Myofibroblastoma of the Breast: Diagnostic Challenges of a Rare Benign Tumor.

Breast myofibroblastomas (MFBs) are rare benign tumors that have recently been recognized as a distinct entity. They are more common in men and often coexist with gynecomastia. Fine needle aspiration cytology or core biopsy should always be performed for triple assessment; however, due to the multiple variants of the tumor, final diagnosis can be safely accomplished only postoperatively with the aid of immunohistochemistry. The main histological characteristic that differentiates MFBs from the majority of malignant mesenchymal breast lesions is the presence of spindle cells in a collagenous background. Low mitotic activity and vimentin and CD34 positivity have been reported as well. Surgical excision retains a pivotal diagnostic and curative role. Local resection is sufficient, but mastectomy can also be advocated either in cases of male patients with coexisting gynecomastia or in females with large tumors. Into this retrospective survey, three cases of breast MFB were consecutively enrolled. Two patients were postmenopausal females and one was male. Relevant literature is briefly reviewed. Clinical features, histogenesis, morphological, and immunochemical findings are discussed, while the role of the current diagnostic and therapeutic management of this uncommon neoplasm is emphasized.

Breast cancer: from "maximum tolerable" to "minimum effective" treatment.

Randomized trials have played a fundamental role in identifying better treatments for most type of diseases, especially in the oncological field. In breast cancer, the shift from "maximum tolerable" to "minimum effective" treatment has been evident since the 1970s and has been based on the results of international randomized trials. The progress of breast surgery represents an excellent model of the evolution of science and the aim of this article is to review the main randomized studies that changed everyday practice in breast surgery.

Conservative mastectomy: extending the idea of breast conservation.

Conservative mastectomy is a surgical technique to remove breast glandular tissue without disruption to the appearance of the breast. The main contraindication to this operation is involvement of the nipple-areola complex. Preservation of a healthy nipple does not affect long-term survival rates. Intraoperative retroareolar biopsy and accurate removal of all mammary tissue are mainstays to guarantee oncological safety of the technique. A complication, however infrequent, is nipple necrosis, which can be treated successfully by local excision. Several breast reconstructive techniques are used during conservative mastectomy and aesthetic results are good in most cases.

Stenting of a gastroduodenal artery aneurysm: report of a case.

We present a case of gastroduodenal artery stenting in a patient with recurrent episodes of pancreatitis due to gastroduodenal artery aneurysm. Stenting was performed under local anesthesia using a 6 × 38-mm primary covered Advanta V12 vascular stent graft. The procedure was successful and the patient is asymptomatic 21 months later. Endovascular treatment of gastroduodenal artery aneurysm should be considered a promising alternative to open surgery, due to lower mortality and morbidity.

Laparoscopic gastric greater curvature plication: results and complications in a series of 135 patients.

Laparoscopic gastric greater curvature plication (LGGCP) is an emerging restrictive bariatric procedure that successfully reduces the gastric volume by plication of the gastric greater curvature. Its main advantages are the reversibility of the technique as well as the lack of foreign materials or gastrectomy. We present our results, focusing on the effectiveness and complications, and on a new modification of the original technique. One hundred and thirty-five patients underwent LGGCP between April 2008 and December 2009. A five-trocar port technique was used, and following dissection of the greater gastric curvature, single plication of the latter was performed under the guidance of a 36-Fr bougie. Modification of the technique included multiple gastric plications. One hundred and four obese women and 31 obese men (mean age of 36 years) underwent LGGCP for weight reduction. Operative time was 40-50 min, and mean hospital stay was 1.9 days (range 1-6 days). After a follow-up of 8-31 months (mean 22.59), the mean percentage of excess weight loss (%EWL) was 65.29. Subgroup analyses based on BMI values showed that %EWL was significantly higher for patients with BMI < 45 kg/m² (group I) compared with patients with BMI > 45 kg/m² (group II) (69.86 vs 55.49, respectively, p = 0.006). Similarly, inadequate weight loss was significantly higher for group II, while the failure of the technique and postoperative complications were comparable. On the other hand, subgroup analysis based on the technique showed that the modification of the technique did not affect the effectiveness or the operative time; however, it reduced early complications dramatically, including prolonged postoperative vomiting and late gastric obstruction, thus affecting the length of hospitalization. Overall complication rate in our series was 8.8% (12/135). Cases of prolonged postoperative vomiting, GI bleeding, and leak were treated conservatively, while one case of portomesenteric thrombosis and three cases of acute gastric obstruction were treated surgically. LGGCP is an emerging technique sparing gastric resection, the use of foreign materials and intestinal bypass. Its effectiveness is satisfactory for patients with BMI < 45 kg/m², and the complication rate is acceptable.

