ABSTRACT
Nasal chondromesenchymal hamartoma [NCMH] is an extremely rare benign lesion arising in the sino-nasal tract. They usually affect children below one year of age. The pathogenesis of these lesions is poorly understood however, they have been associated with pleuropulmonary blastoma [PPB]. Although benign, malignant transformation has been reported in the literature. On literature review, we found 32 reported cases, most of them occurring in children below one year. We report a 14-year-old adolescent male with history of PPB at the age of 6, presenting with bilateral nasal obstruction and decreased sense of smell. In our case report we highlight the association between PPB and NCMH, and describe an unusual presentation
Subject(s)
Humans , Male , Nose Diseases , Pulmonary Blastoma , Lung Neoplasms , Adolescent , Hamartoma/etiology , Hamartoma/pathologyABSTRACT
Tuberous sclerosis [Bourneville Disease, Vogt triad] is an autosomal dominant hereditary disease characterized by hamartomas. It can affect all body organs, but is most commonly seen on the skin, brain, eyes, lungs, heart and kidney. We describe the successful management of a case of forty two years old women who presented to us with complaints of nausea, vomiting, bilateral flank pain
Subject(s)
Humans , Female , Hamartoma/etiology , Electrocardiography , Echocardiography , Magnetic Resonance ImagingABSTRACT
Paciente de 54 años, masculino, con antecedentes de hta, para lo cual se hace estudio encontrándose como hallazgo en rx de tórax nódulo de 5-6 cm que ocupa lóbulo inferior de pulmón izquierdo hacen estudios correspondientes, realizándose tratamiento quirúrgico que arrojó hamartoma condroide...
54-years-old, male patient, with antecedents of hta. A study was indicated, finding a nodule of 5-6 cm in the low lobule of the left lung in thorax rx. The correspondent studies were made, and the surgical treatment showed condroid hamartoma.
Subject(s)
Humans , Male , Middle Aged , Hamartoma/diagnosis , Hamartoma/epidemiology , Hamartoma/etiology , Lung/abnormalities , Lung/surgeryABSTRACT
Relata-se o caso de uma paciente de 60 anos, em menopausa há 14 anos, com presença de nódulo pulmonar à radiografia de tórax diagnosticado no seguimento pós-operatório por neoplasia de mama. A paciente tinha história de mastectomia e linfadenectomia axilar ipsilateral por carcinoma ductal invasivo de mama, assim como de hormonioterapia, quimioterapia e radioterapia adjuvante. Após nodulectomia por vídeotoracoscopia, o exame de congelação mostrou tratar-se de um hamartoma pulmonar. Estudos recentes demonstram que 75 por cento dos pacientes que se submeteram a cirurgia por nódulos pulmonares após mastectomia curativa de câncer de mama apresentaram metástases pulmonares, 11,5 por cento apresentaram câncer primário de pulmão e 13,5 por cento apresentaram lesões benignas, dentre as quais o hamartoma.
We present the case of a 60-year-old female patient who had been in menopause for 14 years and presented a pulmonary nodule on chest X-ray diagnosed in the postoperative follow-up evaluation of breast cancer. The patient had a history of mastectomy and ipsilateral axillary lymphadenectomy for invasive ductal breast carcinoma, as well as of hormone therapy, chemotherapy, and adjuvant radiotherapy. After thoracoscopic nodulectomy, the frozen section analysis revealed a pulmonary hamartoma. Recent studies show that 75 percent of patients who undergo surgery for pulmonary nodules after a curative mastectomy for breast cancer present lung metastases, 11.5 percent present primary lung cancer, and 13.5 percent present benign lesions, including hamartoma.
Subject(s)
Female , Humans , Middle Aged , Breast Neoplasms/pathology , Carcinoma/pathology , Hamartoma/pathology , Lung Diseases/pathology , Breast Neoplasms/therapy , Carcinoma/therapy , Diagnosis, Differential , Hamartoma/etiology , Lung Diseases/etiology , Lung Neoplasms/secondary , Mastectomy , Tomography, X-Ray ComputedABSTRACT
Presentamos tres pacientes con un tumor benigno infrecuente denominado hamartoma fibrolipomatoso del nervio mediano, el cual puede estar o no asociado a macrodistrofia lipomatosa.
