A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study

Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study. Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy which in up to 25% of cases can be extrapulmonary with attachment to the parietal pleura. It is found in pediatric population under 5 years of age. It was initially proposed as a distinct entity by Manivel et al. in 1988. PPB is a proliferation of primitive mesenchymal cells that initially form air-filled cysts lined by benign-appearing epithelium (type I, cystic). Later on, the mesenchymal cells outgrow the cysts with formation of focal solid areas (type II, solid and cystic) and finally, mainly solid mass (type III, solid PPB).

Mielolipoma primario mediastinal / Primary mediastinal myelolipoma

Resumen El mielolipoma es un tumor benigno no funcional, la mayoría de ellos son asintomáticos y descubiertos de forma incidental a través de estudios de imagen o en es tudios de autopsia. Aun cuando la mayoría de los casos se presenta en la glándula suprarrenal, también se han informado en sitios extra-adrenales. Presentamos el caso de una mujer de 65 años de edad con un mielolipoma primario mediastinal. La tomografía computarizada de tórax mostró un tumor ovoide de bordes bien definidos de 6.5 × 4.2 cm, localizado en el mediastino posterior. Se realizó biopsia transtorácica de la lesión y el estudio microscópico reveló elementos hematopoyéticos y tejido adiposo maduro. Aun cuando los estudios de imagen como la tomografía computarizada y la resonancia mag nética son efectivos en el diagnóstico del mielolipoma primario mediastinal, la evaluación histopatológica es esencial para el diagnóstico definitivo.

Abstract Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most commonly occurs in the adrenal gland, it has also been reported at extra-adrenal sites. We present the case of a 65-year-old woman with a primary mediastinal myelolipoma. Computer tomographic scan of the thorax showed an ovoid tumor with well-defined borders of 6.5 × 4.2 cm, located in the posterior mediastinum. A transthoracic biopsy of the lesion was made, and the microscopic observation revealed hematopoietic cells and mature adipose tissue. Although computed tomo graphy and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, histopathological examination is essential for the definitive diagnosis.

Brown tumors of the jaw: an atypical manifestation of primary hyperparathyroidism / Tumores pardos de mandíbula: una manifestación atípica de hiperparatiroidismo primario

Abstract Brown tumors represent the terminal stage of bone remodeling processes in primary hyperparathyroidism. Currently they are rare, and typically affect long bones, pelvis and ribs. Brown tumors may be not included in the initial differential diagnosis of bone disease, espe cially when they are present in atypical localizations. We reported two cases of oral brown tumors as the initial presentation of primary hyperparathyroidism. In the first case, a 44-year-old woman presented a painful and sessile lesion of 4x3 cm over the central body of the mandible which progressively increased in 4-month. The second case involved a 23-year-old woman who was referred with a 3-month history of a painful and ulcerated mass of 2 cm arising from left maxilla, episodes of gingival hemorrhage and difficulty of breathing. Both cases were solitary tumors with no evidence of palpable cervical lymphadenopathy. Incisional biopsy of oral tumors resul ted in giant cell and primary hyperparathyroidism was confirmed by laboratory tests. After parathyroidectomy, histology confirmed adenoma in both cases. Although this type of clinical presentation has almost disappeared in the recent decades, brown tumors should be consi dered in the differential diagnosis of bone oral masses.

Resumen Los tumores pardos son raros y, por lo general, afectan huesos largos, pelvis y costillas. Pueden no estar incluidos en el diagnóstico diferencial inicial como manifestación de enfermedad esquelética, es pecialmente cuando se presentan en localizaciones atípicas. Comunicamos dos casos de tumores pardos orales como presentación inicial de hiperparatiroidismo primario. En el primer caso, una mujer de 44 años presentó una lesión dolorosa y sésil de 4 × 3 cm sobre el cuerpo central de la mandíbula que aumentó de tamaño progresivamente en 4 meses. El segundo caso corresponde a una mujer de 23 años que acudió por presentar una masa dolorosa y ul cerada de 2 cm en maxilar izquierdo de 3 meses de evolución, episodios de hemorragia gingival y difi cultad para respirar. Todos fueron tumores solitarios sin evidencia de linfadenopatía cervical palpable. La biopsia incisional de los tumores orales resultó en células gigantes, y las pruebas de laboratorio confirmaron el hiperparatiroidismo primario. Tras la paratiroidectomía, la histología confirmó adenoma en ambos casos. Los tumores pardos representan la etapa terminal de los procesos de remodelación ósea en el hiperparatiroidismo primario. Aunque este tipo de presentación clínica casi ha desaparecido en las últimas décadas, los tumores pardos deben ser considerados en el diagnóstico diferencial de las masas óseas orales.

