Sclerosing haemangioma of the lung in a 4-year-old child.

Sclerosing haemangioma of the lung (SHL) should be recognised as a distinct clinicopathological entity. It is a benign neoplasm, probably of epithelial origin. Clinically, the tumour is asymptomatic and shows a striking preponderance in middle-aged women. SHL is often detected incidentally, as a round, well-defined homogeneous mass on routine chest radiograms. The diagnosis is based on pathohistologic examination of the biopsy material, therapy is surgical, and prognosis is excellent. We report a case of a 4-year-old boy with SHL, which is extremely rare in childhood.

Heart metastasis of extraskeletal myxoid chondrosarcoma.

Isolated, remote heart metastasis of myxoid chondrosarcoma is extremely rare. We present an unusual case of isolated remote metastasis of extraosseus myxoid chondrosarcoma from the right ankle region to the right ventricle, its clinical course, and treatment in a 46-year-old woman. Although heart biopsy done before the surgery revealed myxoma, histopathologic diagnosis of the heart tumor was confirmed after its surgical resection from the right ventricle. Nine months after the surgery the patient was doing well but, seven months later, she died in the local hospital because of global heart failure. On the autopsy, the only metastatic lesion found was in the heart. The pericardium and heart muscle were infiltrated with the tumor, whereas all other organs were free from malignant dissemination.

Intracardiac juvenile xanthogranuloma in a newborn.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

Ultrastructural analysis and TUNEL demonstrate motor neuron apoptosis in Werdnig-Hoffmann disease.

Werdnig-Hoffmann disease (WHD) is the most severe clinical type of spinal muscular atrophy characterized by loss of lower motor neurons and paralysis. We examined the hypothesis that disease pathogenesis is based on an inappropriate persistence of normally occurring motor neuron programmed cell death. The diagnosis of WHD was made on the basis of clinical findings, electromyoneurography, and biopsy, and further confirmed by mutation analysis of the survival motor neuron (SMN) and neuronal apoptosis inhibitory protein (NAIP) genes using PCR. We used ultrastructural analysis as well as TUNEL and ISEL methods to assess DNA fragmentation, and immunocytochemistry to identify expression of the apoptosis-related proteins bcl-2 and p53. A significant number of motor neurons in the spinal cord of children with WHD were shown to die by apoptosis. As revealed by TUNEL, dying neurons in WHD patients comprised 0.2%-6.4% of the neuron numbers counted. This finding contradicts earlier studies that failed to find such evidence and suggests that early blockade of prolonged motor neuron apoptosis may be a potential therapeutic strategy for WHD.

Massive osteolysis in a girl with agenesis of thyroid C cells.

A rare case of massive osteolysis affecting the pelvis of a young girl is presented. The clinical, radiographic, and histopathological features are described in detail. Septicemia complicated the clinical course and the patient eventually died. Histopathological examination of the pelvic lesion revealed massive osteolysis characterized by prominent osteoclastic activity with extensive bone resorption. A thorough post-mortem histological examination of the thyroid gland showed no C cells. This is an interesting observation, since it is known that thyroid C cells are the primary source of calcitonin. Since the main biological effect of calcitonin is to inhibit osteoclastic bone resorption, there is a possibility that massive osteolysis in our case could have been related to the lack of this hormone. There is a clear need for further investigation regarding the role of thyroid C cells and calcitonin in this puzzling disease.

[The Budd-Chiari syndrome--case report of a patient with hepatic vein occlusion and adenocarcinoma of the lung]. / Budd-Chiarijev sindrom--prikaz bolesnika s okluzijom hepatalnih vena i adenokarcinomom pluca.

The case of a patient with sudden onset of abdominal pain, hepatomegaly and laboratory findings which indicated necrosis of a parenchymal organ is reported. Patient died soon after the admission to hospital after fulminant disease development. At the time of admission diagnosis of hepatic vein occlusion, i.e., Budd-Chiari syndrome was made based on ultrasonographic finding. Obduction confirmed this finding showing lung adenocarcinoma with liver metastases as well as occlusion of all hepatic vein branches with thrombi. This report emphasizes the role of ultrasonographic diagnostics in the early diagnosis of Budd-Chiari syndrome.

Prostatic polyp in the prostatic urethra.

The authors report on a case of prostatic polyp in the urethral bulb of a 35-year-old man. The presence of this lesion in the urethral bulb is quite unusual and may be overlooked on cystocopic evaluation.

Cystosarcoma phyllodes of the prostate.

We report a case of a 40-year-old man with cystosarcoma phyllodes of the prostate. This tumour is very rare in the prostate. The stroma of the tumour was cellular and composed of elongated cells with spindle shaped nuclei, scanty, pale and eosinophilic cytoplasm. Some cells showed nuclear atypia but the cells did not display mitotic activity. We categorised this tumour as benign. The suprapubic prostatectomy was done. The patient is alive and well after three years.

[Sclerosing hemangioma of the lung]. / Sklerozirajuci hemangiom pluca.

The sclerosing hemangioma of the lung is a rare benign tumor. Its histogenesis has not been explained yet. Following the electron-microscopic and immunohistochemical researches, the opinions have been still unhomogeneous. Therefore, it is concluded that is a tumor of epithelial, endothelial, mesenchymal and even mesothelial origin. This study deals with this tumor. Its immunohistochemical analysis points at its epithelial character.