Nephron-sparing surgery for bilateral sporadic giant angiomyolipomas

@#A 40-year-old female complains of right flank plain associated with progressive abdominal enlargement. She had stable vital signs and normal renal function. CT urogram revealed bilateral flank masses suggestive of bilateral giant angiomyolipomas. She was counseled on the various treatment options and opted to undergo open surgical excision. She underwent an open clamp-less partial nephrectomy with no intraoperative events. Operative time was 120 minutes and estimated blood loss was 250cc. She was discharged in good clinical condition on postoperative day 4. Final histopathological analysis revealed angiomyolipoma. Genetic testing was positive for mosaic variant of tuberous sclerosis. After a year of follow up, she remains stable and is maintained on everolimus. Open ischemia-free partial nephrectomy may be done safely for giant renal angiomyolipomas. Radical nephrectomy should be reserved for the last option because the presence of contralateral disease may also require surgical excision in the future.

An excerpt of 2022 European Association for the Study of the Liver clinical practice guidelines on the management of cystic liver diseases / 中华消化杂志

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in-depth review of the relevant literature, this guideline provide recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence-Based Medicine system and categorized as "weak" or "strong" . This guideline aims to provide the best available evidence to aid the clinical decision-making process in the management of patients with cystic liver disease, and presents the readers with translations and summarizations of the above mentioned recommendations.

Retinal Hamartomas in a case of Tuberous Sclerosis

A 21-year-old male patient suffering from epilepsy came for fundoscopic evaluation. Right eye showed a ?at, smooth surfaced well circumscribed, non-calci?ed translucent lesion of 1 disc dioptre size, inferior to the optic disc, obscuring underlying vessels & an opaque, calci?ed, yellow white lesion having irregular surface & margins with mulberry appearance, elevated, supero-nasal to the disc, obscuring the underlying vessels. Adenoma sebaceum, periungual ?bromas, Ash leaf macules were seen on general physical examination suggestive of tuberous sclerosis. MRI brain revealed subependymal nodules & subcortical tubers. OCT showed an optically hyper re?ective mass with retinal disorganization and moth-eaten spaces. 2D echo showed mild Left Ventricular Hypertrophy, diastolic dysfunction, trivial mitral and tricuspid regurgitation. Ultrasonography of abdomen & pelvis showed fatty liver and renal angiomyolipoma. It was interesting to note cardiac & hepatic involvement in addition to the standard tuberous sclerosis complex (TSC) major & minor diagnostic criteria

Ocular Manifestations In Tuberous Sclerosis-A Case Report

Tuberous Sclerosis is a multisystem disorder characterised by hamartomatous growth in any organ. Ophthalmic manifestation can be retinal or non- retinal. A 24 yr. old female diagnosed as Tuberous sclerosis was referred from Dermatology department. Full Ophthalmic examination was done. Patient was diagnosed with adenoma sebaceum of lid and retinal hamartoma in right eye

A Primer on Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Medically Refractory Epilepsy

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

A Primer on Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Medically Refractory Epilepsy

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

Síndrome de Cowden / Cowden syndrome

El síndrome de Cowden es una enfermedad hereditaria, de transmisión autosómica dominante, caracterizada por la presencia de múltiples hamartomas y nódulos en la piel y la mucosa oral, junto con anomalías en mamas, tiroides y pólipos en el tracto gastrointestinal, con un riesgo incrementado de tumores malignos. Se reporta un caso de una paciente con diagnóstico de bocio tóxico nodular, y que presentaba -por los antecedentes y estudios realizados- manifestaciones compatibles con el síndrome de Cowden. El síndrome de Cowden es el síndrome hamartomatoso tumoral del phosphatase and tensinhomolog mejor descrito hasta el momento. Los pacientes con él tienen lesiones mucocutáneas características y un elevado riesgo de cáncer de mama, tiroides, endometrio, colorrectal y renal, así como varias manifestaciones benignas como macrocefalia y gangliocitomadisplásico del cerebelo. Es importante el diagnóstico precoz de este síndrome y el seguimiento a largo plazo, dado el alto riesgo de desarrollar tumores malignos(AU)

