Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor.

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm that appears to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites. Malignant PEComas exist but are very rare. These tumors represent a family of mesenchymal neoplasms, mechanistically linked through activation of the mTOR signaling pathway. Metastatic PEComa is a rare form of sarcoma for which no effective therapy has been described previously and that has a uniformly fatal outcome. Although there is no known effective therapy, the molecular pathophysiology of aberrant mTOR signaling provides a scientific rationale to target this pathway therapeutically. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. On this basis, we report a case of metastatic retroperitoneal PEComa treated with an oral mTOR inhibitor, with everolimus achieving significant clinical response.

Surgical challenges in the treatment of leiomyosarcoma of the inferior vena cava: analysis of two cases and brief review of the literature.

BACKGROUND: Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor of mesenchymal origin. Optimal treatment should include complete resection of the malignant lesion with preservation of venous return. We present our experience from two patients treated in our hospital in the last 3 years. METHODS AND RESULTS: The first case is that of a 54-year-old woman, with a 9 cm a primary IVC leiomyosarcoma extending from the level of the right renal vein to the common iliac veins. The patient underwent radical tumor resection and reconstruction of the IVC with a polytetrafluoroethylene patch. She received adjuvant chemotherapy and is free of recurrence almost 3 years after surgery. The second case is that of a 56-year-old woman presenting with back pain due to an 8-cm retroperitoneal mass in close proximity to the right renal vein. She underwent exploratory laparotomy, where initially the effort of en bloc resection of the mass failed. Eventually, partial resection of the IVC was performed and the defect was primarily repaired. Pathological examination confirmed primary leiomyosarcoma of the IVC. She received adjuvant chemotherapy, but was referred to our hospital with local recurrence 6 months after the operation and is suffering from disseminated abdominal disease almost a year postsurgery. CONCLUSION: Radical surgical en bloc resection is the mainstay of treatment for IVC leiomyosarcomas. Extensive vascular reconstruction techniques may be necessary to restore adequate venous return to the IVC after tumor resection, and combination with adjuvant chemoradiotherapy has been shown to prolong disease-free survival rates.

Does internal stenting of the pancreaticojejunostomy improve outcomes after pancreatoduodenectomy? A prospective study.

AIM: This study's aim is to evaluate the effectiveness of using an internal stent when fashioning a duct-to-mucosa pancreatojejunostomy on preventing pancreatic fistula formation, as well as on the overall outcome for patients undergoing pancreaticoduodenectomy. MATERIALS AND METHODS: Between January 2000 and December 2008, 82 consecutive patients underwent pancreaticoduodenectomy and duct-to-mucosa pancreaticojejunostomy in an isolated jejunal loop, either with or without the aid of an internal stent. The allocation of the patients into group A (n = 41, stented anastomosis) and group B (n = 1, unstented anastomosis) was performed in a strictly alternating way. No statistically significant differences were identified between the two groups regarding age, sex, operative time, intraoperative pathological findings, and comorbidities. The two groups were compared regarding the rate of pancreatic fistula formation, postoperative complications, and hospital stay. RESULTS: In group A, pancreatic fistula formation rate was 4.9%; overall morbidity reached 30%; and hospital stay duration was 13 +/-4 days. In group B, pancreatic fistula formation rate was 2.4%; overall morbidity was 26%; and hospital stay duration extended to 14 +/- 5. According to Clavien's classification, the severity of surgical complications was designated as follows: for group A, 56% of the complications were allocated as grade I, 38% grade II, 4% grade III, 2.5% grade IV, and 0% grade V. The relative values for group B were 53%, 42%, 3%, 2%, and 0%, respectively. In six group A patients (14.7%), the internal stent was found stuck in the pancreatic stump, causing severe back pain requiring analgesic treatment with opioids for four of them. In group B, four patients (9.7%) complained of mild back pain, none of which required regular treatment. No mortalities were recorded in both groups. No statistically significant differences were found between the two groups regarding fistula formation and severity of complications. CONCLUSIONS: Internal stenting of a duct-to-mucosa pancreatojejunostomy does not diminish the rate of pancreatic fistula formation or alter overall patient's outcome.

Surgical approaches of resectable synchronous colorectal liver metastases: timing considerations.

AIM: To compare the safety and efficacy of simultaneous versus two stage resection of primary colorectal tumors and liver metastases. METHODS: From January 1996 to May 2004, 103 colorectal tumor patients presented with synchronous liver metastases. Twenty five underwent simultaneous colorectal and liver surgery and 78 underwent liver surgery 1-3 mo after primary colorectal tumor resection. Data were retrospectively analyzed to assess and compare the morbidity and mortality between the surgical strategies. The two groups were comparable regarding the age and sex distribution, the types of liver resection and stage of primary tumors, as well as the number and size of liver metastases. RESULTS: In two-stage procedures more transfusions were required (4 +/- 1.5 vs 2 +/- 1.8, pRBCs, P < 0.05). Chest infection was increased after the two-stage approach (26% vs 17%, P < 0.05). The two-stage procedure was also associated with longer hospitalization (20 +/- 8 vs 12 +/- 6 d, P < 0.05). Five year survival in both groups was similar (28% vs 31%). No hospital mortality occurred in our series. CONCLUSION: Synchronous colorectal liver metastases can be safely treated simultaneously with the primary tumor. Liver resection should be prioritized over colon resection. It is advisable that complex liver resections with marginal liver residual volume should be dealt with at a later stage.