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.

The Juscelino Kubitschek government and the Brazilian Malaria Control and Eradication Working Group: collaboration and conflicts in Brazilian and international health agenda, 1958-1961 / O governo JK e o Grupo de Trabalho de Controle e Erradicação da Malária no Brasil: encontros e desencontros nas agendas brasileira e internacional de saúde, 1958-1961

Malaria, a disease which was under control in the beginning of Juscelino Kubitschek government, became the most important endemic disease in 1958, when Brazil made a commitment with the World Health Organization to convert its control programs into eradication programs. For this purpose a Malaria Control and Eradication Group was set up under the leadership of the malaria specialist Mário Pinotti. Malaria would become an important bargaining chip in the context of the development policies of Kubitschek. This article focuses on path of the Malaria Control and Eradication Working Group in Brazil, in its varying relationships with the arguments and guidelines established at international level.

A malária, doença que estava controlada no início do governo de Juscelino Kubitschek, torna-se a mais importante endemia em 1958, quando o Brasil assumiu o compromisso com a Organização Mundial da Saúde de converter seus programas de controle em programas de erradicação. Para isso foi instalado um Grupo de Controle e Erradicação da Malária sob a direção do malariologista Mário Pinotti. A malária seria uma importante moeda de negociação no contexto da política de desenvolvimento de Kubitschek. Este artigo tem como foco a trajetória do Grupo de Trabalho de Controle e Erradicação da Malária no Brasil, em suas diferentes relações com as discussões e normativas travadas e estabelecidas em âmbito internacional.

[Angiomiofibroblastoma of the vulva. Report of case]. / Angiomiofibroblastoma de la vulva. Reporte de caso.

Angiomyofibroblastoma is a benign mesenchymal tumor of the lower genital tract described in 1992 by Fletcher and his group. Must be differentiated mainly from aggressive angiomyxoma, which has a high local recurrence rate. We report the case of a 21 year old patient who attended the General Hospital Dr. Miguel Silva, in Morelia, Michoacan, because post-term pregnancy of 41.1 weeks gestation, with a bulky vulvar tumor, asymptomatic with four years of evolution. Cesarean section and vulvar tumor resection were performed. The surgical specimen measured 14 x 10 x 5 cm, had a polypoid aspect, multilobulated, with nodules of varying size. From a microscopic point of view the tumor was well defined, formed by loose connective tissue septa, with stromal fusiform cells and benign characteristics, between collagen fibers, with abundant vessels. The histochemical studies were positive for desmin and vimentin. These anatomopathological findings are diagnostic of vulvar angiomyofibroblastoma, benign course tumor, does not resort nor does it generate metastases after simple surgical resection; however, recurrence in the vagina is reported. Must be differentiated from other mesenchymal neoplasms of the genital tract.

Inflammatory myofibroblastic tumor of the liver.

 Hepatic inflammatory myofibroblastic tumors are uncommon low grade malignant neoplasms. They can be confused clinically and by imaging studies with abscess.

Inflammatory myofibroblastic tumor of the tongue: report of an unusual case in a teenage patient.

Inflammatory myofibroblastic tumor is a rare and distinctive lesion composed of myofibroblastic cells accompanied by an inflammatory infiltration of plasma cells, lymphocytes, and eosinophils that mainly affects viscera and soft tissues of children and young adults. It clinically manifests as a mass with rapid development that may occur at almost any site of the body, but predominantly in the lungs or the upper respiratory tract. It rarely arises in the oral cavity with approximately 15 cases reported in that location. We describe a case of an inflammatory myofibroblastic tumor of the tongue, confirmed by both histopathologic and immunohistochemical analysis, occurring in a 14-year-old boy that spontaneously regressed after the surgical procedures for its diagnosis. Because of some aggressive clinical, histological, and radiological features, this lesion may be confused with a malignant tumor. Therefore, its correct recognition is important to avoid unnecessary extensive and radical therapeutic approaches.

The immunohistochemical profile of oral inflammatory myofibroblastic tumors.

OBJECTIVE: The aim of this study was to demonstrate the immunohistochemical profile of oral inflammatory myofibroblastic tumors (IMTs) along with morphologic analysis. STUDY DESIGN: Three cases diagnosed as oral IMTs were selected to compile an immunohistochemical panel constituted by calponin, caldesmon, Bcl-2, desmin, fibronectin, CD68, Ki-67, S100, anaplastic lymphoma kinase (ALK), α-smooth muscle actin, cytokeratins AE1/AE3, muscle-specific actin, CD34, and vimentin. An oral squamous cell carcinoma with a focal area of desmoplastic stroma was used as control for the stained myofibroblastic cells. RESULTS: All oral IMTs were positive for calponin, revealing a strong and diffuse expression in the spindle-shaped cells. The lesions were also positive for vimentin (3/3), fibronectin (3/3), α-smooth muscle actin (3/3), and muscle-specific actin (1/3) and negative for h-caldesmon, Bcl-2, desmin, CD68, Ki-67, S100, ALK, cytokeratins AE1/AE3, and CD34. CONCLUSIONS: Within the results encountered, the present panel should be of great assistance in the diagnosis of oral IMTs.

