RESUMO
BACKGROUND: Tumour markers are substances produced by the tumour itself, or by the host in response to a tumour. These markers could be measured either in the blood or in body secretions. One of the most common tumour markers used in gastrointestinal diseases is Ca 19-9. It is the marker most used for pancreatic cancer, but can be elevated in many benign processes. Thus, it is not a specific marker. CLINICAL CASE: The case is presented of a male patient with 4 years of moderate abdominal pain, weight loss, and persistent elevation of Ca 19-9. After an extensive work-up, renal and hepatic cysts were found, as well as steatosis and, apparently, a gallbladder polyp. With these findings and the persistent elevation of Ca 19-9, it was decided to operate the patient. An exploratory laparoscopy was performed showing multiple, yellowish nodular lesions all over the hepatic surface suggestive of metastases, as well as simple hepatic cysts. Pathology reported biliary hamartomas, steatosis, and chronic cholecystitis. After 2years of follow up, although there is no evidence of malignant neoplasia, there is still an elevation of Ca 19-9. CONCLUSION: The persistent elevation of Ca 19-9 is probably due to the presence of multiple benign diseases such as steatosis, urolithiasis, hepatic and renal cysts, and cholecystitis. An algorithm is needed for healthy patients with elevated levels of Ca 19-9 marker, in order to lower costs, avoid misdiagnoses, and improve management.
Assuntos
Antígeno CA-19-9/sangue , Hamartoma/sangue , Hepatopatias/sangue , Colecistite/sangue , Colecistite/complicações , Doença Crônica , Diagnóstico Diferencial , Fígado Gorduroso/sangue , Fígado Gorduroso/complicações , Fígado Gorduroso/patologia , Hamartoma/complicações , Hamartoma/patologia , Hamartoma/cirurgia , Humanos , Doenças Renais Císticas/complicações , Hepatopatias/complicações , Hepatopatias/patologia , Hepatopatias/cirurgia , Neoplasias Hepáticas/diagnóstico , Masculino , Pessoa de Meia-Idade , Nefrolitíase/complicaçõesRESUMO
BACKGROUND: Mesenchymal hamartoma of the liver is a rare benign liver tumor in children, usually arising from the right liver lobe and represents about 5 to 6% of all primary hepatic tumors. Complete surgical resection of the tumor is curative. CLINICAL CASE: A 30 months old male presented with epigastrium abdominal pain and a palpable mass over a period of two days with no other symptom. The mass was excised completely. Postoperatively the patient recovered with an uneventful course and was discharge 13 days following surgery. All microscopic findings were consistent with the diagnosis of mesenchymal hamartoma of the liver. CONCLUSIONS: Approximately 75% of mesenchymal hamartoma of the liver occur in the right lobe of the liver. Several diagnostic considerations should be elucidated to differentiate these type of tumors in the left lobe from other benign liver tumors. Sometimes a multidisciplinary approach is necessary to complete a successful complete surgical excision. Our case exemplifies a rare entity in a rare location, an adequate treatment in a third level reference hospital setting.
Assuntos
Hamartoma/cirurgia , Hepatectomia/métodos , Hepatopatias/cirurgia , Dor Abdominal/etiologia , Ductos Biliares/patologia , Biomarcadores Tumorais/análise , Pré-Escolar , Células Epiteliais/patologia , Hamartoma/sangue , Hamartoma/complicações , Hamartoma/diagnóstico por imagem , Humanos , Hepatopatias/sangue , Hepatopatias/complicações , Hepatopatias/diagnóstico por imagem , Masculino , Mesoderma/patologia , Infiltração de Neutrófilos , Células Estromais/patologia , Tomografia Computadorizada por Raios XRESUMO
The gonadotropin releasing hormone (GnRH) secreting hypothalamic hamartoma (HH) is a congenital malformation consisting of a heterotopic mass of nervous tissue that contains GnRH neurosecretory neurons attached to the tuber cinereum or the floor of the third ventricle. HH is a well recognised cause of gonadotropin dependent precocious puberty (GDPP). Long term data are presented on eight children (five boys and three girls) with GDPP due to HH. Physical signs of puberty were observed before 2 years of age in all patients. At presentation with sexual precocity, the mean height standard deviation (SD) for chronological age was +1.60 (1.27) and the mean height SD for bone age was -0.92 (1.77). Neurological symptoms were absent at presentation and follow up. The hamartoma diameter ranged from 5 to 18 mm and did not change in six patients who had magnetic resonance imaging follow up. All patients were treated clinically with GnRH agonists (GnRH-a). The duration of treatment varied from 2.66 to 8.41 years. Seven of the eight children had satisfactory responses to treatment, shown by regression of pubertal signs, suppression of hormonal levels, and improvement of height SD for bone age and predicted height. One patient had a severe local reaction to GnRH-a with failure of hormonal suppression and progression of pubertal signs. It seems that HH is benign and that GnRH-a treatment provides satisfactory and safe control for most children with GDPP due to HH.