Surgical Management of a Giant Inflammatory Hepatocellular Adenoma in a Young Female.

Hepatocellular adenomas are rare and benign primary neoplasms of phenotypically mature hepatocytes. Our understanding of this pathology has greatly improved due to advances in molecular and anatomic knowledge. This article provides an in-depth review of hepatic adenomas (HCA) while presenting the case of a 20-year-old patient with a giant inflammatory hepatocellular adenoma with an atypical presentation, in whom surgical intervention was performed via right hepatectomy. In the post-surgical course, the patient had an in-hospital stay of three days with no complications. During outpatient monitoring via laboratory tests and imaging at eight months, the patient did not present any trace of recurrence.

A Giant Malignant Solitary Fibrous Tumor of the Pancreas: A Case Report and Review of the Literature.

We present a case report of a giant solitary fibrous tumor (SFT) with a review of the literature and discuss its biological features and diagnosis. A 43-year-old man presented to our emergency department with abdominal pain and distension with an evolution of two days. Contrast-enhanced computed tomography (CT) showed a large, well-circumscribed semisolid mass (12 cm x 10 cm x 12 cm) localized in the pancreatic head. The histological diagnosis obtained by endoscopic ultrasound-guided trans-duodenal tumor biopsy with fine-needle aspiration showed proliferating short spindle-shaped cells, suggesting a mesenchymal neoplasia of low grade. We proceeded to a Whipple surgical technique. The histopathological study of the resected tumor confirmed proliferating spindle-shaped cells in the tissue, and one mitotic figure was observed in 10 high-power fields (HPFs). Immunostaining was positive for CD34 and STAT-6. The histological diagnosis was a malignant pancreatic SFT. In the six months posterior to the surgical procedure, the patient has been free of recurrent disease. Preoperative diagnosis is difficult and requires comprehensive evidence including clinical, immunohistochemistry, and histological features. Since there are currently no recognized best practices, we advise total surgical excision and careful clinical monitoring.

Desmoid tumors: diagnostic and therapeutic considerations.

A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies' evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.

Se realizó una revisión bibliográfica de los tumores desmoides, lo cuales afectan los tejidos blandos con un comportamiento localmente agresivo sin capacidad de producir metástasis. Los casos esporádicos se localizan en extremidades y pared torácica; los casos hereditarios tienen predilección intraabdominal y los asociados con el embarazo en la pared abdominal. Las técnicas de imagen evalúan la extensión de la enfermedad. La biopsia con aguja trucut es el estudio de elección para el diagnóstico. Las mutaciones en el gen CTNNB1 o en el gen de APC provocan acumulación anormal de betacatenina en la célula. En esta revisión se hace énfasis en la evolución y cambio de las estrategias terapéuticas y se analizan las actuales herramientas para la toma de decisiones, así como los resultados clínicos. La radioterapia puede tener un papel terapéutico o adyuvante. Los avances en la comprensión de la enfermedad han permitido establecer tratamientos mejor dirigidos y con menor morbilidad; sin embargo, aún existen interrogantes en cuanto a la elección del candidato ideal para la vigilancia o el tratamiento precoz. También se presentan datos relacionados con la calidad de vida y la incertidumbre que genera el diagnóstico en el médico y el paciente.

Tumores desmoides: consideraciones diagnóstico-terapéuticas / Desmoid tumors: diagnostic and therapeutic considerations

Resumen Se realizó una revisión bibliográfica de los tumores desmoides, lo cuales afectan los tejidos blandos con un comportamiento localmente agresivo sin capacidad de producir metástasis. Los casos esporádicos se localizan en extremidades y pared torácica; los casos hereditarios tienen predilección intraabdominal y los asociados con el embarazo en la pared abdominal. Las técnicas de imagen evalúan la extensión de la enfermedad. La biopsia con aguja trucut es el estudio de elección para el diagnóstico. Las mutaciones en el gen CTNNB1 o en el gen de APC provocan acumulación anormal de betacatenina en la célula. En esta revisión se hace énfasis en la evolución y cambio de las estrategias terapéuticas y se analizan las actuales herramientas para la toma de decisiones, así como los resultados clínicos. La radioterapia puede tener un papel terapéutico o adyuvante. Los avances en la comprensión de la enfermedad han permitido establecer tratamientos mejor dirigidos y con menor morbilidad; sin embargo, aún existen interrogantes en cuanto a la elección del candidato ideal para la vigilancia o el tratamiento precoz. También se presentan datos relacionados con la calidad de vida y la incertidumbre que genera el diagnóstico en el médico y el paciente.

