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Inflammatory myofibroblastic tumor is an extremely rare neoplastic lesion with a predilection for aggressive local and recurrent behavior. The tumor tends to occur in the lungs of children and young adults, and although it can develop in older patients and other organs, this is extremely rare. Symptoms are nonspecific and depend on the location and size of the tumor. The gastrointestinal tract is rarely this mass's primary site of origin, and the cecum is an even rarer location. We present the case of an otherwise healthy 55-year-old female who presented with an acute abdomen and a mass in her abdomen; after successful surgery, she fully recovered. Inflammatory myofibroblastic tumor causing acute abdomen was the final diagnosis.
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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.
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Granuloma de Células Plasmáticas , Neoplasias Pulmonares , Criança , Humanos , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/genética , Sistema Nervoso Central/patologia , Receptores Proteína Tirosina Quinases , Pulmão/patologia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/terapia , Neoplasias Pulmonares/patologia , ConvulsõesRESUMO
Primary gastric inflammatory myofibroblastic tumor is a rare neoplasm developed from mesenchymal stem cells, infrequently discussed in the scientific literature. Clinical diagnosis through endoscopy and pathology is challenging for the medical team. We report the case of a female patient with gastric obstruction syndrome due to a 10 cm tumor diagnosed with this disease by histology and immunohistochemistry.El tumor miofibroblástico inflamatorio primario gástrico es una neoplasia rara desarrollada de células madre mesenquimales, e infrecuentemente discutido en la literatura científica. El diagnóstico clínico a través de endoscopia y patología es desafiante para el equipo. Nosotros reportamos el caso de una paciente mujer con síndrome de obstrucción gástrica por un tumor de 10 cm diagnosticado con esta enfermedad usando histología e inmunohistoquímica.
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Neoplasias Gástricas , Humanos , Adulto , Feminino , Peru , Neoplasias Gástricas/diagnóstico , Estudos RetrospectivosRESUMO
El tumor miofibroblástico inflamatorio primario gástrico es una neoplasia rara desarrollada de células madre mesenquimales, e infrecuentemente discutido en la literatura científica. El diagnóstico clínico a través de endoscopia y patología es desafiante para el equipo. Nosotros reportamos el caso de una paciente mujer con síndrome de obstrucción gástrica por un tumor de 10 cm diagnosticado con esta enfermedad usando histología e inmunohistoquímica.
Primary gastric inflammatory myofibroblastic tumor is a rare neoplasm developed from mesenchymal stem cells, infrequently discussed in the scientific literature. Clinical diagnosis through endoscopy and pathology is challenging for the medical team. We report the case of a female patient with gastric obstruction syndrome due to a 10 cm tumor diagnosed with this disease by histology and immunohistochemistry.
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INTRODUCTION: Inflammatory myofibroblastic tumors are neoplasms that occur infrequently, mainly affects children and young adults. It is an intermediate grade fibrotic multinodular neoplasm. DESCRIPTION OF THE CASE: We present the case of a 47-year-old female patient, who underwent emergency umbilical hernioplasty, later developed intestinal obstruction secondary to an inflammatory myofibroblastic tumor. DISCUSSION: In 1939 Brunn described it for the first time, later in 1954 Umiker named it "Inflammatory Myofibroblastic Tumor". The symptoms are nonspecific. In 15 to 40% of patients they are asymptomatic. Cells positive for actin, smooth muscle, vimentin and desmin, in 3367% of cases the cells are positive for ALK, which is present in some malignant lesions. The recommended treatment is radical resection. CONCLUSION: The diagnosis is established by histopathological study, surgery is the cornerstone of treatment.
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Inflammatory myofibroblastic tumor (IMT) is a lesion of intermediate biological potential with local recurrences and rare metastases found in multiple anatomical locations. We present a case of a pure intraarticular IMT of the knee, a location that has not been previously documented, with genetic confirmation of ALK-CARS fusion detected with next-generation sequencing. A 20-year-old healthy male was admitted to the orthopedic oncology department due to several months of pain and restriction in movement of his left knee. On magnetic resonance imaging, multiple intraarticular nodular lesions were seen. The patient underwent 2 synovectomies within the course of 1 year. The initial biopsy was interpreted as nodular fasciitis. The second biopsy revealed exuberant tissue displaying compact fascicles of spindle cells intermixed with myxoid areas in a background of inflammatory cells, highly suggestive for IMT. Due to the unusual intraarticular location, equivocal ALK immunostaining and the differential diagnosis with nodular fasciitis, we performed targeted next-generation sequencing using Archer FusionPlex Sarcoma panel, which can identify multiple fusions in a single assay. An ALK-CARS fusion was found, supporting the diagnosis of IMT. This report emphasizes the added value of broad molecular analysis in cases with unusual clinical presentation, equivocal immunohistochemistry, and a wide differential diagnosis.
