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Spontaneous rupture of a hepatic sarcoma in an adult resembling hydatid cyst. Key words: Undifferentiated embryonic sarcoma. Rupture of liver sarcoma. Hydatid cyst.

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Spontaneous rupture of a hepatic sarcoma in an adult resembling hydatid cyst. Key words: Undifferentiated embryonic sarcoma. Rupture of liver sarcoma. Hydatid cyst. Undifferentiated embryonic sarcoma of the liver USL is a rare primary malignant liver tumour. It is most frequently seen in late childhood, but it is quite exceptional in adults 1,2. Only 11 cases have been reported in in patients over age As clinical presentation in adults it is poor and uncharacteristic USL can reach a large size cm.

Treatment is usually delayed for months due to vague symptoms and erroneous image findings 3. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with tumour-free margins, with or without postoperative chemotherapy 1.

A years-old female was referred to our Hospital Emergency with diffuse abdominal pain more intense in the right upper quadrant. The pain had started about 24 hours earlier. She did not present weight loss, fever, anorexia, vomiting or abdominal distension. She had been waiting for elective surgery for one month with hydatid cyst diagnosis. Physical examination revealed a healthy-looking adult woman.

The liver edge was palpable near 8 cm below the right costal margin. Laboratory data included hemoglobin, Computed tomography scans showed a large mass 18 x 12 cm involving the entire right lobe. It had multicystic appearance with solid portions and calcified capsule. There were capsule interruptions around the tumour and peritoneal free fluid. The patient underwent surgery and peroperatively enormous hepatomegaly and hemoperitoneum cc were observed due to spontaneous rupture in the peritoneal cavity of a tumour involving the entire right lobe of the liver Fig.

Blood was evacuated and right hepatectomy and cholecystectomy were performed, resulting in complete removal of the tumour with tumour-free resection margins. The patient had a postoperative uneventful recovery. No further adjuvant irradiation or chemotherapy was administered. The specimen from the right hepatectomy presented a ruptured capsule opposite to the resected margin.

Most of the parenchyma was replaced by a 15 x 15 x 11 cm multicystic tumour mass with well defined margins but no capsulation. Microscopic examination showed: sarcoma with features reminiscent of a rhabdomyosarcoma. One year after the operation, the patient remains well with no evidence of local recurrence or metastasis.

USL it is composed of undifferentiated mesenchymal cells and it is extremely rare in adulthood, mainly in patients over age As in our case, the tumour is a solitary, well-circumscribed solid-to-cystic lesion, most commonly occurring in the right lobe. Distant metastasis at the time of presentation are relatively uncommon. Because of the rarity of this tumour type in adults no prospective treatment studies or larger series reporting survival data and treatment strategies are available 4.

The median survival of all patients was 29 months. Patients who underwent complete tumour resection followed by adjuvant chemotherapy survived over a median follow-up of This is an important study but it is not a prospective one, groups are not comparable and it contains only a few number of cases. According to some authors 1,5 tumor resection with chemotherapy is indispensable for the treatment of ruptured USL in children. However, in adult patients over 65 years of age, as in our case, it has not yet been established that adjuvant chemotherapy after complete lesion resection can decrease recurrence or metastasis 6.

According to Lenze neoadjuvant therapy may become an option in the strategy for treating adults when diagnosis can be made pre-operatively 4. Our patient was 65 years old, which is older than the peak age group for USL. The patient had a large tumour with multiple cystic areas resembling hydatid cyst as reported in other cases 2,3. With this diagnosis she was waiting for elective surgery. Spontaneous tumour rupture and hemoperitoneum led us to perform an emergency operation. Tumour involved the entire right hepatic lobe but did not involve diaphragm nor inferior vena cava.

Right hepatectomy and cholecystectomy were performed with complete removal of the tumour with free resection margins. No adjuvant irradiation or chemotherapy was administered.

Bernardes, F. Manata, C. Rosenvinge, L. Coutinho, C. Marinho 1 and F. Departments of Surgery, and 1 Pathology. Coimbra University Hospital. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature. J Hepatobiliary Pancreat Surg ; 8: Primary multilocular cystic undifferentiated embryonal sarcoma of the liver in childhood resembling hydatid cyst of the liver. Br J Radiol ; Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.

Eur J Surg Oncol. Undifferentiated embryonal sarcoma of the liver in adults. Cancer ; 10 : The Internet Journal of Pathology ; 3 1 : Bras Patol Med Lab ; 43 6 : Servicios Personalizados Revista. Dear Editor, Undifferentiated embryonic sarcoma of the liver USL is a rare primary malignant liver tumour.

Case report A years-old female was referred to our Hospital Emergency with diffuse abdominal pain more intense in the right upper quadrant. Discussion USL it is composed of undifferentiated mesenchymal cells and it is extremely rare in adulthood, mainly in patients over age Oliveira Departments of Surgery, and 1 Pathology.

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Felty's syndrome is the triad of arthritis, splenomegaly and leukopenia. Additionally you can find many findings in this syndrome occurring in rheumatoid arthritis including pulmonary nodules. We report a patient with clinical and laboratory findings which show the presence of Felty syndrome, pulmonary nodules documents in addition to radiological and histological studies that documented chronic granulomas, bronchiolitis obliterans organizing pneumonia. Cryptogenic organizing pneumonia associated with Felty's syndrome.

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The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Annals of Pediatrics is the Body of Scientific Expression of the Association and is the vehicle through which members communicate. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same.

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Debe compararse siempre con el mejor valor obtenido de previo para cada paciente. Otras Enfermedades obstructivas:. Mantener niveles normales de actividad, incluyendo ejercicio. Evitar los efectos adversos de los medicamentos. Educar a los pacientes para que tomen parte activa en el manejo de su enfermedad. Establecer planes individuales para el manejo de las exacerbaciones. Dar seguimiento regular a cada caso.

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