Abstract A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies’ evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.

A Hormonally Active Malignant Struma Ovarii.

Struma ovarii is a rare monodermal variant of ovarian teratoma that contains at least 50% thyroid tissue. Less than 8% of struma ovarii cases present with clinical and biochemical evidence of thyrotoxicosis due to ectopic production of thyroid hormone and only 5% undergo malignant transformation into a papillary thyroid carcinoma. Only isolated cases of hormonally active papillary thyroid carcinoma developing within a struma ovarii have been reported in the literature. We report the case of a 36-year-old woman who presented with clinical signs and symptoms of hyperthyroidism as well as a left adnexal mass, which proved to be a thyroid hormone-producing, malignant struma ovarii.

Neuroblastoma in the adult. Case report.

BACKGROUND: Neuroblastoma is a common malignancy in infancy but extremely rare in adults. These tumors, commonly found in the abdomen, originate in the sympathetic nervous system. Staging and management are standardized in children and adults, although their prognosis is very different, being more aggressive and with a poorer outcome in the adult. CLINICAL CASE: We present the case of a 31-year-old male with non-specific abdominal pain and constipation. After several studies, a stage III giant retroperitoneal neuroblastoma was diagnosed. We discuss here the evaluation, management and follow-up of the patient. A literature review is presented as well. CONCLUSIONS: Adult neuroblastoma is an unusual disease with an insidious presentation and is usually diagnosed in advanced stages. Unlike its behavior in young patients, in the adult it is more aggressive and with a poor prognosis.

Neuroblastoma en el adulto: informe de un caso / Neuroblastoma in the adult: case report

Introducción: El neuroblastoma es una neoplasia común en la infancia, pero extremadamente rara en el adulto. Se origina del sistema nervioso simpático y su localización más común es abdominal. Su estadificación y tratamiento se han estandarizado en niños y adultos, aunque el pronóstico es muy distinto debido a un comportamiento más agresivo y menor sobrevida en los segundos. Caso clínico: Hombre de 31 años de edad evaluado por dolor abdominal inespecífico y constipación, a quien se le diagnosticó gran neuroblastoma retroperitoneal estadio III. Al no tolerar quimioterapia se realizó cirugía. Se presenta la evaluación, manejo y seguimiento, así como una revisión de la literatura. Conclusiones: El neuroblastoma en el adulto es una enfermedad poco común que cursa con una evolución inicial insidiosa y la presentación suele ser en estadios avanzados. A diferencia del comportamiento en la infancia, en el adulto es más agresivo y con menor sobrevida a pesar de realizar el mismo tratamiento.

BACKGROUND: Neuroblastoma is a common malignancy in infancy but extremely rare in adults. These tumors, commonly found in the abdomen, originate in the sympathetic nervous system. Staging and management are standardized in children and adults, although their prognosis is very different, being more aggressive and with a poorer outcome in the adult. CLINICAL CASE: We present the case of a 31-year-old male with non-specific abdominal pain and constipation. After several studies, a stage III giant retroperitoneal neuroblastoma was diagnosed. We discuss here the evaluation, management and follow-up of the patient. A literature review is presented as well. CONCLUSIONS: Adult neuroblastoma is an unusual disease with an insidious presentation and is usually diagnosed in advanced stages. Unlike its behavior in young patients, in the adult it is more aggressive and with a poor prognosis.

Primary adamantinoma of the rib. Unusual presentation for a bone neoplasm of uncertain origin.

