Paquimeningitis hipertrófica por enfermedad relacionada con IgG4 (ER-IgG4), reporte de un caso / Hypertrophic pachymeningitis due to IgG4-related disease (RD-IgG4). A case report

Introducción: La paquimeningitis hipertrófica (PH) es una entidad clínico-imagenológica caracterizada por un engrosamiento de la duramadre que puede ser focal o difuso manifestada por una variedad de síndromes neurológicos. Etiológicamente se clasifica en infecciosa, neoplásica, autoinmune e idiopática. Se ha demostrado que muchos de estos casos, antes idiopáticos, caen en el espectro de la enfermedad relacionada con IgG4. Objetivo: Describir el caso de una paciente asistida en nuestro servicio por compromiso neurológico por PH con diagnóstico inicial de tumor miofibroblástico inflamatorio (TMI) y diagnóstico final de enfermedad relacionada con IgG4. Caso: Mujer de 25 años con cuadro neurológico de 3 años de evolución caracterizado inicialmente por hipoacusia derecha, que evoluciona con cefalea y diplopía. Se realiza resonancia magnética nuclear (RMN) de encéfalo donde se evidencia engrosamiento paquimeníngeo con compromiso de estructuras vasculonerviosas en la punta del peñasco, seno cavernoso, agujero rasgado y quiasma óptico. Se presenta a la consulta con resultado de biopsia incisional que informa de lesión proliferativa que combina elementos fibrosos, de disposición fascicular o arremolinada con bandas colagenizadas con infiltrado linfoplasmocitario denso y algunos macrófagos, con tinción negativa para ALK 1 y con diagnóstico de tumor miofibroblástico inflamatorio. Por sospecha de enfermedad relacionada con IgG4 (ER-IgG4) se envía pieza de biopsia a revisión y se solicitan estudios complementarios pertinentes. Revisión de biopsia: fibrosis de tipo no estoriforme, infiltrado con predominio linfoplasmocitario, en otros cortes se reconocen también histiocitos y polimorfonucleares, sin granulomas ni atipias. Tinción para gérmenes negativos. Inmunohistoquímica con 50-60 células IgG4+/HPF e intervalo del 15 al 20%, CD68+ en histiocitos, CD1a− y S100...(AU)

Introduction: Hypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. Objective: To describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. Case: A 25-year-old woman with neurological symptoms of 3 years’ evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour.Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested.(AU)

Hypertrophic pachymeningitis due to IgG4-related disease (RD-IgG4). A case report.

INTRODUCTION: Hypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. OBJECTIVE: To describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. CASE: A 25-year-old woman with neurological symptoms of 3 years' evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour. Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. BIOPSY REVIEW: Non storiform fibrosis, predominantly lymphoplasmacytic infiltrate, histiocytes, and polymorphonuclear infiltrate in sectors, without granulomas or atypia. Staining for germs negative. Immunohistochemistry with 50-60 IgG4+/HPF cells and range of 15%-20%, CD68+ in histiocytes, CD1a-, S100-. The patient presented deterioration of visual acuity due to ophthalmic nerve involvement, so glucocorticoid treatment was started in pulses and rituximab with regression of symptoms and imaging improvement of the lesions. CONCLUSION: HP is a clinical imaging syndrome with variable symptoms and aetiologies that poses a diagnostic challenge. In this case the initial diagnosis was inflammatory myofibroblastic tumour, which is a neoplasm of variable behaviour, locally aggressive, and can metastasize; it is one of the main differential diagnoses of IgG4-related disease because they share anatomopathological features, including storiform fibrosis. IgG4-RD is an immune-mediated condition that can have single or multiple involvement. Its diagnosis is complex when it presents with single organ involvement or in non-typical organs (CNS, meninges) in which data are scarce, as in the case of our patient with single organ involvement of the CNS. Although there are classification criteria to guide non-specialists in the diagnosis, the sum of the clinical picture, imaging, laboratory, pathological anatomy, and immunohistochemistry will always be evaluated together for a definitive diagnosis.

