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Introduction: Hepatic epithelioid haemangioendothelioma (HEH) is a rare and highly malignant tumor with a vascular component,-it arises from epithelioid endothelial cells. Only about 500 cases are described in world scientific literature. The aim: To review this rare, malignant pathology, to submit standards of diagnostic and treatment. To present two clinical cases of HEH, which were treated at Klaipeda University hospital. The methods: Two patients were treated at Klaipeda University hospital, period of treatment 2007 – 2013. Both patients were diagnosed with advanced HEH. The results: Patient 1: E.B., 48 years old man. In 21 09 2007 after performed radiological examinations of thoracic cavity and abdominal organs were diagnosed multiple derivatives in the lungs, mediastinum and liver. Biopsy of liver derivatives with immunohistochemistry confirmed the diagnosis of HEH. The patient was diagnosed with advanced HEH. Due to the spread of the disease, surgical treatment was not applied. In 03 07 2008 the patient started chemotherapy treatment (Docetaxel). In 25 07 2008 diagnostic laparoscopy confirmed disease progression, symptomatic treatment was appointed. The patient died in 25 08 2008. Final biopsy with immunohistochemistry confirmed the diagnosis of HEH. The death of the patient was caused by progressive HEH. Patient 2: R.R., 45 years old man. In 29 05 2009 after performed radiological examinations of thoracic cavity and abdominal organs were diagnosed: 3.6 cm. size derivative in mediastinum and 4 cm. size derivative in the liver. Biopsy of liver derivative with immunohistochemistry confirmed the diagnosis of HEH. In 05 06 2009 the patient was operated, resection of liver segments S3 – S4A was performed. After operation performed control computed tomography (CT) of thorax, mediastium derivative decreased to 2 cm. size. The patient felt well and was observed by chemotherapists of Klaipeda University hospital 3 years. In 03 10 2012 after performed computed tomography (CT) of abdominal organs and thoracic cavity disease progression was determined: new derivatives in the liver, 13.5 mm. size in S8 segment and 27.9 mm. size in S5 segment, also 3.7 cm. size derivative in mediastinum and new focals on both sides of the lungs up to 8 mm. size. In 10 01 2013 the patient was operated, radiofrequency ablation was applied for liver derivatives mentioned above. One month later, after performed control computed tomography (CT) of abdominal organs new focals are not observed in the liver, destruction zones are visible after termoablation. The patient condition is fully satisfactory, hereinafter his health should be observed by chemotherapists. Conclusions: Hepatic epithelioid haemangioendothelioma. is a rare, highly malignant tumor. The disease occurs without apparent clinical expression and tends to get worse quickly. Diagnosis is based on radiological studies and biopsy derivatives with immunohistochemistry. Number, size, location of HEH derivatives defines opportunities of surgical treatment. Surgery remains the standard of treatment - liver resection or transplantation. The role of systemic chemotherapy is uncertain. Metastazic disease does not correlate with survival. Differences of treatment results do not allow to conclude exact treatment algorithms, therefore the strategy of the treatment is discussed considering to the each specific clinical case, during the time of multidisciplinary Counsel. doi:10.5200/sm-hs.2013.070

A boy who had been diagnosed with chronic granulomatous disease (CGD) at the age of 6.5 years had a medical history of multiple bacterial infections, including pneumonia, staphylococcal liver abscesses and septicemia, from birth. At the age of 10 years and 4 months he developed an infection that was accompanied by high fever and pulmonary, mediastinal and paravertebral infiltrations. Aspergillus niger was cultured on bronchial secretions obtained by bronchoscopy. Shortly thereafter, proteinuria manifested and progressed to the nephrotic level. A skin biopsy indicated a diagnosis of amyloidosis. An anti-fungal treatment with amphotericin B and other agents, along with surgical pus drainage, intravenous leukocyte mass, interferon-gamma and immunoglobulin infusions, was ineffective, and the patient eventually died from multi-organ failure. The postmortem examination revealed the presence of disseminated aspergillosis and systemic amyloidosis. Although no direct evidence is available that would confirm the causative role of aspergillosis in the development of systemic amyloidosis, to the best of our knowledge this is the first report of a CGD case with complications of both invasive aspergillosis and systemic amyloidosis.

Dermatofibrosarcoma protuberans is a very rare malignant neoplasm in children. It is reported that the classic form of dermatofibrosarcoma has a very limited metastatic potential, while a fibrosarcomous transformation of dermatofibrosarcoma protuberans has a much higher potential. We present a case of a 17-year-old boy with a huge ulcerated mass (17 x 15 cm) on his right thigh. At operation, the pathological diagnosis was dermatofibrosarcoma protuberans with a fibrosarcomous component. All lesions were removed by surgical excision with a margin of 1.5 to 3 cm. After one year, there is no evidence of tumor recurrence.