Condromatosis sinovial extrarticular de pie. Reporte de un caso y revision bibliografica / Extra-articular synovial chondromatosis of the foot. Case report and literature review

La condromatosis sinovial es una lesión poco frecuente caracterizada por la metaplasia cartilaginosa de la membrana sinovial de pequeñas y grandes articulaciones. Se suelen observar múltiples nódulos cartilaginosos y un grado variable de destrucción articular. Las manifestaciones clínicas son variables y dependen de la localización y el estadio de la enfermedad. Nuestra paciente concurrió con una tumoración en la planta del pie a nivel de la raíz del segundo y tercer dedo, de meses de evolución. Luego de los estudios por imágenes correspondientes y evidenciar la extensión dorsal de la lesión, se decidió realizar la resección en bloque a través de doble abordaje; el análisis anatomopatológico informó condromatosis sinovial. La ausencia de extensión intrarticular fue constatada en la cirugía. Este caso representa una manifestación poco frecuente de la patología no solo por su localización, sino también por su ubicación extrarticular. Según nuestra búsqueda bibliográfica, no se han publicado reportes de casos con condromatosis sinovial extrarticular avanzada (grado 3) en el antepié y creemos que debería ser considerada dentro de las lesiones tumorales de aspecto cartilaginoso en esta localización.

Synovial chondromatosis is a rare condition characterized by synovial metaplasia of the synovial layer affecting large and small joints. Multiple cartilaginous nodules and a variable degree of joint destruction are frequently observed. The clinical presentation varies and depends on the localization and grade of the condition. This patient presented with a painful plantar tumor in the root of the second and third finger. After the correct imaging studies en bloc resection using a double approach was performed. The histopathological exam informed synovial chondromatosis. Absence of joint involvement was confirmed during surgery. This case represents a rare presentation of the synovial chondromatosis not only for the localization on the foot but also for its extra-articular location. According to our literature review, case reports on highgrade extra-articular synovial chondromatosis of the forefoot are lacking, and we think that this entity must be considered in the differential diagnosis of the tumoral cartilaginous lesions in this location.

Calcinosis pseudotumoral en esclerodermia / Pseudotumoral calcinosis in systemic sclerosis

La calcinosis es una manifestación frecuente en la esclerodermia sistémica. Sin embargo, la calcinosis pseudotumoral es de presentación excepcional. Las radiografías o tomografía axial computada son de utilidad para el diagnóstico y el seguimiento. Aunque no existe un tratamiento estandarizado efectivo, la remoción quirúrgica de las lesiones sintomáticas y algunos fármacos son útiles en algunos enfermos. Se presenta el caso de una paciente de 23 años con esclerodermia sistémica variedad limitada que desarrolló múltiples lesiones pseudotumorales.

Soft tissue calcinosis is a common cutaneous manifestation in systemic sclerosis. However, pseudotumoral calcinosis is rare. Radiographs or computed tomography are useful tools to diagnose and assess this condition. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment provided benefit for some patients. We report here a case of a 23 year old woman, suffering from limited cutaneous systemic sclerosis, who developed many pseudo tumoural calcinosis lesions.

Lesiones proliferativas más frecuentes del complejo bucomaxilofacial / Most frequent proliferative lesions of the Buco Maxillofacial Complex

