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1.
Journal of Clinical Otorhinolaryngology Head and Neck Surgery ; (12): 338-343, 2023.
Artigo em Chinês | WPRIM | ID: wpr-982745

RESUMO

〓 Objectives: To analyze the pathological and clinical features of nasal respiratory epithelial adenomatoid hamartoma(REAH), and summarize the diagnostic points, to improve the experience of diagnosis and treatment. Methods:The clinical data of 16 patients with REAH were analyzed retrospectively. The clinical manifestations, pathological features, imaging features, surgical treatment and prognosis were summarized. Results:16 cases of REAH were studied, 10 cases(62.50%) were associated with sinusitis, 1 case(6.25%) was associated with inverted papilloma, 1 case(6.25%) was associated with hemangioma. 5 cases(31.25%) had a history of nasal sinus surgery, including 1 case with 3 times of nasal sinus surgery, 1 case with 2 times of nasal sinus surgery, 3 cases with 1 time of nasal sinus surgery; 10 cases(62.50%) occurred in the bilateral olfactory cleft, 2 cases(12.50%) in the unilateral olfactory cleft, 3 cases(18.75%) in the unilateral middle turbinate, 1 case(6.25%) in the nasopharynx. All 16 patients were pathologically diagnosed as REAH. In the patients with lesions located in bilateral olfactory fissures, symmetrical widening of olfactory fissures and lateral displacement of middle turbinate were observed on preoperative sinus CT. The average width of bilateral olfactory fissures was (9.9±2.70) mm. The ratio of wide to narrow olfactory cleft was 1.21 ± 0.19. There was no significant difference in Lund-Mackay score between the two sides(P>0.05). All patients underwent surgery under general anesthesia and nasal endoscopy. The follow-up period ranged from 1 to 66 months, and no recurrence occurred. Conclusion:Preoperative diagnosis of REAH is facilitated by the combination of clinical manifestations and endoscopic and imaging features. Endoscopic complete resection can achieve a good therapeutic effect.


Assuntos
Humanos , Pólipos Nasais/complicações , Estudos Retrospectivos , Seios Paranasais/patologia , Adenoma , Endoscopia/métodos , Hamartoma/cirurgia
2.
J. coloproctol. (Rio J., Impr.) ; 42(2): 190-192, Apr.-June 2022. ilus
Artigo em Inglês | LILACS | ID: biblio-1394415

RESUMO

Retrorectal hamartoma, also called tailgut cyst, is a congenital lesion resulting from the non-regression of embryo remnants of the hindgut. We describe in this work the case report of a 68-year-old man, previously healthy, complaining of rectal bulging for 4 months, which was diagnosed by nuclear magnetic resonance imaging as a multi-loculated and mucinous lesion, suggestive of tailgut cyst. In view of the finding, the lesion was surgically resected, due to the potential for future complications, and, through the anatomopathological analysis, there were no findings of malignancy in the specimen. (AU)


Assuntos
Humanos , Masculino , Idoso , Doenças Retais/diagnóstico por imagem , Hamartoma/diagnóstico por imagem , Doenças Retais/cirurgia , Imageamento por Ressonância Magnética , Hamartoma/cirurgia
3.
Journal of Central South University(Medical Sciences) ; (12): 529-534, 2022.
Artigo em Inglês | WPRIM | ID: wpr-928998

RESUMO

The clinical data for a patient with primary lung adenocarcinoma complicated with pulmonary hamartoma, who admitted to Zunyi Medical University Hospital in September 2020, was retrospectively analyzed. The 62-years-old male visited outpatient service because of dysphagia in March 2015, and the pulmonary nodules were found. In September 2020, the computed tomography indicated the enlarged nodule in the lower lobe of left lung with lobulation, and there was ground glass nodule in the upper lobe of left lung. After thoracoscopic wedge surgery, the primary pulmonary adenocarcinoma in the upper lobe of left lung and pulmonary hamartoma in the lower lobe of left lung were confirmed by pathology. Whole exon sequencing revealed that kinesin family member 20B (KIF20B) gene was not expressed in lung adenocarcinoma, but was expressed in pulmonary hamartoma. The clinical manifestations of lung adenocarcinoma complicated with pulmonary hamartoma was not typical, which could locate in the same side and different sides of the lung. The imaging manifestations of the 2 kinds of tumors were diverse and can not be completely distinguished. The pathological examination after surgery is the gold standard, and the possibility of malignant transformation of pulmonary hamartoma should be warned.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Adenocarcinoma de Pulmão/complicações , Hamartoma/cirurgia , Cinesinas , Pulmão/patologia , Neoplasias Pulmonares/cirurgia , Estudos Retrospectivos
4.
Arch. argent. pediatr ; 117(5): 519-522, oct. 2019. ilus
Artigo em Inglês, Espanhol | LILACS, BINACIS | ID: biblio-1054975