Adamantinomas are rare, low-grade malignant intra-osseous tumors composed of epithelial and mesenchymal elements, which show a marked predilection for the tibia and fibula of young adult male patients. Although cases of adamantinoma located to the axial skeleton have been reported either as recurrent or metastatic disease, only two cases of primary adamantinoma located to the thoracic wall have been previously described. In this study we present the clinical, radiological and histopathological features of a 24-year-old male with a slow growing, solid-cystic, painful mass, located to the right 11th rib, which was morphological and immunohistochemically diagnosed as a primary classic adamantinoma. Radiological studies showed a multiloculated lesion with a solid component. The patient underwent a whole surgical resection of the lesion. Histologically, multiple foci of epithelial cells with basaloid and squamous components were found intermixed within a fibrous stromal tissue. Immunohistochemical analysis demonstrated expression of cytokeratins, EMA, vimentin and other epithelial markers. Primary affection of the rib is an unusual feature of classic adamantinomas.

[Endocrine tumors of the pancreas: experience in the ABC Medical Center]. / Tumores endocrinos del páncreas: experiencia en el Centro Médico ABC.

OBJECTIVES: To analyze presentation, diagnosis and treatment of islet cell tumors at the ABC Medical Center. MATERIALS AND METHODS: Medical records of the 7 patients with endocrine tumors diagnosed between 1995 and 2005 were reviewed and analyzed, with emphasis to clinical, biochemical and radiological characteristics, surgical treatment and outcome. RESULTS: There were 3 insulinomas, 1 gastrinoma, 1 VIPoma, and 2 non-functioning tumors. All insulinomas presented the Whipple's triad. The tumor was localized before surgery in 2 cases. In all patients intraoperative ultrasound confirmed the tumor and enucleation was performed in all three. The patient with gastrinoma was diagnosed by endoscopy in the presence of metastatic disease, therefore no surgical treatment was performed. The patient with VIPoma, presented the typical secretory diarrhea. A tumor in the pancreatic head was found and it was resected by pancreaticoduodenectomy. Histology revealed a malignant lesion. Both non functioning tumors were found by imaging studies, one benign tumor was treated by central pancreatectomy and the other was malignant and underwent distal en-block pancreatectomy. Immunohistochemistry was positive for VIP in the benign lesion. Two of the 3 malignant tumors have died and one is alive with recurrent disease. CONCLUSIONS: Distribution of islet cell tumors in our series followed the usual patterns. In all functioning lesions hormonal production was identified before surgery. Imaging studies localized the tumor in 7 of the 8 patients. Surgical resection cured all benign tumors.

Expresión difusa de EMA y Glut-1, en tres casos de neurofibromas. Evidencia inmunohistoquímica de diferenciación perineural extensa / Diffuse expresion of EMA and Glut-1 in three cases of neurofibromas. Immunohistochemical evidence of extensive perineurial differentiation

Antecedentes: Los neurofibromas son tumores originadosde la vaina del nervio periférico formados por diversos componentescelulares endoneruales. La presencia de células perineurales,dentro de la proliferación del neurofibroma, es generalmenteescasa. Métodos: De 15 neurofibromas encontrados enun periodo de cuatro años (1999-2003) en los archivos deldepartamento de patología del Hospital ABC, de la ciudad deMéxico, tres casos presentaron extensa diferenciación perineuralevidenciada por medio de la inmunomarcación con EMA,GLUT-1. Resultados: En los tres neurofibromas hubo numerosascélulas perineurales EMA y GLUT1 positivas, distribuidasen forma difusa, que representaron aproximadamente 40% de lapoblación celular del tumor. Los pacientes fueron dos mujeres(20 años de edad con tumor subcutáneo en región sacra; y de 58años de edad con lesión subungueal en dedo pulgar derecho) yun hombre (de 61 años de edad con tumor mesentérico). Lascélulas perineurales eran delgadas con procesos citoplásmicosbipolares eosinófilos y formando fascículos laminares distribuidasirregularmente en todo el tumor, siendo particularmenteprominentes en zonas con estroma mixoide. Conclusión: Informamos3 casos de neurofibromas (NF) con numerosas célulasfusiformes positivas por imunohistoquímica para el antígeno demembrana epitelial (EMA) y a la proteína transportadora deglucosa 1 (GLUT-1), lo que indica la presencia de células perineuralesdentro de la lesión. Esta morfología de células perineuralesen NF es difícil de observar con H&E por lo que el usode inmunomarcación con EMA y GLUT-1 es necesario paracomprobar la presencia de células perineurales en los neurofibromas.La diferenciación perineural extensa, aparentemente notiene implicación pronostica alguna

