SEOM Clinical Guideline of management of soft-tissue sarcoma (2016).

Soft-tissue sarcomas are uncommon and heterogeneous tumors of mesenchymal origin. A soft-tissue mass that is increasing in size, greater than 5 cm, or located under deep fascia are criteria for suspicion of sarcoma. Diagnosis, treatment, and management should preferably be performed by a multidisciplinary team in reference centers. MRI and lung CT scan are mandatory for local and distant assessment. A biopsy indicating histological type and grade is needed previous to the treatment. Wide surgical resection with tumor-free tissue margin is the primary treatment for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not likely of being improved with reexcision. Neoadjuvant and adjuvant chemotherapy improve survival in selected cases, usually in high-grade sarcomas of the extremities. In the case of metastatic disease, patients with exclusive lung metastasis could be considered for surgery. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. New drugs have shown activity in second-line therapy and in specific histological subtypes.

Renal primary angiosarcoma.

Angiosarcomas account for 2% of all soft tissue sarcomas and of them, primary renal angiosarcomas represent 1%. Twenty-four cases have been published in the English specialised literature. We report the second case to be described in a middle-aged female, with pulmonary metastases at diagnosis, and fatal outcome despite surgery and chemotherapy.

Renal primary angiosarcoma

Angiosarcomas account for 2% of all soft tissue sarcomas and of them, primary renal angiosarcomas represent 1%. Twenty-four cases have been published in the English specialised literature. We report the second case to be described in a middle-aged female, with pulmonary metastases at diagnosis, and fatal outcome despite surgery and chemotherapy (AU)

Disseminated classic Kaposi's sarcoma.

Kaposi's sarcoma (KS) is characterised by proliferation of vascular endothelial and lymphoreticular cells, frequently with a multicentric expression developed from a single node and evolving to multiple cutaneous lumps or plaque-like appearance. Four types of KS with similar histological patterns have been described in terms of their clinical and epidemiological features: classic KS, endemic (African) KS, iatrogenic KS and epidemic (AIDS-related) KS. The differences in clinical features are quite relevant: classic KS is usually limited to the lower extremities; whereas immunodeficiency-related diseases frequently involve several organs. A case of a 67-year-old woman with metastatic KS and unproven immunodeficiency is presented.

Disseminated classic Kaposi's sarcoma

Kaposi's sarcoma (KS) is characterised by proliferation of vascular endothelial and lymphoreticular cells, frequently with a multicentric expression developed from a single node and evolving to multiple cutaneous lumps or plaque-like appearance. Four types of KS with similar histological patterns have been described in terms of their clinical and epidemiological features: classic KS, endemic (African) KS, iatrogenic KS and epidemic (AIDS-related) KS. The differences in clinical features are quite relevant: classic KS is usually limited to the lower extremities; whereas immunodeficiency-related diseases frequently involve several organs. A case of a 67-year-old woman with metastatic KS and unproven immunodeficiency is presented (AU)

Gliomatosis Cerebri: diagnóstico y tratamiento con temozolomida, a propósito de un caso / No disponible

La gliomatosis cerebri (GC) constituye un raroproceso neoplásico cerebral primario de crecimientodifuso, infiltrativo y no destructivo, de naturalezaglial. El término GC implica afectación de al menosdos lóbulos cerebrales con posible extensión al tallocerebral, cerebelo, médula espinal y espacio subaracnoideo.Las manifestaciones clínicas son inespecíficasy, aunque las pruebas de neuroimagen (fundamentalmentela RMN) muestran alteraciones características,el diagnóstico definitivo requiere confirmaciónhistológica. El tratamiento con radioterapiapuede estabilizar o mejorar la función neurológicaen algunos pacientes sin implicaciones en lasupervivencia global. Recientemente han surgidonuevas alternativas terapéuticas esperanzadoras, comoel empleo de quimioterapia con temozolomida.Presentamos el caso de un varón de 49 años diagnosticadode gliomatosis cerebri y tratado con temozolomidaen monoterapia

Gliomatosis cerebri (GC) is a rare difuse,infiltrative and non destructive primary brain tumorfrom glial origin The term GC implies the affectionof two or more brain lobes with possible extensionto brain stem, cerebellum, spinal cord andsubarachnoid space.Clinical features are unspecific, so diagnosiscomes from characteristic features in neuroimaginstudies (fundamentally IRM) but histologicalconfirmation is required for diagnosis. Radiotherapytreatment can improve or stabilizeneurological function in some patients, its impacton survival has not been demonstrated. Encouragingalternative treatment, as chemotherapy treatmentwith temozolomide, has recently arisen.We present a case of GC in a 49 years old maletreated with temozolomide as a single therapy