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Articulação do Joelho/patologia , Proteínas de Fusão Oncogênica/genética , Neoplasias de Tecidos Moles/diagnóstico , Membrana Sinovial/patologia , Aminoacil-tRNA Sintetases/genética , Quinase do Linfoma Anaplásico/genética , Biópsia , Procedimentos Cirúrgicos de Citorredução , Diagnóstico Diferencial , Fasciite/diagnóstico , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/imunologia , Articulação do Joelho/cirurgia , Imageamento por Ressonância Magnética , Masculino , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/imunologia , Neoplasias de Tecidos Moles/cirurgia , Sinovectomia , Membrana Sinovial/diagnóstico por imagem , Membrana Sinovial/imunologia , Adulto JovemRESUMO
Inflammatory myofibroblastic tumor (IMT) is a very rare lesion of unknown etiology. Cases of IMT involving the appendix are exceptional, and they can mimic malignant appendicular tumors. We present a case of a 65-year-old man who presented to our emergency room on septic shock and acute abdomen secondary to visceral perforation. The patient underwent exploratory laparotomy; massive bowel dilatation was encountered, along with 3 L of purulent intraperitoneal fluid and a perforated appendicular mass of 6 cm. An appendicectomy was performed. Histopathologic examination established the diagnosis of inflammatory pseudotumor with appendiceal perforation. This study constitutes the 14th confirmed case report of an appendicular IMT. It is important to include IMT in differential diagnoses of appendicular masses to avoid excessive resections.
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INTRODUCTION: Calcifying fibrous pseudotumor is a rare benign lesion with few peritoneal and mesenteric cases in pediatric population described. Its course is mainly asymptomatic, which is why diagnosis corresponds mostly to incidental findings. CLINICAL CASE: Authors present the case of a 9-year-old patient with abdominal pain, and intra-abdominal mass finding in abdominal tomography. The histopathological study realized established diagnosis of calcifying fibrous pseudotumor, which is why programmed resection of the mass was performed by laparotomy. Follow-up was performed during one year, without evidence of recurrences through ecography. COMMENTS: Authors discuss the diagnostic and therapeutic approach in this patient compared to that described in the literature.
INTRODUCCION: El pseudotumor fibroso calcificado es una lesión benigna, con pocos casos de localización peritoneal y mesentérica descritos en la población pediátrica. Su curso es principalmente asintomático, por lo cual el diagnóstico corresponde en su mayoría a hallazgos incidentales. CASO CLINICO: Se presenta el caso de una paciente de 9 años con dolor abdominal y hallazgo de masa entra-abdominal en tomografía de abdomen. El estudio histopatológico realizado estableció el diagnóstico de pseudotumor fibroso calcificado, por lo que se llevó a resección programada de la masa mediante laparotomía. El seguimiento de la paciente fue durante un año, sin evidencia de recurrencias en ecografía. COMENTARIOS: Se discute la aproximación diagnóstica y terapéutica en esta paciente comparado con lo descrito en la literatura.
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Laparotomia/métodos , Mesentério/patologia , Doenças Peritoneais/diagnóstico , Dor Abdominal/etiologia , Calcinose/patologia , Criança , Feminino , Seguimentos , Humanos , Doenças Peritoneais/patologia , Doenças Peritoneais/cirurgia , Tomografia Computadorizada por Raios X/métodosRESUMO
INTRODUCCIÓN: El tumor miofibroblástico inflamatorio (TMI) es una neoplasia benigna infrecuente, de comportamiento clínico impredecible. OBJETIVOS: describir 3 casos de TMI diagnosticados entre marzo 2014 y enero 2018 en Hospital Clinico San Borja Arriaran, y realizar una revisión actualizada de la literatura. CASO 1: Adolescente de género masculino de 14 años de edad, hospitalizado por dolor abdominal, diagnosticado de invaginación yeyunoyeyunal secundaria a un tumor de pared intestinal. La histología fue compatible con un tumor miofibroblástico inflamatorio. CASO 2: Adolescente de género femenino, edad 12 años, hospitalizada por neumonía y dolor lumbar en estudio asociado a pérdida de peso. Se diagnosticó una masa retroperitoneal que comprometía el músculo psoas derecho, músculos paravertebrales, vértebras, riñón derecho y diafragma ipsilateral. Se efectuó biopsia por punción cuyo resultado fue compatible con un tumor miofibroblástico inflamatorio. CASO 3: Preadolescente de género femenino de 11 años de edad, hospitalizada para estudio de infección del tracto urinario a repetición. Se identificó un tumor vesical y la biopsia mostró ser compatible con tumor miofibroblástico inflamatorio. CONCLUSIÓN: Debido al comportamiento variable del tumor miofibroblástico inflamatorio, el manejo de este dependerá de la localización, la expresión del anaplasic like lymphoma (ALK), el comportamiento del tumor y la posibilidad de resección.
