Endoscopic or surgical ampullectomy for intramucosal ampullary tumor: the patient populations are not the same.

PURPOSE: To evaluate and compare the results of endoscopic ampullectomy (EA) and surgical ampullectomy (SA) for benign tumors. MATERIAL AND METHOD: From 2005 to 2018, 66 patients were eligible for ampullectomy (adenoma, ultrasound grading≤T1, N0). The principal endpoint was the rate of complete resection: R0 resection without local recurrence and no recurrence after a single operative procedure and with final pathology confirming the operative indications. Secondary endpoints were: morbidity, mean length of stay (LOS), preoperative and final pathology results, complete (or incomplete) resection, mean duration of follow-up, local recurrence rate and late complications (biliary stricture). RESULTS: Among the 41 patients eligible for EA, 36 ampullectomies were performed, while five were referred for SA because of intracanalar invasion that had not been diagnosed initially. The rate of complete treatment was 48% (13/27), the morbidity was 10%; the LOS was 3.3days; no adenomatous lesion was found on the definitive pathology in 9 patients (25%). The resection was R1 in 30% but there were no R2 resections. The median duration of follow-up was 20months, the rate of local recurrence was 22% and the rate of late biliary stricture was 2%. Among the 30 patients who were eligible for SA, (25 plus the five referred by endoscopists after intracanalar invasion was found), SA was eventually performed in 24: five patients were converted to pancreatoduodenectomy, one patient was found to have metastatic disease and had no resection. An extemporaneous frozen-section pathology examination was performed in 22 of the 24 SA patients and confirmed healthy margins on the bile ducts and pancreatic ducts. The rate of complete treatment was 71% (17/24), morbidity was 35%; LOS was 10days; seven patients (29%) were found to have invasive adenocarcinoma, and six of these (86%) underwent subsequent pancreatoduodenectomy. None of the resections were R1 or R2; median follow-up was 21months; there was no local recurrence; biliary stricture rate was 8%. CONCLUSION: The short-term and long-term results of patients undergoing endoscopic or surgical ampullectomy are different. The lesions resected by EA were less advanced, with simpler postoperative course. The lesions for which SA was performed were more advanced, and had more morbid sequelae, but with a better rate of complete treatment and better long-term results.

Serum value of procalcitonin as a marker of intestinal damages: type, extension, and prognosis.

BACKGROUND: Ischemic and necrotic damages are complications of digestive diseases and require emergency management. Nevertheless, the decision to surgically manage could be delayed because of no sufficiently preoperative accurate marker of ischemia diagnosis, extension, and prognosis. METHODS: The aim of this study was to assess the predictive value of serum procalcitonin (PCT) levels for diagnosing intestinal necrotic damages, their extension, and their prognosis in patients with ischemic disease including ischemic colitis and mesenteric infarction by a gray zone approach. Between January 2007 to June 2014, 128 patients with ischemic colitis and mesenteric infarction (codes K55.0 and K51.9) were operated, for whom data on PCT were available. We perform a retrospective, multicenter review of their medical records. Patients were divided into subgroups: ischemia (ID group) versus necrosis (ND group); the extension [focal (FD) vs. extended (ED)] and the vital status [deceased (D) vs. alive (A)]. RESULTS: PCT levels were higher in the ND (n = 94; p = 0.009); ED (n = 100; p = 0.02); and D (n = 70; p = 0.0003) groups. With a gray zone approach, the predictive thresholds were (i) for necrosis 2.473 ng/mL, (ii) for extension 3.884 ng/mL, and (iii) for mortality 7.87 ng/mL. CONCLUSION: In our population, PCT could be used as a marker of necrosis; especially in case of extended damages and reflects the patient's prognosis.

Primary cutaneous T-cell lymphoma presenting as mycosis fungoides with a T-/null-cell phenotype: report of two cases.

Variations in the clinical and histological presentation of cutaneous T-cell lymphoma (CTCL) can hamper diagnosis. We report two cases of a novel presentation of CTCL characterized by an aberrant immunophenotype with complete loss of pan T-cell antigens including T-cell receptor ß chain and showing the clinical and histopathological appearance of erythrodermic and plaque-stage mycosis fungoides.

