Pseudotumor inflamatorio hepático: caso clínico y revisión de la literatura / Hepatic Inflammatory Pseudotumor Mimicking Cholangiocarcinoma: A Rare Case and Literature Review

Presentamos el caso de un paciente de 49 años, de sexo masculino, que consulta en el servicio de urgencias por un cuadro de dos meses de evolución, caracterizado por compromiso del estado general, baja de peso, dolor abdominal, sensación febril y elevación de los parámetros inflamatorios. Al estudio imagenológico se observa una voluminosa lesión hepática, asociada a dilatación de la vía biliar y adenopatías en hilio hepático, espacio porto-cavo y retroperitoneales (inter-cavo-aórticos), que plantea dentro de los diagnósticos diferencias un colangiocarcinoma intrahepático. Basados en esta sospecha se realiza una segmentectomía y linfadenectomía regional. El estudio histopatológico e inmunohistoquímico de la pieza quirúrgica, evidencia un proceso inflamatorio linfoplasmocitario, con la presencia de células plasmáticas IgG4 positivas, compatible con una enfermedad asociada a IgG4. Posterior a la resección se decide manejo expectante, evolucionando el paciente de forma favorable, asintomático y sin signos de recurrencia. Presentamos un caso y una breve revisión de la literatura de un pseudotumor inflamatorio hepático, entidad poco frecuente y de comportamiento benigno.

We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.

INTRODUCTION: The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND: The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION: Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS: The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT: Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION: IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

[Hepatic Inflammatory Pseudotumor Mimicking Cholangiocarcinoma: A Rare Case and Literature Review]. / Pseudotumor inflamatorio hepático: caso clínico y revisión de la literatura.

We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.

Pseudotumor inflamatorio hepático, una entidad de difícil diagnóstico / Inflammatory pseudotumor of the liver: a condition hard to diagnose

RESUMEN El pseudotumor inflamatorio hepático es una lesión muy infrecuente, sin una etiología ni patogenia claras. Su diagnóstico preoperatorio no es habitual pero, en caso de realizarse, puede evitar la cirugía. Presentamos el caso de un paciente joven, con antecedente de lupus cutáneo que, tras debutar con una pancreatitis aguda, presenta episodios de colangitis y cuyos hallazgos radiológicos no permiten descartar la presencia de un colangiocarcinoma, por lo que se realiza hepatectomía izquierda, siendo el diagnóstico histológico final de pseudotumor inflamatorio hepático.

ABSTRACT Inflammatory pseudotumors of the liver are rare and lack clear etiology and pathogenesis. The preoperative diagnosis is seldom made but it avoids unnecessary surgery. We report the case of a young male patient with a history of cutaneous lupus and episodes cholangitis after an acute pancreatitis. As the imaging tests could not rule out cholangiocarcinoma, a left liver resection was performed, and the final histologic diagnosis was inflammatory pseudotumor of the liver.

Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

Tumor miofibroblástico inflamatorio del colon / Inflammatory Myofibroblastic Tumor of the Colon

RESUMEN Introducción: Los tumores miofibroblásticos son tumores benignos de origen mesenquimal, de etiología incierta, y frecuente en niños y jóvenes. Objetivo: Describir un nuevo caso de tumor miofibroblástico abdominal. Caso clínico: Se presenta una paciente de 20 años con antecedentes de salud con dolor en abdomen derecho, se realiza tomografía de abdomen contrastada que informa una masa hiperdensa no homogénea en flanco derecho. Se realiza resección quirúrgica del tumor y el estudio anatomopatológico informa un tumor miofibroblástico inflamatorio. Su pronóstico es favorable porque es una entidad benigna y no metastiza. Conclusiones: Los tumores miofibroblásticos de colon son infrecuentes, benignos, que al diagnosticarlos deben resecarse ampliamente para evitar la recidiva(AU)

ABSTRACT Introduction: Myofibroblastic tumors are benign tumors of mesenchymal origin, of uncertain etiology, and frequent in children and young people. Objective: To describe a new case of abdominal myofibroblastic tumor. Clinical case: The case is presented of a 20-year-old female patient with a health history and pain in the right abdomen. Contrasted abdominal tomography is performed, which shows an inhomogeneous hyperdense mass in the right flank. Surgical resection of the tumor was performed. The pathological study reported an inflammatory myofibroblastic tumor. Its prognosis is favorable because it is a benign entity that does not metastasize. Conclusions: Colon myofibroblastic tumors are infrequent, benign, and when diagnosed, they must be widely removed to avoid recurrence(AU)

Inflammatory myofibroblastic tumor of the liver in an elderly woman following a second liver biopsy: a case report.