INTRODUCTION: The inflammatory myofibroblastic tumor is an infrequent benign neoplasm with unpredictable cli nical behavior. OBJECTIVES: to describe three clinical cases at the San Borja Arriarán Clinical Hospital between March 2014 and January 2018 and to carry out an updated review of the literature. CASE 1: 14-year-old male adolescent, hospitalized due to abdominal pain, diagnosed with jejunojejunal intus susception secondary to an intestinal wall tumor. The histology was compatible with an inflamma tory myofibroblastic tumor. CASE 2: 12-year-old female adolescent, hospitalized due to pneumonia and low-back pain under study associated with weight loss. A retroperitoneal mass was diagnosed involving the right psoas muscle, paravertebral muscles, vertebrae, right kidney, and ipsilateral dia phragm. A puncture biopsy was performed and the result was compatible with an inflammatory myofibroblastic tumor. CASE 3: 11-year-old female pre-adolescent, hospitalized to study recurrent urinary tract infection. A bladder tumor was identified, and the biopsy showed compatibility with inflammatory myofibroblastic tumor. CONCLUSION: Due to the variable behavior of the inflammatory myofibroblastic tumor, its management will depend on the location, expression of the anaplastic lymphoma kinase (ALK), tumor behavior, and the resection possibility.
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Humanos , Masculino , Feminino , Criança , Adolescente , Neoplasias Retroperitoneais/diagnóstico , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias Intestinais/diagnóstico , Neoplasias Retroperitoneais/patologia , Neoplasias da Bexiga Urinária/patologia , Miofibroblastos/patologia , Inflamação/diagnóstico , Inflamação/patologia , Neoplasias Intestinais/patologiaRESUMO
Inflammatory myofibroblastic tumor or inflammatory pseudotumor was first described by Brunn in 1932. Umiker et al. named it so in 1954 for its ability to mimic malignancy clinically and radiologically [Med Pediatr Oncol 2000; 35(5): 484-7]. This tumor, characterized by its rareness, affects mainly the lung and the orbit. Histologically, the tumor is characterized by the presence of fibroblasts, myofibroblasts, polymorphs, lymphocytes, and neutrophils. This case report and review of literature present a male patient, 37 years old, with a bulky mediastinal lesion at the topography of the 4th rib. Histopathology reveals Hodgkin's Lymphoma (HL) concomitant with inflammatory myofibroblastic tumor (IMT). This report aims to emphasize the relevance of differential diagnoses to a better medical assistance. To the best of our knowledge, a case with this characteristic has never been seen before.
