Laurent Coubeau

Successful emergency resection of a massive intra-abdominal hemophilic pseudotumor.

An intra-abdominal pseudotumor is a rare complication of hemophilia. Surgical treatment is associated with high morbidity and mortality rates and reported cases are scarce. We present a 66-year-old Caucasian male suffering from severe hemophilia type A treated for 10 years with Factor VIII. Major complications from the disease were chronic hepatitis B and C, cerebral hemorrhage and disabling arthropathy. Twenty-three years ago, retro-peritoneal bleeding led to the development of a large intra-abdominal pseudotumor, which was followed-up clinically due to the high surgical risk and the lack of clinical indication. The patient presented to the emergency department with severe sepsis and umbilical discharge that had appeared over the past two days. Abdominal computed tomography images were highly suggestive of a bowel fistula. The patient was taken to the operating room under continuous infusion of factor VIII. Surgical exploration revealed a large infected pseudotumor with severe intra-abdominal adhesions and a left colonic fistula. The pseudotumor was partially resected en bloc with the left colon leaving the posterior wall intact. The postoperative period was complicated by septic shock and a small bowel fistula that required reoperation. He was discharged on the 73(rd) hospital day and is well 8 mo after surgery. No bleeding complications were encountered and we consider surgery safe under factor VIII replacement therapy.

Tacrolimus (TAC) and Single Intra-Operative High-Dose of r-ATG Induction vs. Tacrolimus Monotherapy as Immunosuppression (IS) in Adult Liver Transplantation (LT): One-Year Results of an Investigator-Driven, Prospective, Randomized, Controlled Trial (RCT)

Introduction The role of induction therapy using anti-lymphocytic serum is poorly understood in LT as it is its utility in tolerance induction. We aimed to assess efficacy of a high-dose of rabbit anti-lymphocytic serum (r-ATG, Grafalon®, Neovii) administered intra-operatively in combination with TAC (Prograft®, Astellas) in primary adult LT. Materials and Methods Investigator-driven, single-centre, open-label, prospective RCT stratifying pts into an intra-operative dose of 9 mg/kg r-ATG followed by TAC monotherapy maintenance IS (TAC-ATG group; n=97) and TAC monotherapy IS (TAC group, n=109).The primary endpoint was minimization of IS to monotherapy within 12 months. Safety endpoints were patient (PS) and graft survival (GS). The secondary endpoint was one-year biopsy-proven acute cellular rejection (ACR). 200 patients were required to provide a power of 80% to detect a difference of 9% in attaining TAC monotherapy within 12 mo, accepting 5% level of &agr; error. Means were compared with t-test and proportions with Pearson’s &khgr;2 test. Time to clinical rejection, PS and GS were analysed with the Kaplan–Meier method and compared with the log-rank test. All patients had similar clinical, biochemical and histological follow-up (FU) including protocol biopsies. ACR treatment was based on concordance of Banff score > 5 and biochemical score (including bilirubin, platelet and eosinophilia) >2. Steroid-resistant rejection (SRR) was defined as absence of response to 3-5 pulses of 200 mg methylprednisolone. Mean FU for TAC-ATG and TAC groups were 87 and 95 months. Results 78/80 (97.5%) TAC-ATG and 100/101 (99.0%) TAC pts were steroid free (p=0.429). A second immunosuppressant (steroids, mycophenolate, azathioprine or mTOR inhibitor) was administered in 29/80 (36.3%) TAC-ATG and 35/101 (34.7%) TAC pts (p=0.823). One-year mean TAC trough level was 5.19 (±3.06) mg, in TAC-ATG and 5.04 (±2.80) mg in TAC pts (p=0.738). No statistically significant differences in one-year PS (83% TAC-ATG vs. 92% TAC pts, p=0.260) and GS (76% TAC-ATG vs. 90% TAC pts, p=0.054) were observed. A 6-9 Banff score was seen in 27/107 (25.2%) TAC and 14/83 (16.9%) TAC-ATG pts (p=0.164). No difference was observed in relation to the number of treated rejection (15% TAC-ATG vs. 18% TAC pts developed steroid-sensitive rejection (SSR), p=0.449); 2% TAC-ATG and 3% TAC pts (p=0.628) developed SRR. Chronic rejection was diagnosed in 1% in TAC-ATG vs. 4% in TAC pts (p=0.307). Discussion This first ever-done RCT comparing TAC and single intra-operative high-dose of r-ATG induction vs. TAC monotherapy as IS in adult LT did not show any benefit in relation to IS minimization nor survival. Rejection treatment based on concordance between histology and biochemistry was similar in both groups. Conclusions The studied induction protocol did not offer short-term clinical benefit. Long-term results have to be awaited in order to analyse its’ influence on tolerance induction. (EudraCT 2006-004830-34). Unrestricted grant from Neovii - Fresenius for immunological research not presented in this study.

