[Tumor and transplantation]. / Tumor und Transplantation.

Tumor treatment and transplantation-associated with unavoidable mandatory immunosuppression-appear to be unreconcilable opposites. The clinical reality shows, however, that transplantation in many early stage primary tumors is the most effective treatment. The essential immunosuppression after transplantation can however promote tumor recurrence. Immunosuppression also leads to a significant increased rate of de novo tumors-in all organ transplant recipients. However, not all immunosuppressant drugs have the same effect on tumors. In experimental and clinical settings, the class of mTOR inhibitors has a clear antitumoral effect and is recommended as the immunosuppression treatment of choice in patients with increased tumor risk. The purpose of this review is to provide the reader with the scientific background regarding the clinical problem of tumors and transplantation.

[Multifocal epithelioid hemangioendothelioma treated by liver transplantation--case report]. / Multifokalni epiteloidni hemangioendoteliom jetre lijecen ortotopnom transplantacijom jetre--prikaz bolesnika.

Multifocal epithelioid hemangioendothelioma of the liver is a rare primary tumor with a variable course of disease. A case is presented of a 27-year-old female patient with multiple hepatic lesions on ultrasonography, suspect of metastatic tumor of the liver. Serum tumor markers were not elevated, while clinical examination of the lungs, gastrointestinal and gynecologic systems did not confirm the presence of a primary tumor process. Metastatic tumor and primary hepatocellular tumor were ruled out by fine needle aspiration cytology. Along with a characteristic immunophenotype of the vascular cell endothelium (positive for CD31 and CD34), high proliferation demonstrated by the analysis of argyrophilic nucleolar organization regions (AgNOR) and DNA aneuploidy, cytomorphological pattern suggested the diagnosis of angiosarcoma. Histopathologic finding corresponded to epithelioid hemangioendothelioma. Ten years after orthotopic liver transplantation, the patient is free from disease relapse, with regular follow up testing. Hemangioendothelioma of the liver is characterized by multifocality, which excludes resection; thus, liver transplantation is the method of choice. Therefore, preoperative diagnostic workup is of utmost importance to differentiate it from other primary and metastatic tumors of the liver.

Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel anti-angiogenetic treatments?

Primary hepatic epithelioid hemangioendothelioma (HEH) is a rare, low-grade malignant neoplasm of endothelial origin, with an unpredictable clinical course and prognosis. No standard therapeutic strategies are still available for HEH, due to the infrequency of the disease and to its variable natural history that limit the identification of the most effective treatment. In the absence of metastatic disease, surgical resection or liver transplantation represent the treatment of choice for HEH, while several antineoplastic agents have been proposed in the presence of metastatic nonresectable disesase. Herein, we describe the biological characterization and the clinical course of a primary HEH progressively responsive to treatment with intermediate doses of interferon-alpha (IFN)-alpha2a. Furthermore, based on the newly-identified expression of endoglin (CD105) on HEH, we discuss the clinical potential of novel anti-angiogenetic approaches to the disease.

[Clinicopathologic and immunohistochemical study of pulmonary epithelioid hemangioendothelioma].

OBJECTIVE: To study the clinical and pathological characteristics of pulmonary epithelioid hemangioendothelioma. METHODS: Four cases of pulmonary epithelioid hemangioendothelioma were studied by histopathologic and immunohistochemical examination of lung biopsy specimens. RESULTS: There were 3 female and 1 male, age 28 to 40 years. Clinically the tumor presented as multiple bilateral small nodules in the lung. Histologically, crown-like clusters of epithelioid tumor cells were obtained which filled in the alveoli locating at the periphery of the tumor nodules, while the central part of the nodules contained myxoid to hyaline matrix. The overall architecture of the lung was still preserved. Additionally, intracytoplasmic vacuoles were seen in tumor cells within which red blood cells were sometimes identified. Tumor cells generally lacked pleomorphism, mitotic activity and necrosis. They were immunohistochemically positive for CD31 and CD34. AE1/AE3 staining was positive in some cases. CONCLUSIONS: Pulmonary epithelioid hemangioendothelioma often occurs in a middle-aged woman and represents a distinct clinical pathological entity.