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Follicular dendritic cell (FDC) tumor is an uncommon neoplasm. It generally presents as a slow-growing, painless mass, without systemic symptoms. Histological features usually include low grade spindle cell proliferation. This tumor occurs primarily in lymph nodes, especially cervical and axillary, however, involvement of extranodal sites such as the tonsils, spleen, liver, and gastrointestinal tract has been reported. Inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDCT) is a rare, distinctive histological subtype of this low-grade malignant neoplasm, with consistent Epstein-Barr virus (EBV) association. The differential diagnosis with other fibro-inflammatory tumor proliferations, as inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT), may be challenging. In the present article, two cases of IPT-like FDCT of the spleen are presented, with a broad overview of the literature: one 77-year-old male and one 70-year-old female. A large immunohistochemical panel should be used for diagnosis, as no single specific and totally sensitive markers are available, including markers for CD21, CD23, CD35, CNA42, and clusterin. Individual cases may express one or more of these markers, so that all of them should be investigated. In situ hybridization for EBV is constantly positive. Immunostaining for ALK should be negative, as it is present in roughly half of the cases of IMT. This panel should be used in combination of clinical, laboratory, and topographic evidences. Importantly, inclusion of this lesion as a possible option in clinical and pathological investigation represents the basis for a correct diagnosis (AU)
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Humanos , Neoplasias Esplênicas , Imuno-Histoquímica , Células Dendríticas Foliculares , Proliferação de Células , Miofibroblastos , Granuloma de Células PlasmáticasRESUMO
El tumor miofibroblástico inflamatorio de laringe es una patología rara dentro de los tumores de cabeza, cuello y de vía aérea, con una baja prevalencia, existiendo escasa literatura al respecto. Corresponde a un tumor benigno que generalmente se presenta como hallazgo incidental en relación a cuerdas vocales con mayor frecuencia, que puede ser de carácter asintomático o presentarse con clínica de obstrucción de vía aérea como estridor y disnea progresiva, además de disfonía. Puede afectar a pacientes de cualquier edad, siendo más frecuentes en adultos en edad media. El diagnóstico se hace a través de radiología y visualización directa mediante laringoscopía con la consiguiente obtención de muestra y estudio histopatológico. El tratamiento consta en la resección quirúrgica completa de la lesión, asociado o no a terapia esteroidal, sin embargo la curación completa no se logra en todos los casos producto de la naturaleza recidivante de la lesión.
The inflammatory myofibroblastic umor of the larynx is a rare condition in tumors of head, neck and airway, with a low prevalence, and scarce existing literature. It corresponds to a benign tumor that usually occurs as an incidental finding in relation to vocal cords more frequently, which can be asymptomatic or present with clinical character of airway obstruction, as progressive stridor and dyspnea, plus dysphonia. It can affect patients of any age are more common in adults in middle age. The diagnosis is made by radiology and direct laryngoscopy with visualization by obtaining sample and histology. Treatment consists of surgical removal of the entire lesion with or without steroid therapy, however complete healing is not achieved in all the cases product of the recurrent nature of the patology.
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Humanos , Masculino , Idoso , Neoplasias Laríngeas/cirurgia , Neoplasias Laríngeas/diagnóstico , Neoplasias de Tecido Muscular/cirurgia , Neoplasias de Tecido Muscular/diagnóstico , Prognóstico , Neoplasias Laríngeas/patologia , Granuloma de Células Plasmáticas , Inflamação , Neoplasias de Tecido Muscular/patologiaRESUMO
Los tumores miofibroblásticos son lesiones de presentación infrecuente, especialmente en localizaciones extrapulmonar y genital, con pocos casos descritos en la literatura internacional a nivel ovárico. Su diagnóstico, basado en la histopatología, es un reto para el patólogo, dada la heterogeneidad de su inmunohistoquímica. La mayoría de los casos presentan un comportamiento benigno, sin embargo, algunos subtipos pueden recurrir y/o presentar agresividad local y potencial metastatizante. Se han descrito algunos factores pronósticos de potencial maligno pero que desafortunadamente no han podido predecir en todos los casos el comportamiento biológico de la enfermedad. El pilar del tratamiento continúa siendo la cirugía con la extensión apropiada para proporcionar márgenes quirúrgicos negativos y disminuir el potencial de recidiva.
Myofibroblastic tumors are infrequent, particularly in extrapulmonary and genital sites, with few cases described within the ovaries in the international literature. The diagnosis, based on histopathology is always a challenge for the pathologist, due to the heterogeneity of the immunohistochemistry. Their behaviour is mostly benign, although some subtypes may recur, be locally aggressive, and have potential to metastasise. Prognostic factors for malignant potential have been described, unfortunately without predicting the biological behaviour in all cases. The mainstay of treatment is surgery, taking into account an appropriate extension to provide negative surgical margins, thus decreasing the risk of recurrence.