RESUMO

El hamartoma mesenquimal rabdomiomatoso es una lesión cutánea rara descripta por primera vez en 1986 como "hamartoma de músculo estriado". En general, se presenta en la región de la cabeza y el cuello de los recién nacidos. En este artículo, describimos el caso de una niña de 38 días con un apéndice cutáneo congénito en la región perianal. En el examen físico, no se observaron anomalías congénitas ni otras lesiones cutáneas. En el examen histopatológico, se observó un hamartoma con fibras de músculo esquelético desorganizadas. El diagnóstico diferencial incluyó apéndice cutáneo, trago accesorio y fibroma péndulo. El hamartoma mesenquimal rabdomiomatoso se diferencia de las lesiones mencionadas debido al componente de músculo estriado. Dado que no conlleva el riesgo de recurrencia ni de transformación a neoplasia maligna, no es muy relevante diferenciarlo de estas lesiones. Sin embargo, es importante establecer el diagnóstico correcto porque aproximadamente un tercio de los casos se asocian con anomalías congénitas. Asimismo, es necesario un diagnóstico histopatológico en los niños con ubicación perianal debido a las manifestaciones clínicas similares al rabdomiosarcoma.


Rhabdomyomatous mesenchymal hamartoma is a rare dermal lesion which was first described in 1986 as "striated muscle hamartoma". It usually develops in the head and neck region of newborns. We report a 38-day-old girl with a congenital skin tag in the perianal region. Physical examination did not reveal any congenital abnormalities or other dermal lesions. Histopathological examination showed a hamartoma with disorganized skeletal muscle fibers. The differential diagnosis includes skin tag, accessory tragus and soft fibroma. Rhabdomyomatous mesenchymal hamartoma differs from the listed lesions with its striated muscle component. Since it does not carry the risk of recurrence and malignant transformation, it is not very important to distinguish it from these lesions. However, a correct diagnosis is important because approximately one third of the cases are associated with congenital anomalies. Also, histopathological diagnosis should be made in children with perianal localization due to similar clinical manifestation of rhabdomyosarcoma.


Assuntos
Humanos , Feminino , Lactente , Rabdomioma/diagnóstico , Hamartoma/diagnóstico , Neoplasias do Ânus , Rabdomioma/cirurgia , Rabdomioma/patologia , Hamartoma/cirurgia , Hamartoma/patologia
6.
Revista Digital de Postgrado ; 7(1): 38-40, jun. 2018. ilus
Artigo em Espanhol | LILACS, LIVECS | ID: biblio-1096378

RESUMO

El Hamartoma Condromesenquimal Nasal (HCMN) es una neoplasia descrita por McDermott et al en 1998 en pacientes pediátricos, con pocos casos reportados en adultos. Este tumor está constituido por tejido condroide o cartilaginoso así como por tejidos mesenquimales. Es una lesión expansiva, localmente destructiva y en algunos casos está descrita la extensión intracraneal. El comportamiento es habitualmente benigno y el tratamiento consiste en la resección quirurgica. Se presenta un caso de un paciente masculino de 8 meses de edad, quien presentaba aumento de volumen en región periorbitaria derecha, con pseudoptosis palpebral, paresia del nervio oculomotor derecho y rinorrea hialina. Mediante la Tomografia Computarizada (TC) craneal y la Resonancia Magnética (RM) cerebral se observó lesión ocupante de espacio naso fronto etmoidal. El estudio histopatológico e inmunohistoquimico determinó la presencia de tumor mesenquimal fibrocartilaginoso osificante: Hamartoma Condromesenquimal. Se revisan las características y el tratamiento de este tumor poco frecuente(AU)