Primary rectal linitis: a rare clinical condition.

Rectal linitis is a rare tumor with a poor prognosis that may be seen as a primary tumor or in association with gastric linitis plastica. In this article we describe the morphological appearance on CT and magnetic resonance imaging, which is typical. We also discuss the oncologic management, which is not well defined, including the potential value of neo-adjuvant treatment.

Nonuraemic calciphylaxis: response to treatment with pamidronate and negative pressure therapy.

Calciphylaxis is a rare cause of skin ulcerations and necrosis in patients with both normal renal and parathyroid function. Although calciphylaxis appears to be on the increase, treatments are mainly empirical, especially for wound care. The lesions in calciphylaxis are typically very painful and carry a high risk of infection, with sepsis being the leading cause of death in this serious disease. We report two cases of nonuraemic calciphylaxis, which responded to treatment with pamidronate and wound management by negative pressure system.

Innate mucosal-associated invariant T (MAIT) cells are activated in inflammatory bowel diseases.

Inflammatory bowel diseases are characterized by a deregulated immune response targeting the gut bacterial flora. Mucosal-associated invariant T (MAIT) cells are major histocompatibility complex (MHC) class Ib-restricted innate-like lymphocytes with anti-bacterial functions. They display an effector/memory phenotype and are found in large numbers in the blood, mucosae and liver. They have also been implicated in inflammatory diseases such as multiple sclerosis. Therefore, we aimed to analyse the possible involvement of MAIT cells in Crohn's disease (CD) and ulcerative colitis (UC). To this end, a phenotypical and functional analysis of MAIT cells isolated from the blood of healthy subjects, CD and UC patients was undertaken. MAIT cells were also quantified in ileal biopsies of CD patients. The frequency of blood MAIT cells was specifically reduced in IBD patients compared with healthy donors, whereas it was dramatically greater in the inflamed versus healthy tissue. MAIT cells were activated as they expressed significantly more the Ki67 antigen, and this was accompanied by phenotypical changes such as increased expression of natural killer (NK)G2D and B and T lymphocyte attenuator (BTLA). Finally, in-vitro-activated MAIT cells from CD and UC patients secreted significantly more interleukin (IL)-17, together with a decreased interferon (IFN)-γ in CD but an increased IL-22 in UC. These data show that MAIT cells are activated in IBD, which results in an increased recruitment towards the inflamed tissues, an altered phenotype and a switch in the pattern of cytokine secretion. This is the first demonstration that MAIT cells are immune players in IBD, whose precise functions in this context need to be addressed.

Intraoperative consultation in digestive surgery. A consecutive series of 800 frozen sections.

AIM: To assess indications and quality of frozen sections in digestive surgery. PATIENTS AND METHODS: All the frozen sections from the department of digestive surgery from Amiens hospital performed between 01/07/2006 and 01/07/2010 were assessed. Assessment of frozen section forms, reading of pathology reports, and reviewing of frozen section slides were performed. RESULTS: Eight hundred frozen sections were performed in 349 patients. From one to 14 surgical specimens were sent for frozen section (mean 2.3). Frozen sections were performed in 77% of the cases for cancer surgery (n=268), most of the time pancreatic surgery (28.4%) and liver surgery (24.6%). Frozen sections were performed in 69% of the cases for diagnosis, in 29% of the cases to assess surgical margins and in 2% of the cases to assess if tissue specimen was appropriate for pathological diagnosis. Frozen sections were sent all days of the week (except Saturday and Sunday), during all the year, between 8 H 30 and 17 H 15. Thirty-seven percent of the cases were sent between 12 H and 14 H. Response time was 15 minutes (3 to 57 minutes). Rate of differed diagnoses was 2%. Rate of discordant diagnoses was 3.4%. CONCLUSION: Frozen section is a rapid and accurate tool in digestive surgery. Local adjustment of the organization of the Pathology Department could enhance the rapidity and the quality of pathology diagnoses.

Letter: A case of generalized guttate psoriasis induced by etanercept with relapse after abatacept.