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Humanos , Feminino , Ovário , Neoplasias , Prognóstico , Terapêutica , Imuno-Histoquímica , Agressão , Diagnóstico , Margens de ExcisãoRESUMO
BACKGROUND: Inflammatory pseudotumor is a little known and uncommon condition. The debate continues whether it represents an inflammatory lesion or is a true neoplasm. It is considered a reactive process usually characterized by irregular growth of inflammatory cells. It has been described at various sites, the most common being the lung. The aim of this report is to emphasize the difficulty in the initial diagnosis. CLINICAL CASE: We present the case of a 56-year-old male who reports an 8-month history of dry cough, dyspnea, fatigue, weakness and weight loss of 20 kg. We performed two biopsies, one positive for malignancy without response to medical treatment and the second reporting chronic granulomatous inflammation. The patient underwent sternotomy, revealing a tumor of 20 × 17 × 10 cm, weighing approximately 2 kg. The tumor was dependent on the anterior mediastinum surrounding large vessels, and venous brachiocephalic, pericardium and both pleuras with firm adhesions to the right lung. Pathological report was as follows: inflammatory myofibroblastic tumor with positive immunohistochemistry for CD20 and CD3. Postoperative course was satisfactory and 1 year after surgery there was no evidence of recurrence. CONCLUSION: Inflammatory pseudotumor is a benign neoplasm of unknown origin with a chronic course. It can simulate a malignant tumor, causing constitutional manifestations, airway obstruction, cardiac alterations or other symptoms according to their location. Diagnosis is based on radiological features and direct biopsy. Treatment of choice is complete resection of the tumor with a favorable long-term outcome.
ANTECEDENTES: el pseudotumor inflamatorio es una afección poco frecuente y conocida de la que aún se debate si es una lesión inflamatoria o se trata de una verdadera neoplasia. Se considera un proceso generalmente reactivo caracterizado por crecimiento irregular de células inflamatorias. Se ha descrito en diversos sitios y la localización más frecuente es el pulmón. El objetivo de este caso es denotar la dificultad diagnóstica inicial. Caso clínico: paciente masculino de 56 años de edad, con ocho meses de evolución del padecimiento, con tos seca, disnea progresiva, astenia, adinamia y pérdida ponderal de 20 kg. Se realizaron dos biopsias; una positiva a malignidad sin respuesta al tratamiento médico y la segunda que reportó inflamación crónica granulomatosa. En la esternotomía se encontró un tumor de 20 × 17 × 10 cm, con peso aproximado de 2 kg, dependiente del mediastino anterior que rodeaba grandes vasos, el tronco braquiocefálico venoso al igual que el pericardio; ambas pleuras con adherencias firmes al pulmón derecho. El reporte histopatológico fue de: tumor miofibroblástico inflamatorio con inmunohistoquímica positiva para CD20 y CD3. La evolución postoperatoria fue satisfactoria y a un año de la cirugía sin evidencia de recurrencia. CONCLUSIÓN: el pseudotumor inflamatorio es una neoplasia benigna de origen indeterminado y de evolución crónica que puede simular un tumor maligno, con manifestaciones constitucionales y de obstrucción aérea, cardiaca o según su localización. El diagnóstico se basa en las características radiológicas y la biopsia directa, el tratamiento de elección consiste en la resección completa del tumor, con resultado favorable a largo plazo.
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Granuloma de Células Plasmáticas/patologia , Doenças do Mediastino/patologia , Doenças Torácicas/patologia , Antígenos CD20/análise , Complexo CD3/análise , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/epidemiologia , Granuloma de Células Plasmáticas/cirurgia , Humanos , Masculino , Doenças do Mediastino/diagnóstico , Doenças do Mediastino/cirurgia , Pessoa de Meia-Idade , Doenças Torácicas/diagnóstico , Doenças Torácicas/cirurgiaRESUMO
Background: Inflammatory myofibroblastic tumor (IMT) is a rare disease, usually benign, although with possible progression to malignancy. The clinical features depend on its location. If the resection is completed, surgery is curative, but recurrence is possible. The diagnosis is always histopathological. Case report: We report a 20 years old female admitted for a progressive abdominal pain lasting 24 hours. The patient was subjected to an appendectomy but in the postoperative period she continued with nausea and vomiting. The patient was operated again, finding a small bowel tumor with multiple adhesions, occluding the intestinal lumen and a Meckel diverticulum. The pathological study of the tumor reported the presence of an inflammatory myofibroblastic tumor. The patient had an uneventful postoperative outcome.
Introducción: El tumor miofibroblástico inflamatorio (TMI) es una enfermedad poco frecuente, en general benigna, aunque con posible evolución a malignidad. Las características clínicas dependen del lugar en el que esté ubicado. La cirugía es curativa siempre y cuando la resección sea total; aunque no se excluye la posibilidad de recidivas. El diagnóstico siempre es histopatológico. Dadas sus características clínicas y sitios de presentación se ha denominado de distintas formas e incluso sigue siendo motivo de estudio a fin de entender completamente su fisiopatología. Caso clínico: Presentamos un caso de esta rara entidad con un cuadro clínico de un abdomen agudo.