The Chondromesenchymal Nasal Hamartoma (CMNH) is a rare neoplasm described in 1998 by McDermott et al in the pediatric age, with few cases reported in adults. This tumor is composed by chondroid or cartilaginous tissue as well as mesenchymal tissues. It is an expansive lesion, locally destructive and in some cases intracranial extension is described. Its behavior is usually benign and its treatment consists of surgical resection. The authors present an 8-month-old male patient with increase volume in the right periorbital region, palpebral ptosis, paresis of the right oculomotor nerve and hyaline rhinorrhea. Computed Tomography (CT) and Cerebral Magnetic Resonance Imaging (MRI) were perfomed and revealed a nasofrontoethmoidal space occupying lesion; a histopathological and inmunohistochemical study resulted in osseous fibrocartilaginous mesenchymal tumor: chondromesenchymal hamartoma. We review the characteristics and treatment of this rare tumor(AU)


Assuntos
Humanos , Masculino , Lactente , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/fisiopatologia , Hamartoma/cirurgia , Hamartoma/patologia , Pediatria , Espectroscopia de Ressonância Magnética , Tomografia
7.
Rev. bras. cir. plást ; 30(2): 303-310, 2015. ilus
Artigo em Inglês, Português | LILACS | ID: biblio-1005

RESUMO

INTRODUÇÃO: A síndrome de Proteus é uma doença complexa e rara, classificada nos grupo das hamartoses. Foi primeiramente descrita em dois pacientes, em 1979, por Cohen e Hayden. Existe dificuldade no diagnóstico, sendo comum a confusão com síndromes de Klippel-Trenaunay-Weber, neurofibromatose ou Stuge-Weber. Apresentamos dois casos tratados no Serviço de Cirurgia Plástica e Reparadora da Universidade Federal do Paraná. MÉTODO: Paciente masculino (caso 1), que chegou ao serviço aos 6 anos de idade, tendo como principais apresentações lipomatoses e assimetrias. A segunda paciente (caso 2) deu entrada no serviço com 20 anos de idade e diagnóstico de síndrome de Klippel-Trenaunay-Weber, que posteriormente mostrou se tratar de síndrome de Proteus. CONCLUSÃO: A hipótese etiológica mais aceita para a doença é genética. Acredita-se que exista mosaicismo somático e que a doença seja letal no estado não mosaico. Morte prematura é bastante frequente. Entretanto, a sequela mais comum é a ocorrência de tumores incomuns. O cuidado dos pacientes portadores da síndrome é um desafio devido às suas consequências médicas e psicossociais.


INTRODUCTION: Proteus syndrome is a complex and rare disorder classified as a hamartomatous disease. It was first described in two patients in 1979, by Cohen and Hayden. Proteus syndrome is difficult to diagnose, and is often confused with Klippel-Trenaunay-Weber syndrome, neurofibromatosis, or Sturge-Weber syndrome. In this study we describe two patients who were treated at the Plastic and Reconstructive Surgery Service of the Federal University of Paraná. METHOD: A 6-year-old male patient (case 1) presented to the Service with lipomatosis and asymmetry, as the primary findings. A 20-year-old (case 2) was admitted to the Service with a diagnosis of Klippel-Trenaunay-Weber syndrome, which later was shown to be Proteus syndrome. CONCLUSION: The etiological hypothesis that is most accepted for this disease is genetic. It is believed that somatic mosaicism may occur during pathogenesis, which can be lethal in the mosaic state. Premature death is common. However, the most common sequelae are the occurrence of unusual tumors. The care of patients with this syndrome is a challenge due to medical and psychosocial consequences.


Assuntos
Humanos , Masculino , Feminino , Criança , História do Século XXI , Adulto Jovem , Cirurgia Plástica , Síndrome do Hamartoma Múltiplo , Síndrome de Proteu , Doenças Raras , Gigantismo , Hamartoma , Doenças Genéticas Inatas , Lipomatose , Cirurgia Plástica/métodos , Síndrome do Hamartoma Múltiplo/cirurgia , Síndrome do Hamartoma Múltiplo/mortalidade , Síndrome do Hamartoma Múltiplo/patologia , Síndrome de Proteu/cirurgia , Síndrome de Proteu/patologia , Doenças Raras/patologia , Gigantismo/cirurgia , Gigantismo/patologia , Hamartoma/cirurgia , Hamartoma/patologia , Doenças Genéticas Inatas/cirurgia , Doenças Genéticas Inatas/patologia , Lipomatose/cirurgia , Lipomatose/patologia
8.
J. coloproctol. (Rio J., Impr.) ; 34(4): 260-264, Oct-Dec/2014. ilus
Artigo em Inglês | LILACS | ID: lil-732567

