Hepatoblastoma en niños / Hepatoblastoma in children

El Hepatoblastoma es el principal tumor maligno primario del hí gado en ni ños. El objetivo de este art ículo es recordar que el Hepatoblastoma se presenta principalmente antes de los 3 a ños de vida, y que siempre que palpemos una masa abdominal, pense- mos que es maligno hasta que no se dem - uestre lo contrario. El paciente del presente caso es un lactante masculino de 15 meses de edad, procedente de Santa B árbara , ingresado en la sala de Lactantes del Hos pital Nacional " Mario Catarino Rivas " ( HNMCR ) el 03 de marzo 2010 , con histo ria de dos meses de evoluci ón de masa en cuadrante superior derecho. Se le realiz ó resecci ón total del tumor y luego se inicio quimioterapia. La biopsia report ó Hepato blastoma patr ón morfol ógico cl ásico (Fetal)...(AU)

Hepatoblastoma: Experience from a single center.

Background: The cornerstones of successful treatment of hepatoblastoma (HB) include preoperative chemotherapy followed by complete anatomical resection of tumor, followed by chemotherapy. Advances in chemotherapy in the last 2 decades have been associated with a higher rate of tumor response and possibly a greater potential for resectability. Aims: We analyzed our single center experience with neoadjuvant chemotherapy (NACT) and surgery in HBs. Settings and Design: Our study included all children with HBs who received NACT and underwent surgical excision from January 1997 to July 2004. Materials and Methods: Patient characteristics, clinical features, clinical course, treatment modalities, and long-term outcome were analyzed. Results: There were 9 boys and 3 girls, aged 5-60 months (median age at tumor diagnosis was 24 months). All received NACT containing cisplatin and doxorubicin. Of the 12 children, 9 underwent hepatectomy and among them, 4 patients each had right and left hepatectomy and 1 patient underwent right extended hepatectomy. After surgery, all patients completed rest of the chemotherapy course (total 6 cycles). R0 resection was carried out in all the 9 cases with no life-threatening complications. Conclusions: Our experience of the 9 cases, although less in number, reaffirms the advantages of NACT followed by surgery. The prognosis for patients with resectable tumors is fairly good in combination with chemotherapy.

Fine needle aspiration cytology of hepatoblastoma: a study of 20 cases.

Hepatoblastoma (HBL) is the most common primary malignant hepatic tumor in children. The aim of the present study is to describe cytological findings of HBL, to subclassify it, and to discuss differential diagnoses. Twenty cases were taken from records of Cytopathology department, Gujarat Cancer and Research Institute, Ahmedabad. The aspiration smears were stained with Papanicolaou stain andlor Giemsa stain. Cytological and architectural criteria were applied to aspiration smears. Fifteen cases (75%) of HBL were diagnosed in the patients below the age of two years. The commonest presentation was found to be lump in abdomen. On the basis of cytoarchitectural features, HBL was classified in two groups undifferentiated and differentiated. Morphologically, the tumor cells were commonly arranged in acinar pattern, papillary pattern, or in sheets. FNA cytology alone had some limitations in the diagnosis of HBL. Hence, cytoarchitecture in combination with clinicalfeatures, imaging techniques and serum a-fetoprotein levels were helpful for specific diagnosis of HBL and to rule out various others differential diagnosis of small round cell tumor. The cytological differential diagnosis between differentiated HBL and Hepatocellular carcinoma (HCC) was found to be very difficult.

Advanced hepatoblastoma: successful liver resection following preoperative chemotherapy.

We present a case of advanced hepatobastoma in a 9-month-old child who was treated with chemotherapy (three cycles of PLADO regimen conisting of cisplatin and Adriamycin) followed by surgical excision (left lobectomy). At 1-year follow up, the is doing well and is disease-free.

Ultrasound guided fine needle aspiration cytology for diagnosis of mass lesions of liver.

The therapeutic and prognostic evaluation of malignant neoplasia of liver depends mostly on morphologic diagnosis. Ultrasound guided fine needle aspiration cytology (FNAC) of liver has been proved to be a rapid, reliable and cost-effective method for cytological diagnosis of hepatic mass lesions. This study was undertaken to find out the frequency of different benign and malignant space occupying lesions (SOL) of liver from patients attending for FNAC in Mymensingh. Ultrasound guided FNAC was performed on 108 patients with hepatic mass lesions from September 2, 2001 to August 19, 2003. There were 67 (62.0%) males and 41 (37.96%) females with a mean age 53 year (SD +/- 14) ranging from 2 to 83 years. Samples were adequate in 101 (93.5%) and inadequate in 7 (6.5%). Out of 101 adequate samples only one was benign (liver abscess) and 100 were malignant. Of the malignant lesions hepatoblastoma was in 1 (1%), hepatocellular carcinomas in 8 (8%), metastatic adenocarcinomas in 73 (73%) and unclassified malignancies were in 18 (18%). Most of the hepatocellular carcinomas occurred in males (7 in 8). The frequency of metastatic adenocarcinoma was more in males (39 vs. 34) but the difference is not significant (p > 0.05). Ultrasound guided FNAC of liver is a rapid, reliable and cost-effective diagnostic method. It can be practice in any centres where ultrasound facility and specialist pathologists are available.