Hepatoblastoma en un paciente pediátrico a propósito de un caso

La incidencia de hepatoblastoma alrededor del mundo permanece constante entre 0.5 y 1.5 casos por millón de niños por año. En los Estados Unidos de América se reporta para el hepatoblastoma una incidencia anual de aproximadamente 1 por millón en niños menores de 15 años de edad. En Ecuador, en una investigación realizada en la ciudad de Cuenca, ocupa el séptimo lugar entre los tumores pediátricos. Se trata de un tumor infrecuente, cuya incidencia parece aumentar en los últimos años. Puede aparecer de forma aislada o integrarse en el contexto de un síndrome de predisposición. Presentamos el caso de un paciente pediátrico, femenina, preescolar de 3 años de edad, sin antecedentes perinatales de importancia, producto de la tercera gesta, nacida por cesárea por distocia de presentación a las 39 semanas. Cuenta con esquema de vacunación completo para la edad. Como antecedentes patológicos personales requiere una hospitalización por enfermedad diarreica aguda a los 2 años. Sin antecedentes quirúrgicos, antecedentes patológicos familiares de tía materna con hipotiroidismo. Se realizó exámenes complementarios de sangre y de imagen, los cuales revelaron una masa abdominal dependiente de hígado compatible con hepatoblastoma con niveles de AFP superiores a 1000ng/ml

The incidence of hepatoblastoma around the world remains constant between 0.5 and 1.5 cases per million children per year. In the United States of America, an annual incidence of approximately 1 per million is reported for hepatoblastoma in children under 15 years of age. In Ecuador, in a study carried out in the city of Cuenca, it ranks seventh among pediatric tumors. It is an infrequent tumor, its incidence seems to have increased in recent years. It can appear in isolation or be part of a predisposing syndrome. We present the case of a 3-year-old preschool female pediatric patient with no significant perinatal history, product of a third pregnancy, born by cesarean section due to presentation of dystocia at 39 weeks. She had a complete vaccination for her age. As past medical history, she was hospitalized for acute diarrheal disease at 2 years of age. She had no surgical history, family pathological history except for a maternal aunt with hypothyroidism. Complementary blood and imaging tests were performed, which revealed an abdominal liver-dependent mass, compatible with hepatoblastoma with AFP levels greater than 1000 ng/ml.

Plaque brachytherapy – Radiating positivity

Background: Plaque brachytherapy is an evolving, yet effective globe and vision-sparing modality for the treatment of intraocular tumors by transscleral irradiation of the tumor base with a radioactive implant. The American Brachytherapy Society (ABS) along with the collaboration of the international multicenter Ophthalmic Oncology Task Force (OOTF) was assembled to reach a consensus regarding establishing practice guidelines and setting standards of care for intraocular tumors. The advent of plaque brachytherapy has revolutionized the outcomes of intraocular tumors, thus ensuring globe salvage, reducing morbidity and mortality, and avoiding cosmetic disfigurement. A well-planned dosimetry for plaque brachytherapy results in achieving local tumor control and excellent prognosis. Purpose: This technique provides the advantage of focal radiation, thus eliminating the damage to the adjacent structures, minimal periorbital tissue damage, absence of cosmetic disfigurement owing to lack of retarded bone growth as seen in external beam radiotherapy. Thus, it reduces the risk of metastasis, and with the recent advances, it provides a shorter duration of treatment. Synopsis: In this video, we shall display the concept of plaque brachytherapy, the various types of plaques available, different radiations sources, planning dosimetry and calculations, target disease spectrum, surgical placement, and post-radiation outcomes in terms of local tumor control and prognosis. Highlights: This video highlights the history, basic principles and techniques of plaque brachytherapy and provides an understanding of its applications in the world of ocular oncology.

Lesiones cervicofaciales tumorales y pseudotumorales pediátricas en Villa Clara. Años 2010-2019

Caracterizar las lesiones cervicofaciales tumorales y pseudotumorales en niños en Villa Clara es una necesidad creciente por las alteraciones físicas, estéticas y psicológicas que pueden ocasionar. Se realizó un estudio transversal y descriptivo en el Servicio de Cirugía Maxilofacial Pediátrico de esta provincia, en el período 2010-2019. La población estuvo constituida por 101 niños con estudio histológico concluyente de lesión tumoral benigna, maligna o pseudotumoral de la región cervicofacial. Se concluyó que los tumores y pseudotumores en la región cervicofacial no tuvieron relación con la edad, género, ni color de la piel, en los niños estudiados. En esta serie predominaron los tumores benignos. El tumor maligno de mayor prevalencia fue el Linfoma de Burkitt. Existió alta correlación entre los diagnósticos clínico e histológico.

