23), a mediator which induces renal phosphate loss. The biochemical picture is characterized by hypophosphatemia, decreased tubular reabsorption of phosphates, low or inappropriately normal serum calcitriol and high or unusually normal levels of FGF-23 plasma. This paraneoplastic syndrome is called tumorinduced or oncogenic osteomalacia. There are a limited series of published cases, although it has been increasingly accepted in recent years. Diagnosis may be complex given its low incidence, the difficulties in localizing the tumors and heterogeneity in histopathologic interpretation. Complete surgical removal has healed, but there may be recurrences whereas phosphorus and calcitriol oral supplements offer alternative medical treatment (AU)

Tumor fibrohistiocítico plexiforme con metástasis ganglionar: reporte de un caso / Plexiform fibrohistiocytic tumor with nodal metastasis: a case report

El tumor fibrohistiocítico plexiforme (TFHP) es una rara neoplasia mesenquimal de malignidad intermedia, con alta tasa de recidiva local y potencial metastásico ganglionar o pulmonar. Suele afectar tejidos blandos superficiales de extremidades superiores de niños y adultos jóvenes, con predilección femenina. Presentamos el caso de un niño de 10 años con tumoración de crecimiento progresivo, dependiente del plano cutáneo en rodilla derecha con adenopatías inguinales ipsilaterales. Se le realiza resección amplia de la lesión con linfadenectomía inguinocrural. El estudio histopatológico reveló una neoplasia de patrón plexiforme con componente bifásico (células mononucleares tipo histiocitos asociado a células gigantes tipo osteoclasto, CD68 positivas y células fusiformes, actina de músculo liso positivas); favoreciendo el diagnóstico de esta entidad con metástasis ganglionar (AU)

Plexiform fibrohistiocytic tumour (TFHP) is a rare mesenchymal neoplasm of intermediate malignancy but with a high rate of local recurrence and potential for lymph node or pulmonary metastases. It usually affects superficial soft tissues of the upper extremities of children and young adults and is more frequent in females. We report a case of a 10 year old boy with progressive tumour growth, under the skin in the right knee with ipsilateral inguinal lymphadenopathy. He underwent wide resection of the lesion with inguinal lymphadenectomy. Histopathology revealed a plexiform pattern with a biphasic component (mononuclear histiocytic cells associated with CD68 positive osteoclast-like giant cells and smooth muscle actin-positive spindle cells); favoring the diagnosis of TFHP with lymph node metastasis (AU)

Potencial terapéutico de las células madre derivadas de la médula ósea en el infarto cerebral / Therapeutic potential of bone marrow stem cells in cerebral infarction

Introducción. Las células madre constituyen una alternativa terapéutica que se encuentra en fase de experimentación para el infarto cerebral. Objetivo. Mostrar la evidencia científica existente sobre el potencial terapéutico de las células madre de la médula ósea en esta enfermedad. Desarrollo. El infarto cerebral representa el 80% de las enfermedades cerebrovasculares. La trombólisis constituye la única terapia aprobada, pero, por su estrecha ventana terapéutica, sólo se aplica a un bajo porcentaje de los pacientes. De manera alternativa, los tratamientos neurorrestauradores, como el de células madre, pueden aplicarse en períodos más prolongados. Por esta razón se efectuó una búsqueda bibliográfica en PubMed con el empleo de las palabras clave ‘stem cells’, ‘bone marrow derived mononuclear cells’ y ‘stroke’. Se encontraron evidencias de seguridad y eficacia de dichas cé- lulas en diferentes momentos evolutivos del infarto cerebral. Se identificaron estudios que en clínica y preclínica las recolectaron por punción medular y en sangre periférica, y las trasplantaron directamente en el área infartada o por vía intravascular. El efecto terapéutico se relaciona con sus propiedades de plasticidad celular y liberación de factores tróficos. Conclusiones. El concentrado de células mononucleares autólogas, obtenido en sangre periférica o por punción de la médula ósea, y trasplantado por vía intravenosa, es una factible opción metodológica que permitirá rápidamente incrementar el número de ensayos clínicos en diferentes etapas evolutivas del infarto cerebral. Esta terapia muestra seguridad y eficacia; sin embargo, deben ampliarse las evidencias que avalen su generalización en humanos (AU)

