Large Retroperitoneal Perivascular Epithelioid Cell Neoplasm (PEComa): A Case Report and a Brief Review

Objective: To describe a case of retroperitoneal perivascular epithelioid cell tumor (PEComa) and to discuss the main features of this rare pathology. Introduction: PEComas represent a rare cluster of neoplasms with uncertain origin; their precursor cells are spindle-shaped and characterized by a myomelanocytic phenotype, so only immunohistochemical staining makes a definitive diagnosis possible. To date, less than three hundred cases are reported in Literature and retroperitoneal site accounts for 7-8% of overall locations. Case Report: Middle-aged female has visited for abdominal pain and urinary complaints; physical findings and imaging demonstrated a huge inhomogeneous mass occupying right abdomen and arising from renal capsule. After multidisciplinary evaluation, patient has been addressed to open surgery and an en-bloc resection of the mass, with right nephrectomy and adrenalectomy. Immunohistochemical staining made a diagnosis of PEComa possible. After an uneventful postoperative stay, the patient entered a follow up protocol, without signs of local recurrence and distant metastases. Conclusion: Retroperitoneal PEComa often presents as a bulky mass with renal and adrenal involvement. Surgical resection should be aimed to obtain a complete removal with negative margins; this makes compartment surgery and en-bloc resection mandatory. Immunostaining is the key methods for a correct diagnosis

Trattamento multidisciplinare nella sindrome di Budd-Chiari: caso clinico

La sindrome di Budd-Chiari (BCS) è caratterizzata dall’ostacolo al deflusso delle vene sovraepatiche; nei casi in cui l’ostruzione è limita - ta alle vene sovraepatiche, il confezionamento di uno shunt portocavale permette una rapida risoluzione della sintomatologia. Se l’ostruzione coinvolge anche la vena cava inferiore, il trattamento è affidato a pro - cedure combinate che offrono migliori garanzie di successo. Gli Autori riportano il caso di una paziente trattata con successo mediante uno shunt portocavale latero-laterale preceduto dal posizio - namento di uno stent cavale autoespandibile. Ad un follow-up di 85 mesi la paziente è in buone condizioni generale. Il posizionamento di uno stent infracavale associato ad uno shunt portocavale latero-laterale sembrerebbe essere il trattamento ideale nei casi acuti o subacuti di BC

Total shunting and elective management of variceal bleeding

A 20-year experience with treatment of esophageal varices in patients with cirrhosis is reported. Considering that total shunts are well tolerated immediately after operation (hospital mortality rate for all elective procedures being 6.4%), that they offer a good protection against rebleeding (rebleeding variceal rate of 7.6%), and that they offer the same long-term survival as given by other shunts (5- and 10-year survival rates of 57% and 31%, respectively), the authors affirm that these kinds of shunts are still useful in well selected cases. Late follow-up results of a prospective randomized trial of elective mesocaval shunts compared to portacaval shunt have shown no significant differences in operative mortality, rebleeding rates, encephalopathy rates, or survival. Based on this information, the authors currently use portacaval shunt as their operation of choice

Preoperative Transcatheter Arterial Embolization and En-Bloc Resection for Giant Non-Functioning Left Adrenocortical Carcinoma: A Case Report

Objective: To demonstrate efficacy and relative safety of compartment surgery in a case of large sized adrenocortical carcinoma (ACC), whenever associated with preoperative transcatheter arterial embolization (TAE) and adjuvant treatment with mitotane. Introduction: ACC is a rare illness; non-functioning lesions account for 40-55% of patients, may reach a large size, and show a clear attitude to infiltrate neighbouring organs. Case Report: Middle aged male visited for a huge left abdominal mass; contrast CT scan showed origin from the left adrenal lodge and possible extension of the tumor to surrounding structures. Preoperative adrenal work-up confirmed non-functioning nature of the adrenal mass. In order to allow a safe compartment excision and to decrease blood loss, preoperative angiography and TAE was performed. Patient underwent en-bloc resection of the mass, together with nephrectomy and distal splenopancreasectomy. No postoperative transfusions were required. Patient received adjuvant therapy with mitotane for 36 months since surgery, without any sign of tumor recurrence. Conclusion: An aggressive surgical approach seems justified also in locally advanced and infiltrating ACC; compartment resection is mandatory in order to obtain adequate resection margins. Preoperative TAE decreases intraoperative blood loss and need for transfusions. Adjuvant treatment with mitotane may contribute to reach remarkable disease-free survival

Surgical treatment of hepatocellular carcinoma in elderly cirrhotic patients: personal experience

BACKGROUND: Due to the longer life expectancy of the world's population, the number of elderly cirrhotic patients undergoing surgery for hepatocellular carcinoma (HCC) is increasing. Our study evaluates the benefits of hepatic resections for HCC in cirrhotic patients aged over 65, analysing the early and long-term surgical results. PATIENTS AND METHODS: We retrospectively considered a series of 46 patients receiving hepatic resection for HCC. The clinicopathological data and surgical outcome of 14 (30.4%) patients aged 65 or older (group I) were evaluated and compared to the 32 (69.6%) younger than 65 (group II). RESULTS: No operative mortality was recorded in either group. The hospital mortality rate was 7.1% (1 out of 14) in group I and 9.4% (3 out of 32) in group II (p = 1.00). Hospital morbidity did not differ significantly in the two groups (21.4% vs 34.4%; p = 0.50). At follow-up (median 34 months, interquartile range: 12-63) 3 patients from group I (21.4%) and 16 patients from group II (50%) experienced tumor recurrence (p = 0.14). The five-year disease-free survival rate for group I and group II was 71.4% vs 28.2%, respectively (p = 0.05). The overall 5-year survival rate for group I and group II was, respectively, 77.4% vs 41.8%, (p = 0.3). CONCLUSION: Elderly cirrhotic patients with HCC can benefit from hepatic resection as well as younger patients; age by itself should not be considered a contraindication to surgery

Multidisciplinary treatment in Budd-Chiari syndrome: clinical case

Budd-Chiari Syndrome (BCS) is characterized by obstruction of hepatic venous outflow. When obstruction is limited to the suprahepatic veins, portocaval shunting provides an immediate relief of symptoms. If the obstacle results also from narrowing of the inferior vena cava (IVC), multimodality treatments seem to offer safer and easier alternative. In the patient herein reported, combination of side-to-side portocaval anastomosis with a cavo-atrial shunt through an expandible metallic stent provided immediate relief of symptoms. The patient is doing well after 85 months from combined treatment. In conclusion infracaval stenting combined to side-to-side portocaval shunting should represent the treatment of choice in acute or subacute forms of BCS