Characterizing tumoral and pseudotumoral cervicofacial lesions in children in Villa Clara is a growing need due to the physical, aesthetic and psychological alterations that they can cause. A cross-sectional and descriptive study was carried out in the pediatric maxillofacial surgery service of this province from 2010 to 2019. The population consisted of 101 children with conclusive histological study of benign and malignant tumoral or pseudotumoral lesions of the cervicofacial region. We concluded that tumors and pseudotumors in the cervicofacial region were not related to age, gender or skin color in the studied children. In this series, benign tumors predominated. The most prevalent malignant tumor was Burkitt's lymphoma. There was a high correlation between clinical and histological diagnoses.

Onychomatricoma on the fourth toenail: A rare tumor in a rare localization

Onychomatricoma is a rare benign tumor of the nail matrix, characterized by finger-like projections that invade the nail plate. The fingernails of Caucasian women are most commonly affected. Because this tumor can easily mimic other more prevalent ungual diseases, it is crucial to be acquainted with its characteristic clinical and histopathologic features. The authors present a case of a 40-year-old man with an onychomatrichoma in the fourth left toenail, which was initially misdiagnosed and treated as onychomycosis

Epidermoid cyst of the brain: A common cystic lesion in rare location

Epidermoid cysts are common benign tumors comprising around 1% and 2% of all intracranial tumors. Their usual locations include the parasellar region and cerebellopontine angle, and less commonly, the Sylvian fissure, suprasellar region, cerebral, and cerebellar hemispheres. Epidermoid cysts located in the brain stem are rare. These epidermoid cysts are similar to epidermoids arising in the skin which contain cheesy and flaky-white soft pultaceous material. Epidermoid cysts are very slow-growing tumors having a similar growth pattern of the epidermal cells of the skin and develop from the remnants of epidermal elements during the closure of the neural groove and disjunction of the surface ectoderm with neural ectoderm between the 3rd and 5th weeks of embryonic life. The ideal treatment of choice is the removal of cystic components with the complete resection of the capsule. We are presenting an interesting case of an epidermoid cyst in the frontal lobe in a 42-year-old male along with radiological investigations.

Ileum neuroendocrine tumor: Case report and literature review

Ileal neuroendocrine tumors are rare primary epithelial neoplasm arising from enterochromaffin cells. A 65-year-old male presented with complaints of recurrent abdominal pain of 1 year duration. The pain was more aggravated in last 7 days associated with vomiting, loss of appetite and diarrhea. On radioimaging ultrasonography finding impression of small bowel mass lesion suggestive of ileac carcinoid tumor with ischemic bowel disease was given. Computerized tomograph abdomen pelvis showed a welldefined hypodense, round, hetergenous, enhancing mass measuring 2.6x1.6x1.2 cm., showing arterial enhancement in mesentery in close relation to ileum and metastatic lymph node mass- indicating likeness of neuroendocrine tumor. The surgical resection of ileum with mass and enlarged mesenteric nodes were done. On gross examination ileum specimen on cut open showed multiple, irregular mucosal thickenings with foci of ulceration. The submocosa showed single, circumscribed, round tumor measuring 3 x 2.5x1.5 cm. Cut section of tumor was yellowish, tan, well circumscribed and solid. The mesenteric lymph nodes were enlarged. The microscopic examination ileum showed a tumor composed of uniform, round cells having to oval nuclei with salt and pepper chromatin. Tumor cells were arranged in trabecular, nests, ribbons and in areas pseudo glandular pattern. Increased mitotic activity was noted (4/10 high power field). Mesenteric lymph nodes were involved by tumor. On histopathology reported as Well-differentiated neuroendocrine tumors (NET), G2, intermediate grade of ileum. The immunohistochemical stain were positive for synaptophysin, chromogranin A. The Ki-67 expression was <1%. Herewith we are presenting rare case of well-differentiated neuroendocrine tumor NET, G2, intermediate grade of ileum for its clinical, radio imaging ,pathological features and management.

Analysis of Various Histopathological Patterns in Pleomorphic Adenoma - An Institutional Case Study

Introduction: Pleomorphic adenoma, the most common benign mixed salivary gland tumor which accounts for 70-80%, is a biphasic tumor with dual origin from epithelial and myoepithelial elements. It shows a malignant transformation rate of 6%. Microscopically, pleomorphic adenoma characteristically displays vast morphologic diversity. This present study is done to analyze prevalence rates and histomorphological variations to better elucidate the pattern of occurrence, diagnosis and treat- ment plan of pleomorphic adenoma. Materials and Methods: This are a retrospective study which was conducted in a private institution, Chennai. Based on the proportion of parenchymal and stromal tumor components, the cases were classified into four subtypes as proposed by Foote and Frazell. Morphological patterns, cellular patterns, capsular alterations and Stromal components were analyzed and statisti- cally evaluated. Results: Our analysis showed an overall 0.87% of pleomorphic adenoma cases being reported were associated with minor salivary glands followed buccal mucosa and palate. All the reported cases showed Type II pattern with histological cellular pat- tern of spindle cells and ductal morphological pattern. Conclusion: We observed the great diversity of morphological aspects of the stroma in pleomorphic adenoma, in which many cases showed variation in morphology and cellularity associated with tumor location.