Manejo multidisciplinario de tumores hepáticos malignos en el niño: una experiencia nacional reciente / Multidisciplinary management of hepatic malignant tumors in children: report of 6 cases

Background: malignant hepatic tumors (Mht) are rare in children. Among them hepatoblastoma (HB) is the most common. Aim: to report the results of the multidisciplinary management in 6 consecutive children: five HB and one metastatic Wilms tumor (MWT). Patients and methods: the mean age of patients was 42 months. All HB patients had elevated serum alfafetoprotein (median 150,000 ng/ml). All patients received preoperative chemotherapy: HB patients received carboplatin/doxorrubicin alternating with cisplatin, and the MWT patient, vincristine alone. Surgery included two formal right and two formal left hepatectomies, one extensive central resection with partial left segmentectomy, and one lateral segmentectomy. Extracorporeal circulation was used in the child with atrial involvement. All patients received postoperative chemotherapy. Results: all tumors had variable regresion on preoperative chemotherapy. Complete resection with negative margins was achieved in all patients. The degree of tumor necrosis on histology ranged from 60 percent to 90 percent. Alfafetoprotein levels fell to under 10 ng/ml in all HB cases, one to three months after surgery. All patients survive free of disease at a median follow up of 19 months. Conclusion: a multidisciplinary approach including the well timed used of chemotherapy and surgery is highly effective in the management of pediatric malignant tumors

Diagnóstico citológico de hepatoblastoma: estudo de caso com ênfase nos achados citopatológicos / Cytological diagnosis of hepatoblastoma: case report with emphasis to cytopathological findings

Os autores apresentam um caso de hepatoblastoma diagnosticado mediante punçäo por agulha fina, sendo este o primeiro descrito na literatura latino-americana. O paciente era do sexo masculino e tinha oito meses, apresentando intensa irritabilidade, com achado de hepatomegalia volumosa de superfície regular. O estudo citopatológico definiu o diagnóstico de hepatoblastoma do tipo fetal. Dada a escassez de relatos na literatua acerca da importância da punçäo aspirativa como método diagnóstico pré-operatório em lesöes hepáticas pediátricas, esse estudo vem enfatizar a importância da adoçäo deste método na rotina diagnóstica

Comparación de la biopsia por aspiración con aguja fina y la biopsia quirúrgica en el diagnóstico de tumores en niños / Comparison between diagnosisi by fine needle aspiration biopsy and conventional biopsy in children's tumors

Introducción. La biopsia por aspiración con aguja fina (BAAF) es un método de diagnóstico útil, inocuo y rápido para el estudio inicial de los tumores superficiales y profundos que ha sido utilizado cada vez con mayor frecuencia en niños. Fue propósito de este trabajo evaluar la precisión diagnóstica del procedimiento en el diagnóstico inicial de tumores en niños. Material y métodos. Se revisaron todas las BAAF diagnosticadas en un período de 24 meses y se seleccionaron aquellas con diagnóstico de tumor o masa tumoral que tuvieran además estudio histopatológico subsecuente ya sea mediante biopsia o extirpación total de la lesión. Se compararon los diagnósticos de ambos procedimientos y se juzgó conveniente calcular la sensibilidad y especificidad. Resultados. De 89 BAAF registradas en el período de estudio solamente 34 tuvieron estudio histopatológico subsecuente. La gran mayoría de las lesiones correspondieron a neoplasias malignas. En 30 casos el diagnóstico de la BAAF estuvo de aucerdo con el de la biopsia quirúrgica y no hubo acuerdo en 4. La sensibilidad del método fue del 97 por ciento y la especificidad del 33 por ciento. Conclusiones. Los resultados de este estudio indican que la BAAF es confiable para el diagnóstico inicial de tumores superficiales y profundos en niños