Subject(s)
Hamartoma/etiology , Hamartoma , Median Nerve/pathology , Fibroma , Lipomatosis , Magnetic Resonance Spectroscopy , Radiography , UltrasonographyABSTRACT
Tuberous sclerosis - also called tuberous sclerosis complex (TSC) - is a rare, multi-system genetic disorder affecting cellular differentiation & proliferation, which results in hamartoma formation in many organs. The classic triad of clinical features comprises mental retardation, epilepsy & skin lesion, but these three features are not always present. Mrs. Jahanara Khatun, a 30 years old lady presented with maculo-papular skin lesions over the face, neck, shoulder since her six years of age, a lump in the right lumber region for four years, pain in the right lumber region associated with passage of blood clot in urine for 15 days. Her family history was very characteristic. One of her elder brother had developed same type of skin lesions. Again her 13 years old daughter had developed same type of skin lesions since seven years of age & she was mentally retarded. Clinical examination revealed normal mentation, pin head sized yellowish red translucent discrete waxy papules situated in the face, neck, shoulder. A large tender firm irregular mass in the right lumber region, which was ballotable & moved with respiration. USG revealed bilateral retroperitoneal masses with the involvement of right kidney & formation of renal artery aneurysm. The patient undergone right sided nephrectomy & histopathology of the specimen showed features suggestive of renal angiomyolipoma. The patient was diagnosed as a case of definite tuberous sclerosis complex as she had two major feature of revised diagnostic criteria - facial angiofibromas & renal angiomyolipoma.
Subject(s)
Adult , Female , Hamartoma/etiology , Humans , Tuberous Sclerosis/complicationsABSTRACT
El nevo de Becker es un hamartoma cutáneo hiperpigmentado e hipertricósico, de localización usualmente unilateral, de presentación más frecuente en sexo masculino. La mayoría de los casos se manifiestan durante la pubertad, pero se ha publicado actualmente la existencia de casos congénitos. Excepcionalmente presenta una agregación familiar, con una herencia autosómica dominante. Se postula una influencia androgénica en su desarrollo, dada su mayor presentación durante la adolescencia. Este nevo puede asociarse a malformaciones cutáneas, esqueléticas y musculares, describiéndose dos nuevos síndromes recientemente relacionados con la presencia del nevo de Becker. Se presenta un resumen de actualidad en cuanto a su patología y tratamiento
Subject(s)
Humans , Hamartoma/diagnosis , Nevus/diagnosis , Clinical Evolution , Diagnosis, Differential , Hamartoma/complications , Hamartoma/etiology , Hamartoma/surgery , Hypertrichosis/diagnosisABSTRACT
Los tumores disontogénicos son lesiones proliferativas que se originan como un trastorno del desarrollo e incluyen una variedad de neoformaciones: Hamartomas, coristomas y el complejo grupo de los teratomas. La mayoría de estas lesiones son benignas con excepción de los teratomas. Son entidades algunas de ellas frecuentes en la clínica y otras son excepcionales. El tratamiento es quirúrgico y el pronóstico es bueno en la mayoría de los casos. Es un grupo de lesiones poco conocidas en la clínica oftalmológica. Se hace una revisión completa del tema en tres partes. Los hamartomas pueden ser simples o complejos y las características histopatológicas y la taxonómicas dependen de sus componentes tisulares. Afectan a la órbita, párpados, conjuntiva, córnea y túnicas internas
Subject(s)
Humans , Teratoma/classification , Teratoma/etiology , Choristoma/classification , Choristoma/etiology , Neoplasms, Germ Cell and Embryonal/pathology , Eye Neoplasms/classification , Eye Neoplasms/etiology , Hamartoma/classification , Hamartoma/etiology , Germ Cells/pathology , Eye Diseases/pathology , PrognosisABSTRACT
