An aggressive case of pseudomyogenic haemangioendothelioma of bone with pathological fracture and rapidly progressive pulmonary metastatic disease: case report and review of the literature.

Pseudomyogenic haemangioendothelioma (PMH) is a rare recently described vascular tumour typically presenting with soft tissue disease in distal extremities of young adults. Multi-focal and multi-layered involvement is commonly recognised. The majority of cases described so far have shown an indolent clinical course and distant metastatic spread is rare. We report a case of PMH in an 82-year-old male diagnosed following a pathological fracture of the distal tibia. Further bone lesions were identified in the fibula, patella and distal femur. The patient was found to have multiple nodules suspicious for pulmonary metastases on a CT scan at the time of diagnosis that showed significant progression at a follow-up scan 4 weeks later. To our knowledge, this is the first reported case of PMH presenting with a pathological fracture. The rapid progression of bone and distant metastatic disease in this case is highly unusual given the typically indolent clinical course reported in the literature to date.

Pseudomyogenic hemangioendothelioma: report of an additional case with aggressive clinical course.

: Pseudomyogenic hemangioendothelioma is a recently described vascular tumor that occurs predominantly in the distal extremities of young adults. Because of multifocal presentation, epithelioid morphology, and strong cytokeratin expression, the tumor was frequently misdiagnosed as epithelioid sarcoma. However, substantial immunohistochemical studies revealed an endothelial nature. It has been currently considered a tumor of intermediate malignancy with frequent local recurrence but low risk of distant metastasis. In this report, we describe a case of pseudomyogenic hemangioendothelioma occurring in a 22-year-old man who presented with multifocal disease in the lower extremity and developed bilateral pulmonary metastases within a short period.

Composite hemangioendothelioma of the spleen with multiple metastases: CT findings and review of the literature.

ABSTRACT: Composite hemangioendothelioma (CHE) is a rare vascular neoplasm of intermediate malignant potential. Only 52 cases have been reported in the English literature, and one case previously reported occurred in the spleen. The purpose of our study was to report a 65-year-old man diagnosed as CHE primary arising from the spleen with multiple metastases.Clinical and imaging features, laboratory tests, and pathological results about CHE were described in detail in this study.The patient presented with multiple lesions in bilateral lungs and spleen that had been incidentally detected by computed tomography (CT). Except for thrombocytopenia, other laboratory tests were not significant. The CT scan of the abdomen revealed multiple round-like and irregularly mixed density masses with unclear borders in enlarged spleen. And contrast enhancement showed mild heterogeneous enhancement. CT scan also showed widespread liver, ribs, lungs, and vertebral bodies metastases. This diagnosis was confirmed by histopathological examination. The patient underwent splenectomy and still survives with tumors after six months followed-up.Due to the lack of specificity of clinical features and laboratory tests, it is necessary to combine imaging features and pathological findings to make a correct diagnosis.

Primary malignant bone neoplasm: a case report of dedifferentiated chondrosarcoma in the rib and review of the literature.

Dedifferentiated chondrosarcoma (DDCS) is a rare but highly malignant primary bone neoplasm, which is resistant to radiotherapy and chemotherapy. There remains uncertainly as to the best treatment of this disease and how to improve its prognosis. In this paper we reported a case of DDCS and reviewed the related literatures in order to provide references to throw a light on the histogenesis, diagnosis and therapy of this disease.

Composite haemangioendothelioma with lymph-node metastasis: an unusual presentation at an uncommon site.

A 48-year-old woman presented with red papules on the thigh. Histopathological examination indicated pyogenic granuloma, and the patient was treated with total excision in 2003 and electrocauterization in 2005. Three months later, upon recurrence of the lesions, a diagnosis of composite haemangioendothelioma (CHE) was made. The patient was treated by total excision and lymph-node dissection, which revealed inguinal lymph-node metastasis. Despite the surgery, a further local recurrence occurred, subsequently treated by wide excision en bloc, with adjuvant radiotherapy and chemotherapy. Although CHE is defined as a vascular tumour with low-grade malignancy, the local recurrences and lymph-node metastases resulted in treatment difficulties in this case. Unlike earlier cases, the tumour in our patient presented as localized numerous small papulonodules, and lymph-node metastasis was detected within a relatively short time. Dermatologists and pathologists should be aware of this rare condition and include it in the differential diagnosis of vascular lesions.

Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome.