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Humanos , Adulto , Feminino , Enteropatias/cirurgia , Enteropatias/complicações , Granuloma de Células Plasmáticas/cirurgia , Granuloma de Células Plasmáticas/complicações , Abdome Agudo/etiologia , Divertículo Ileal/cirurgia , Divertículo Ileal/complicaçõesRESUMO
Tumores miofibroblásticos inflamatórios raramente acometem vias urinárias ou crianças, comumente mimetizando neoplasias malignas nos exames de imagem. Foram descritos apenas 35 casos desses tumores na bexiga de crianças, segundo a literatura recente. Os autores apresentam o caso de uma criança com um tumor miofibroblástico vesical que evoluiu favoravelmente após ressecção cirúrgica completa.
Inflammatory myofibroblastic tumors rarely affect the urinary tract or children, and frequently mimic malignancy on imaging studies. According to the recent literature, only 35 cases of such bladder tumors in children have been reported. The authors present the case of a child with a bladder myofibroblastic tumor with favorable progression following complete surgical resection.
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Humanos , Feminino , Adolescente , Neoplasias da Bexiga Urinária/diagnóstico , Pelve , Dor Abdominal , Biópsia , Hematúria , Espectroscopia de Ressonância Magnética , Tomografia Computadorizada por Raios X , Ultrassonografia Doppler , Redução de PesoRESUMO
El tumor miofibroblástico inflamatorio tiene un comportamiento predominantemente benigno pero en ocasiones puede mostrar grados variables de agresividad. Se localiza en diferentes órganos, el pulmón es el sitio más frecuente, seguido por la cavidad abdominal. Se presentan 2 casos con tumor miofibroblástico inflamatorio. Caso N° 1 femenino de 5 años con palidez cutáneo mucosa, hiporexia, dolor abdominal, fiebre, soplo cardíaco y hepatoesplenomegalia. Ultrasonido, tomografía y estudio radiológico contrastado: tumor en cuerpo gástrico con engrosamiento de sus paredes sugestivo de enfermedad linfoproliferativa. Estudio endoscópico: lesión verrugosa, en curvatura mayor y cuerpo alto. Biopsia: no concluyente. Caso N° 2 femenino de 14 años con estreñimiento agudo, dolor abdominal, masa palpable en mesogastrio e hipogastrio. Ultrasonido y tomografía: lesión ocupante de espacio en mesenterio, quística. Las dos pacientes presentaron anemia severa, leucocitosis y trombocitosis. Se realizó laparotomía exploradora en ambas, caso N° 1: tumor sólido en curvatura mayor gástrica, se hizo resección completa mediante gastrectomía parcial. Caso N° 2: tumor que surge del mesocolon transverso, quístico, ocupando todo el abdomen, 90% fue resecado. Diagnosticó histopatológico: tumor miofibroblástico inflamatorio. Evolución satisfactoria en ambos casos. Esta neoplasia debe sospecharse en niños para evitar terapias radicales, la cirugía conservadora es suficiente como tratamiento en la mayoría de los casos
The inflammatory myofibroblastic tumor behaves predominantly benign but can sometimes show varying degrees of aggressiveness. It is located in different organs, the lung is the most frequent, followed by the abdominal cavity. We present 2 cases of inflammatory myofibroblastic tumor. Case No. 1 female 5 years old with pale skin mucosa, anorexia, abdominal pain, fever, heart murmur, and hepatosplenomegaly. Ultrasound, CT and contrast radiological study: tumor in the stomach with thickening of their walls suggestive of lymphoproliferative disease. Endoscopic Study: warty lesion on greater curvature and high body. Biopsy inconclusive. Case No. 2 female 14 years with severe constipation, dominal pain, palpable mass in mesogastrium and hypogastric. Ultrasound and CT: space-occupying lesion in the mesentery, cystic. The two patients had severe anemia, leukocytosis and thrombocytosis. Laparotomy was performed in both Case No. 1: solid tumor greater curvature gastric resection was completed by partial gastrectomy. Case No. 2: tumor that arises from the transverse mesocolon, cystic, occupying the entire abdomen, 90% were resected. Pathological diagnosis: inflammatory myofibroblastic tumor. Satisfactory in both cases. This neoplasm should be suspected in children to prevent radical therapies, surgery, conservative treatment is sufficient in most cases