Mastectomia conservadora: uma técnica promissora / Conservative mastectomy: a promising technique

A presença do complexo areolo papilar (CAP) contribui para uma melhor autoimagem corporal e para satisfação pessoal das pacientes. A mastectomia nipple sparing, que consiste na remoção de todo o parênquima mamário, conservando-se a pele da mama e o CAP; essa é uma técnica inovadora que surge para satisfazer aspectos cosméticos sem prejuízo oncológico. No Instituto Europeu de Oncologia (IEO),iniciamos a aplicação dessa técnica em 2002, tratando até o momento 2.487 pacientes. Apresentamos, pois, nossa experiência com a técnica.

The presence of the nipple is known to improve the body image and patient satisfaction. Conservative mastectomy is an emerging technique that couples oncological safety and cosmesis by entirely removing the breast parenchyma sparing the breast skin and nipple-areola complex (NAC). At European Institute of Oncology (IEO) we starting performing this technique in 2002 and sofar we have treated 2,487 patients. We present our experience with the technique.

Recurrent pulmonary embolism due to giant hepatic hamangioma treated with hepatectomy under vascular exclusion.

Giant hepatic hemangiomas are benign tumors that measure more than 4 cm and are usually asymptomatic. Pulmonary embolism (PE) is an extremely rare manifestation of giant hepatic hemangiomas. We report a case of a 44-year-old woman who suffered of recurrent pulmonary emboli that, after thorough work up, were attributed to thrombi formation inside a giant hepatic hemangioma. A right hepatectomy under vascular exclusion was performed and the hemangioma, measuring 17 cm, was resected. Two years later the patient remains asymptomatic. The report highlights the value of investigating giant liver hemangiomas in case of PE. In such cases, the hemangioma should be resected preferably under occlusion of the venous outflow of the liver to avoid PE intraoperatively.

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.

Combination of right nephrectomy and total pancreaticoduodenectomy for Von Hippel-Lindau disease.

CONTEXT: Von Hippel-Lindau disease is an inherited syndrome of multiorgan neoplasia caused by a germline mutation in the von Hippel-Lindau gene and can include central nervous system tumors, renal cell carcinomas and benign pancreatic cystic tumors. CASE REPORT: We report the case of a 56-year-old patient who had a past history of cerebellar hemangioblastoma and presented with abdominal pain. Imaging revealed renal tumors and multiple pancreatic tumors which caused duodenal and pancreatic duct compression. The patient was treated with a combination of radical right nephrectomy, total pancreaticoduodenectomy and splenectomy. Pathology identified a multifocal unilateral clear cell renal carcinoma which interestingly coexisted with multiple large pancreatic serous microcystic adenomas with infiltration of the fibrous capsule. CONCLUSION: In past cases of von Hippel-Lindau disease, pancreatic adenomas with malignant transformation have not been reported. In our case, the infiltration of the fibrous capsule by parenchymal cells may indicate malignant transformation.

Intranodal palisaded myofibroblastoma: a case report.

Intranodal palisaded myofibroblastoma is a rare benign soft tissue tumor, almost always arising from inguinal lymph nodes. It usually presents as a painless, slow-growing inguinal mass. We report herein a case of an intranodal palisaded myofibroblastoma occurring in a 36-year-old man. The salient clinicopathologic features of this unusual tumor are presented and the literature is briefly reviewed.