A 36-year-old woman was diagnosed with compensated cirrhosis of liver secondary to Budd-Chiari syndrome (BCS) and had undergone stenting of a thrombosed left hepatic vein. Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. Four months later, magnetic resonance cholangiopancreatography showed an advanced stage of HHE with infiltration of the common bile duct and vascular invasion with a blocked stent, with metastasis to the spleen and dorsolumbar vertebrae. We believe this is the first reported case of an HHE developing in the background of BCS.

Metastasizing splenic littoral cell hemangioendothelioma.

Littoral cell angioma is a unique splenic tumor that is generally considered to be benign. We present a case of a low-grade littoral cell splenic tumor that metastasized to the liver and retroperitoneum 4 years after splenectomy. Although the splenic lesion showed the typical morphology of a littoral cell angioma, it also contained areas with unusual solid nests of cytologically bland, plump cells with clear cytoplasm. The liver was diffusely infiltrated exclusively by cells with similar clear cell features. Both splenic and liver lesions demonstrated identical immunophenotypes, typical of littoral cell angioma, expressing CD31, CD68, CD21, and CD163, although negative for CD8 and CD34. A single prior description of a littoral cell hemangioendothelioma showed nuclear atypia and necrosis, and this is the first case report of a splenic littoral cell hemangioendothelioma with a completely bland histologic appearance. This case suggests that the presence of solid areas of clear cells in a littoral cell angioma may be a marker of low-grade malignant potential in these tumors.

Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases.

The clinical, morphologic, and follow-up findings in 32 patients with a hitherto rarely reported tumor of the liver are reported. The study comprised 20 women (62.5 per cent) and 12 men (37.5 per cent) ranging in age from 19 to 86 years (average, 49.65 years). The tumors were discovered incidentally in four patients (12.5 per cent). Four patients (12.5 per cent) had jaundice attributable to the tumors; one of these patients experienced liver failure. One patient presented with an acutely painful abdomen due to hemoperitoneum. The remaining patients had nonspecific complaints. Grossly, the tumors were often multiple and involved both lobes of the liver. They were generally white and firm to hard. Microscopically, the neoplastic cells infiltrated sinusoids and intrahepatic veins of all sizes. Two types of tumor cells were identified--dendritic and epithelioid. Tumor cells were also vasoformative and synthesized Factor VIII-related antigen. Nine patients survived five years or longer. Two of these patients were alive five years, two nine years, one 12 years, and one 15 years after the onset of disease. Three patients died seven, ten, and 28 years, respectively, after the initial diagnosis. This vascular tumor has distinctive morphologic features that allow differentiation from sclerosing carcinoma and angiosarcoma. Although the prognosis is much more favorable than that for angiosarcoma, extrahepatic metastases occurred in nine of the patients (28 per cent) in this series. The biologic behavior of the tumor may be related in part to its matrix, which may show inflammation, dense sclerosis, and calcification.

Angiosarcoma of the liver in children.

The clinico-pathological features of four cases of pediatric hepatic angiosarcomas are described. One case was initially diagnosed in infancy and underwent resection of a left-sided benign hemangioendothelioma of the liver. Further resection of a lesion in the right liver was followed by malignant transformation. Primary hepatic resection of the tumor was not possible in three other cases, and all received courses of chemotherapy without significant tumor shrinkage. One child underwent liver transplantation but died 4 months later of immunosuppression complications. The remaining three children died of disseminated malignancy. Histological verification of malignancy was difficult in three cases in which there was discrepancy between the rapid growth of the liver tumor and the microscopic features of benign hemangioendothelioma.

Sudden death due to rupture of an omental metastatic tumor arising from cardiac angiosarcoma. A case report.

A 32-year-old man underwent surgical excision of a malignant hemangioendothelioma of the heart and received multidisciplinary treatment. Thirty-three months later, he underwent a second surgical treatment for a recurrent tumor of the posterior chest wall of the right thorax. Five months after this surgery (thirty-eight months after the initial surgery), a second recurrent tumor in the right thorax developed. Although radiotherapy and recombinant interleukin-2 were administered, anemia of unknown origin (hemoglobin 6.7 g/dl) developed. The patient died of sudden shock due to a rupture of an omental metastatic tumor. The patient survived for 41 months after the first surgical resection. We present this case because it is the first reported case of sudden death due to bleeding from an omental metastatic tumor, and because our patient was the second-longest survivor after surgical treatment for cardiac angiosarcoma.