Introduction. Stem cells are an alternative therapy for cerebral infarction that is still in the experimental phase. Aims. To report on the existing scientific evidence on the therapeutic potential of bone marrow stem cells in this disease. Development. Cerebral infarction accounts for 80% of cerebrovascular diseases. Thrombolysis is the only approved therapy, but, owing to its narrow therapeutic window, it is only applied to a low percentage of patients. Conversely, neurorestorative treatments, such as stem cells, can be applied over longer periods of time. For this reason a literature search was conducted on PubMed using the key words ‘stem cells’, ‘bone marrow derived mononuclear cells’ and ‘stroke’. Evidence was found of the safety and effectiveness of such cells at different points in the development of the completed stroke. Results included studies that, in the clinical and preclinical period, collected them by spinal puncture and in peripheral blood, and transplanted them either directly into the infarcted area or intravenously. The therapeutic effect is related with their cell plasticity and trophic-factor releasing properties. Conclusions. Autologous mononuclear cell concentrate, obtained from peripheral blood or by puncturing the bone marrow and transplanted intravenously, is a feasible methodological option that will make it possible to quickly increase the number of clinical trials conducted at different stages of the development of a completed stroke. This therapy has proved itself to be safe and effective; nevertheless, further evidence is needed to endorse its generalised use in humans (AU)

Angiofibroma de células gigantes en mucosa yugal: una entidad rara en una localización infrecuente / A rare entity in a rare location: giant cell angiofibroma in the oral mucosa

El angiofibroma de células gigantes es una neoplasia mesenquimal benigna poco habitual descrita inicialmente en la órbita y con publicaciones posteriores en otras localizaciones menos frecuentes. En este sentido, la ubicación en la cavidad oral es muy poco habitual. Los hallazgos histológicos son los de una proliferación de células fusiformes dispuestas en un estroma fibromixoide sin patrón definido y con la presencia característica de células gigantes multinucleadas, en ocasiones dispuestas en torno a espacios pseudovasculares. Aportamos un nuevo caso de esta entidad en la cavidad oral y describimos los datos histológicos, inmunohistoquímicos y moleculares reseñados en la bibliografía más reciente (AU)

Giant cell angiofibroma is a rare benign mesenchymal neoplasm originally described in the ocular orbit. Since then, it has been reported in several less frequent extraorbitary locations, including the oral cavity. Histologically the tumour is composed of a disorganized proliferation of spindle cells in a fibromyxoidstroma with giant multinucleated cells around pseudovascular spaces. We report a new case of this entity in the oral cavity together with an up-to-date review of the histological, immunohistochemical and molecular features reported in the literature (AU)

Angiomixoma agresivo pélvico: diagnóstico en un examen de salud periódico de una trabajadora expuesta a radiaciones ionizantes / Agressive angiomyxom of the pelvis: diagnosis in an annual health examination of a worker exposed to ionizing radiation

El angiomixoma agresivo (AA) fue descrito por Steeper y Rosai en 1983 para definir una neoplasia mesenquimal mixoide de crecimiento lento localizada en la región pélvica, genital y/o perineal. Se tratan de tumores benignos, poco frecuentes, infiltrativos localmente, raramente metastatizantes y muy recidivantes. La prevalencia mujer/hombre es de 6:1, siendo más frecuente en la edad fértil por estar relacionada con los niveles hormonales de estrógenos y progesterona. Clínicamente el AA cursa con sintomatología inespecífica y variable en relación al tamaño tumoral y a su crecimiento lento, de ahí la importancia de realizar un diagnóstico diferencial precoz tanto macroscópico como microscópico. El diagnóstico se basa en pruebas de imagen (Resonancia Magnética, Tomografía Computarizada), histológicas e inmunohistoquímicas, siendo estas determinantes a la hora de la exéresis completa tumoral, tratamiento de elección en estos casos, al no haber sido demostrada la eficacia de otras terapias como la radio o quimioterapia

Aggressive angiomyxoma (AA) was described in 1983 by Steeper y Rosai as a myxoid mesenchymal neoplasm of the pelvic, genital and/or perineal region with slow growth. The AA is an uncommon and benign neoplasm, although it is a locally aggressive neoplasm. It is rarely metastasize and shows a high rate of recurrence. The prevalence rate female/male is 6:1, with a higher frequency in the childbearing age due to its relation with oestrogen and progesterone levels. The clinical symptoms of the AA are several and non-specific. Those symptoms are related to the size and the slow growth of the tumours. For this reason it is extremely important to do an early macroscopic and microscopic differential diagnosis. The specific diagnosis of this tumour is based on imaging test (CTscan, MRI), as well as an histopathological and immunohistochemical study, which are critical to carry out a complete surgical resection of the tumour, which is the main treatment in this case. There are no evidences of the successful of radiotherapy and chemotherapy