RESUMO

Introduction: Tailgut cysts or cystic hamartomas are rare developmental tumors of the pre-sacral space. Their true incidence is not well known but we must think on it when we have a patient with a multicystic uncapsulated tumor at presacral space. Tailgut cysts are often asymptomatic, and in other cases present with chronic perineal pain, constipation or rectal tenesmus. They must be treated to avoid complications such as infections (perianal fistula or abscess) and malignant degenerations (usually adenocarcinoma). Case report: One patient with anal pain was diagnosed with a perianal abscess. He was operated but at the second month review a MRI revealed a multilocular lesion in the presacral suggesting the presence of a retrorectal cystic hamartoma. An elective operation was performed by endoanal surgical approach using TAMIS. En bloc resection of the cyst was achieved with safe margins. The pathology result reported retrorectal cystic hamartoma. The patient was asymptomatic with no signs of recurrence in subsequent controls. Our experience, despite being based on one case, is evidence that TAMIS (Transanal Minimally Invasive Surgery) allows a minimally invasive dissection with similar benefits as the use of TEM/TEO devices. (AU)


Introdução: Tail gut cysts ou hamartomas císticos são tumores raros de desenvolvimento do espaço pré-sacral. Sua verdadeira incidência não é bem conhecida, mas devemos pensarnele quando temos um paciente com um tumor uncapsulated multicística no espaço présacral. Hamartomas císticos são muitas vezes assintomáticas, em outros casos, apresentase com dor perineal crônica, constipação ou tenesmo retal. Eles devem ser tratados para evitar complicações, como infecções (fístula perianal ou abscesso) e degenerações malignas (geralmente adenocarcinoma). Relato de Caso: Um paciente com dor anal foi diagnosticado com um abscesso perianal. Ele foi operado, mas na revisão do segundo mês uma ressonância magnética revelou uma lesão multilocular no pré-sacral, sugerindo a presença de um hamartoma cístico retrorretal. Uma operação eletiva foi realizada por abordagem cirúrgica endoanal usando TAMIS. A resseção em bloco do cisto foi conseguido com margens seguras. O resultado do exame patológico relatou hamartoma cístico retrorretal. O paciente encontrava-se assintomático, sem sinais de recidiva em controles posteriores. A nossa experiência, apesar de ser baseado em um dos casos, as provas que TAMIS (cirurgia transanal minimamente invasiva) permite um esvaziamento minimamente invasivo com benefícios similares como o uso de dispositivos de MET/TEO. (AU)


Assuntos
Humanos , Masculino , Adulto , Cirurgia Colorretal/métodos , Procedimentos Cirúrgicos Minimamente Invasivos , Hamartoma/cirurgia , Reto/cirurgia , Imageamento por Ressonância Magnética , Cistos/patologia , Hamartoma/diagnóstico
9.
Rev. chil. cir ; 66(2): 170-174, abr. 2014. ilus
Artigo em Espanhol | LILACS | ID: lil-706536

RESUMO

Introducción: Los hamartomas y la hiperplasia estromal pseudoangiomatosa nodular (PASH) son entidades infrecuentes en la patología tumoral mamaria, sin embargo, pueden simular al cáncer de mama. La única herramienta certera preoperatoria es la biopsia. El tratamiento quirúrgico es curativo con bajo índice de recurrencia. Objetivo: El objetivo de este trabajo es exponer un caso de hamartoma mamario con PASH asociado cuya presentación inicial fue sugerente de probable patología maligna. Caso Clínico: Mujer de 44 años, ingresa a controles por probable patología mamaria maligna, la mamografía fue informada como BIRADS 0, la ecografía mamaria como BIRADS US: 5, la biopsia CORE informó PASH. Se realizó mastectomía parcial objetivando en la biopsia diferida un hamartoma mamario con extensa hiperplasia estromal pseudoangiomatosa. Discusión: Los hamartomas y la PASH son patologías sin presentación clínica específica, los hallazgos radiológicos del hamartoma pueden ser patognomónicos, no así en la PASH; en el caso expuesto el estudio anatomopatológico demostró una asociación de estas patologías, la cual se describe desde un 16 por ciento a un 71 por ciento de los casos de hamartoma mamario. El análisis de toda la pieza quirúrgica es fundamental para establecer un diagnóstico definitivo.


