1/8. Progressive development of diffuse liver hemangiomatosis.Diffuse liver hemangiomatosis is extremely rare. The etiology and natural history of the disease are unknown. It is also unclear whether tumor growth is induced or modulated by drug therapy. Tumor recurrence after ablative therapy has not been described in patients with diffuse liver hemangiomatosis. Diffuse hemangiomatosis of the left hepatic lobe was suspected in a 35-year-old woman by ultrasonography, CT and hepatic arteriography, and confirmed by laparotomy and biopsies. The patient denied any drug or estrogen use. The tumor was removed by left hepatectomy. Two and six years later, the patient was again hospitalized with progressive tumor growth into the right hepatic lobe. Although diffuse liver hemangiomatosis is a rare disease, its diagnosis should be considered in patients with progressive tumor growth in one or both hepatic lobes. The absence of drug intake or estrogen use does not exclude the diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. The successful treatment of diffuse neonatal hemangiomatosis with flashlamp pulsed dye laser.Diffuse neonatal hemangiomatosis is a rare disease with the distinctive features of multiple hemangiomas of the skin and visceral organs. These lesions have been treated with systemic corticosteroids, interferon-alpha, and their combination. We report a patient with diffuse neonatal hemangiomatosis who had multiple cutaneous and hepatic hemangiomas. Single therapy with the flashlamp pulsed dye laser was effective for cutaneous hemangiomas, whereas the hemangiomas of the liver remained stable and no liver dysfunction or hemorrhage has occurred so far, even with no treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Maffucci's syndrome (hemangiomatosis osteolytica): a report of four cases.Maffucci's syndrome is a congenital nonfamilial syndrome combining dyschondroplasia, (enchondromatosis) and hemangiomatosis. It is a rare disease; only 200 cases have been reported throughout the world in the past 140 years. Over the past 20 years, four patients have been admitted with signs and symptoms consistent with Maffucci's syndrome. Three were children ages 3, 7, and 9 years. The fourth was 23 years old. Two were male and two female. All had hemangiomas at birth, and all had skeletal deformities and enchondromas. All complained of pain and heaviness of the involved extremity. Three patients had the arterial inflow evaluated with arteriograms, and one had magnetic resonance imaging. Two also had venograms. Two patients had excision of their hemangiomas, and one had sclerotherapy and compression therapy. All had bone biopsies performed. None of the enchondromas or the soft tissue lesions had undergone sarcomatous transformation.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome--three case reports and review of the literature.Ollier's disease is a rare disease characterized by constitutional bone dysplasia with multiple enchondromas. The combination of haemangioma and chondromatoses is known as Maffucci's syndrome. Malignant degeneration of bone dysplasia into chondrosarcoma is a well-known complication, but a lesion of the base of the skull is exceptional. It is a slowly growing low-grade malignant tumour. Three new cases of chondrosarcoma of the base of the skull occurring in one patient with Marfucci's syndrome and two patients with Ollier's disease are reported and these cases are discussed in the light of the literature. A multidisciplinary approach, comprising surgery and radiotherapy, achieved good results.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Blue rubber Bleb Nevi as a cause of intussusception.The Blue rubber Bleb nevus syndrome is a rare disease characterized by a distinctive type of hemangioma which involves the skin and the gastrointestinal tract. In the latter location, these lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may act as a leading point for an intussusception. The diagnosis of intussusception in children older than 3 or 4 yr is frequently difficult and delayed. In a child with typical skin lesions of the Blue rubber Bleb nevus syndrome, an acute illness with manifestations of intestinal obstruction should indicate the possibility of an associated intussusception.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. portal vein aneurysm in the liver associated with multiple vascular malformations.portal vein aneurysm (PVA) includes focal dilatation of the portal vein, and was formerly thought to be a rare disease. We report a 46-year-old man with chronic aggressive hepatitis and intrahepatic portal vein aneurysm communicating with the hepatic vein. Hemangiomas in the liver and intracranial arteriovenous malformation (AVM) were also found. To our knowledge, this is the first report of a case of PVA in a patient with congenital intracranial AVM. As the PVA in this patient communicated with the hepatic vein, and as hemangiomas in the liver and intracranial AVM were also present, the pathogenesis in this patient seems to have been congenital anomaly of the vasculature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Cystic adrenal mass: pseudocyst associated with hemangioma.We present a 70-year-old man with cystic adrenal tumor associated with hemangioma. The pathogenesis of this rare disease is discussed with special reference to its diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Familial idiopathic myelofibrosis and multiple hemangiomas.Idiopathic myelofibrosis (MF) is a rare disease in childhood. The clinical spectrum is very variable. Familial idiopathic MF has been recorded exceptionally. In previous reports idiopathic MF in childhood has been described in association with congenital anomalies and with chromosome abnormalities, although neither of these features have been reported in a familial context. We report two sisters with idiopathic MF and multiple eruptive hemangiomas. Details of their clinical signs, laboratory findings, and histologic features are described.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |