Kaposiform haemangioendothelioma in an adult: lack of response to topical sirolimus and response to radiotherapy.

Kaposiform haemangioendothelioma (KHE) is a rare, primarily paediatric tumour with only a handful of case reports in the adult population. Given the paucity of evidence, this article is important in raising awareness of radiotherapy as a suitable and effective treatment in the adult population with KHE and highlights the potential limitations of topical sirolimus in these tumours.

Multifocal hemangioendothelioma of the lumbar spine and response to surgical resection and radiation.

BACKGROUND CONTEXT: Epithelioid hemangioendothelioma rarely occurs in the lumbosacral spine, with very few case reports of spinal hemangioendothelioma in the literature. There is variability in aggressiveness of these lesions without established treatment guidelines. PURPOSE: The aim was to present a case of epithelioid hemangioendothelioma in the lumbar spine, including magnetic resonance imaging (MRI) findings, which rapidly progressed over a 2-month period as regional multifocal lumbosacral spinal lesions with epidural extension causing severe spinal canal stenosis. STUDY DESIGN/SETTING: This was a case report in a university hospital setting. PATIENT SAMPLE: The sample included an otherwise healthy adult male with low back pain. METHODS: Multimodality imaging was performed to help with diagnosis and management including computed tomography, MRI, and positron emission tomography (PET). The patient was treated by embolization, L5 corpectomy and L4-S1 stabilization, and radiation therapy. The diagnosis was confirmed by tissue biopsy. RESULTS: The patient initially presented with severe back and leg pain after a vertebroplasty for an L5 compression fracture at an outside hospital where biopsy was negative for malignancy. Magnetic resonance imaging showed diffuse abnormality of L5 with several smaller lesions in the sacrum. Due to progressive pain 2 weeks after the vertebroplasty, the patient underwent an L5 laminectomy, L4-S1 instrumented posterior fusion, and attempted partial corpectomy for stenosis. At this surgery, the L5 corpectomy was aborted owing to profound bleeding. Pathology was again negative for malignancy. Presumed to be an atypical hemangioma, the lesion was embolized before repeat surgery where the thecal sac was decompressed by partial L5 corpectomy. Biopsy at this time revealed a vascular neoplasm, with hemangioendothelioma not excluded. Approximately 2 months after the stabilization procedure, the patient had increasing pain and bilateral lower extremity weakness. Magnetic resonance imaging was performed and demonstrated marked local progression of disease with new multifocal lesions involving L4 through S2 vertebrae and new severe spinal canal stenosis. These lesions were subsequently treated with localized radiation therapy. Magnetic resonance imaging 2 months after radiation therapy showed significant regression of the epidural tumor although a new metastatic lesion was discovered at T6 vertebra. CONCLUSIONS: Spinal hemangioendothelioma is a rare disease and can present in variable forms, including as a multifocal regional process--which may be mistaken for infection. Additionally, there are no standard treatment protocols for this entity. We present the extensive imaging and treatment of a single case of rapidly progressive lumbar epithelioid hemangioendothelioma, which to our knowledge has not been described with this multifocal appearance in the lumbar spine.

Congenital kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon successfully treated with low-dose radiation therapy.

Kaposiform hemangioendothelioma (KHE) associated with Kasabach-Merritt phenomenon is a life-threatening vasculopathy. The current mainstay treatment for KHEs is corticosteroids and chemotherapy, but these medications do not work for all patients and carry significant side effects. We report a neonate with a large congenital KHE who responded extremely well to low-dose radiation therapy.

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.

[Giant vascular tumour in an adult: tufted angioma or kaposiform hemangioendothelioma]. / Tumeur vasculaire géante de l'adulte: angiome en touffe ou hémangioendothéliome kaposiforme.

INTRODUCTION: Tufted angioma and kaposiform hemangioendothelioma are two rare benign but aggressive vascular tumours that occur mainly in children. OBSERVATION: A 72 year-old man consulted for a 50 cm wide vascular tumour of the right shoulder which was increasing for 10 years. On histological examination there were features of tufted angioma and kaposiform hemangioendothelioma. DISCUSSION: The tumour of this patient was atypical because of its big size never described before. The histological association of aspects which could correspond to tufted angioma and kaposiform hemangioendothelioma seems to confirm recent publications which support the hypothesis that these two tumours are two evolutive stages of one and only entity.