Introduction: Hamartoma and nodular pseudoangiomatous stromal hyperplasia (PASH) are inusual breast tumor entities, however, can simulate breast cancer. The only accurate tool is preoperative biopsy. Surgical treatment is curative with low recurrence rate. Objective: To present a case of mammary hamartoma associated with PASH whose initial presentation was suggestive of probable malignancy. Case Report: Woman, 44 years old, admitted to controls because of probable malignant breast disease, mammography was reported as BIRADS 0, breast ultrasound as BIRADS U.S: 5, CORE biopsy reported PASH. Partial mastectomy was performed. On delayed biopsy mammary hamartoma with extensive stromal hyperplasia pseudoangiomatosa was diagnosed. Discussion: PASH and hamartomas are diseases with no specific clinical presentation, radiological findings may be pathognomonic of hamartoma, while not in PASH. In the reported case the pathological study showed an association of these conditions, described in a 16% to 71% breast hamartoma cases. The analysis of the entire surgical specimen is essential for a definitive diagnosis.


Assuntos
Humanos , Adulto , Feminino , Angiomatose/cirurgia , Angiomatose/diagnóstico , Doenças Mamárias/cirurgia , Doenças Mamárias/diagnóstico , Hamartoma/cirurgia , Hamartoma/diagnóstico , Diagnóstico Diferencial , Hiperplasia , Mastectomia Segmentar
10.
Einstein (Säo Paulo) ; 12(2): 234-236, Apr-Jun/2014. graf
Artigo em Inglês | LILACS | ID: lil-713011

RESUMO

A full-term male neonate with anorectal anomaly and external perineal anomalies was referred to our service. Physical examination showed an epithelized perineal mass with cutaneous orifices, which had urine fistulization, hipotrofic perineal musculature, bilateral congenital clubfoot, hipospadic urethra, criptorquidy bilateral with nonpalpable testis and imperforate anus. A colostomy was constructed immediately after birth. The child underwent excision of perineal mass, bilateral orchidopexy, Duplay neourethroplasty and coloanal anastomosis at 3 months of age. The histopathological examination of the perineal mass revealed a hamartoma.


Recém-nascido a termo do sexo masculino encaminhado ao nosso serviço por anomalia anorretal e anomalias perineais externas. O exame físico revelou massa perineal epitelizada, com orifícios cutâneos que apresentavam saída de urina, musculatura perineal hipotrófica, pé torto congênito bilateral, uretra hipospádica, criptorquidia bilateral com testículos não palpáveis e ânus imperfurado. Logo após o nascimento, o paciente foi submetido à colostomia. Aos 3 meses de idade, a criança foi submetida à excisão da massa perineal, orquidopexia bilateral, neouretroplastia a Duplay e anastomose coloanal. A análise anatomopatológica da massa perineal indicou hamartoma.


Assuntos
Humanos , Recém-Nascido , Masculino , Anormalidades Múltiplas , Anus Imperfurado/complicações , Criptorquidismo/complicações , Hamartoma/complicações , Períneo/anormalidades , Anus Imperfurado/cirurgia , Criptorquidismo/cirurgia , Hamartoma/diagnóstico , Hamartoma/cirurgia , Doenças Raras/complicações , Doenças Raras/cirurgia
11.
Rev. otorrinolaringol. cir. cabeza cuello ; 74(2): 151-154, 2014. ilus
Artigo em Espanhol | LILACS | ID: lil-726166

RESUMO

Los pólipos linfangiomatosos son malformaciones congénitas de tipo hamartomatosas caracterizados histológicamente por una proliferación linfática vascular con distintos grados de componente fibroso, adiposo y linfático, cubiertos por un epitelio escamoso. Dado a que se conocen por distintos nombres en la literatura, sólo se han descrito alrededor de 30 casos de pólipos linfagiomatosos como tal. De etiopatogenia desconocida, se presentan como una masa polipoidea o papilomatosa en las amígdalas palatinas, con sintomatología variable. Su diagnóstico definitivo es histológico tras una resección completa. No se han reportado casos de malignización ni recurrencia. En el presente trabajo se reporta el caso de un paciente de 5 años con historia de crecimiento amigdalino bilateral de dos años de evolución. La biopsia definitiva demuestra una poliposis linfangiomatosa de amígdalas palatinas y adenoides.