Inflammatory myofibroblastic tumor (IMT) is a rare benign lesion of unknown etiology reported in numerous anatomic sites. Hepatic IMT is rare. It is composed of a dominant spindle cell proliferation with a variable inflammatory component that has unique histological appearance. The tumor is more common in women, who often present with fever of unknown origin or other vague, nonspecific symptoms. A 74-year-old female presented with fever of unknown origin. Abdominal CT-Scan showed focal mass effect with luminal narrowing at the distal sigmoid colon, highly suggestive of a sigmoid neoplasm as well as numerous hypodense lesions scattered throughout the liver, likely representing metastatic disease. Liver biopsy revealed an IMT. Patient was treated conservatively and remained without symptoms. It is extremely difficult to differentiate an IMT from neoplastic disease. Most cases require complete resection to obtain an accurate diagnosis. This entity should be considered in the differential diagnosis in patients with hepatic lesions to avoid unnecessary surgical procedures.

Vanek's polyp or inflammatory fibroid polyp. Case report and review of the literature.

BACKGROUND: Intussusception in an adult must make us suspect the presence of a tumor (benign or potentially dangerous) as the most frequent cause. Accurate diagnosis is of great importance in order to provide appropriate treatment and improve patient prognosis. CLINICAL CASE: We report the case of a 42-year-old male with abdominal pain. We performed a CT and found a small bowel intussusception. Definitive diagnosis according to the surgical specimen was inflammatory fibroid polyp (Vanek's polyp). CONCLUSIONS: Vanek's polyp is a benign lesion that occurs most frequently in the stomach and secondarily in the small bowel. Generally, it is uncommon, and its etiology is not completely known. Accurate diagnosis is done with immunohistochemistry. Because of the consequences that depend on the size and location of the lesion, it may be considered a malignant lesion. Treatment is resection.

Adult intussusception with perforation and secondary peritonitis. Case report.

BACKGROUND: Intussuception is an uncommon condition in adults. It is usually secondary to an organic lesion that may be malignant. The most common clinical presentation is as a partial bowel obstruction that requires surgical management. Preoperative diagnosis remains difficult; therefore, this paper presents a case report and a brief review of adult intussusception. CLINICAL CASE: We present the case of a 24-year-old female with a 36-h evolution of lower abdominal pain with nausea, vomiting and diarrhea and a previous episode 8 days earlier. Pneumoperitoneum was observed on chest x-ray and surgery was decided upon. Peritonitis due to ileoileal intussusception was found, caused by an inflammatory fibroid polyp with microperforations. Small bowel resection with end-to-end anastomosis was performed and the patient had an uneventful recovery. CONCLUSIONS: Adult intussusception is an infrequent condition with nonspecific symptoms such as pain, nausea and vomiting. With more frequent use of tomography in patients with abdominal pain, correct diagnosis can be achieved. Treatment requires resection of the involved bowel without attempted reduction.

Endoscopic ultrasound and fine needle aspiration in chronic pancreatitis: differential diagnosis between pseudotumoral masses and pancreatic cancer.