Angiossarcoma epitelióide ósseo: relato de um caso, com estudo imuno-histoquímico / Epithelioid angiosarcoma of bone: report of a case with immunohistochemical study

Angiossarcoma primário do osso é muito raro, representando menos de 1 por cento de todos os angiossarcomas. Neoplasias vasculares também são incomuns, ocorrendo em 14 por cento dos tumores malignos primários do osso. Os autores relatam caso em coluna lombar que merece atenção porque inicialmente tratava-se de um hemangioendotelioma, um tumor de malignidade intermediária, definido pelos critérios estabelecidos por Stout. Foi tratado com embolização local e radioterapia. Após dez anos, recidivou, com maior grau de malignidade, fenômeno interpretado como progressão tumoral, com caracterísitcas de Angiossarcoma epitelióide. O estudo imuno-histoquímico revelou positividade para marcadores vasculares (Fator VIII, CD31 e CD34). O paciente evoluiu com metástases pulmonares. Em revisão bibliográfica, não foi encontrado nenhum relato de caso semelhante, tendo em vista a longa evolução e progressão tumoral após dez anos de seguimento e expressão do fenotipo epitelióide.

Primary sarcomas of the lung: a clinicopathological and immunohistochemical study of 14 cases.

The clinicopathological features and immunohistochemical findings in 14 primary sarcomas of the lung collected over a 30-year-period are presented. This represents one sarcoma per 550 bronchogenic carcinomas undergoing resection in this centre. The study group comprised six leiomyosarcomas, five malignant peripheral nerve sheath tumours, two haemangiopericytomas and one epithelioid haemangioendothelioma. The majority of cases occurred in men (nine males: five females), with mean age at presentation of 54 years for men and 47 years for women. All leiomyosarcomas were seen in men, whereas malignant peripheral nerve sheath tumours showed no particular sex preponderance. Leiomyosarcomas were larger tumours than malignant peripheral nerve sheath tumours, mean tumour diameter 15 cm (range 10-25 cm) compared to 9.5 cm (7-15 cm), respectively. All leiomyosarcomas were situated intraparenchymally whereas two of the five malignant peripheral nerve sheath tumours were endobronchial in site. Extrathoracic metastates were seen at death in two of the six leiomyosarcomas but not in any of the malignant peripheral nerve sheath tumours. Overall survival was 28 months although for the leiomyosarcoma/malignant peripheral nerve sheath tumour group alone survival was 8 months. Tumour grading appeared to be a more useful prognostic factor than tumour site (endobronchial/parenchymal) or tumour size. Haemangiopericytoma and epithelioid haemangioendothelioma were associated with a more favourable prognosis.

Primary epithelioid hemangioendothelioma of the brain.

Epithelioid hemangioendothelioma is an uncommon form of endothelial neoplasm, one of intermediate-grade malignancy and relatively favorable prognosis. Herein we report the third and fourth cases described in the central nervous system and compare their clinical and pathologic properties with those arising at systemic sites. Both patients presented with signs and symptoms of a mass lesion with seizures present in both cases. Imaging studies revealed well-demarcated mass lesions with surrounding edema. Gross total removal was accomplished surgically in both cases One case was partially cystic and nodular; the other was firm, focally gritty, and cartilaginous in appearance. Histologically they were identical to hemangioendotheliomas of other locales: multinodular neoplasms with regional variation in cellularity, cords and clusters of epithelioid cells with variable attempts at lumen formation, and spindled cells associated with a fibromyxoid matrix. Immunohistochemical stains confirmed the endothelial natures of the cells. These cases and those previously reported were treated with surgical excision. The few patients described have ranged in age from infants to older adults. Some patients have had residual neurologic defects, but no deaths due to tumor have been reported.