Lymphangiomatous polyps are hamartomatous congenital malformations. They are histologically characterized by a vascular lymphatic proliferation associated with fibrous, adipose and lymphatic components covered by squamous epithelium. There are only 30 cases described in the literature by the name of lymphangiomatous polyp, since it has multiple denominations. Even though their etiopathogenesis is unknown, their clinical presentation is described as a polypoid mass in the palatine tonsils, which may have multiple manifestations. The diagnosis is made histologically after complete resection. There have not been reports of malignant transformation nor recurrence. We present a case of a five year old patient with history of bilateral palatine tonsil growth. Final biopsy described lymphangiomatous polyps of adenoids and palatine tonsils.


Assuntos
Humanos , Masculino , Pré-Escolar , Tonsila Palatina/cirurgia , Tonsila Palatina/patologia , Hamartoma/cirurgia , Hamartoma/patologia , Pólipos/patologia , Tonsilectomia , Adenoidectomia , Tonsila Faríngea/cirurgia , Tonsila Faríngea/patologia
14.
J. bras. pneumol ; 37(6): 796-800, nov.-dez. 2011. ilus, tab
Artigo em Português | LILACS | ID: lil-610913

RESUMO

OBJETIVO: Tumores benignos primários da traqueia e dos brônquios principais são incomuns. A broncoscopia intervencionista permite o diagnóstico e o tratamento de algumas dessas lesões. MÉTODOS: Revisamos quatro casos tratados endoscopicamente em nossa instituição. RESULTADOS: Dois pacientes tinham hamartoma, e dois pacientes apresentaram lipoma endobrônquico. Em todos os casos, a técnica de intervenção para a ressecção foi o uso de alça de polipectomia e eletrocautério. A única complicação relatada foi um episódio de broncoespasmo. CONCLUSÕES: O tratamento broncoscópico minimamente invasivo é um método seguro e efetivo para o tratamento bem-sucedido de alguns tumores benignos da via aérea principal, com um baixo índice de complicações.


OBJECTIVE: Primary benign tumors of the trachea and main bronchi are uncommon. Interventional bronchoscopy allows the diagnosis and the treatment of some of these lesions. METHODS: We reviewed four cases endoscopically treated at our institution. RESULTS: Two patients had hamartoma, and two patients had endobronchial lipoma. In all of the cases, the interventional technique for the resection was the use of a polypectomy snare and electrocautery. The only complication reported was one episode of bronchospasm. CONCLUSIONS: Minimally invasive bronchoscopic resection is a safe, effective method for treating selected benign tumors of the main airway and has a low complication rate.


Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Brônquicas/cirurgia , Broncoscopia/métodos , Hamartoma/cirurgia , Lipoma/cirurgia , Broncoscopia/efeitos adversos
15.
West Indian med. j ; 60(6): 678-680, Dec. 2011.
Artigo em Inglês | LILACS | ID: lil-672834

RESUMO

Although 75% of intussusceptions occur within the first two years of life, they can also develop in teenage years. This is a case report of a 13-year old boy with an ileocolorectal intussusception from a large caecal hamartoma (10 x 6 x 2 cm3) adjacent to the ileocaecal valve. Partial resection of the ascending colon and terminal ileum was performed, and the pathology of the resected mass revealed a hamartoma. Ileocolorectal intussusception secondary to hamartoma represents a particularly rare event in the paediatric population. With early surgical intervention, this patient's outcome was uneventful.


Aunque el 75% de las intususcepciones ocurren en los primeros dos años de vida, pueden también desarrollarse en el período de la adolescencia. Éste es el reporte del caso de un niño de 13 años con una intususcepción ileocolorectal a partir de un hamartoma de ciego de gran tamaño (10 x 6 x 2 cm3) adyacente a la válvula ileocecal. Se realizó una resección parcial del colon ascendente y el íleo terminal, y la patología de la masa resecada reveló un hamartoma. La intususcepción íleocolorectal derivada secundariamente a partir de un hamartoma, representa un caso particularmente raro dentro de la población pediátrica. Una intervención quirúrgica temprana, hizo posible que este paciente tuviera una evolución clínica sin graves consecuencias.