Introducción: las lesiones proliferativas son entidades que se presentan en la cavidad bucal. Algunas son de origen traumático y otras son neoplasias. Con frecuencia no son bien diagnosticadas en la clínica, por la similitud que pueden presentarse entre ellas. Objetivo: determinar las características de las lesiones proliferativas diagnosticadas y la coincidencia entre el diagnóstico clínico y el histopatológico. Método: se realizó un estudio retrospectivo de las biopsias procesadas en cinco años en el Departamento de Patología de la Facultad de Estomatología de La Habana atendiendo a edad y sexo del paciente, tipo de lesión, localización anatómica y coincidencia entre el diagnóstico clínico y el histopatológico. Resultados: se encontró que de las lesiones proliferativas, las pseudotumorales aportaron el 18,1 por ciento y las neoplasias benignas el 5,8 por ciento. En el grupo de edad de 10 a 19 años las lesiones pseudotumorales representaron el 7,78 por ciento y las neoplasias benignas el 1,78 por ciento. En el grupo de 60 años o más, las lesiones pseudotumorales aportaron el 22,67 por ciento del total de lesiones proliferativas. El sexo femenino resultó afectado por lesiones pseudotumorales en 61,3 por ciento y las neoplasias benignas afectaron al sexo masculino en un 55 por ciento. La hiperplasia fibroepitelial representó el 54,22 por ciento de las pseudotumorales, mientras que el nevus aportó el 8,67 por ciento entre las neoplasias benignas. Los diagnósticos clínicos de las lesiones pseudotumorales coincidieron en un 50,4 por ciento con los histológicos, para las neoplasias benignas la coincidencia fue del 53,2 por ciento. Conclusiones: las lesiones pseudotumorales predominaron sobre las neoplasias benignas, con un incremento marcado de ambos tipos de lesiones a partir de los 40 años. En el sexo femenino predominaron las lesiones pseudotumorales, y en el masculino predominaron las neoplasias benignas. Se encontró una baja coincidencia entre el diagnóstico clínico e histopatológico para ambos tipos de lesiones(AU)

Introduction: proliferative lesions are entities that are present in the buccal cavity. Some of them are of traumatic origin and others are neoplasias. Frequently, they are not well diagnosed in the clinical practice because of the similarity that can exist among them. Objective: to determine the characteristics of the proliferative lesions diagnosed and the coincidences between clinical and histopathological diagnosis Method: a retrospective study of the biopsies processed during five years in the Department of Oral Pathology of the Faculty of Stomatology of Havana was conducted dealing with age, sex, type of lesion, anatomic location and similarities between the clinical and histopathological diagnosis. Results: it was found that, of the proliferative lesions, the pseudotumoral ones reported the 18.1 percent and the benign neoplasias, the 5.8 percent. In the age group 10-19 years, pseudotumoral lesions represented the 7.78 percent and the benign neoplasias the 1,78 percent. In the group of 60 years or older, pseudotumoral lesions reported the 22.67 percent of the total of proliferative lesions. The female sex resulted to be affected by pseudotumoral lesions in a 61,3 percent and the benign neoplasias effected the male sex in a 55 percent. Fibroepithelial hyperplasia represented the 54.22 percent of the pseudotumoral ones, whereas the nevus reported the 8.67 percent among the benign neoplasias. The clinical diagnosis of the pseudotumoral lesions matched up a 50.4 percent with the histological; for benign neoplasias, the coincidence was 53.2 percent. Conclusions: pseudotumoural lesions predominated on benign neoplasias, with a marked increase in both types of lesions after 40 years. Pseudotumoural lesions predominated in the female sex, whereas the benign neoplasias predominated in the male sex. There was a low coincidence between the clinical and histopathological diagnosis for both types of lesions(AU)

Calcinosis tumoral / Tumoral calcinosis

La calcinosis tumoral (CT) es una enfermedad pseudotumoral rara, de etiología desconocida, que afecta a tejidos blandos periarticulares. Se diagnostica en base a las imágenes radiográficas, tomografía axial computarizada (TAC) y resonancia magnética nuclear (RNM), y su tratamiento es quirúrgico. En este trabajo se presenta un caso de CT y se revisan los conceptos actuales de esta infrecuente enfermedad.

Tumoral calcinosis is a rare pseudotumoral disease ofunknown etiology affecting periarticular soft tissues. Thediagnosis may be advice by radiographic, CT scan and MRimages. Surgery is the elective treatment. A new case oftumoral calcinosis is here presented, reviewing the currentconcepts of this infrequent process.