Cementoblastoma asociado a primer molar permanente. Reporte de un caso / Cementoblastoma associated with a first permanent molar. A case report

Resumen Objetivo: El cementoblastoma es una neoplasia odontogénica benigna de origen mesenquimático relativamente poco frecuente que se asocia a las raíces de las piezas dentarias. Se considera que es la única neoplasia verdadera de origen cemental. El objetivo del presente trabajo es describir el caso clínico de un cementoblastoma de grandes dimensiones y realizar una revisión bibliográfica a fin de establecer pautas de diagnóstico y tratamiento, señalando sus diferenciales con patologías de similares características como la displasia cemento ósea y el fibroma cemento osificante. Caso clínico: Se describe el caso de un paciente pediátrico que presenta un aumento de volumen de considerables dimensiones asociado al primer molar permanente mandibular. Teniendo en cuenta las características clínicas y radiográficas, y con un diagnóstico presuntivo de cementoblastoma, se realizó la extirpación quirúrgica de la lesión junto con la pieza dentaria asociada, confirmándose el diagnóstico inicial por medio del estudio anatomopatológico.

Abstract Aim: Cementoblastoma is a relatively infrequent benign odontogenic neoplasia of mesenchymal origin that is associated with the roots of teeth. It is considered to be the only true cemental originated neoplasm. The objective of this article is to describe the clinical case of a large cementoblastoma and to carry out a bibliographical review to establish diagnostic and treatment guidelines, pointing out its differences with pathologies of similar characteristics, such as the cemento-osseous dysplasia and the cemento-ossifying fibroma. Clinical case: The case of a pediatric patient who presents a considerable increase in the volume associated with the mandibular first permanent molar is described. Considering the clinical and radiographic characteristics, and with a presumptive diagnosis of cementoblastoma, a surgical extraction of the lesion along with the associated tooth was performed, confirming the initial diagnosis by an anatomopathological study.

Seguridad y factibilidad de la reconstrucción microquirúrgica en tumores de cabeza y cuello en pacientes mayores de 70 años / Safety and feasibility of free-flap reconstruction in head and neck tumors in patients aged 70 years or older

RESUMEN Antecedentes: debido al aumento en la expectativa de vida, se ha incrementado la incidencia de tumores de cabeza y cuello en pacientes añosos. Objetivo: evaluar los resultados de la reconstrucción con colgajos microquirúrgicos luego de la resección radical (RRMC) de tumores de cabeza y cuello en pacientes de 70 años o mayores. Material y métodos: se analizó una serie de pacientes sometidos a RRCM por tumores de cabeza y cuello en el período 2000-2020. Se dividió la muestra en dos grupos: G1: ≥ de 70 años y G2: < de 70 años. Se analizaron variables demográficas, quirúrgicas, posoperatorias y factores de riesgo de trombosis del colgajo en los pacientes ≥ de 70 años. Resultados: se incluyó un total de 178 pacientes, 61 en G1 y 117 en G2. Ambos grupos fueron homogéneos respecto del sexo, IMC (índice de masa corporal), alcoholismo, tabaquismo, tratamiento neoadyuvante e incidencia de HPV (virus del papiloma humano). Hubo mayor cantidad de pacientes con riesgo ASA ≥ III en G1 vs. G2; (p: 0,005). En G1, 33 (54%) correspondieron a estadio oncológico ≥ III vs. 99 (87%) en G2 (p: 0,001). Cuarenta y dos (69%) pacientes en G1 recibieron adyuvancia vs. 94 (83%) en G2 (p: 0,02) y no hubo diferencias en la morbimortalidad global y en fallas del colgajo. El sexo femenino fue el único factor de riesgo de trombosis del pedículo vascular (p: 0,05). Conclusión: la RRCM para tumores de cabeza y cuello es factible y segura en pacientes añosos, con una incidencia de morbimortalidad similar a la del resto de la población.