Cowden syndrome is a hereditary disease, of autosomal dominant transmission, and characterized by the presence of multiple hamartomas and nodules in the skin and oral mucosa, and also with abnormalities in the breast, thyroid, and polyps in the gastrointestinal tract with an increased risk of malignant tumors. It is reported a case of a patient with a diagnosis of toxic nodular goiter, and who presented -due to the antecedents and studies carried out- manifestations compatible with the Cowden syndrome. Cowden syndrome is the hamartomatous tumor syndrome of phosphatase and tensin homolog which is better described so far. Patients having it present characteristic mucocutaneous lesions and a high risk of breast, thyroid, endometrial, colorectal and renal cancers, as well as several benign manifestations such as macrocephaly and gangliocytoma of the cerebellum. Early diagnosis of this syndrome and long-term follow-up are important given the high risk of developing malignant tumors(AU)

Odontoma compuesto. presentación de un caso / Odontoma compound. presentation of a case

Se presentó el caso clínico de un niño de 8 años de edad, que acude a consulta de Estomatología del Policlínico "Mártires de Jamaica", del municipio Manuel Tames, de la provincia Guantánamo, acompañado de la madre preocupada por la no erupción del diente 21. Al examen clínico se observó ausencia clínica del 21 con espacio para su ubicación con ligero aumento de volumen en el fondo del surco vestibular a nivel de la zona de consistencia dura a la palpación. Se indicó radiografía panorámica observándose imagen radiolúcida con numerosas formaciones radioopacas en su interior que asemejan estructuras dentarias y una banda radiolúcida en su periferia que presuntivamente se diagnosticó como un odontoma compuesto, se remite al segundo nivel de atención para excéresis y diagnóstico definitivo. Se discuten algunas características de su etiología, diagnóstico y tratamiento(AU)

It was presented the clinical case of an 8-year-old boy, who was attended in the clinic of "Mártires de Jamaica" Polyclinic, in Manuel Tames municipality of Guantanamo province, accompanied by the mother concerned about the non-eruption of tooth 21 .On clinical examination, was observed absence of tooth 21 with space for its location with slight increase in volume at the bottom of the vestibular sulcus at the level of the area hard to palpation. Panoramic x-ray is indicated, with a radiolucent image with numerous radio opaque formations that resemble dental structures and a radiolucent band in its periphery presumptively diagnosed as a compound odontoma. It is referred to the second level of attention for excresis and definitive diagnosis. Some characteristics of its etiology, diagnosis and treatment were discussed(AU)

Síndrome de Peutz-Jeghers: novos dados e bases moleculares sobre uma doença rara / The Peutz-Jeghers syndrome: new data and molecular bases on a rare disease

A síndrome de Peutz-Jeghers é uma associação de polipose no trato gastrointestinal do tipo familiar e pigmentação melânica mucocutânea, particularmente nos lábios, mucosa bucal e polpa digital. Há poucas décadas, algumas centenas de casos haviam sido descritos na literatura, o que torna a doença não tão rara. O objetivo deste trabalho foi relatar casos clínicos em que os pacientes eram portadores da síndrome. Em um dos casos, tia e sobrinha evidenciaram a implicação genética da doença. Dados clínicos, bases moleculares e resultados histopatológicos, com a utilização de anticorpos (Ac) monoclonais, mostram a relevância do trabalho. Portadores crônicos da síndrome após décadas, ainda não diagnosticados, procuram o cirurgião-dentista para tratamentos diversos, dando-lhe oportunidade e responsabilidade na detecção da doença, devido ao risco de desenvolvimento de tumores malignos no trato gastrointestinal.

The Peutz-Jeghers syndrome is a familiar association of polyposis in the gastrointestinal tract and melanic mucocutaneous pigmentation, particularly in the lips, oral mucosa and fi ngertips. A few decades few hundred cases had been described in the literature wich makes the disease not so rare. The objective of this study is to report clinical cases where patients were carriers of the syndrome. In one of the cases the aunt and nice showed the genetic implication of the disease. Clinical data, molecular basis and histopathological results with monoclonal antibodies (Ac), show the relevance of this work. Chronic carriers of the syndrome after decades, still undiagnosed, seek the dentist for various treatments giving him the opportunity and responsibility to detect the disease due to the risk of development of malignant tumors in the gastrointestinal tract.