Schistosoma mansoni granuloma in late evolutive phase, in a case of tumoral form in man / Granuloma equistossomótico em fase evolutiva tardia, em um caso de forma tumoral no homem

INTRODUCTION: Authors describe human schistosomal granuloma in late chronic phase, from the morphological and evolutionary viewpoints. METHODS: The study was based on a histological analysis of two fragments obtained from a surgical biopsy of peritoneum and large intestine of a 42-year-old patient, with a pseudotumoral form mimicking a peritoneal carcinomatosis associated to the schistosomiasis hepatointestinal form. RESULTS: Two hundred and three granulomas were identified in the pseudotumor and 27 in the intestinal biopsy, with similar morphological features, most in the late chronic phase, in fibrotic healing. A new structural classification was suggested for granulomas: zone 1 (internal), 2 (intermediate) and 3 (external). CONCLUSIONS: Regarding granuloma as a whole, we may conclude that fibrosis is likely to be controlled by different and independent mechanisms in the three zones of the granuloma. Lamellar fibrosis in zone 3 seems to be controlled by matrix mesenchymal cells (fibroblasts and myoepithelial cells) and by inflammatory exudate cells (lymphocytes, plasmocytes, neutrophils, eosinophils). Annular fibrosis in zone 2, comprising a dense fibrous connective tissue, with few cells in the advanced phase, would be controlled by epithelioid cells involving zone 1 in recent granulomas. In zone 1, replacing periovular necrosis, an initialy loose and tracery connective neoformation, housing stellate cells or with fusiform nuclei, a dense paucicellular nodular connctive tissue emerges, probably induced by fibroblasts. In several granulomas, one of the zones is missing and granuloma is represented by two of them: Z3 and Z2, Z3 and Z1 or Z2 and Z1 and, ultimately, by a scar.

INTRODUÇÃO: Os autores descrevem o granuloma esquistossomótico no homem, na fase crônica tardia, do ponto de vista morfológico e evolutivo. MÉTODOS: O estudo baseou-se na análise histológica de dois fragmentos obtidos de biópsia cirúrgica do peritônio e do intestino grosso de um paciente de 42 anos de idade, com a forma pseudotumoral mimetizando carcinomatose peritoneal associada à forma hepatointestinal da esquistossomose. RESULTADOS: Foram identificados 203 granulomas no pseudotumor e 27 na biópsia intestinal, com aspectos morfológicos semelhantes, a maioria na fase crônica tardia, em cura por fibrose. Foi sugerida nova classificação estrutural para os granulomas: zona 1 (interna), zona 2 (intermediária) e zona 3 (externa). CONCLUSÕES: Considerando o granuloma como um todo, concluímos que, provavelmente, a fibrose é comandada por mecanismos diferentes e independentes nas três zonas do granuloma. A fibrose lamelar na zona 3 parece ser comandada pelas células mesenquimais da matriz (fibroblastos e células mioepiteliais) e pelas células do exsudato inflamatório (linfócitos, plasmócitos, neutrófilos, eosinófilos). A fibrose anular na zona 2, composta por conjuntivo fibroso denso, pouco celular na fase avançada, seria comandada pelas células epitelioides que envolvem a zona 1 nos granulomas recentes. Na zona 1, substituindo a necrose periovular, a neoformação conjuntiva inicialmente frouxa, rendilhada, albergando células estreladas ou com núcleos fusiformes, surge um conjuntivo denso, paucicelular, nodular, provavelmente induzido pelos fibroblastos. Em muitos granulomas falta uma das zonas descritas e o granuloma é representado apenas por duas delas: Z3 e Z2, Z3 e Z1 ou Z2 e Z1 e, no final, por uma cicatriz.

Fibromatosis mandibular infantil: presentation of a case / presentación de un caso / Infantile mandibular fibromatosis

La fibromatosis constituye una entidad pseudotumoral poco frecuente. Esta se clasifica en juvenil y del adulto. Se caracteriza por una gran agresividad local con tendencia a la recurrencia y a no metastizar. Histológicamente está formada por una neoformación de tejido conectivo fibromatoso que invade las estructuras adyacentes. Se presenta una paciente femenina de 12 años que acude a consulta con un aumento de volumen en la mandíbula derecha de cuatro meses de evolución. En los estudios radiológicos se observa una lesión osteolítica muy vascularizada la cual fue tratada quirúrgicamente con una resección segmentaria mandibular. La evolución post-operatoria es favorable después de seis años de seguimiento.