Pancreatic leakage after pancreaticoduodenectomy: the impact of the isolated jejunal loop length and anastomotic technique of the pancreatic stump.

OBJECTIVES: To evaluate the impact of the length of the isolated jejunal loop and the type of pancreaticojejunostomy on pancreatic leakage after pancreaticoduodenectomy. METHODS: One hundred thirty-two consecutive patients who underwent a pancreaticoduodenectomy were studied according to the length of the isolated jejunal loop (short loop, 20-25 cm vs long loop, 40-50 cm) and the type of pancreaticojejunostomy (invagination vs duct to mucosa). RESULTS: The use of the long isolated jejunal loop was associated with a significantly lower pancreatic leakage rate compared with the use of a short isolated jejunal loop (4.34% vs 14.2%, P < 0.05). In addition, the use of duct-to-mucosa technique was associated with significantly lower incidence of postoperative pancreatic fistula compared with the invagination technique (4.2% vs 14.5%, P < 0.05). Finally, patients with a short isolated jejunal loop compared with patients with a long loop had increased morbidity (50.7% vs 27.5%, P < 0.05) and prolonged hospital stay (16.3 +/- 1.9 days vs 10.2 +/- 2.3 days, P < 0.05). Overall mortality rate was 1.5%. CONCLUSIONS: The use of a long isolated jejunal loop and a duct-to-mucosa pancreaticojejunostomy is associated with decreased pancreatic leakage rate after pancreaticoduodenectomy.

Ischemic preconditioning confers antiapoptotic protection during major hepatectomies performed under combined inflow and outflow exclusion of the liver. A randomized clinical trial.

BACKGROUND: Extensive experimental studies and a few clinical series have shown that ischemic preconditioning (IPC) attenuates oxidative ischemia/reperfusion (I/R) injuries in liver resections performed under inflow vascular control. Selective hepatic vascular exclusion (SHVE) employed during hepatectomies completely deprives the liver of blood flow, as it entails simultaneous clamping of the portal triad and the main hepatic veins. The aim of the present study was to identify whether IPC can also protect hepatocytes during liver resections performed under SHVE. METHODS: Patients undergoing major liver resection were randomly assigned to have either only SHVE (control group, n = 43) or SHVE combined with IPC--10 min of ischemia followed by 15 min of reperfusion before SHVE was applied (IPC group, n = 41). RESULTS: The two groups were comparable with regard to age, liver resection volume, blood loss and transfusions, warm ischemic time, and total operative time. In liver remnant biopsies obtained 60 min post-reperfusion, IPC patients had significantly fewer cells stained positive by TUNEL compared to controls (19% +/- 8% versus 45% +/- 12%; p < 0.05). Also IPC patients had attenuated hepatocyte necrosis, systemic inflammatory response, and oxidative stress as manifested by lower postoperative peak values of aspartate transaminase, interleukin-6, interleukin-8, and malondialdehyde compared to controls. Morbidity was similar for the two groups, as were duration of intensive care unit stay and extent of total hospital stay. CONCLUSIONS: In major hepatectomies performed under SHVE, ischemic preconditioning appears to attenuate apoptotic response of the liver remnant, possibly through alteration of inflammatory and oxidative pathways.

Intrapancreatic accessory spleen issues: diagnostic and therapeutic challenges.

CONTEXT: Intrapancreatic accessory spleen is an exceptionally rare entity, commonly mistaken for a pancreatic neoplasm. Preoperative diagnosis based on radiologic imaging features is difficult. Aspiration cytology may be misleading due to poor sampling. CASE REPORT: We report a very rare case of an intrapancreatic accessory spleen which radiologically and cytologically mimicked a neuroendocrine pancreatic tumor. CONCLUSION: Modern imaging techniques will probably lead to an increasing number of intrapancreatic accessory spleens being discovered, posing significant diagnostic and therapeutic challenges.