Seudotumor inflamatorio y tumor miofibroblástico inflamatorio. Criterios diagnósticos y diferencias pronósticas / Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences

Introducción El seudotumor inflamatorio (PTI) y el tumor miofibroblástico inflamatorio (TMI) son 2 entidades muy poco frecuentes que se incluían antiguamente en la misma categoría; sin embargo, en la actualidad se consideran 2 enfermedades diferentes debido al origen neoplásico del TMI. Nuestro objetivo es compartir nuestra experiencia en el manejo de estos 2 tipos de lesiones que debemos tener en cuenta en el diagnóstico diferencial de masas o nódulos pulmonares. Métodos Fueron estudiados retrospectivamente 13 pacientes con diagnóstico anatomopatológico de PTI o TMI, intervenidos entre los años 2008 y 2019. Registramos la información pre y postoperatoria de cada uno, así como el análisis de supervivencia. Resultados De los 13 pacientes, 8 eran varones y 5 mujeres. La media de edad de presentación fue de 53,5 años. En 6 pacientes se practicó una segmentectomía atípica, en 6 fue necesario realizar una lobectomía y en un caso una neumonectomía. En todos los casos se consiguió una resección completa. El diagnóstico fue posible gracias a la histología, técnicas de inmunohistoquímica (IHQ) y de hibridación fluorescente in situ (FISH) determinando la expresión de IgG4 y el reordenamiento de ALK, respectivamente. Tras una mediana de seguimiento de 49 meses no se observaron datos de recidiva locorregional ni a distancia en los pacientes estudiados. Conclusión El PTI y el TMI son tumores poco frecuentes con muy buen pronóstico. El diagnóstico de ambas entidades se basa principalmente en técnicas anatomopatológicas específicas. La cirugía tiene, en la mayor parte de las ocasiones, un papel tanto diagnóstico como terapéutico (AU)

Introduction Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. Methods Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. Results Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didńt find any loco-regional or distant recurrence in the patients studied. Conclusion IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role (AU)

Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences.

INTRODUCTION: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. METHODS: Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. RESULTS: Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didn't find any loco-regional or distant recurrence in the patients studied. CONCLUSION: IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role.

[Inflammatory myofibroblastic tumour of the vocal cord. A case report and revision of the literature]. / Tumor miofibroblástico inflamatorio en cuerda vocal. Reporte de un caso y revisión de la bibliografía.

Inflammatory myofibroblastic tumour (IMT) is a rare entity that can occur in practically any location. Although it has an increased incidence in infancy and adolescence, cases of IMT in the head and neck are more frequent in adults. We report the case of a 74-year-old male who presented with a two month history of dysphonia. Laryngoscopy and cervical TAC revealed a nodular lesion affecting the anterior half of the left vocal cord. He underwent endoscopic laser cordectomy. Histopathology concluded that the lesion was an IMT. Cases of IMT in the head and neck are infrequent and in the vocal cord extremely rare, with only a few previously reported cases.

Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences. / Seudotumor inflamatorio y tumor miofibroblástico inflamatorio. Criterios diagnósticos y diferencias pronósticas.

INTRODUCTION: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. METHODS: Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. RESULTS: Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didnt find any loco-regional or distant recurrence in the patients studied. CONCLUSION: IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role.

Tumor miofibroblástico inflamatorio: presentación variable de una misma patología / Inflammatory myofibroblastic tumor: variable presentation of the same pathology

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.

Tumor miofibroblástico de laringe: reporte de caso clínico y revisión de literatura / Miofibroflástico larynx tumor: case report and literature review

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.

Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: (18)F-FDG PET/CT findings.