Estudio clínico genético en pacientes con complejo de esclerosis tuberosa / Clinical and genetic study patients with tuberous sclerosis complex

El complejo de esclerosis tuberosa (CET) es una enfermedad autosómica dominante multisistémica producida por mutaciones en los genes supresores de tumores TSC1 o TSC2. Objetivo: Caracterizar clínica y genéticamente pacientes pediátricos con diagnóstico de CET. Pacientes y Método: Estudio descriptivo de registros clínicos de 42 pacientes pediátricos controlados en un servicio de neuropsiquiatría infantil con diagnóstico de CET y estudio genético en 21 de ellos. Se amplificó por reacción en cadena de la polimerasa y secuenció el exón 15 del gen TSC1 y los exones 33, 36 y 37 del gen TSC2. Se analizó la relación entre las mutaciones encontradas con la severidad y evolución clínica. Resultados: En el 61,9% de los pacientes las manifestaciones comenzaron antes de los 6 meses de edad. Las manifestaciones iniciales de CET más frecuentes fueron las crisis convulsivas (73,8%) y el hallazgo de rabdomiomas cardiacos (16,6%). Durante su evolución, todos los pacientes presentaron compromiso neurológico; el 92,9% presentó epilepsia. Todos los pacientes presentaron máculas hipomelanóticas, 47,6% pangiofibromas faciales, 23,8% parches de Shagreen, 47,6% rabdomiomas cardiacos y 35,7% hamartomas retinianos. El estudio genético realizado a 21 pacientes identificó 2 mutaciones heterocigotas patogénicas en TSC1 y una en TSC2. Este último paciente presentaba un fenotipo clínico más severo. Conclusiones: Las manifestaciones neurológicas y dermatológicas fueron las más frecuentes en los pacientes con CET. Se identificaron 2 mutaciones patogénicas en el gen TSC1 y una en el gen TSC2. La mutación en TSC2 se manifestó en un fenotipo clínico más severo.

Tuberous sclerosis complex (TSC) is a multisystem autosomal dominant disease caused by mutations in the tumor suppressor genes TSC1 or TSC2. Objective: To characterize clinically and genetically patients diagnosed with TSC. Patients and Method: Descriptive study of clinical records of 42 patients from a pediatric neuropsychiatry department diagnosed with TSC and genetic study in 21 of them. The exon 15 of TSC1 gene and exons 33, 36 and 37 of TSC2 gene were amplified by polymerase chain reaction and sequenced. The relationship between the mutations found with the severity and clinical course were analyzed. Results: In 61.9% of the patients the symptoms began before 6 months of age. The initial most frequent manifestations of TSC were new onset of seizures (73.8%) and the detection of cardiac rhabdomyomas (16.6%). During the evolution of the disease all patients had neurological involvement; 92.9% had epilepsy. All patients presented hypomelanotic spots, 47.6% facial angiofibromas, 23.8% Shagreen patch, 47.6 heart rhabdomyomas and 35.7% retinal hamartomas. In the genetic study of 21 patients two heterozygous pathogenic mutations in TSC1 and one in TSC2 genes were identified. The latter had a more severe clinical phenotype. Conclusions: Neurological and dermatological manifestations were the most frequent ones in patients with TSC. Two pathogenic mutations in TSC1 and one in TSC2 genes were identified. The patient with TSC2 mutation manifested a more severe clinical phenotype.