Assuntos
Adolescente , Humanos , Masculino , Doenças do Ceco/complicações , Hamartoma/complicações , Doenças do Íleo/etiologia , Intussuscepção/etiologia , Doenças Retais/etiologia , Doenças do Ceco/cirurgia , Hamartoma/cirurgia , Doenças do Íleo/cirurgia , Valva Ileocecal , Intussuscepção/cirurgia , Doenças Retais/cirurgia
17.
Saudi Journal of Gastroenterology [The]. 2011; 17 (1): 20-22
em Inglês | IMEMR | ID: emr-112922

RESUMO

Mesenchymal hamartoma of liver [MHL] is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL. All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically. The total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 +/- 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one [11.11%].: MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory


Assuntos
Humanos , Masculino , Feminino , Mesoderma/patologia , Hamartoma/cirurgia , Hepatopatias/patologia , Seguimentos
19.
Cir. & cir ; 77(6): 487-491, nov.-dic. 2009. ilus
Artigo em Espanhol | LILACS | ID: lil-566450

RESUMO

Introducción: El hamartoma fibroso de la infancia es una proliferación benigna de los tejidos blandos, de presentación infrecuente; 91 % de los casos ocurre durante el primer año de edad y afecta más frecuentemente al sexo masculino. Se caracteriza por ser una lesión subcutánea de morfología característica con patrón de crecimiento organoide trifásico. Caso clínico: Lactante masculino de seis meses de edad, con tumor en la región plantar medial del pie izquierdo, el cual fue resecado completamente. En el estudio histopatológico se informó como hamartoma fibroso de la infancia. Conclusiones: Puede localizarse en cualquier sitio anatómico, aunque 5 a 10 % afecta las extremidades inferiores existen pocos casos informados en el pie. En biopsias pequeñas es importante su diagnóstico diferencial con otras lesiones fibroadiposas para el adecuado tratamiento. La resección quirúrgica amplia con márgenes libres de lesión confiere un buen pronóstico a los pacientes.


BACKGROUND: Fibrous hamartoma of infancy (FHI) is an infrequent benign proliferation of the soft tissues. Ninety one percent of cases occur during the first year of life. FHI is characterized as a subcutaneous lesion with characteristic morphology with a triphasic organoid growth pattern. CLINICAL CASE: We present the case of a 6-month-old male infant with a tumor in the medial plantar region of the left foot, which was completely withered. Histopathological study reported a fibrous hamartoma of infancy. CONCLUSIONS: Fibrous hamartoma of infancy most frequently affects males. It may be localized at any anatomic site, although 5-10% of cases affect the lower limbs. Few cases are reported in the foot. This is a lesion with a characteristic morphological pattern; however, in small biopsies, its differential diagnosis is important with other fibroadipose lesions for appropriate treatment. Ample surgical resection with lesion-free borders confers a good prognosis for these patients.


Assuntos
Humanos , Masculino , Lactente , Doenças do Pé , Hamartoma , Doenças do Pé/diagnóstico , Doenças do Pé/cirurgia , Hamartoma/diagnóstico , Hamartoma/cirurgia
20.
West Indian med. j ; 58(6): 607-609, Dec. 2009. ilus
Artigo em Inglês | LILACS | ID: lil-672552

RESUMO

A case of cutaneous rhabdomyomatous mesenchymal hamartoma in a 6-year old Afro-Caribbean girl is reported with review of the literature. The lesions were fine, located on the central face and became inapparent after six months. Spontaneous regression of these lesions has not been previously reported. Although rare, continued reporting will facilitate the elucidation of the clinical features and natural history of these lesions and the relationship to disordered embryogenesis.


Un caso de hamartoma mesenquimal rhabdomiomatoso cutáneo en una niña afrocaribeña de seis años de edad, se reporta junto con una revisión de la literatura. Las lesiones eran tenues, localizadas en la parte central de la cara, y se hicieron aparentes luego de seis meses. La regresión espontánea de estas lesiones no se ha reportado con anterioridad. Aunque sean raras, reportarlas de manera continuada facilitará la dilucidación de los rasgos clínicos y la historia natural de estas lesiones, así como su relación con una embriogénesis desordenada.


Assuntos
Criança , Feminino , Humanos , Neoplasias Faciais/patologia , Hamartoma/patologia , Rabdomioma/patologia , Administração Tópica , Anti-Inflamatórios/administração & dosagem , Antifúngicos/administração & dosagem , Desonida/administração & dosagem , Neoplasias Faciais/tratamento farmacológico , Neoplasias Faciais/cirurgia , Hamartoma/tratamento farmacológico , Hamartoma/cirurgia , Cetoconazol/administração & dosagem , Indução de Remissão , Rabdomioma/tratamento farmacológico , Rabdomioma/cirurgia
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