Miofibroblastoma de la mama femenina: reporte de un caso / Miofibroblastoma of the female breast: a case report

El miofibroblastoma constituye una entidad benigna de la mama de presentación muy infrecuente, que afecta principalmente la mama masculina. Su principal característica histológica es su estirpe mesenquimal caracterizada por la proliferación de células fusiformes rodeadas de colágeno y que derivan de los fibroblastos. El tratamiento principal es la cirugía, con tendencia a la preservación de la glándula sobre todo en la mujer. Presentamos el caso de una paciente femenina de 64 años de edad, con un tumor solitario, de crecimiento progresivo durante 5 años, hasta que decide solicitar asistencia médica, planteándose el diagnóstico preoperatorio de una lesión de tipo mesenquimal; la cual posterior al tratamiento quirúrgico y mediante estudios inmunohistoquímicos se confirma la presencia de un miofibroblastoma clásico, siendo estos tumores infrecuentes en la práctica clínica diaria, debiendo ser considerados al momento de hacer diagnóstico diferencial.

The miofibroblastoma is a benign tumor of the breast of very infrequent presentation that mainly affects the male breast. The main histological characteristic is the mesenchymal ancestry characterized by the proliferation of plump and spindle cells surrounded by collagen and that derive from fibroblasts. The surgery is the main treatment with preservation of the breast in female patients. We presented a case of a female patient of 64 years old, which presents a solitary tumor with progressive growth by 5 years, until she decides to ask for medical aid, considering the preoperating diagnosis of an tumor of mesenchymal type; which subsequent to the surgical treatment and confirmed by immunohistochemistry the presence of miofibroblastoma of the breast, being this finding very unusual in the clinical practice, and to be considered at the time of making differential diagnosis.

Rare benign breast tumor.

We report the case of a female patient with an incidental finding at routine mammography evaluation which consisted of a benign spindle cell tumor, namely Breast Myofibroblastoma. It is arranged in fascicles with interspersed broad bands of hyalinized collagen with variable immunohistochemical reactivity to desmin, vimentin, smooth muscle actin and CD 34. It is usually not reactive to cytokeratins and S-100 as seen in the myoepitheliomas. Recurrence of the lesion after excisional surgical procedure is not documented at medical literature. It is important to recognize the benign nature of this neoplasm to prevent extensive mutilating surgical procedures.

Congenital peribronchial myofibroblastic tumor: a case report.

We describe a rare occurrence of congenital peribronchial myofibroblastic tumor of the lung presenting in early fetal life. A female patient in the 24th week of gestation who presented with polyhydramnios was admitted for examination. Ultrasound examination revealed a mass compromising the lungs. Because the intrauterine fetal death was revealed by the ultrasound, delivery was induced. Necropsy revealed a pulmonary lesion compromising the left lower pulmonary lobe together with hepatomegaly. Microscopic analysis of the lung showed a lesion with a storiform arrangement of spindle cells with focal peribronchial distribution. Hepatic architecture was diffusely altered by fibrotic tissue. Immunohistochemical analysis on the pulmonary lesion showed high vimentin positivity in the fusocellular components, pointing to the mesenchymal nature of the lesion. Significant differentiation of smooth muscle tissue, as indicated by high HHF35 positivity, was also observed. Electron microscopy on the pulmonary lesion revealed elongated cells with some cytoplasmatic processes, a finding that is also compatible with mesenchymal differentiation.

Miofrioblastoma mamário em axila / Mammary myofibroblastoma in axila

Miofibroblastoma é um tumor benigno raro de mama, de origem mesenquimal. A localização em região axilar é ainda mais incomum. Tendo em vista a dificuldade em se estabelecer o diagnóstico diferencial baseado apenas nos achados clínicos e imaginológicos, a confirmação histopatológica é imprescindível no diagnóstico diferencial com tumor oculto de mama.

Myofibroblastoma is a rare and benign mesenchymal breast neoplasm. The axillary topography is further more uncommon. The histologic identification is important because the differencial diagnosis with malignant tumours of the breast. This report a case of mammary myofibroblastoma in axilla, wich was initially diagnosticated as an occult lobular carcinoma.