[Spindle-cell hemangioendothelioma of the pharynx managed by radiotherapy]. / Hémangioendothéliome à cellules fusiformes du pharynx traité par irradiation.

Spindle cells hemangioendotheloma generally reaches the extremities. Only one oropharyngeal localisation was described in the literature. We report the observation of a 41 years old patient who was operated twice for this localization. The patient presented in 2005 with a third recurrence with important local extension contraindicating surgical treatment. An external radiotherapy was carried out with a good local control of the lesion. In the light of this observation we will discuss physiopathology of this rare lesion and the various therapeutic options.

Treatment options for malignant hemangioendotheliomas of the thyroid.

BACKGROUND: Malignant hemangioendotheliomas of the thyroid are rare tumors predominantly seen in areas with endemic goiter such as the Alpine regions. The estimated incidence of the disease is between 0.15 and 0.25 per 100.000 inhabitants annually for Western Austria. The tumor is regarded as radio-resistant, and its prognosis is reported to be dismal. PATIENTS AND METHODS: Between 1982 and 1999, 12 cases with immunohistochemically confirmed malignant hemangioendotheliomas of the thyroid were referred for postoperative or palliative treatment. There were 8 men and 4 women with a median age of 67 years (range, 55-81 years). With surgery, clear margins were achieved in 5, microscopic residues were left in 3, and gross residual disease in 3 patients. One patient had an inoperable primary tumor. Postoperative radiotherapy was given to 8 cases, 6 of them received the radiosensitizer razoxane on radiation days. Total tumor doses ranged between 54 and 65 Gy. Two patients with clear margins at surgery received no adjuvant radiotherapy and were observed only. RESULTS: Local tumor control was achieved in 11 of 12 patients; 5 lived longer than 5 years. The median survival time of all cases is presently 14 months (range, 0.5-196 months). If 3 cases with metastasis at diagnosis were left out of the analysis, the median survival is 70 months. Noteworthy is a complete regression of 2 lung metastases in a 72-year-old man treated with the combination of vindesine, razoxane, and radiotherapy; the patient is still in complete remission more than 94 months after 3-year maintenance therapy with vindesine and razoxane. Fibrinogen, factor VIII, and factor VIII-related antigen in the serum could serve as surrogate markers during the follow-up. The tolerance to the combined modality treatment was good to fair, local chemoradiation reactions of normal tissues have to be considered as the principal toxicity. It may also be of interest that 5 of 12 patients were exposed to vinyl chloride and other polymeric materials during their working life. CONCLUSIONS: This small series indicates that the course of the disease is not uniformly bleak and that the resistance to radiotherapy reported in the literature has to be questioned. It appears that adequate surgery together with rapid radiation therapy in combination with razoxane, a radiosensitizer and agent that can normalize tumor blood vessels, is able to improve the local control rate and thus perhaps to alter the natural history of this disease. In addition, the data offer new evidence of the occurrence of vinyl-chloride-induced angiosarcomas outside the liver, and support similar observations that have already been published in case reports.

[Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study]. / Charakterystyka kliniczna naczyniopochodnych nowotworów zlosliwych u dzieci -- raport Programu Guzów Rzadkich PPGGL.

UNLABELLED: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. THE AIM OF THE STUDY: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms. MATERIAL AND METHODS: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients). RESULTS: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression. CONCLUSIONS: The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.

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.

[Malignant hemangioendothelioma of the thyroid gland: new results on pathogenesis, therapy and prognosis]. / Das maligne Hämangioendotheliom der Schilddrüse: Neue Resultate zur Pathogenese, Therapie und Prognose.