ABSTRACT Background: The higher life expectancy has increased the incidence of head and neck tumors in elder patients. Objective: the aim of this study was to evaluate the outcomes of free flap reconstructions after radical resection (FFRR) of head and neck tumors in patients aged 70 years or older. Material and methods: We analyzed a series of patients undergoing FFR due to head and neck tumors between 2000-2020. The patients were divided into two groups: G1: ≥ 70 years, and G2: < 70 years. The demographic, operative and postoperative variables and the risk factors for flap thrombosis in patients ≥ 70 years were analyzed. Results: A total of 178 patients were included, 61 in G1 and 117 in G2. Both groups were homogeneous regarding sex, BMI (body mass index), alcohol consumption, smoking habits, neoadjuvant treatment, and incidence of HPV (human papillomavirus). The incidence of ASA grade ≥ III was significantly higher in G1 vs. G2; (p: 0,005). In G1, 33 patients (54%) corresponded to cancer stage ≥ III vs. 99 (87%) in G2 (p: 0.001). Forty-two (69%) patients in G1 received adjuvant therapy vs. 94 (83%) in G2 (p = 0.02) and there were no differences in overall morbidity and mortality and in flap failure. Female sex was the only predictor of vascular flap thrombosis (p = 0.05). Conclusion: FFRR in head and neck tumors is feasible and safe in elderly patients, with morbidity and mortality rates similar to those of the general population.

Hemorragia de intestino delgado en un paciente con Neurofibromatosis tipo 1

Presentamos el caso de un paciente masculino de 32 años con antecedente de Neurofibromatosis tipo 1, que se presenta por hemorragia de intestino delgado activo, diagnosticada inicialmente al observar sangrado en ileoscopía, al cursar con inestabilidad hemodinámica se realiza angiotomografía abdominal identificando a nivel de yeyuno medio una masa con captación de contraste y sangrado activo por lo cual se realiza una angiografía con embolización arterial de la rama que irriga dicha zona. Con el paciente estable, se realizó una enteroscopía anterógrada de doble balón, observando una lesión subepitelial, ulcerada, se realiza tatuaje endoscópico y finalmente se envía a cirugía para resección mediante laparoscopia. El estudio anatomopatológico fue compatible con un tumor estromal gastrointestinal (GIST) yeyunal.

We present the case of a 32-year-old male patient with a history of Neurofibromatosis type 1, who presented with active small bowel bleeding, initially diagnosed by observing bleeding in ileoscopy, presenting with hemodynamic instability, abdominal angiotomography was performed, identifying a mass with contrast enhancement and active bleeding at the middle jejunum level, for which an angiography with arterial embolization of the branch that supplies said area is performed. With the patient stable, a double-balloon antegrade enteroscopy was performed, observing a subepithelial, ulcerated lesion, endoscopic tattooing was performed and finally surgery was sent for resection by laparoscopy. The pathology study was compatible with a jejunal gastrointestinal stromal tumor (GIST).

Estrategia quirúrgica en un paciente con tumor agresivo del tracto gastrointestinal en cuarta porción de duodeno

Los tumores del estroma gastrointestinal son tumores infrecuentes y se corresponden con un 1 % de todas las neoplasias gastrointestinales; la localización duodenal solo representa entre 3-5 %. Se realizó este reporte de caso con el propósito de divulgar la estrategia quirúrgica seguida con un paciente portador de tumor invasivo del estroma gastrointestinal (>10 cm) de localización atípica, en cuarta porción del duodeno. El internamiento fue por tumor abdominal y anemia. El ejercicio clínico consistió en identificar una masa abdominal izquierda con contacto lumbar. Las pruebas diagnósticas realizadas fueron: pruebas de química sanguínea, ultrasonido abdominal, tomografía axial computadorizada y endoscopia digestiva con biopsia que confirmó el diagnóstico. El procedimiento quirúrgico fue resección de la cuarta porción de duodeno y primeras asas yeyunales, con restablecimiento de la funcionabilidad intestinal mediante duodeno (segunda porción)-yeyunostomía latero-lateral. La cirugía fue interrumpida por inestabilidad hemodinámica del paciente y cuatro días después fue llevado otra vez al salón de operaciones por presentar peritonitis, con salida de pus por los drenajes abdominales, que fue solucionada con lavado de la cavidad. La morbilidad estuvo acompañada por una fístula pancreática. En el tercer tiempo quirúrgico se realizó resección del tumor residual, nefrectomía izquierda y control de la fístula pancreática. Después de un año el paciente se encuentra libre de enfermedad tumoral. Se puede concluir que la estrategia de manejo en pacientes con tumores del estroma gastrointestinal de localización atípica representa un reto para el cirujano como miembro del grupo multidisciplinar y depende de la extensión del tumor, el estado del paciente y el manejo oportuno del equipo quirúrgico.