Abatacept is the first in a new class of agents for the treatment of rheumatoid arthritis. We report a case of guttate psoriasis in a patient treated with abatacept for rheumatoid arthritis. This patient had the same reaction with etanercept in the past.

Sudden death caused by aortic dissection in a patient with polycystic kidney disease.

A 43-year-old man presented at the emergency medical unit with chest pain. The results of a clinical examination were normal, apart from sternum pain (without radiation) on palpation. The patient had no respiratory problems and the pain was relieved by paracetamol. The electrocardiogram, laboratory tests and chest X-ray were normal. However, the man was found dead the next morning. In the autopsy, we noted the presence of haemopericardium, aortic dissection (starting from the vessel's origin and extended to the aortic arch and on through the diaphragm), polycystic kidney disease and liver cysts. In adult autosomal dominant polycystic kidney disease (ADPKD) patients, the main causes of death are ruptured intracerebral aneurysms, coronary artery disease, congestive heart failure, valvular heart disease and ruptured abdominal aortic aneurysms. Aortic dissection is considered to be rare cause of sudden death in ADPKD sufferers. ADPKD can have serious consequences for the vascular system. The families of confirmed ADPKD sufferers must be informed and screened as early as possible, in order to prevent renal and cardiovascular complications.

Gastric metastases. An endoscopic series of ten cases.

We report a series of ten cases of the clinical, endoscopic and pathological features of gastric metastases. Patients were six women and four men between 54 and 88 years old, with gastric metastases from breast carcinoma (4), lung carcinoma (4) and melanoma (2). Patients underwent an upper gastrointestinal endoscopy for epigastralgia (2), hematemesis (2), dysphagia (1) and anemia (5). On endoscopy, tumors appeared as nodules with a central ulceration (5), an ulceration (4) or simulating linitis plastica (1). Metastases were located in the cardia (2), fundus (5) and antrum (3). Primary tumors had been diagnosed between one day and 20 years before upper endoscopy. Eight patients had multivisceral metastases. The microscopic features of the gastric metastases resembled a primary gastric cancer in eight cases. Thanks to clinical data, the pathologist confirmed the diagnosis of gastric metastases on immunohistochemistry. Nine patients died in the eight-month follow-up period. Gastric metastases are rare, occur at a late stage of the neoplastic disease, and have a poor prognosis. Diagnosis of gastric metastases is difficult because they simulate primary gastric cancer on endoscopy and on microscopic examination. A correct diagnosis is based on good communication between gastroenterologists and pathologists.

[Eosinophilic dermatosis associated with hematological disorders: A clinical, histopathological and immunohistochemical study of six observations]. / Dermatose éosinophilique associée aux hémopathies : étude clinique, histopathologique et immunohistochimique de six cas.

BACKGROUND: Eosinophilic dermatosis of hematologic disease (EDH) or insect bite-like reaction is a pruritic dermatitis described mostly in patients with chronic lymphocytic leukaemia (CLL). We describe six patients with the disorder in association with CLL and other blood dyscrasias. PATIENTS AND METHODS: We reviewed the medical records of patients with EDH seen between 2004 and 2009 in our department and re-examined histological slides. RESULTS: Mean age at dermatosis onset was 75.6 years and the sex ratio was 1. There were three CLL, two mantle-cell lymphomas and one MALT-type lymphoma. The dermatitis was quite polymorphic, with erythematous papules, wheals and plaques. The initial skin lesions appeared at the same time as or after the diagnosis of haematological neoplasm. Their reappearance heralded relapse of the blood disease in three cases. Histologically, all lesions had a dense dermal infiltrate of small, mostly CD4+ T-cells, with numerous eosinophils. In three patients, there was marked folliculotropism, resembling folliculotropic T-cell lymphoma. In most cases, EDH disappeared after appropriate chemotherapy for the blood disorder. DISCUSSION: Our cases show that the clinical expression of EDH is quite polymorphic. Its appearance may precede relapse of or may indicate prompt search screening for blood dyscrasia. The most efficient treatment of this dermatosis appears to be specific chemotherapy for the blood dyscrasia. There is reason to believe that a population of T-helper 2 (Th2) lymphocytes, reactive to malignant B-cells, induces tissue eosinophilia, mainly through production of interleukin (IL)-5, among other cytokines. Eosinophils appear to be the main effector cells.