Sudden death with malignant hemangioendothelioma originating in the pericardium--a case report.

The authors report a rare case of a malignant hemangioendothelioma (MH) originating in the pericardium. In this case, a metastatic skin lesion was found first, and subsequently the existence of a primary cardiac lesion was confirmed. Generally, primary cardiac tumors grow slowly, and the prognosis of MH is relatively good. In this case, however, the patient died suddenly during the creation of a pericardial window for drainage. An autopsy showed that the MH originated from a pericardial lesion in the right atrium.

Malignant hemangioendothelioma presenting as multifocal intraskeletal lesions during pregnancy. A case report.

BACKGROUND: Malignant hemangioendothelioma is a neoplasm of vascular origin characterized by irregular vascular channels lined with atypical endothelial cells. CASE: A gravida at 32 weeks' gestation presented with diffuse back and lower leg pain and was diagnosed with multifocal malignant hemangioendothelioma of bone. Computed tomography of the chest also demonstrated a small right atrial density. Three weeks later the patient became septic, and cesarean section was performed. After several cycles of chemotherapy, clinical improvement was noted. However, disease progression was noted thereafter, and the patient died one and a half years after the diagnosis. CONCLUSION: Our case was a primary multifocal malignant hemangioendothelioma of bone arising during pregnancy. Considering the absence of pulmonary involvement, it is unlikely that the skeletal lesions represented metastatic deposits from a cardiac primary. With such extensive skeletal disease, the right atrial density probably was a metastatic deposit.

Cytology of hemangioendothelioma.

The cytologic appearance is described of hemangioendothelioma in fine needle aspiration smears from a young male who presented with recurring bilateral lesions within two years. The cytomorphologic pattern was extremely cellular, with a monomorphic population of cells, some of which showed a tendency to form rosettelike or pseudoacinar formations.

Epithelioid hemangioendothelioma of spleen with intrasplenic metastasis: ultrasound and computed-tomography appearance.

The US and CT appearances of epithelioid hemangioendothelioma of the spleen with intrasplenic metastasis have not been previously reported. We described a 29-yr-old female with such a disease. Abdominal US study revealed a large mass in the upper pole and multiple small nodules in the rest of the spleen. CT scan also showed similar lesions of hypodensity which were not apparently enhanced by contrast medium.

Hemopneumothorax due to pulmonary metastasis with malignant hemangioendothelioma: CT and pathological findings.

Malignant hemangioendothelioma is a rare vascular tumor. The disease frequently recurs and eventually metastasizes to the lung in most cases. Hemopneumothorax developing soon after metastasis to the lung indicates on unfavorable prognosis. We had an opportunity to examine a patient who had malignant hemangioendothelioma in the occipital scalp and developed hemopneumothorax following metastasis to the lung. We report the computed tomographic (CT) and pathological finding in the patient, and review the literature on hemopneumothorax.

Scintigraphic features of malignant epithelioid hemangioendothelioma.

The scintigraphic features of malignant epithelioid hemangioendothelioma of the liver have not been previously documented. In particular, there are no reports on the nature of gallium uptake in these rare tumors. The case reported is a young woman with primary hepatic epithelioid hemangioendothelioma. Metastatic disease in the skull was detected on a Tc-99m MDP bone scan. A Ga-67 study demonstrated that this tumor was not gallium avid.

Primary malignant hemangioendothelioma of jejunum.

Primary malignant hemangioendothelioma is a rare tumor. We report a patient with a malignant jejunal hemangioendothelioma which had metastasized to the regional lymph nodes and the liver.

[A case of metamorphopsia restricted to faces and different familiar objects]. / Etude d'un cas de métamorphopsie limitée aux visages et à certains objets familiers.

The case of a 19-year old patient suffering of transient metamorphopsia restricted to familiar faces and familiar objects is reported. This clinical sign resulted from a small right occipitotemporal haemorrhage due to a sub-cortical metastasis. The patient claimed that faces are distorted and look more pleasant. There were neither visual field defects nor visual agnosia. MRI revealed a small high signal area in the right fusiform gyrus. The structural and functional aspects of the metamorphopsia are documented and discussed in relation to aperceptive prosopagnosia. More specifically, it is suggested that facial metamorphopsia and aperceptive prosopagnosia express the same underlying disorder differing only in terms of severity.