Introduction: Neurofibromas belong to the group ofneoplasms arising from nerve sheath and are composed of amixture of endoneurial components. Usually, they containonly few perineurial cells within its cellular proliferation.Patients and Methods: Fifteen (15) neurofibromas wereretrieved from the department of pathology files (ABC hospitalin Mexico City), during a 5 years period (1999-2003).Three cases presented an extensive perineurial differentiationthat was made evident by immunostaining with EMAand Glut-1. Results: In the three selected cases, EMA andGlut-1 positive perineurial cells represented a significantcellularity percentage (approximately 40%). Two patientswere females (age 20, with a subcutaneous tumor in thesacral region; and age 58, with a subungueal tumor in theright thumb) and one was male (age 61, with a mesenterictumor). Perineurial cells appear as spindle- shaped withbipolar cytoplasmic processes irregularly distributedthroughout, but especially at the periphery of the lesion andin areas with myxoid stroma. Conclusions: Three neurofibromascontaining numerous EMA and Glut-1-positive,slender, spindle-shaped, perineurial cells are reported. Perineurialcells are difficult to identify by H&E. EMA andGlut-1 immunostainings are imperative to prove the amountof perineurial differentiation that seems to have no prognosticimplications

Tumor fibroso solitario de la pleura: informe de un caso / Fibrous solitary tumor of the pleura. Report of a case

Se informa el caso de un hombre de 72 años con un tumor fibroso solitario que ocurrió como un nódulo pulmonar único adyacente a la arteria pulmonar izquierda. Este tumor midió 1.5 cm de diámetro y mostró superficie lisa, café rosada y consistencia firme. Microscópicamente la neoplasia estaba bien delimitada y compuesta por células fusiformes con núcleo oval y cromatina fina, Separadas por colágena, sin mitosis ni pleomorfismo. Desde el punto de vista inmunohistoquímico, fue intensamente positivo para CD34 (QBend/10) y vitamina, y focalmente positivo para actina. La proteína s-100 y la queratina (CKAE1-3) resultaron unifromemente negativas. La inmunohistoquímica es útil para hacer diagnóstico diferencial con mesotelioma sarcomatoide. El tumor fibroso solitario es positivo para CD34 y negativo para queratina, mientras que el mesotelioma sarcomatoide es positivo para queratina y negativo para CD34. Es importante tratar de predecir la conducta del tumor. Se clasifica como maligno si mide más de 10 cm, tiene arriba de 10 mitosis por 10 campos de gran aumento (40x), es hipercelular o muestra zonas de necrosis. Se ha encontrado que la accesibilidad del tumor para su resección total y la existencia de un pedículo neoplásico son los más importantes criterios de benignidad. Se ha utilizado el análisis de ploidía (CAS-200) y el Ki-67 (marcador de proliferación celular) con la intención de separar los casos de conducta agresiva de los de conducta no agresiva

Plombage: a surgical procedure not to be forgotten yet

Un paciente de 82 años fue enviado a nustro hospital con el diagnóstico de neoplasia maligna pulmonar o pleural, debido a la presencia de adenopatía supraclavicular y gran opacidad en un hemitórax. Al interrogarlo, mencionó haber sido operado 42 años antes de plombage con aceite, como tratamiento de tuberculosis pulmonar, sin quimioterapia específica; permaneció asintomático durante 38 años, hasta que presentó un primer episodio de adenopatía supraclavicular, que cedió con hidracidad del ácido isonicotínico oral durante dos meses. En esta ocasión, la tomografía axial computarizada y la resonancia magnética nuclear mostraron que parte del aceite había escapado del interior de tórax hacia el cuello, entre las costillas; se trató con régimen de tres drogas antituberculosas y permanece en condiciones satisfactorias 12 meses después. Una buena historia clínica y el conocimiento de la historia de la cirugía torácica pueden prevenir errores diagnósticos como el que comunicamos