[A case of pancreatic schwannoma successfully treated by enucleation]. / Schwannome pancréatique traité avec succès par énucléation : à propos d'un cas.

Benign pancreatic schwannoma is a very rare cystic and solid tumor, which poses a diagnostic and therapeutic challenge. Herein, we present a case of 4-cm solitary benign schwannoma of pancreatic uncinate process, with pancreas divisum, incidentally discovered in a 53-year-old woman. Preoperative diagnosis was established by endoscopic ultrasound and fine needle aspiration. Simple enucleation of the tumor from the surrounding parenchyma was successfully performed. No complications were found after the operation.

[Conservative treatment of an aortoduodenal fistula after paraaortic lymphadenectomy in hepatobiliary malignancy surgery with aortic endoprosthesis]. / Traitement conservateur d'une fistule aortoduodénale après curage interaorticocave dans le cadre d'une chirurgie hépatobiliaire carcinologique par endoprothèse aortique.

Lower gastrointestinal bleeding from a primary aortoduodenal fistula is unusual and usually fatal. Postoperative aortoduodenal fistula after biliary surgery is a very rare complication. We report hence a 69-year-old female patient who underwent a main bile duct resection with extended paraaortic lymphadenectomy for a cholangiocarcinoma. Acute melena with hemoglobin drop occurred on postoperative day 24. Initial CT-scan showed an aortic pseudoaneurysm with aortoduodenal fistula. An aortic endoprosthesis with endoscopic drainage of periaortic collections allowed successful treatment.

[Low-molecular-weight heparin-induced bullous haemorrhagic dermatosis associated with cell-mediated hypersensitivity]. / Dermatose bulleuse hémorragique associée à une réaction d'hypersensibilité retardée sous héparine de bas poids moléculaire.

BACKGROUND: Heparin-induced bullous hemorrhagic dermatosis is a rare, recently described side-effect of subcutaneous heparin injection. We describe a patient simultaneously presenting distant haemorrhagic bullae and eczematous reaction at the low molecular-weight heparin (LMWH) injection sites. PATIENTS AND METHODS: Subcutaneous enoxaparin sodium was initiated in a 51-year-old patient and was replaced a few days later by tinzaparin sodium. Forty-eight hours later, annular, erythematous and vesicular plaques appeared at the injection sites (thighs). Small hemorrhagic bullae were noted on the abdominal skin at the same time. Skin biopsies revealed respectively eczematous dermatitis and an intraepidermal blister filled with red blood cells. Direct immunofluorescence was negative. Standard laboratory investigations and coagulation studies were unremarkable. Skin lesions disappeared ten days after discontinuation of LMWH. Patch tests and intradermal tests were negative. DISCUSSION: The case described herein shares the stereotypical clinical picture previously reported, namely small, multiple, haemorrhagic bullae on normal skin, appearing at remote sites five to 21 days after the start of subcutaneous heparin treatment. Despite the absence of clear management guidelines, it is obviously tempting to stop the heparin if there are too many bullae for fear of more clinically significant and dangerous mucous membrane lesions. The underlying physiopathological mechanism is poorly understood; no coagulation abnormalities were recorded. In addition, our patient presented an eczematous reaction at the injection sites, raising the possibility of a type IV hypersensitivity reaction. The association of these two cutaneous side effects of heparin is perhaps not purely coincidental.

[Hepatic Epithelioid hemangioendothelioma: a rare liver tumor]. / L'hémangioendothéliome épithélioïde du foie: une tumeur hépatique rare.

Epithelioid hemangioendothelioma of the liver is a rare primary malignant tumor of vascular origin. It most frequently occurs in middle age adults and clinical findings are non-specific. Histological diagnosis may be difficult to achieve with lesions containing a large myxoid component, and immunohistochemical staining is required. A few imaging features may suggest the correct diagnosis, and radiologists should be familiar with these findings. We will illustrate the potential role of PET-CT at the time of initial presentation. Prognosis is variable and optimal management is based on a case by case analysis, ranging from clinical and imaging follow-up to liver transplantation.