Insights into the epithelial mesenchymal transition phenotype in cancer of unknown primary from a global microRNA profiling study

PURPOSE: We sought to study the microRNA regulation of epithelial mesenchymal transition (EMT), the acquisition of migratory, mesenchymal-like properties of epithelial cells, in cancer of unknown primary (CUP). PATIENTS AND METHODS: We studied the global expression profile of 982 microRNAs by means of microarray technology in 68 CUP cases immunohistochemically characterised as EMT-positive (n = 5 by % of cells or n = 10 by a semiquantitative H-score) or EMT-negative. RESULTS: EMT-suppressive miRNAs such as miR-203 and members of the miR-200 family (miR-200a,b,c and miR-141) presented a 2.45 to 3.64-fold lower expression level in the EMT-positive cases without, however, reaching statistical significance. MiR-205, a squamous tissue-specific marker, was very variable in the data set. Excluding CUP cases with squamous cell histology, miR-205, miR-203 and the miR-200 family exhibited a trend of downregulation in EMT-positive cases. A similar pattern of miRNA expression was detected when the comparison took place between EMT-positive vs EMT-negative cases according to the H-score. Moreover, miR-203, miR-205 and miR-200c were numerically downregulated in those tumours with high expression of the EMT marker N-cadherin. CONCLUSIONS: The EMT-suppressive miR-203 and miR-200 family were consistently but non-significantly downregulated in CUP with the EMT phenotype. A larger study is warranted to further explore the role of microRNAs in CUP (AU)

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Hemangiomas y hamartomas hepáticos en un lactante con síndrome de Beckwith-Wiedemann asociado a displasia mesenquimal placentaria / Hepatic hemangiomas and hamartomas in children with Beckwith-Wiedemann syndrome associated with placental mesenchymal dysplasia

La displasia mesenquimal placentaria (DMP) es una rara patología que afecta al desarrollo de la vascularización placentaria y condiciona la aparición en el recién nacido de hamartomas hepáticos y alteraciones hematológicas, asociándose además en 1 de cada 4 casos al desarrollo del síndrome de Beckwith-Wiedemann (BWS), con la que comparte un origen genético común. Presentamos el caso de un recién nacido afectado de BWS asociado a DMP, que además de los hamartomas hepáticos descritos en la bibliografía, presentó como hallazgo casual lesiones hepáticas de tipo sólido con diagnóstico anatomopatológico de hemangiomas hepáticos con marcador Glut-1 positivo, molécula con implicaciones en la respuesta terapéutica y el pronóstico a largo plazo de estas lesiones. El tratamiento con propranolol es efectivo en estos casos, ya que consigue disminuir el tamaño de las lesiones, como en el caso que presentamos (AU)

The placental mesenchymal dysplasia (DMP) is a rare disease that affects the development of placental vascularization and conditions the appearance of liver hamartomas and blood disorders in newborns. In addition it is associated with Beckwith-Wiedemann syndrome (BWS) in 25% of the cases, sharing a common genetic origin. We report a case of both entities (DMP and BWS) in a male newborn, who developed not only liver hamartomas as described in the literature but also liver hemangiomas with positive marker Glut-1 as a new finding; this molecule is related to a better therapeutic response and long-term prognosis. The patient recieved treatment with propranolol with successfull reduction of the lesions size (AU)

Tumor fibroso solitario primario en tiroides: comunicación de un caso y revisión de la literatura / Primary solitary fibrous tumour of the thyroid: a case report and review of the literature

El tumor fibroso solitario es una neoplasia mesenquimal de localización tiroidea muy poco usual. Comunicamos el caso de un varón de 42 años con resección del lóbulo tiroideo derecho tras un diagnóstico citológico de proliferación folicular. Macroscópicamente la lesión correspondía a un nódulo encapsulado de 2,5 cm de diámetro máximo. Histológicamente la lesión estaba constituida por una proliferación de células fusiformes que engloban y atrapan a los folículos tiroideos, positivas para vimentina, CD34 y BCL2. El estudio ultraestructural confirmó la naturaleza miofibroblástica de las células neoplásicas (AU)