Síndrome de Cowden. Presentación de un caso infrecuente / Cowden syndrome. Presentation of an infrequent case

El síndrome de Cowden es una enfermedad hereditaria, de transmisión autosómica dominante, caracterizada por la presencia de múltiples hamartomas y nódulos en piel y mucosa oral, junto con anomalías en mama, tiroides y pólipos en el tracto gastrointestinal, tendiendo a malignizarse sobre todo en mama y tiroides. El diagnóstico precoz a partir de las lesiones en piel y alteraciones digestivas pueden redirigir el estudio buscando malignidades en tiroides y en caso que sea una fémina en mama. Se presenta un caso clínico que debuta por trastornos digestivos que se interpretan como un sangramiento digestivo alto, al realizar la endoscopia se observan múltiples pólipos duodenales que junto a las alteraciones de la piel hacen el diagnóstico. La presencia de pápulas faciales cutáneas y la papilomatosis de la mucosa oral son los criterios clínicos mayores para establecer el diagnóstico del síndrome de Cowden debiendo alertar al clínico ante la posibilidad de otras lesiones neoplásicas, a veces ocultas, sobre todo en mama, tiroides y tubo digestivo con carácter maligno.

Cowden syndrome is an inherited disease, of dominant auto somatic transmission, characterized by the presence of multiple hamartomas and nodules in skin and oral mucosa, together with anomalies in breasts and thyroids, and polyps in the gastrointestinal tract; they tend to become malignant, above all in breast and thyroids. The precocious diagnosis after finding skin lesions and digestive disturbances should redirect the study, looking for malignancy in thyroids and, in cases of women patients, in breast. We presented a clinical case debuting with digestive disturbances interpreted as upper digestive bleeding. During the endoscopy, several duodenal polyps were observed that together with skin disturbances led to the diagnosis. The presence of skin facial papules and the oral mucosa papillomatosis are the bigger clinical criteria to stablish the Cowden syndrome diagnosis; they should raise the clinician alert toward the possibility of other neoplastic lesions, sometimes hidden, especially in the breast, thyroids and alimentary canal, with malignant character.

Giant hypothalamic hamartoma associated with an intracranial cyst in a newborn

We report the case of a giant hypothalamic hamartoma with a large intracranial cyst in a neonate. On ultrasonography, the lesion presented as a lobulated, mass-like lesion with similar echogenicity to the adjacent brain parenchyma, located anterior to the underdeveloped and compressed left temporal lobe, and presenting as an intracranial cyst in the left cerebral convexity without definite internal echogenicity or septa. The presence of a hypothalamic hamartoma and intracranial neurenteric cyst were confirmed by surgical biopsy. The association of a giant hypothalamic hamartoma and a neurenteric cyst is rare. Due to the rarity of this association, the large size of the intracranial cyst, and the resulting distortion in the regional anatomy, the diagnosis of the solid mass was not made correctly on prenatal high-resolution ultrasonography.

The importance of the dentist in the diagnosis of cowden syndrome – Case report

Cowden Syndrome is an autosomal dominant disorder characterized by the development of several hamartomas in a variety of tissues, including the skin, gastrointestinal tract, adipose tissue, and bone and connective tissues. This syndrome is associated with a high risk of developing malignancies, especially breast, thyroid and endometrium cancers. This report presents a case of a 53-year-old patient who sought assistance at the Dentistry Department of the University Hospital of Brasilia (HUB) to receive basic dental treatment. The current and past medical history and oral and facial manifestations led to the diagnosis of Cowden syndrome. This case report aims to highlight the importance of a dental surgeon in the diagnosis of this syndrome, based on the observation of oral manifestations and medical history.

A Síndrome de Cowden é uma desordem hereditária autossômica dominante, caracterizada pelo desenvolvimento de diversos hamartomas em vários tipos de tecidos, incluindo a epiderme, trato gastrointestinal, tecido adiposo e tecidos ósseo e conjuntivo. Essa síndrome é associada a um risco elevado de desenvolvimento de malignidades, especialmente câncer de mama, tireoide e endométrio. Esse relato apresenta o caso de um paciente de 53 anos de idade que procurou o Departamento de Odontologia do Hospital Universitário de Brasília (HUB) para tratamento dental básico. História médica atual e passada e manifestações orais e faciais levaram ao diagnóstico de síndrome de Cowden. Este relato de caso apresenta a importância do cirurgião-dentista no diagnóstico desta síndrome, com base na observação das manifestações orais e história médica.