The fibromatosis is a rare pseudotumoral entity. It is classify in juvenile fibromatosis and adult fibromatosis. The fibromatosis is characterized for a local aggressivity, recurrence but not metastases. Histopathological findings have indicated that fibromatosis is an invasive neoformation of fibromatous connective tissue involving adjacent structures. A 12 year-old female patient is presented that goes to clinic with increase of volume in right mandible of four months of evolution. On radiography studies is observed wide area highly vascular of osteolysis at level of the mandibler body. The lesion was carried out surgically and a segmentary mandible resection was done. After six years of follow out she has presented a satisfactory evolution.

Pseudotumoral hepatic tuberculosis with pericardial abscess.

We report the postmortem findings in a 28-year-old immunocompetent male patient, a rare case of tuberculous liver abscesses with concomitant pericardial abscess in the absence of pleuropulmonary or splenic involvement, who continued to be a diagnostic dilemma. This case report illustrates the difficulty in reaching the correct diagnosis in case of hepatic masses, which are most often confused with carcinoma of the liver, primary or metastatic and, hence, have been aptly referred to as pseudotumoral hepatic tuberculosis in the past.

Fascitis nodular en región facial en edades pediàtricas / Nodular fasciitis present in facial area in children

Propósito: Presentar 3 casos de fascitisnodular de región facial. Método: Se realizó un estudio de los pacientes que comprendió examen físico, anàlisis de laboratorio y radiológico. Se describió la conducta quirúrgica o médica realizada en cada caso y su diagnóstico anatomopatológico. Resultados: Se mostró el método científico como única vía de llegar al diagnóstico certero, a pesar de enfrentar el reto de diagnosticar una entidad nosológica inusual en la cirugía maxilofacial. Se discutió la evolución de cada paciente y la literatura consultada. Conclusiones: Resulta muy útil la presentación de estos casos, pues esta tumoración presenta una localización muy poco frecuente en cara y su rápido crecimiento y evolución clínica son habitualmente difíciles de interpretar(AU)

Aim: To present 3 cases of nodular fasciitis in facial area. Method: A study was carried out in patients including physical examination, laboratory and radiologic analysis. Surgical or medical behavior was described present in each case with an anatomical and the pathological diagnosis was made. Results: Scientific method was the only route to achieve an accurate diagnosis despite the challenge of diagnosing an uncommon disease entity in maxillofacial surgery. In each patient the course was discussed as well as a literature review. Conclusions: The presentation of these cases is very essential since this tumor has a not much location in the face and its fast developmental and clinical course are often difficult to interpret(AU)

Fibromatosis maddibular: presentacion de un caso / Mandible fibroattosis: a case presetaation

La fibromatosis constituye una entidad pseudotumoral poco frecuente, cuya presentación en el maxilar es excepcional. Se caracteriza por una gran agresividad local con tendencia a la recurrencia y por no metastatizar. Puede involucrar hueso o el tejido blando. Se presenta un paciente masculino de 2 años de edad, que acude a consulta con aumento de volumen en región mandibular derecha, de 4 meses de evolución, acompañado de dolor a la presión local. Radiográficamente, se observa amplia zona osteolítica a nivel del cuerpo mandibular que expande la cortical, la adelgaza y en diferentes zonas la destruye totalmente. Se realiza tratamiento quirúrgico bajo anestesia general. El diagnóstico se correspondió con una fibromatosis de la mandíbula. Después de 5 meses se ha presentado una evolución satisfactoria sin recidiva.

