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 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.

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.

Radiation therapy for hemangioendothelioma: the university of Florida experience.

OBJECTIVES: The benefit of radiotherapy (RT) for unresectable hemangioendotheliomas or patients with a high risk of local recurrence is unclear. This single-institution report describes the long-term effectiveness of RT for hemangioendothelioma. METHODS: From 1976 to 2009, 14 patients with nonmetastatic hemangioendothelioma were treated with RT at our institution. Median patient age was 45 years (range, 20 to 71 y). Nine patients had hemangioendothelioma of the extremities and 5 had spinal tumors. Eleven tumors originated from bone. Most tumors (n=12) were ≤5 cm in diameter. Nine patients had multifocal tumors. Four patients underwent surgery and postoperative RT, whereas 10 had RT alone. All 4 operative patients had microscopic negative margins. Median RT dose was 52.2 Gy (range, 45 to 60 Gy) for RT alone and 62.2 Gy (range, 60 to 64.8 Gy) for postoperative patients. Seven patients received 1.5-2 Gy once daily and 7 patients received 1.2 Gy twice daily. The median follow-up was 10.3 years (range, 0.1 to 28.0 y). RESULTS: The 10-year local control, cause-specific survival, and overall survival rates were 100%, 86%, and 73%, respectively. Two patients experienced a distant metastasis and died within 3 months of starting definitive RT. Three patients died of intercurrent illness at a median of 10.5 years after treatment. Nine patients had no evidence of disease at most recent follow-up. No patients experienced greater than grade 1 acute or late toxicity from RT. CONCLUSIONS: With no local recurrences and minimal risk of toxicity, our data suggest that RT can effectively manage this disease and radical surgery compromising function or cosmesis may be safely avoided with moderate-dose RT.

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.

Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and childhood. This tumor results in poor prognosis, and therefore, development of a more effective treatment is needed. METHODS AND RESULTS: We describe an 11-year-old boy presenting with left facial palsy caused by aggressive KHE of the left temporomastoid region. He was treated with paclitaxel-based chemotherapy, because of the difficulty with complete surgical resection for anatomic factor, multiple lung metastases on diagnosis, and no response to conventional treatments. This treatment reduced the volume of primary tumor and lung metastatic lesions, but the efficacy was transitory. CONCLUSIONS: Paclitaxel-based chemotherapy for aggressive KHE may be effective, therefore the multimodality therapy including paclitaxel of aggressive KHE, particularly in the head and neck, needs to be investigated in further studies.

A rare angiosarcoma: retiform haemangioendothelioma.

OBJECTIVE: We report the case of a rare angiosarcoma, retiform haemangioendothelioma, in an 18-year-old young man, which presented as a recurrent ulcerating lesion of the left pinna. METHOD: Case report and literature review of retiform haemangioendothelioma. This is a low grade angiosarcoma with a high local recurrence rate and low metastasis rate, and was first described in 1994 by Calonje et al. RESULTS: This patient represents only the third report of lymph node metastasis in a case of retiform haemangioendothelioma. To date, 31 cases of the tumour have been reported. Histological diagnosis of this group of vascular neoplasms can be challenging, as their histopathological appearance is intermediate between haemangioma and angiosarcoma. CONCLUSION: Surgical excision remains the primary treatment modality, with adjuvant radiotherapy recommended in patients with large tumour size, local recurrence and lymph node metastasis, as seen in this case.

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.

Infantile hemangioendothelioma: A case report and discussion.

Infantile hepatic hamangioendothelioma type II is similar to angiosarcoma in terms of histomorphology and behavior. Various presentations of this lesion have been reported in the literature, e.g. cases with a hepatic mass, cutaneous hemangiomas, heart failure, etc. We report on a patient, male/2 years, who had two jejunal masses and a hepatic mass accompanied by lower GI bleeding and intestinal obstruction. The two jejunal masses and the hepatic lesion were diagnosed as angiosarcoma histomorphologically (IHHE type II), and were positive for vascular markers (CD31 and CD34) on immunohistochemistry. The patient had no skin lesions. We report this case and provide a literature review because of the unusual presentation and the overall rarity of this entity.

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.

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.

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.

Malignant vascular tumors in young patients.

BACKGROUND: To the authors' knowledge there are few published series of malignant vascular tumors in patients age < or = 21 years. METHODS: The authors retrospectively documented the clinical presentation, pathology, treatment, and outcome of patients age < or = 21 years with malignant vascular tumors treated between 1970-1995 at Memorial Sloan-Kettering Cancer Center. The histologic sections were rereviewed to confirm the diagnosis. RESULTS: Four patients were identified with angiosarcoma and two with malignant hemangioendothelioma. Five patients were female and one was male; the median age at diagnosis was 11.8 years (range, 8 months-21 years). The tumor involved the skin in one patient, soft tissue in one patient, bone in two patients, and internal organs in two patients. One patient had associated diffuse angiomatosis and another had the Klippel-Trenaunay-Weber syndrome. This patient received prior radiation therapy to the primary site with the subsequent development of a vascular sarcoma. None of the patients had distant metastases at diagnosis. Resection was attempted in five patients and completed in four. Chemotherapy alone was given to three patients whereas radiation therapy alone and radiation plus chemotherapy were administered to one patient each. The median follow-up was 4.9 years (range, 1 month-12 years). There were three deaths from progressive disease and two deaths from locoregional recurrences. Reexcision and radiotherapy controlled one local recurrence. Another patient developed recurrence to regional lymph nodes and further dissemination. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease underwent an incomplete excision or biopsy alone. CONCLUSIONS: Malignant vascular tumors are rare in the first two decades of life and when they do occur are very aggressive. Complete resection is curative for patients with localized lesions.