Hamartomas hipotalámicos: distintas formas de debut. Casos clínicos / Hypothalamyc hamartomas: different ways of clinical debut. Cases report

Los hamartomas hipotalámicos son malformaciones no neoplásicas de sustancia gris compuestas por neuronas hiperplásicas. Suelen ser lesiones pequenas localizadas en la base del cerebro, en el piso del tercer ventrículo y, generalmente, asintomáticas. Sin embargo, pueden ocurrir con alteraciones conductuales-cognitivas, crisis epilépticas y/o signos de pubertad precoz central en función de la localización en la que se encuentren. Se presentan dos pacientes de 2 años 8 meses y 7 años, con presencia de hamartomas hipotalámicos diagnosticados tras el estudio de pubertad precoz central. La paciente de menor edad presenta, además, crisis gelásticas, típicamente asociadas a hamartomas hipotalámicos. Tras los hallazgos clínicos y radiológicos, se trataron con análogos de gonadotropinas, y se observó una regresión de los signos puberales y una no progresión del tamano de los hamartomas.

Hypothalamic hamartomas are benign tumors of gray substance composed by hyperplasic neurons. They are usually asymptomatic small masses with extensions into the third ventricular cavity. In some instances they can cause cognitive behavioral alterations, seizures and/or central precocious puberty depending on the location. Here we present two cases of central precocious puberty due to hypothalamic hamartomas at 2(8/12) and 7 years of age. The younger patient also presents gelastic seizures, typically associated with hypothalamic hamartomas. After the clinical and radiological findings, they started treatment with GnRH analogues and a regression of the puberty signs without progression in the hamartomas size was observed.

Odontoma, revisión de la literatura a propósito de un caso clínico / Odontoma, literature review regarding a clinical case

Los odontomas son los tumores odontogénicos más frecuentes. Se presenta una revisión de la literatura, seguida de un reporte de un caso de odontoma complejo en el maxilar superior en un paciente de sexo masculino de 10 años de edad asociado con la presencia de piezas deciduas. El tumor fue enucleado y se realizó la correspondiente biopsia para confirmación de el diagnóstico clínico.

Odontomas are the most common odontogenic tumors. A literature review of the odontomas is presented followed by a case report of a complex odontoma of the anterior maxillar in a 10 year old male patient, associated with the persistence of deciduous teeth. The tumor was enucleated and the corresponding biopsy was performed which confirmed the clinical diagnosis

Neural, renal and retinal hamartomas with cutis vertis gyrata a rare presentation in tuberous sclerosis complex.

Tuberous sclerosis complex characterised by multiple benign tumours, is caused by mutation in the genes TSC1 and TSC2 coding for Hamartin and Tuberin respectively. We report a case of a 17 year old female patient who presented with classical Vogt’s triad characterized by seizures, mental retardation and adenoma sebaceous. She presented with Cutis Vertis Gyrata in addition to multiple retinal astrocytic hamartomas in her right eye with retinal pigment epithelium changes in both eyes and a normal anterior segment examination. Magnetic resonance imaging (MRI) of the brain showed subependymal giant cell astrocytomas and cortical tubers. Ultrasound of the abdomen showed bilateral renal angiomyoliposis. We are presenting this case as retinal hamartomas with five Major criteria are seen along with secondary Cutis Vertis Gyrata which is an extremely rare presentation of Tuberous sclerosis. Absence of most of the Minor criteria is not to be excluded.

Epidermal nevus syndrome associated with ocular symblepharon and gum hypertrophy – A rare variant.

Epidermal nevi are congenital hamartomas of embryonal ectodermal origin classified on the basis of their main component. The component may be sebaceous, apocrine, eccrine, follicular or keratinocytic. An estimated 1/3 of individuals with epidermal nevi have involvement of other organ systems; hence this condition is considered to be epidermal nevus syndrome. Solomon defined epidermal nevus syndrome as a sporadic neuro-cutaneous linkage of congenital ectodermal defects in the skin, brain, eyes and skeleton, now also referred to as Solomon syndrome. The syndromes are uncommon. Mortality and morbidity are associated with systemic anomalies.