Gastrointestinal stromal tumors are rare tumors and correspond to 1% of all gastrointestinal neoplasms; duodenal location only represents between 3-5%. This case report was made for disclosing the surgical strategy followed in a patient with invasive gastrointestinal stromal tumors (>10 cm) of atypical location in the duodenum fourth portion. Hospitalization was due to abdominal tumor and anemia. The clinical exercise consisted of identifying a left abdominal mass with lumbar contact. The diagnostic tests performed were: blood chemistry tests, abdominal ultrasound, computerized axial tomography, and digestive endoscopy with biopsy that confirmed the diagnosis. The surgical procedure was resection of the duodenum fourth portion and the first jejunal loops, with restoration of intestinal function through duodenum (second portion) lateral jejunostomy. The surgery was interrupted due to the patient's hemodynamic instability, and four days later he was taken back to the operating room due to peritonitis, with pus coming out of the abdominal drains, which was resolved by washing the cavity. Morbidity was accompanied by a pancreatic fistula. In the third surgical time, resection of the residual tumor, left nephrectomy, and control of the pancreatic fistula were performed. After one year the patient is free of tumor disease. The management strategy in patients with atypically located gastrointestinal stromal tumors represents a challenge for the surgeon as a member of the multidisciplinary group and depends on the extent of the tumor, the patient's condition, and the surgical team timely management.

Tumores cardíacos benignos operados en un Centro de Cirugía Cardiovascular Regional 2015 - 2021

Introducción: Los tumores cardíacos primarios se caracterizan por su baja prevalencia, son principalmente mixomas y se presentan frecuentemente de forma asintomática. Objetivos: Identificar el tipo histológico más común, edad de presentación, tipo de cirugías y sobrevida de un grupo de pacientes tratados por Tumores Cardíacos Primarios (TCP) en el Hospital Regional de Temuco. Métodos: Revisión de fichas clínicas de 14 pacientes portadores de TCP entre marzo 2015 y diciembre 2021. Resultados: El tipo histológico más común fue el mixoma (85,7%), seguido por el fibroelastomas papilar (14,3%). La edad promedio fue 62 años (39-85), 9 fueron mujeres y 5 hombres. Los antecedentes mórbidos más comunes fueron: Insuficiencia Cardíaca Congestiva (ICC), Hipertensión Arterial (HTA) y Accidente Vascular Encefálico (AVE). La localización anatómica más común fue la Aurícula izquierda (92%). El tratamiento en el 92% de los casos fue resección aislada y en el 7% restante resección y reparación con parche. Conclusiones: Nuestros resultados son concordantes con la literatura.

Background: primary cardiac tumors are characterized by a low prevalence. Most of them are myxomas and asymptomatic. Aim: To describe the most common histological type, the age of presentation, type of surgery performed and survival of a group of patients operated on for Primary Cardiac Tumors (PCT) in the Hospital Regional de Temuco (Chile). Methods: Review of clinical records of 14 patients with PCT operated on between March 2015 and December 2021. Results: By far the most common histological type was a myxoma (85.7%), followed by a papillary fibroelastoma (14.3%). Mean age was 62 years (39-85), 9 were women and 5 men. The most common associated medical conditions were Congestive Heart Failure (CHF), Arterial Hypertension and Stroke The usual anatomical location was the left atrium (92%). Surgical treatment was isolated resection in 92% of cases and along with a patch repair in the remaining patient. Conclusion: good results were obtained, similar to those described in the literature.

Tumor fibroso solitario (TFS) parafaríngeo: reporte de caso y revisión de literatura

El espacio parafaríngeo es una región donde puede surgir un amplio espectro de tumores, que son poco frecuentes y comprenden solo el 0,5 al 1,5% de las neoplasias de cabeza y cuello. Generalmente, son hallazgos y suelen presentarse como masas asintomáticas. Incluye neoplasias primarias, extensión directa de regiones adyacentes y enfermedad metastásica. La literatura describe múltiples patologías e histologías que pueden surgir de esta zona, entre ellas, tenemos los tumores fibrosos solitarios, que son un espectro de neoplasias mesenquimatosas fibroblásticas de histogénesis incierta, que generalmente involucran la pleura. Son lesiones muy poco frecuentes y su localización parafaríngea es excepcional, siendo su primer reporte en la literatura en 1993. Se presenta el caso de un tumor fibroso solitario del espacio parafaríngeo evaluado en nuestro centro, con su enfrentamiento diagnóstico, resolución quirúrgica y seguimiento respectivo, con el objetivo de presentar esta patología poco frecuente a nuestros colegas y contribuir al conocimiento médico.