Clinical and cytogenetic analysis of an intracranial inflammatory myofibroblastic tumor induced by a ventriculoperitoneal shunt.

The authors report the first case of an intracranial inflammatory myofibroblastic tumor (IMT) associated with the placement of a ventriculoperitoneal shunt, which occurred in a 7-year-old boy. Neuroradiological features showed a mass surrounding the ventricular catheter. The lesion was completely resected. Histological study revealed the mass to be an IMT. The patient's postoperative course was complicated by a local recurrence requiring a second surgery. Cytogenetic analysis of the sample by comparative genome hybridization revealed several chromosomal amplifications and regional losses. The occurrence of IMT in the CNS has rarely been reported. For treatment of this condition, the authors recommend a total removal of the shunt with a mass excision to prevent local recurrence.

Inflammatory myofibroblastic tumor of the alveolar mucosa of the mandible.

Inflammatory myofibroblastic tumor is a rare lesion composed of myofibroblastic spindle cells accompanied by inflammatory infiltrate. The objective of this paper is to report an uncommon case of inflammatory myofibroblastic tumor located in the alveolar mucosa of the mandible. A 33-year-old male presented an asymptomatic tumoral lesion, firm, pedunculated, pink-colored, covered by smooth mucosa, with focal ulceration, measuring 30x20x20 mm, located in the left posterior alveolar mucosa. Clinical diagnosis was soft tissue tumor. An excisional biopsy was made. Microscopic examination showed compact fascicular spindle cells proliferation with a diffuse inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. Large ganglion-like cells were observed. The lesional cells were immunopos-itive to vimentin, a-smooth muscle actin, muscle specific actin, and CD68. Negative immunostain was observed to S-100, Bcl-2, Ki-67, desmin, CD34, and cytokeratin. A diagnosis of inflammatory myofibroblastic tumor was performed. After 28 months of follow-up there was no recurrence. Although no evidence of oral inflammatory myofibroblastic tumor recurrence or malignant transformation has been reported, it has been observed that in inflammatory myofibroblastic tumor of other regions a prolonged follow-up is necessary after surgical excision.

Low-grade myofibroblastic sarcoma of the parapharyngeal space.

Low-grade myofibroblastic sarcoma was recently described as representing malignant mesenchymal tumours that show myofibroblastic differentiation; few cases have been reported. Here, a low-grade myofibroblastic sarcoma of the parapharyngeal space is described. A 42-year-old man presented with swelling on the right side of the temporal bone. Based on histological and immunohistochemical features, the diagnosis of low-grade myofibroblastic sarcoma was established. The tumour had invaded the orbit and the brain, and therefore surgical excision was not possible. There are thought to have been no cases affecting this region reported previously in the English-language literature.

Mammary myofibroblastoma: report of two cases with fine-needle aspiration cytology and review of the cytology literature.

Fine-needle aspirates from two histologically, immunohistochemically, and ultrastructurally confirmed mammary myofibroblastomas (MM) of two elderly women revealed abundant, randomly arranged single and clustered benign spindle-shaped mesenchymal cells with scant cytoplasm and elongated or oval nuclei displaying a finely granular chromatin pattern and inconspicuous nucleoli. In one case a few cells showed inconspicuous nuclear grooves. The aspirated tumor cells from the other case stained positively with desmin and CD34 antibodies and negatively with cytokeratin and S-100 protein antibodies, in keeping with an MM. A review of the literature was briefly presented.

[Tumors with rhabdomyoblasts and neuroblasts]. / Tumores con rabdomioblastos y neuroblastos.

A metallic impregnation technique, Del Río Hortega's double impregnation, to evidence neuroblasts and rhabdomyoblasts, with recommendations as to the times of impregnation to obtains better results is evaluated. The images obtained are highly demonstrative both of the embryo elements of the primitive mesenchyma from myoblast, myotube, a rhabdomyoblastic ribbon cell with transverse streaks to the neoplastic elements of this lineage and to the neuroblasts with their extensions. The study material includes a renal tumor of Wilms with rhabdomyoblasts and neuroblasts and a cerebellum neuroblastoma with a rhabdomyoblastic element. These injuries are considered dysembryoplasic. We also studied two botryoid embryo rhabdomyosarcomas, begin one or them unusually found in a menopausic woman, two Müller mixed tumors of the uterus and Fallopian tubes, a rhabdomyoma in pharynx and three human embryos from aborted material between 5 and 13 weeks old. We emphasize the use of double impregnation to study rhabdomyoblasts and neuroblasts. In rhabdomyosarcomas, structures and elements similar to those of the embryo stage--racketoid and ribbon cells, myotubes and rhabdomyoblasts--can be observed. The tumor of Wilms is a dysembryoplasic tumor composed of renal blastem where we have found rhabdomyoblasts and neuroblasts. In the cerebellum neuroblastoma we observe rhabdomyoblasts with certain organism (ectomesenchyma). Finally, we describe a rather infrequent case of a cervix botryoid rhabdomyosarcoma of a menopausic woman.