Nefroblastomatosis difusa bilateral: Presentación de caso / Bilateral Diffuse Nephroblastomatosis: Case Report

En los niños, la nefroblastomatosis ocurre como resultado de la persistencia de blastema metanéfrico en el riñón. Este artículo presenta el caso de la presentación más rara de esta enfermedad, que es la universal, en un recién nacido que fallece por una nefropatía rápidamente progresiva. Los hallazgos imaginológicos son relevantes para el diagnóstico, la estatificación y la diferenciación de la enfermedad. Se compara con el tumor de Wilms y sus hallazgos en imágenes. La tomografía axial computarizada y la resonancia magnética tienen alta sensibilidad y pueden detectar lesiones tan pequeñas como de 5 mm, por lo cual es importante conocer las características de esta patología y su comportamiento en las diferentes modalidades de imágenes.

In children, nephroblastomatosis occurs as a result of persistent metanephric blastema in the kidney. This article presents the most unusual presentation of this disease, which is the universal, in a newborn who died of a rapidly progressive nephropathy. Imaging findings are relevant for the diagnosis, staging and differentiation of the disease. We compare it to Wilms tumor and its imaging findings. CT and magnetic resonance imaging have high sensitivity and can detect lesions as small as 5mm. For this reason, it is important to know the characteristics of this pathology and its behavior in different imaging modalities

Linfangioma de cabeça e pescoço: levantamento de casos / Lymphangioma of the head and neck: survey of cases

Os linfangiomas são tumores benignos de vasos linfáti-cos que afetam geralmente a cabeça e o pescoço. Esses podem ter origem congênita ou constituir lesões que se desenvolvem ao longo da vida, acometendo sobretudo crianças. Podem ser tratados cirurgicamente, por subs-tâncias esclerosantes ou por meio de radioterapia, no entanto, sua recidiva acontece entre 10% e 40% dos casos. Materiais e método: o objetivo deste estudo foi realizar uma pesquisa epidemiológica dos casos de lin-fangioma registrados nos laboratórios de Patologia do Instituto de Ciências Biológicas da Universidade de Pas-so Fundo (ICB/UPF) e do Hospital São Vicente de Paulo de Passo Fundo/RS (HSVP) desde o ano 1987 até 2012. Resultados: ao todo, foram catalogados 78 casos de lin-fangioma. Desses, 69 acometeram a região de cabeça e de pescoço (88%) e os demais estavam localizados em intestino, baço, costelas, membros superiores, peito e axila, sendo a primeira década de vida a faixa etária mais acometida. O aumento de volume local foi o sinal mais observado. Após a aplicação do teste qui-quadrado a 5% de significância, notou-se haver relação entre o gênero dos pacientes e a localização dos linfangiomas (p=0,0003). Conclusão: verificou-se que os homens, na primeira década de vida, foram os mais acometidos. Houve relação entre sexo e localização, sendo mais co-muns os linfangiomas de crânio em pacientes do sexo masculino. Além disso, nos linfangiomas estudados foi observada a presença de infecção local e o tratamento foi realizado por meio de cirurgia

Odontoma compuesto: casos clínicos y revisión de la literatura / Compound odontoma: case reports and review of the literature

Objetivo: el objetivo de este trabajo es describir los odontomas compuestos desde una perspectiva actual, a partir de casos clínicos. Casos clínicos: se presentan cuatro casos de niños a los que se les realizó la enucleación de un odontoma, cuyas biopsias confirmaron el diagnóstico inicial. En los controles a distancia, no se registraron recidivas en ninguno de los casos. Conclusión: un odontoma compuesto está constituido por esmalte, dentina y, ocasionalmente, cantidades variables de cemento y pulpa que forman un conjunto de estructuras similares a dientes denominadas dentículos. Su etiología es desconocida. Son asintomáticos, por lo cual, generalmente, se diagnostican como hallazgos radiográficos. El tratamiento consiste en la escisión quirúrgica del odontoma.