A 30-year-old female presented with a 3-month history of erosive stomatitis and bullous lesions, along with recurrent episodes of abdominal pain. She was found to have a retroperitoneal lump in left lumbar region. Skin biopsy revealed bullous disorder. CT guided biopsy of the retroperitoneal mass was suggestive of inflammatory myofibroblastic tumor (IMT). She was started on oral steroids and supportive care, and surgery was being planned when she developed respiratory failure. CT chest revealed vertebral metastases. PET/CT for whole body work up revealed a left para-aortic mass along with multiple skeletal metastases. The patient was kept on conservative management. After 3 months, the patient has shown clinical improvement, and an exploratory laparotomy is now being planned for the excision of the tumor, followed by chemotherapy. This case of retroperitoneal IMT is rare in terms of skeletal metastases with paraneoplastic pemphigus.

Tumor miofibroblástico de origen ovárico: comportamiento agresivo y fatal / Myofibroblastic tumour of ovary origin: aggressive and fatal behaviour

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.

Tumor miofibroblástico inflamatório da vesícula biliar: relato de caso / Inflammatory myofibroblastic tumor of the gallbladder: case report

Introdução: tumor miofibroblástico inflamatório (TMI) é um tumor benigno, não metastizante, que ocorre por proliferação de miofibroblastos com potencial para infiltração e recorrência local, sendo o TMI primário da vesícula biliar é uma entidade rara. Relato de Caso: relatamos um caso de um homem de 62 anos, que realizou colecistectomia por colecistite aguda. O exame histopatológico revelou um tumor miofibroblástico inflamatório da vesícula biliar com marcadores positivos para vimentina, actina de músculo liso, desmina, CD68, CD34, CD45, ALK-1 e KI-67. Conclusão: tumores miofibroblásticos inflamatórios de vesícula biliar podem simular quadro de colecistite aguda. O método terapêutico deve ser a ressecção cirúrgica para evitar recorrência local. O diagnóstico pré-operatório é difícil e deve-se ter alto índice de suspeição mesmo em pacientes sem achados radiológicos.

Background: inflammatory myofibroblastic tumor (IMT) is a nonmetastasizing benign tumor that occurs by proliferation of myofibroblasts with a potential for infiltration and local recurrence is the primary IMT gallbladder is a rare entity. Case Report: we report a case of a 62 year-old man, who performed cholecystectomy for acute cholecystitis. The histopathology examination revealed an inflammatory myofibroblastic tumor of the gallbladder with positive markers for vimentin, smooth muscle actin, desmin, CD68, CD34, CD45, ALK-1 and KI-67. Conclusion: Inflammatory myofibroblastic tumor of gallbladder can mimic acute cholecystitis. The therapeutic method should be surgical resection to avoid recurrence site. The preoperative diagnosis is difficult and should have a high index of suspicion in patients without radiological findings.

[Intrathoracic-mediastinal myofibroblastic tumor. Report and experience of one case]. / Tumor miofibroblástico intratorácico-mediastinal. Reporte y experiencia en un caso.

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.

Tumor miofibroblástico inflamatório da bexiga em criança: relato de caso / Inflammatory myofibroblastic tumor of the bladder in a child: a case report

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.

Tumor miofibroblastico inflamatorio: presentación de dos casos pediátricos / Inflammatory myofibroblastic tumor: presentatiom of two pediatric cases

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

Tumor miofibroblástico inflamatório peritonial: revisão da literatura / Peritoneal inflammatory myofibroblastic tumor: a literature review

Introdução: o tumor miofibroblástico inflamatório (tmi) ao longo das últimas duas décadas saiu de dentro da grande categoria de pseudotumor inflamatório, devido as suas distintas características clínicas, patológicas e moleculares, o pseudotumor é um termo genérico aplicado a uma variedade de entidades neoplásicas e não neoplásicas que compartilham uma aparência histológica em comum, ou seja, uma proliferação de células citologicamente parecida, geralmente com infiltrado inflamatório crônico. (...) (AU)

Tumor miofibroblástico inflamatório peritonial: revisão da literatura

Introdução: o tumor miofibroblástico inflamatório (tmi) ao longo das últimas duas décadas saiu de dentro da grande categoria de pseudotumor inflamatório, devido as suas distintas características clínicas, patológicas e moleculares, o pseudotumor é um termo genérico aplicado a uma variedade de entidades neoplásicas e não neoplásicas que compartilham uma aparência histológica em comum, ou seja, uma proliferação de células citologicamente parecida, geralmente com infiltrado inflamatório crônico. (...)