The parapharyngeal space is a region where a wide spectrum of tumors can arise, which are rare and comprise only 0.5% to 1.5% of head and neck neoplasms. They are generally findings and usually present as asymptomatic masses. Includes primary neoplasms, direct extension from adjacent regions, and metastatic disease. The literature describes multiple pathologies and histologies that can arise from this area, among them, we have solitary fibrous tumors, which are a spectrum of fibroblastic mesenchymal neoplasms of uncertain histogenesis, which are generally the pleura. It is a very rare lesion and its parapharyngeal location is exceptional, being its first report in the literature in 1993. We present the case of a solitary fibrous tumor of the parapharyngeal space evaluated in our center, with its diagnos- tic confrontation, surgical resolution and respective follow-up, with the aim of presenting this rare pathology to our colleagues and contributing to medical knowledge.

Angiosarcoma gastrointestinal multifocal, con compromiso extenso de estómago, intestino delgado y colon. Reporte de un caso / Multifocal gastrointestinal angiosarcoma, with extensive gastric, small intestine and colon involvement. A case report

Introducción. El angiosarcoma es una neoplasia vascular originada a nivel del endotelio, de baja frecuencia, conocido por su agresividad y crecimiento acelerado. Alcanza solo el 1 al 2 % del total de los sarcomas. La presentación gastrointestinal es infrecuente y su incidencia es poco conocida debido al escaso reporte de casos en la literatura. Suele identificarse en etapas avanzadas debido a la dificultad del diagnóstico histopatológico por sus características morfológicas, siendo necesario aplicar tinciones especiales o estudio inmunohistoquímico. Caso clínico. Por su interés y singularidad, presentamos el caso de un paciente masculino de 54 años, quien consultó con hemorragia digestiva profusa, anemización y requerimiento de terapia transfusional. Resultados. Durante el proceso diagnóstico, en la endoscopia encontraron diversas lesiones multifocales que se extendían por gran parte del tracto gastrointestinal. El estudio histopatológico mostró angiosarcoma gastrointestinal. Discusión. El angiosarcoma del tracto gastrointestinal es extremadamente infrecuente, de difícil diagnóstico y bajas posibilidades de manejo curativo, con opciones terapéuticas limitadas, lo que configura un mal pronóstico a corto plazo

Introduction. Angiosarcoma is a vascular neoplasm originating from endothelial cells, known for its aggressiveness, accelerated growth and reduced frequency. Reach only 1 to 2% of total sarcomas. Gastrointestinal presentation is extremely rare, the true incidence is poorly known, due to the limited reports of this entity in the literature. It is usually identified in advanced stages in view of the difficulty of the histopathological diagnosis, attributable to its morphological characteristics, being necessary to apply special stains or immunohistochemical study. Case report. Due to their interest and uniqueness, we present the case of a 54-year-old male patient, who presented with a profuse gastrointestinal bleeding, anemia, and requirement for transfusion therapy. Results. The endoscopy detected several multifocal lesions that extended most of the gastrointestinal tract. The histopathological study showed gastrointestinal angiosarcoma. Discussion. Angiosarcoma of the gastrointestinal tract is extremely uncommon, difficult to diagnose and has low possibilities of curative management, with limited therapeutic options, which configures a poor prognosis in the short term

Wide Excision of a Desmoid Tumor of the Anterior Abdominal Wall with Subsequent Reconstruction.

Desmoid tumors are benign tumors of mesenchymal origin with a complex course of the disease, due to absence of a capsule, infiltrative growth, heterogeneous structure and shape, the true extent of which is difficult to assess before surgery. Despite its benign nature, the recurrence rate reaches 25-50% of cases, and surgical treatment is often accompanied by a number of difficulties with a decrease in the quality of life of patients. In the presented clinical case, a wide excision of desmoid tumor was performed, followed by reconstruction, which ensured a good postoperative result while preserving oncological principles.

Low Expression of E-Cadherin and Cdh1 Variants Associated with Diffuse Gastric Cancer

ABSTRACT Background Reduced or null expression of E-cadherin protein is a frequent cause of diffuse gastric cancer (DGC). More than 50% of patients with DGC present somatic variants in CDH1 gene. Objectives The objectives of this study were to study E-cadherin expression and identify variants in the CDH1 gene in gastric tumors of patients with DGC. Methods We studied 18 Mexican DGC patients who attended a hospital of the Mexican Social Security Institute; E-cadherin expression was determined by immunohistochemistry, and variants were identified by Sanger sequencing in promoter and coding regions. Predictive analysis was performed using PolyPhen-2 and HOPE software. Results We found that 56% of DGC patients showed reduced expression of E-cadherin. All patients carried CDH1 variants; overall, 12 different CDH1 variants were identified. Predictive analysis revealed that the rs114265540 variant was probably damaging, with a value of 0.985, indicating a functional impact on the E-cadherin protein. Variants rs34939176 and rs33964119 were identified as risk factors for DGC (odds' ratios [OR] = 31.3, 95% CI 6.3-154.0, p < 0.001; OR = 6.1, 95% CI 2.0-19.0, p < 0.001, respectively) given their elevated frequency and by comparing it with those reported for MXL population in the 1000 Genomes Project database. Conclusions In this Mexican population, the percentage of diffuse gastric tumors with reduced expression of E-cadherin was similar to that reported in other populations. All gastric tumors of DGC patients studied had somatic CDH1 gene variants; however, the rs114265540, rs34939176, and rs33964119 variants were importantly related to DGC.