Mixed hepatoblastoma in an adult--a case report and literature review

Hepatoblastoma is thought to originate from embryonal hepatic tissue, and most of these tumors occur in children under the age of 2 years. Hepatoblastoma in adults is extremely rare, and the prognosis is much worse than the mixed hepatoblastoma of childhood. We experienced a case of mixed hepatoblastoma in a 51 year old female patient. She had been suffering from a mild pain and a palpable lump in the epigastric area. Serum AFP was 43,850 ng/ml. Computerized tomography and selective abdominal angiography showed a large low-density mass. With a suspicion of hepatocellular carcinoma of the left lobe, a left lateral segmentectomy was performed. The external surface showed a huge protruding mass and the capsule was previously ruptured. On section, the tumor was a 11 x 7 cm sized expanding mass which had a variegated surface composed of yellow-white friable tissue with multifocal hemorrhagic areas. Microscopic examination revealed a tumor consisted of epithelial and mesenchymal elements. The mesenchymal cells were spindle in shape and proliferated over the whole tumor with focal osteosarcomatous differentiation. The epithelial components showed well-differentiated hepatocellular carcinoma-like areas, poorly differentiated acinar or tubular structures.

Hepatoblastoma: experiencia en el tratamiento de 20 niños / Hepatoblastoma: experience in the treatment of 20 children

Introducción: el hepatoblastoma es el principal tumor maligno primario del hígado en niños. El objetivo del presente trabajo es analizar el perfil clínico, tipo de cirugía, complicaciones postoperatorias y sobrevida libre de enfermedad de acuerdo a la modalidad quirúrgica empleada, en niños con hepatoblastoma. Material y métodos: se revisaron los expedientes de pacientes con hepatoblastoma atendidos entre enero de 1985 y diciembre de 1995. Se registraron edad, sexo, manifestaciones clínicas, exámenes de laboratorio, alfa-feto proteína pre y postoperatoria, estudios de gabinete, tipo de procedimiento quirúrgico, complicaciones trans y postoperatorias variedad histológica, estudio, tipo de quimioterapia y sobrevida libre de enfermedad. Resultados: se analizaron 20 pacientes de cuatro meses a 13 años de edad. La principal e invariable manifestación clínica fue una masa abdominal. En once pacientes se encontraron niveles de alfa-feto proteína entre 100-10,000 ng/mL. En 12 pacientes se practicó hemihepatectomía; en cuatro, trisegmentectomía y en el resto, biopsia incisional diagnóstica. Hubo sangrado masivo en cuatro pacientes durante el transoperatorio; ninguno falleció. Todos los pacientes en estadio IV fallecieron. El tipo de resección, completa o incompleta, no fue estadísticamente significativo (p=0.49) para la sobrevida; ésta fue 7/20 con un seguimiento de cuatro a 104 meses. Conclusiones: 1. los niveles de alfa-feto proteína son útiles para la viginalcia y el diagnóstico de pacientes con hepatoblastoma. 2. La mayor sobrevida ocurre en pacientes operados y que recibieron quimioterapia. 3. Un segundo abordaje quirúrgico es preferible después de la citorreducción con quimioterapia en pacientes con tumores muy voluminosos o multicéntricos. 4. La conducta actual para el tratamiento del hepatoblastoma debe ser cirugía más quimioterapia independientemente del estadio

Procesos hemorrágicos hepáticos no traumáticos. Utilidad de la tomografía axial computada / Non traumatic liver hemorrhagic process. CT evaluation

Se estudiaron 42 pacientes (17 hombres y 25 mujeres), dos de ellas embarazadas y una en puerperio, para valorar la utilidad de la TAC en la detección de procesos hemorrágicos no traumáticos (PHNT) en 42 hepatopatías locales y difusas asociadas a hepatomegalia y masas palpables subcostales. Dieciocho pacientes (43 por ciento) presentaron PHNT. En 12 casos, sospechados por clínica, laboratorio, rayos X y US (67 por ciento) y en otros 15 por TAC (83 por ciento). Dos requirieron arteriografías (11 por ciento) y uno RM (6 por ciento) ante tomografías dudosas. Tres PHNT se localizaron en segmento hepático IV, tres en IV y V, dos en el VI, once en ambos hemihígados y uno en hemihígado izquierdo. Los PHNT se comprobaron mediante biopsia, estudios postmorten, aspiración dirigida o laparotomía. El US proporcionó imagen diagnóstica de la hepatopatía en 86 por ciento de los casos (36/42) y la TAC en 93 por ciento (39/42). Se destaca la utilidad de la TAC para sospechar PHNT en adenoma hepatocelular (3/3), hematomas de embarazo y puerperio (3/3), quistes hepáticos (1/4), policistosis (2/2), y colangiocarcinoma (1/1), así como sus limitaciones en hemangiomas gigantes (2/7) y metástasis (2/10)