Treatment of intimal sarcoma of peripheral veins.

INTRODUCTION: Intimal sarcoma is an extremely rare group of undifferentiated pleomorphic sarcoma arising from the intimal layer of vessels accounting for only 1% of all sarcomas, intimal sarcoma of large veins are even less common. CASES PRESENTATION: We present two cases of intima sarcoma, one originated form the basilar vein and the other from the cephalic vein, the first one was treated with surgery and postoperative chemotherapy followed by Radiotherapy (RT), the second case was treated with isolated limb perfusion followed by marginal resection and RT. Both patients progressed to the lungs in a short time, the first case was treated with metastasectomy of the lung and is without evidence of disease 7months after surgery; the second case treated with isolated limb perfusion has stable disease. DISCUSSION: Intimal sarcoma are very aggressive tumors, with a high metastatic potential, the two patients progressed to lung in a short time (2months) after local treatment. Both cases exhibit good response to chemotherapy and metastasectomy with a disease - free period of 7months. CONCLUSION: We propose that given the aggressive behavior of these tumors, they should be treated with chemoradiotherapy postoperative, either by systemic chemotherapy or isolated limb perfusion for the limp sparing surgery in this histology.

Metastatic pheochromocytoma to liver without elevation of metanephrines and catecholamines.

INTRODUCTION: Malignant pheochromocytoma represents 10% of all patients with pheochromocytoma. Of these cases, only 5-9% presents without elevation of metanephrines and catecholamines. PRESENTATION OF CASE: A 43-year-old female patient presented with an abdominal tumor. An exploratory laparotomy was performed and the final report was a pheochromocytoma. After ten years, multiple liver lesions were detected and surgical treatment was performed. Pathological evaluation revealed a malignant pheochromocytoma with negative margins after 5 years of follow-up without evidence of disease. DISCUSSION: The recurrence rate of malignant pheochromocytoma is 15-20% at ten years and a 5-year survival rate that ranges from 50% to 80%. The presence of synchronous metastases is rare (10-27%), but have been reported until 20 years later with the most common metastatic sites being the local lymph nodes, bone (50%), liver (50%) and lung (30%). The prognostic factor such as size >6cm, age over 45 years, synchronous metastasis and no tumor excision are related with poor prognosis. CONCLUSION: Surgical treatment offers the best survival rate and the only chance of cure so far and the goal is an R0 resection as in our case. So it should be the treatment of choice.