BACKGROUND: Malignant hemangioendothelioma of the thyroid is a rare tumor predominantly described in areas with endemic goiter like the Alpine regions. The estimated incidence of the disease is between 0.15 and 0.25 per 100,000 inhabitants per year for Western Austria. The prognosis is reported to be dismal. MATERIALS AND METHODS: Between 1982 and 1995, 10 cases with immunohistochemically confirmed malignant hemangioendotheliomas of the thyroid were referred to our department for postoperative or palliative treatment. Two patients with clear margins at surgery received no adjuvant radiotherapy and were only observed. By surgery, clear margins (R-0 resection) were achieved in 5, microscopic residuals (R-1) were left in 3, and gross residual disease in 1 patient. One patient had an inoperable primary tumor. Postoperative radiotherapy was administered in 6 cases, 4 of them additionally received the radiosensitizer razoxane. Total tumor doses ranged between 58 and 65 Gy. RESULTS: Local tumor control was achieved in 9 of 10 patients; 4 of 10 lived longer than 4 years. The median survival time has not yet been reached and is presently between 7.5 and 21+ months.--Noteworthy is a complete regression of 2 lung metastases in a 72-year-old man by a combination of vindesine, razoxane and radiotherapy. The patient is still in complete remission under a maintenance therapy with vindesine and razoxane since 14 months.--It may also be of interest that 4 of the 10 patients were strongly exposed to vinyl chloride and other polymeric materials during their occupational life. CONCLUSIONS: This small series may indicate that the outcome of this disease may not be uniformly deleterious, and that the resistance to radiotherapy reported in the literature may be questioned.--The data offer new evidence of the occurrence of vinyl chloride-induced angiosarcomas outside the liver, and support observations which have already been published in case reports.

[Assessment of radiotherapy effects on brain tumors by dynamic susceptibility contrast MR imaging].

Dynamic susceptibility contrast MR imaging(DSC MRI) was performed on eleven patients with brain tumors before and after radiotherapy. Their confirmed diagnoses were as follows: hemangioendothelioma(n = 1), meningioma(n = 1), low grade astrocytoma(n = 1), glioblastoma(n = 2), and brain metastasis(n = 6). The purpose of this study is to determine whether this technique is clinically useful for monitoring radiotherapeutic effect on brain tumors. Region of interest(ROI) analyses were performed on both the brain tumor and the contralateral normal area to evaluate the therapeutic effect semiquantitatively. The calculated subtraction images, rCBV maps, were also produced for the visual evaluation of the change of tumor vascular beds. The results showed that DSC MRI was clinically useful because of the capability to offer additional functional information.

[Angiosarcoma of the hand. Case report]. / Angiosarkom der Hand. Falldarstellung.

Angiosarcoma is a very rare malignant bone tumour. The case of a 45-year-old man is presented with a primary epithelioid hemangioendothelioma in the fourth metacarpus. After radical extirpation of the tumour radiation followed. The function of the hand was fully preserved. Five years after operation the patient is free of disease.

Cerebello-pontine angle epithelioid haemangioendothelioma in a 4-year-old boy.

A 4-year-old boy presented with a cerebello-pontine angle tumour thought to be an invasive acoustic schwannoma. Subtotal resection of the tumour was performed and followed by radiotherapy; histologically the lesion was an epithelioid haemangioendothelioma.

Malignant haemangioendothelioma of bone in a HbSC disease patient--a case report.

We report here a 35-year-old man with sickle cell disease (SCD), who presented in 1989 with pain in the (R) hip of 7 years duration and swelling of the (R) calf of 3 months duration. Clinical examination revealed a hard tender mass in the (R) calf. Histology of the (R) calf mass revealed haemangioendothelioma (HE), similar to the histology of the (R) iliac bone mass obtained in another institution previously. He was treated with external radiotherapy with the 1.25 megavoltage beam to antero-posterior fields of the (R) hemipelvis and (R) calf, with good response. Chemotherapy was subsequently administered using 6 cycles of VAC regime. The patient remained in remission for 12 months. In 1991 he had lymphoedema of (R) lower limb and received further radiotherapy and chemotherapy after an isotope bone scan had revealed disease activity in the (R) hemipelvis, (R) femur and (L) upper tibia. He responded again with complete regression of the lymphoedema and remained well until April 1993 when the lymphoedema recurred. He died while being evaluated for further treatment. Although there is no evidence to suggest that SCD confers any protection from development of neoplasms, the co-existence of SCD with a neoplasm is not common. We consider the occurrence of HE of bone, a rare malignancy, in a HbSC patient worthy of reporting.

Hemangioendotelioma ósseo: relato de uma caso e revisäo de literatura / Bone hemangioendothelioma: report of a case and review of the literature

Os autores relatam um caso de hemangioendotelioma ósseo e tecem comentários sobre sua confusa nomenclatura, diagnóstico, evoluçäo e tratamento, comparando seu caso com os encontrados na literatura. Os autores lembram também a raridade de sua ocorrência