Tumores con rabdomioblastos y neuroblastos / Tumors with rhabdomyoblasts and neuroblasts

Se evalúa uma técnica de impregnación metálica, doble impregnación de Del Río Hortega, para evidenciar rabdomioblastos y neuroblastos, con recomendaciones sobre los tiempos de impregnación para obtener mejores resultados. Las imágenes obtenidas son muy demostrativas, tanto de los elementos embrionarios del mesénquima primitivo desde mioblasto, miotubo a célula acintada rabdomiobástica con estraciones transversales, hasta los elementos neopláticos de esta estripe. Y de los neuroblastos con sus prolongaciones. El material de estudio incluye un Tumor de Wilms renal con rabdomioblastos y neuroblastos, y un Neuroblastoma de cerebelo con componente rabdomioblástico. A estas lesiones se las considera desembrioplasias. Además se estudiaron 2 Rabdomiosarcomas embrionarios botrioides, uno de ellos de presentación inusual en una mujer menopáusica, 2 Tumores müllerianos mixtos de útero y trompa de Falopio, un Rabdomioma de faringe, y 3 embriones humanos de material de aborto entre 5 y 13 semanas. Destacamos la utilidad de la doble impregnación para estudiar rabdomioblastos y neuroblastos. En los Rabdomiosarcomas se pueden ver estructuras y elementos comparables con los de la etapa embrionaria: células raquetoides, acintadas, miotubos, rabdomioblastos. El Tumor de Wilms es un tumor disembrioplásico y está constituido por el blastema renal. En él hemos encontrado rabdomioblastos y neuroblastos. En el Neuroblastoma del cerebelo servamos rabdomioblastos con cierta organicidad (ectomeséquima). Y por último describimos un caso poco frecuente de Ragdomiosarcoma botrioide de cuello uterino en una mujer menopáusica.

Tumores con rabdomioblastos y neuroblastos / Tumors with rhabdomyoblasts and neuroblasts

Se evalúa uma técnica de impregnación metálica, doble impregnación de Del Río Hortega, para evidenciar rabdomioblastos y neuroblastos, con recomendaciones sobre los tiempos de impregnación para obtener mejores resultados. Las imágenes obtenidas son muy demostrativas, tanto de los elementos embrionarios del mesénquima primitivo desde mioblasto, miotubo a célula acintada rabdomiobástica con estraciones transversales, hasta los elementos neopláticos de esta estripe. Y de los neuroblastos con sus prolongaciones. El material de estudio incluye un Tumor de Wilms renal con rabdomioblastos y neuroblastos, y un Neuroblastoma de cerebelo con componente rabdomioblástico. A estas lesiones se las considera desembrioplasias. Además se estudiaron 2 Rabdomiosarcomas embrionarios botrioides, uno de ellos de presentación inusual en una mujer menopáusica, 2 Tumores m³llerianos mixtos de útero y trompa de Falopio, un Rabdomioma de faringe, y 3 embriones humanos de material de aborto entre 5 y 13 semanas. Destacamos la utilidad de la doble impregnación para estudiar rabdomioblastos y neuroblastos. En los Rabdomiosarcomas se pueden ver estructuras y elementos comparables con los de la etapa embrionaria: células raquetoides, acintadas, miotubos, rabdomioblastos. El Tumor de Wilms es un tumor disembrioplásico y está constituido por el blastema renal. En él hemos encontrado rabdomioblastos y neuroblastos. En el Neuroblastoma del cerebelo servamos rabdomioblastos con cierta organicidad (ectomeséquima). Y por último describimos un caso poco frecuente de Ragdomiosarcoma botrioide de cuello uterino en una mujer menopáusica. (AU)

Estadiamento de Enneking dos tumores músculo-esqueléticos / Enneking staging of musculoskeletal tumors

O autor apresenta a traduçäo e adaptaçäo para a língua portuguesa do estadiamento dos tumores do sistema músculo-esquelético proposto pelo Prof. Willian Enneking e utilizado mundialmente. O autor faz um resumo dos principais tópicos do estadiamento, explicando o texto através de esquemas adaptados das conferências do Prof. Enneking

[Granular cell tumor of the vulva]. / Tumor de células granulares en la vulva.

Granular cell tumor is a relatively uncommon entity of unknown etiology and uncertain histogenesis. A case of granular cell tumor of the vulva is presented with a discussion of the clinical features, pathological characteristics and the histogenic theories of its origin.