The introduction of a simple maneuver to reduce the risk of postoperative bleeding after major hepatectomies.

BACKGROUND/PURPOSE: In major hepatectomies, postoperative increases in central venous pressure (CVP) may cause suture failure and massive bleeding. The aim of our study is to test the application of an intraoperative maneuver to reduce the risk of postoperative bleeding. METHODS: Our study included 172 consecutive patients who had major liver resection with selective hepatic vascular exclusion and sharp transection of the liver parenchyma. An intraoperative maneuver (5 s occlusion of the hepatic vein) was applied in an alternating way, and the patients were assigned to two groups: Cohort A (n = 86), that was granted the maneuver, and Cohort B (n = 86), that was used as a control group. RESULTS: In Cohort A, application of the maneuver was successful in demonstrating bleeders under low CVP levels. Cohort A had lower rate of massive bleeding requiring emergency reoperation (2.3 vs 5.8%, P = 0.049), less postoperative blood transfusions (13 vs 24%, P = 0.042), lower morbidity (20 vs 35%, P < 0.045) and shorter hospital stay compared to Cohort B. CONCLUSIONS: Hepatectomies conducted under low CVP are prone to postoperative hemorrhage which can be prevented if the final bleeding control is performed under high pressure in the hepatic veins. Application of our testing maneuver effectively unmasked previously undetectable bleeding veins.

A rare coexistence of adrenal cavernous hemangioma with extramedullar hemopoietic tissue: a case report and brief review of the literature.

BACKGROUND: Cavernous hemangiomas of the adrenal gland are rare, benign, non-functioning neoplastic tumors. To our knowledge, 55 cases have been reported in the literature to date. CASE PRESENTATION: We report the first case of a large, non-functioning adrenal cavernous hemangioma that was incidentally found during the preoperative staging workup of a 75 year old woman with left breast adenocarcinoma. Imaging with US, CT scan and MRI showed a heterogeneous 8 cm mass with non-specific radiological features that was located on the left adrenal gland. The mass was surgically excised and pathology revealed an adrenal hemangioma with areas of extramedullar hemopoiesis. CONCLUSION: Although adrenal hemangiomas are rare and their preoperative diagnosis is difficult, they should always be included in the differential diagnosis of adrenal neoplasms.

Does clamping during liver surgery predispose to thrombosis of the hepatic veins? Analysis of 210 cases.

AIM: To test whether clamping during liver surgery predisposes to hepatic vein thrombosis. METHODS: We performed a retrospective analysis of 210 patients who underwent liver resection with simultaneous inflow and outflow occlusion. Intraoperatively, flow in the hepatic veins was assessed by Doppler ultrasonography during the reperfusion phase. Postoperatively, patency of the hepatic veins was assessed by contrast-enhanced CT angiography, when necessary after 3-6 mo follow up. RESULTS: Twelve patients (5.7%) developed intraoperative liver remnant swelling. However, intraoperative ultrasonography did not reveal evidence of hepatic vein thrombosis. In three of these patients a kinking of the common trunk of the middle and left hepatic veins hindering outflow was recognized and was managed successfully by suturing the liver remnant to the diaphragm. Twenty three patients (10.9%) who developed signs of mild outflow obstruction postoperatively, had no evidence of thrombi in the hepatic veins or flow disturbances on ultrasonography and contrast-enhanced CT angiography, while hospitalized. Long term assessment of the patency of the hepatic veins over a 3-6 mo follow-up period did not reveal thrombi formation or clinical manifestations of outflow obstruction. CONCLUSION: Extrahepatic dissection and clamping of the hepatic veins does not predispose to clinically important thrombosis.

Mixed acinar-endocrine carcinoma of the pancreas: a case report and review of the literature.