Hibernoma mimicking liposarcoma

A 26-year-old man was referred to the ophthalmology department with a 1-week history of blurred vision of the left eye; there was no pain or redness. Ophthalmological examination found unilateral choriocapillaropathy with optic nerve oedema. He was in a stable relationship and had not travelled abroad recently, nor had he been in contact with any animals or pets. He had a history of ulcerative colitis diagnosed 2 years earlier for which he was treated for 1 year with azathioprine and oral corticosteroids. Since then he had been totally asymptomatic. Physical examination found no abnormalities. Blood analysis, including blood count, serum C-reactive protein, electrolytes, creatinine, liver enzymes, and protein electrophoresis, was normal. Serologic tests for Lyme borreliosis, Bartonella organisms, syphilis, and HIV were also negative. Serum angiotensin-converting-enzyme level was within the normal range but lysozyme was slightly increased at 21 mg/L (normal range <17 mg/L). Because sarcoidosis associated posterior uveitis was the suspected diagnosis an 18fluorodeoxyglucose PET/CT scan was done. This showed a hypermetabolic (standardised uptake value maximum [SUVmax], 3·3) lipomatous abdominal tumour measuring 12 cm × 5·6 cm × 22·3 cm with an extension into the crural hole, highly suggestive of a liposarcoma. The tumour was surgically resected, which required a mono-bloc resection with careful dissection of the femoral nerve. Surprisingly, pathological examination of the mass showed multivacuolated immature adipocytes—consistent with a diagnosis of an hibernoma. The ophthalmological pathology he presented with improved spontaneously within 1 month leading to a presumed diagnosis of isolated sarcoidosis posterior uveitis. Hibernomas are rare, benign, soft tissue tumours of young adults, mostly found in the thigh, shoulder, back, neck, and chest—although they can arise in retroperitoneum. They are slow growing, non-infiltrative, often asymptomatic tumours composed of mitochondriarich brown adipose tissue normally found in large amounts in hibernating animals and newborn babies. The elevated SUVmax, caused by the high mitochondrial content and increased metabolic activity, is a pitfall in the differential diagnosis of soft tissue neoplasms. Interestingly in this case, the SUVmax was lower than most reported cases but closer to SUVmax rates usually attributed to well differentiated liposarcomas. Retroperitoneal liposarcomas usually occur in middle-aged or older adults and are invasive in nature; liposarcomas observed in our patient’s age group are usually myxoid liposarcomas and these are rarely found retroperitoneally.

Abdominal Organ Transplantation: An Overview

Since the implementation in clinical practice of abdominal vascularized organ transplantation in the 1950s, enormous technical progresses have been made both in relation to organ procurement and implantation. These surgical refinements together with improvement of the medical care of the immunosuppressed recipient have transformed vascularized organ transplantation into a curative therapy of many end-stage organ failures. The different technical (r)evolutions made during the last 50 years in kidney, pancreas, liver, intestine, parietal, and even uterus transplantation are highlighted in this chapter. As the ever-increasing demand for organ transplantation is unfortunately paralleled by an ever-increasing organ shortage, special attention has also been given to different possibilities allowing to safely expand the, postmortem as well as the living donor, organ pool. Finally the more and more frequently applied combined abdominal and/or thoracic organ transplantations are also addressed.

Leiomyosarcoma of the Inferior Vena Cava Level II Including Both Renal Veins : Surgical Approach.

Abstract A 66 years old male developping an acute lower right limb oedema due to an extended venous thrombosis of the common femoral and iliac veins was diagnosed to have a leiomyosarcoma of the inferior vena cava (IVC) involving both renal veins. The characteristics and management of this level II IVC leiomyosarcoma are discussed with particular attention to the renal vein reconstruction and neo-adjuvant therapy.