El Síndrome de Peutz-Jeghers (SPJ) es una entidad rara autosómica dominante, caracterizada por lesiones hiperpigmentadas en boca, manos y pies; con presencia de pólipos gastrointestinales que ocasionan cuadros de anemia aguda o crónica, obstrucción intestinal y dolor abdominal. Histológicamente, estos pólipos son hamartomas en los que, estudios recientes establecen hoy un riesgo real para transformación neoplásica maligna. Las endoscopías altas y bajas y la enteroscopía intraoperatoria con polipectomía respectiva, constituye el tratamiento de elección, mejorando cualitativamente el pronóstico en estos pacientes. Describimos en esta oportunidad un caso familiar de una paciente, mujer de 24 años con cuadro suboclusivo a repetición, asi mismo el de su hermano, con similar clínica; además el de su madre fallecida de carcinoma de colon presentando ella también el mismo síndrome; y finalmente el hijo de la paciente, de un año y medio de edad que presenta lesiones hiperpigmentadas en los labios
Subject(s)
Humans , Female , Adult , Endoscopy, Gastrointestinal/statistics & numerical data , Intestinal Polyps/surgery , Intestinal Polyps/physiopathology , Intestinal Polyps/genetics , Intestinal Polyps/pathology , Hamartoma/diagnosis , Hamartoma/etiology , Hyperpigmentation/diagnosis , Hyperpigmentation/etiologyABSTRACT
This is the case of a 55-year-old male with cirrhosis who required a LeVeen shunt for relief of refractory ascites. After eight months he developed recurrence of the ascites and a erythematous patch around the surgical scar. Skin biopsy revealed a proliferation of small and medium-sized vessels throughout the dermis. The acquired form of nevus flammeus is rare an is usually preceded by trauma. This is the report of a patient with an acquired nevus flammeus associated to an obstructed peritoneovenous shunt
Subject(s)
Humans , Male , Middle Aged , Abdomen , Postoperative Complications/etiology , Hamartoma/etiology , Biopsy , Liver Cirrhosis, Alcoholic/complications , Postoperative Complications/pathology , Peritoneovenous Shunt/adverse effects , Diabetes Mellitus, Type 1/complications , Hamartoma/pathologyABSTRACT
Descreve um caso clínico de paciente com esclerose tuberosa com o objetivo de auxiliar o pronto reconhecimento da doença pelo oftalmologista, permitindo um diagnóstico sindrômico que promoverá melhor integraçäo entre as especialidades médicas adequadas ao tratamento da doença
Subject(s)
Humans , Female , Adolescent , Hamartoma/etiology , Retina/pathology , Tuberous Sclerosis/complications , Tuberous Sclerosis/diagnosis , Tuberous Sclerosis/geneticsABSTRACT
El hamartoma de músculo liso, es una lesión cutánea habitualmente congénita, que fue inicialmente descrita bajo otras denominaciones (tumor de músculo liso de la piel, nevo de músculo liso, hamartoma organoide, etc.) En este trabajo presentamos nuestra experiencia con 3 casos, dos de ellos congénitos
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Adult , Hamartoma/diagnosis , Muscle, Smooth/pathology , Hamartoma/etiology , Hamartoma/pathology , Nevus/diagnosis , Skin Neoplasms/diagnosis , Skin Neoplasms/pathologyABSTRACT
Se presenta cuatro casos de angiomiolipoma renal tratados en el Servicio de Urologia del Hospital de Oncología del Centro Médico Nacional, IMSS. Dos de los casos se relacionan con esclerosis tuberosa, uno en su forma típica y otro de variedad frustrada, y dos casos no tienen relación con esta enfermedad. Se comentan los hallazgos, del ultrasonido y principalmente de la tomografia axial computarizada, lo cual demuestra ser específica al detectar densidad de tejido graso (índice de atenuación de 0 a -25 UH) característica del angiomiolipoma, lo que permite que en la actualidad el tratamiento de este tumor tienda a ser conservador y que, en caso de requerirse operación quirúrgica, ésta sea limitada