The fibromatosis constitutes some entities not very frequent pseudotumorals whose presentation in the maxillary one is exceptional. They are characterized by a great local aggressiveness with tendency to the recurrence and for none metastasize it can involve bone or the soft tissue. A 2 year-old masculine patient is presented that goes to consultation with increase of volume in region right mandible, of four months of evolution, accompanied by pain to the local pressure. Radiography is observed wide area osteolysis at level of the body mandible that expands the cortical one, it loses weight it and in different areas it destroys it totally. He is carried out surgical treatment under it general anesthetizes. Diagnosis, Fibromatosis of the jaw. After five months you evolution has presented a satisfactory evolution without relapse.

Infarto pseudotumoral del cerebelo / Malignant cerebellar infarction

El diagnóstico de infarto cerebeloso en su presentación inicial puede ser difícil, su reconocimiento tardío puede asociarse a graves complicaciones. Aunque sólo representa 2 por ciento a 3 por ciento de los infartos encefálicos, afecta a un importante número de pacientes, muchos de los cuales son jóvenes. De todos los infartos de cerebelo sólo 10 por ciento evolucionará en forma maligna, siendo denominado infarto pseudotumoral de cerebelo. Las causas más habituales son la embolia, la disección de la arteria vertebral y la aterotrombosis. La arteria cerebelosa póstero-inferior es la más frecuentemente comprometida, y en un tercio de los casos se encuentra una fuente cardioembólica. Su rasgo distintivo es el efecto de masa, el mismo que ocasiona compresión de troncoencéfalo e hidrocefalia aguda, generando un deterioro cuantitativo de conciencia. Las neuroimágenes, tomografía computada y resonancia magnética de encéfalo, son de vital importancia para establecer la presencia de un infarto cerebeloso e identificar potenciales complicaciones. Es importante recordar que la tomografía computada es menos sensible que la resonancia magnética para establecer el diagnóstico. El manejo especializado multidisciplinario y la implementación de las medidas de soporte generales y específicas pueden mejorar las posibilidades de sobrevida y recuperación funcional. Ante la presencia de un deterioro de conciencia, un abordaje quirúrgico agresivo pareciera ser la mejor opción de tratamiento.

The diagnosis of cerebellar infarction at initial presentation can be difficult, delayed recognition can be associated with serious complications. Although representing only 2 percent to 3 percent of brain infarcts, affects a significant number of patients, many of whom are young. Only 10 percent of cerebellar infarcts evolve into malignant form, being named pseudotumoral cerebellar infarction. Common causes include embolism, vertebral artery dissection and atherothrombosis. The postero-inferior cerebellar artery is the most frequently committed, and one third of cases there is a cardioemboIic sourse. Its distinguishing feature is the mass effect, causing brain stem compression and acute hydrocephalus, and generating a quantitative impairment of consciousness. The brain imaging, computed tomography and magnetic resonance imaging of brain, are of vital importance to establish the presence of a cerebellar infarct and identify potential complications. It is important to remember that computed tomography is less sensitive than magnetic resonance for diagnosis. The multidisciplinary specialized management and implementation of measures of general and specific support can improve the chances of survival and functional recovery. In the presence of impaired consciousness, an aggressive surgical approach appears to be the best treatment option.

Pseudotumoral form of neuroschistosomiasis: report of three cases

Central nervous system (CSN) involvement in schistosomiasis is an ectopic manifestation with a large variety of clinical forms, including pseudotumoral, which occurs in isolated cases and is rare. Three patients with epidemiological indications of this pathology were examined; the clinical picture included lower-back pain irradiating to lower limbs, associated with progressive flaccid paraparesis and sphincterial disturbances in cases in which the spinal chord was involved; while in cases with encephalitic impairment, headache, dizziness and cerebellar syndrome, characterized by dysarthria and right-side dysgraphia, were present. Magnetic resonance imaging (MRI) showed a growing process in all cases; cerebrospinal fluid (CSF) characteristics and biological markers were compatible with neuroschistosomiasis (NS). Biopsy of the lesions confirmed this diagnosis in one case. After specific treatment with schistosomicides and corticosteroids, clinical, radiological and laboratorial improvement was observed.