Outcomes of Extended Curettage with and without Bone Allograft for Grade II Giant Cell Tumors around the Knee. A Retrospective Comparative Study / Resultados da curetagem estendida com e sem aloenxerto ósseo para tumores de células gigantes de grau II no joelho. Um estudo comparativo retrospectivo

Abstract Objective This is the first study to establish the utility of extended curettage with or without bone allograft for Grade II giant cell tumors GCTs around the knee joint with the aim of exploring postoperative functional outcomes. Methods We retrospectively reviewed 25 cases of Campanacci grade II GCTs undergoing extended curettage between January 2014 and December 2019. The participants were divided into two groups: one group of 12 patients underwent extended curettage with bone allograft and bone cement, while the other group of 13 patients underwent extended curettage with bone cement only. Quality of life was assessed by the Revised Musculoskeletal Tumor Society Score and by the Knee score of the Knee Society; recurrence and complications were assessed for each cohort at the last follow-up. The Fisher test and two-sample t-tests were used to compare the categorical and continuous outcomes, respectively. Results The mean age was 28.09 (7.44) years old, with 10 (40%) males and 15 females (60%). The distal femur and the proximal tibia were involved in 13 (52%) and in 12 (48%) patients, respectively. There was no significant difference in the musculoskeletal tumor society score (25.75 versus 27.41; p= 0.178), in the knee society score (78.67 versus 81.46; p= 0.33), recurrence (0 versus 0%; p= 1), and complications (25 versus 7.69%; p= 0.21). Conclusions Extended curettage with or without bone allograft have similar functional outcomes for the knee without any major difference in the incidence of recurrence and of complications for Grade II GCTs. However, surgical convenience and cost-effectiveness might favor the bone cement only, while long-term osteoarthritis prevention needs to be investigated to favor bone allograft.

Resumo Objetivo Este é o primeiro estudo a estabelecer a utilidade da curetagem estendida com ou sem enxerto ósseo em tumores de células gigantes (TCGs) de grau II na articulação do joelho com o objetivo de explorar os resultados funcionais pós-operatórios. Métodos Revisamos retrospectivamente 25 casos de TCGs de grau II de Campanacci submetidos a curetagem estendida entre janeiro de 2014 e dezembro de 2019. Os participantes foram divididos em 2 grupos: um grupo de 12 pacientes foi submetido a curetagem estendida com aloenxerto ósseo e cimento ósseo, enquanto o outro grupo, com 13 pacientes, foi submetido a curetagem estendida apenas com cimento ósseo. A qualidade de vida foi avaliada pela Pontuação Revista da Musculoskeletal Tumor Society (MTS, na sigla em inglês) e pela Pontuação da Knee Society (KS, na sigla em inglês), enquanto as taxas de recidiva e complicações foram avaliadas em cada coorte na última consulta de acompanhamento. O teste de Fisher e os testes t de duas amostras foram usados para comparação de resultados categóricos e contínuos, respectivamente Resultados A média de idade dos pacientes foi de 28,09 (7,44) anos; 10 (40%) pacientes eram do sexo masculino e 15 (60%) pacientes eram do sexo feminino. O fêmur distal e a tíbia proximal foram acometidos em 13 (52%) e 12 (48%) dos pacientes, respectivamente. Não houve diferença significativa na pontuação revista da MTS (25,75 versus 27,41; p= 0,178), na pontuação da KS (78,67 versus 81,46; p= 0,33) e nas taxas de recidiva (0 versus 0%; p= 1) e complicações (25 versus 7,69%; p= 0,21). Conclusões A curetagem estendida com ou sem aloenxerto ósseo tem resultados funcionais semelhantes em pacientes com TCGs de grau II no joelho, sem qualquer diferença importante na incidência de recidivas e complicações. No entanto, a conveniência cirúrgica e o custo-benefício podem favorecer a utilização apenas de cimento ósseo, enquanto a prevenção da osteoartrite em longo prazo precisa ser investigada para favorecer o enxerto ósseo.