Tumor miofibroblástico inflamatório peritonial: revisão da literatura / Peritoneal inflammatory myofibroblastic tumor: a literature review

Introdução: o tumor miofibroblástico inflamatório (tmi) ao longo das últimas duas décadas saiu de dentro da grande categoria de pseudotumor inflamatório, devido as suas distintas características clínicas, patológicas e moleculares, o pseudotumor é um termo genérico aplicado a uma variedade de entidades neoplásicas e não neoplásicas que compartilham uma aparência histológica em comum, ou seja, uma proliferação de células citologicamente parecida, geralmente com infiltrado inflamatório crônico...

Un nuevo caso de pseudotumor inflamatorio de la tráquea / A new case of inflammatory tracheal tumor

El pseudotumor inflamatorio de tráquea es poco frecuente y puede simular un tumor cuando se localiza en la luz traqueal o bronquial. El objetivo del presente artículo fue presentar un nuevo caso y revisar la literatura especializada. Se trató de una paciente de 23 años de edad, con cuadro de disnea de varios años de evolución, tratada con el diagnóstico de asma bronquial. Fue intervenida de urgencia por empeoramiento de la disnea y diagnóstico de tumor intratraqueal, según estudio broncoscópico. Se practicó la resección de 5 anillos traqueales con anastomosis término-terminal. La evolución posoperatoria fue satisfactoria y la paciente se encuentra libre de recidiva 5 años después de la operación(AU)

The inflammatory tracheal pseudotumor is rare and it may mimic a tumor when it is located in the tracheal or bronchial light. The objective of this article was to present a new case and to review the specialized literature. The case of a 23-year-old patient with a picture of dyspnea of several years of evolution treated with diagnosis of bronchial asthma was reported. She underwent emergency surgery due the worsening of dypsnea and to the diagnosis of intratracheal tumor according to a bronchoscopic study. The resection of 5 tracheal rings was performed with termino-terminal anastomosis. The postoperative evolution was satisfactory and the patient has been free of relapse five years after the operation(AU)

Un nuevo caso de pseudotumor inflamatorio de la tráquea / A new case of inflammatory tracheal tumor

El pseudotumor inflamatorio de tráquea es poco frecuente y puede simular un tumor cuando se localiza en la luz traqueal o bronquial. El objetivo del presente artículo fue presentar un nuevo caso y revisar la literatura especializada. Se trató de una paciente de 23 años de edad, con cuadro de disnea de varios años de evolución, tratada con el diagnóstico de asma bronquial. Fue intervenida de urgencia por empeoramiento de la disnea y diagnóstico de tumor intratraqueal, según estudio broncoscópico. Se practicó la resección de 5 anillos traqueales con anastomosis término-terminal. La evolución posoperatoria fue satisfactoria y la paciente se encuentra libre de recidiva 5 años después de la operación(AU)

The inflammatory tracheal pseudotumor is rare and it may mimic a tumor when it is located in the tracheal or bronchial light. The objective of this article was to present a new case and to review the specialized literature. The case of a 23-year-old patient with a picture of dyspnea of several years of evolution treated with diagnosis of bronchial asthma was reported. She underwent emergency surgery due the worsening of dypsnea and to the diagnosis of intratracheal tumor according to a bronchoscopic study. The resection of 5 tracheal rings was performed with termino-terminal anastomosis. The postoperative evolution was satisfactory and the patient has been free of relapse five years after the operation(AU)