CONTEXT: Pseudotumoral chronic pancreatitis can be difficult to differentiate from pancreatic carcinoma. OBJECTIVE: To evaluate the role of endoscopic ultrasound and fine needle aspiration in differentiating between inflammatory masses and malignancies in chronic pancreatitis. DESIGN: Retrospective study. SETTING: Tertiary care endoscopy unit. PATIENTS AND INTERVENTIONS: Between February 1997 and December 2006, 69 pancreatic head masses from patients with alcoholic chronic pancreatitis underwent EUS-FNA using a linear echoendoscope and 22-gauge needles. Final diagnoses were obtained from surgery or clinical follow-up. The patients were subdivided into two groups: pseudotumoral chronic pancreatitis and pancreatic cancer. RESULTS: Pseudotumoral masses and adenocarcinoma were found in 58 and 11 patients, respectively. The size of the lesions and the clinical presentation were similar in both groups, but the cancer patients were older than the patients with pseudotumoral masses (P=0.020). Fourteen of the 58 (24.1%) pseudotumoral masses were misdiagnosed as cancers, and 4 of the 11 (36.4%) cancers were erroneously diagnosed as pseudotumoral masses when evaluated by EUS alone. EUS-FNA confirmed the final diagnosis in 66 of the 69 (95.7%) cases. Cytopathology correctly classified 8 of the 11 (72.7%) malignancies and all benign cases. Three of the 11 (27.3%) cancers were misdiagnosed as pseudotumoral masses, and no pseudotumoral mass was diagnosed as a cancer. In two cases, the specimens were inadequate for cytopathological assessment. The sensitivity, specificity, positive and negative predictive values, and the diagnostic accuracy of EUS-FNA were 72.7%, 100%, 100%, 95.1% and 95.7%, respectively. CONCLUSIONS: The diagnostic accuracy of endoscopic ultrasound alone for differentiating between pseudotumoral masses and pancreatic cancer arising from chronic pancreatitis is unsatisfactory. Fine needle aspiration of these tumors significantly improves diagnostic capability.

[Myofibroblast tumor]. / Tumor miofibroblástico inflamatorio de colón. Análisis clínico, radiológico y morfológico.

OBJECTIVE: Our objective was to present a case of inflammatory myofibroblastic tumor of the colon. BACKGROUND: Inflammatory myofibroblastic tumors are uncommon. They originate from soft tissues, their appearance is more frequent in childhood and your adulthood and these tumors are composed of myofibroblastic cells, leukocytes, and plasmatic cells. More frequently, these tumors originate in lungs, mesentery, liver and spleen; intestinal appearance is uncommon. METHOD: In a 42 year-old male with a lump in right iliac fossa, a colonic inflammatory myofibroblastic tumor infiltrating ileocecal valve was found. Right hemicolectomy was performed. After a 10-month follow-up, no complications were found and no adjuvant therapy was needed. CONCLUSIONS: Inflammatory myofibroblastic tumors have been well described, but, experience is quite limited; Symptoms are consequences of compressed organs in the vicinity. At present, no early detection devices are available, but genetical evaluations for the patient's family members may contribute to unravel a pattern of transmission of the disease.

Pseudotumor inflamatorio esplénico en un paciente pediátrico / Inflammatory pseudotumor of the spleen: report of a case in a child

Se describe un caso de pseudotumor inflamatorio esplénico en un niño de 4 años. Los hallazgos clínicos fueron limitados a: dolor abdominal difuso y tumoración palpable en hipocondrio izquierdo. La ecografía y la TC mostraron una masa sólida homogénea en el polo inferior del bazo, con captación irregular del contraste endovenoso. Se realizó esplenectomía laparoscópica con diagnóstico histopatológico de pseudotumor inflamatorio. Esta localización es extremadamente rara, especialmente en niños, siendo este el cuarto caso reportado en pacientes pediátricos y el de menor edad (AU)

Tumores primitivos de pulmón en pediatría / Primary pulmonary neoplasms in children

Los tumores primitivos pulmonares, endobronquiales y parenquimatosos, son extremadamente raros en niños, por lo que la experiencia individual en el diagnóstico, tratamiento y pronóstico es limitada. Nuestro trabajo muestra las formas de presentación, métodos de diagnóstico empleados y resultados en 17 pacientes que se atendieron en nuestro hospital entre los años 1991 y 2000. De los métodos de imágenes, la radiología permitió evaluar la sospecha de masa pulmonar, el US demostró su naturaleza sólida, quística o mixta y la presencia o no de derrame pleural, mientras que la TC delimitó su localización, extensión y metástasis, datos imprescindibles para el tratamiento quirúrgico adecuado. En los pacientes pediátricos que tienen lesión pulmonar ocupante de espacio, así como en los que debutan con neumotórax o hemotórax espontáneos, debe tenerse en cuenta la posibilidad de tumor primitivo de pulmón, debiéndose profundizar los estudios mediante US, TC y endoscopía. Las lesiones congénitas quísticas tienen riesgo de malignización, por lo que su tratamiento debe ser quirúrgico (AU)