The thyroid gland is an unusual location for solitary fibrous tumours. We report a case of a 42 year old male with a cytological diagnosis of follicular proliferation. Macroscopically the lesion corresponded to an encapsulated nodule of 2.5 cm. Histologically, it showed a proliferation of spindle cells encompassing thyroid follicles, positive for vimentin, CD34 and BCL2. The ultrastructural study confirmed the myofibroblastic nature of the proliferative cells (AU)

Histiocitoma fibroso angiomatoide Presentación de un caso y revisión de la literatura / Angiomatoid fibrous histiocytoma. A case report and a review of the literature

El histiocitoma fibroso angiomatoide es una neoplasia mesenquimal de malignidad intermedia e histogénesis incierta que se da frecuentemente en las extremidades de niños o adultos jóvenes. Su morfología es peculiar, y está constituida como una lesión circunscrita de células fusiformes, infiltrado linfoplasmocitario periférico, cavidades quísticas rellenas de sangre y frecuente expresión de desmina. Nosotros presentamos un nuevo caso y revisamos la literatura, en particular con respecto a acontecimientos genéticos, de diferenciación, morfológicos, inmunohistoquímicos y de diagnóstico diferencial (AU)

Angiomatoid fibrous histiocytoma is a mesenchymal neoplasm of intermediate grade malignancy which is most commonly found in the extremities of children and young adults. Its histogenesis is uncertain and its morphology is unusual as it is a circumscribed lesion with spindle cells, peripheral lymphoplasmacytic infiltrate, blood-filled cystic cavities and frequent desmin expression. We report a new case and review the literature, paying special attention to genetic developments, differentiation, morphology, immunohistochemistry and differential diagnosis (AU)

Condrosarcoma mesenquimal del maxilar superior: un tumor raro de la cavidad oral / Mesenchymal chondrosarcoma of upper maxilla: a rare tumor of the oral cavity

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Histiocitoma fibroso maligno. Un sarcoma excepcional en la glándula mamaria / Malignant fibrous histiocytoma. An exceptional sarcoma in the mammary gland

Introducción. El histiocitoma fibroso maligno constituye la neoplasia sarcomatosa más frecuente en los adultos, pero la mama es una localización excepcional. Presentamos el caso de una mujer que comenzó con una tumoración de crecimiento progresivo en la mama derecha. Caso clínico. Mujer de 68 años que consulta por autopalpación de un nódulo en la mama derecha que ha crecido de forma progresiva en los últimos meses. La mamografía y la ecografía muestran una imagen nodular con bordes bien definidos, situada en intercuadrantes superiores de mama derecha, sin adenopatías axilares. Se decidió intervención quirúrgica y el estudio histológico definitivo fue informado como neoformación mesenquimal fusocelular con patrón estoriforme. El estudio inmunohistoquímico fue compatible con un histiocitoma fibroso maligno. Conclusión. Es primordial el diagnóstico diferencial de esta entidad clínica debido a la variabilidad histológica de los tumores sarcomatosos. Sus características clínicas y radiológicas pueden hacerlo pasar desapercibido, pero su comportamiento agresivo hace necesario un diagnóstico precoz, lo cual permitirá un tratamiento adecuado para lograr el aumento en la supervivencia(AU)

Introduction. Malignant fibrous histiocytoma is the most common sarcomatous neoplasm in adults. Localization in the breast, however, is exceptional. We report the case of a woman who presented with progressive tumoral growth in the right breast. Case report. A 68-year-old woman consulted for a self-palpated nodule in the right breast that had grown steadily in the last few months. Mammography and ultrasound showed a nodule with well-defined borders, located in the upper inner quadrant of the right breast. There was no axillary lymphadenopathy. Surgery was performed and the histological examination gave a definitive diagnosis of mesenchymal spindle cell neoplasm with storiform pattern. Immunohistochemical analysis was compatible with a diagnosis of malignant fibrous histiocytoma. Conclusion. Due to the histological variability of sarcomatous tumors, differential diagnosis is paramount in malignant fibrous histiocytoma. Because of their clinical and radiological features, malignant fibrous histiocytoma can be overlooked. Because these tumors are aggressive, an early diagnosis is essential to allow appropriate treatment and to increase survival(AU)