INTRODUCTION: Pancreatic tumors usually display either a ductal, an acinar or an endocrine differentiation. Mixed exocrine and endocrine pancreatic tumors are extremely rare. There have been a few reports of the rare entity of mixed acinar-endocrine carcinoma of the pancreas, where the endocrine cells represent more than 30% of the tumor. We herein describe a case of such a pancreatic tumor in an asymptomatic patient. CASE PRESENTATION: A 74-year-old male patient with no evident clinical symptoms was referred for surgical resection of a large mass located on the pancreatic head, which was confirmed by an abdominal U/S, CT and MRI. FNA of the mass under endoscopic ultrasound guidance showed the cytology specimen to comprise of cells with morphological and immunohistochemical characteristics of endocrine pancreatic neoplasms. The patient underwent a modified Whipple's procedure and his post-operative course was uneventful. Pathological examination of the tumor revealed a mixed acinar-endocrine carcinoma of the pancreas. CONCLUSION: Mixed tumors of the pancreas are extremely rare and their clinical features and pathogenesis remain unclear. The endocrine component seems to influence their prognosis favorably.Therefore, aggressive surgical therapy remains the only well established line of treatment for these tumors. Further accumulation of clinical cases will help clarify the clinical course and the optimal therapy for these unusual tumors.

Special problems encountering surgical management of large retroperitoneal schwannomas.

BACKGROUND: Retroperitoneal schwannomas are rare, usually benign tumors that originate in the neural sheath and account for only a small percentage of retroperitoneal tumors. The aim of this clinical study is to present our experience in managing retroperitoneal schwannomas with a review of the current literature and to point out the surgical technical difficulties we faced, due to the tumor's strange behavior that eroded the vertebra in two cases without causing malignant invasion. METHODS: We reviewed the medical files of 69 patients treated in our department for retroperitoneal tumors from January 1991 until December 2006. Five patients had retroperitoneal schwannomas according to pathology report. RESULTS: There were two male and three female patients, with a mean age of 56 years (range 44-67 years). All patients were asymptomatic and none suffered from von Recklinghausen disease. Imaging workup included ultrasonography, computed tomography and magnetic resonance imaging. One patient, after having a non-diagnostic computed tomography fine needle aspiration (CT-FNA), underwent exploratory laparotomy and incisional biopsy that established the diagnosis of schwannoma. After complete excision of the tumors, postoperative course was uneventful in all patients. Tumors' maximum diameter was 12.7 cm (range 7-20 cm). No recurrences were detected during the follow up period (6-75 months). CONCLUSION: Preoperative establishment of diagnosis is difficult in case of retroperitoneal schwannomas, however close relationship of retroperitoneal tumors with adjacent neural structures in imaging studies should raise a suspicion. Complete surgical resection is the treatment of choice. Histology and Immunohistochemistry confirms the diagnosis.

One year after laparoscopic "tight" sleeve gastrectomy: technique and outcome.

BACKGROUND: Laparoscopic sleeve gastrectomy (LSG) is currently gaining ground as a new option for the treatment of morbid obesity. The main advantages of this procedure are less postoperative food restrictions, no vomiting, and absence of late complications due to the lack of foreign implants. The aim of this study is to present our experience with this new bariatric technique. METHODS: Ninety three obese patients (65 females and 28 males) who underwent LSG between September 2005 and September 2007 were studied in terms of postoperative complications and weight loss. RESULTS: Mean age was 38.37 +/- 10.81 years (range 19-69) and mean preoperative weight and body mass index (BMI) were 139.12 +/- 24.03 kg (range 100-210) and 46.86 +/- 6.48 kg/m(2) (range 37-66), respectively. Mean follow-up was 12.51 +/- 4.15 months (range 3-24). There were no mortalities, but there were four major and four minor postoperative complications. The mean postoperative excess weight loss (EWL) was 58.32 +/- 16.54%, while mean BMI dropped to 32.98 +/- 6.54 kg/m(2). Mean EWL 3, 6, 12, and 24 months after the operation was 31%, 53%, 67%, and 72%, respectively. Superobese patients (BMI > 50 kg/m(2)) lost less weight. CONCLUSION: In the short term, LSG is a safe and highly effective bariatric operation more suitable for intermediate morbidly obese patients with BMI between 40 and 50 kg/m(2).