Isolated oesophageal Crohn's disease mimicking oesophageal carcinoma treated by Merendino procedure.

Oesophageal involvement is a very rare presentation of Crohn's disease. It can occur as an isolated mass causing dysphagia and can be mistaken for malignancy. Here, we report a case of a 75-year-old woman presenting with dysphagia and weight loss. Gastroscopy showed an ulcerating mass, and her barium swallow showed a bird beak appearance at the level of the gastro-oesophageal junction (GEJ). Repetitive biopsies were inconclusive. Fluorodeoxyglucose-positron emission tomography showed high glucose uptake (standardised uptake value: 10.2) at the level of the GEJ. Endoscopic ultrasound classified the lesion as uT3N1. Step-by-step surgical exploration revealed an oesophageal mass. A frozen section examination showed an absence of malignancy and the presence of inflammatory tissue. A partial oesophagogastrostomy was performed, and reconstruction was achieved by a Merendino procedure. Definitive histopathological examination revealed isolated oesophageal Crohn's disease.

A rare presentation of small diaphragmatic epidermoid cyst with extremely elevated serum CA19-9 level.

Epidermoid cysts are rare lesions that can occur anywhere in the body. They are associated with elevated serum levels of CA 19-9. The spleen represents the most common site of intra-abdominal localisation. Only two cases of diaphragmatic epidermoid cyst are reported in the literature. We present the case of a 61-year-old woman with a small suprasplenic subdiaphragmatic cyst discovered during the investigation of left flank pain. The establishment of an adequate diagnosis was challenging due to the difficulty in specifying the exact localisation of the cyst, the extremely elevated CA 19-9 level of 19,000 and the high uptake on 18-fluoro-2-deoxy-D-glucose positron emission tomography. The definitive diagnosis followed complete surgical excision. Intra-abdominal epidermoid cysts are usually discovered incidentally on imaging for another reason. The cyst is lined by squamous epithelium responsible for the secretion of CA 19-9. The elevation of serum CA 19-9 is due to small rupture or increased intraluminal pressure followed by diffusion to the bloodstream. Surgery with en-bloc resection represents the optimal treatment to avoid any risk of recurrence. The definitive diagnosis is established by demonstrating positive immunohistopathological staining of epithelial cell to CA 19.9.

Intraperitoneal follicular dendritic cell sarcoma: role of chemotherapy and bone marrow allotransplantation in locally advanced disease?

We describe a case of a 44 year-old woman diagnosed with follicular dendritic cell sarcoma (FDCS). FDCS is a very rare disease affecting the dendritic antigen presenting cells and is often misdiagnosed. Surgery is considered the best treatment modality, followed by chemotherapy. In our case, surgical excision was not possible, therefore the patient received two lines of chemotherapy followed by bone marrow allotransplantation, then a third line of chemotherapy with a complete metabolic response seen on PET/computed tomography (CT) follow-up 29 months later. A review of the literature has been performed.

Increased nerve density in deep infiltrating endometriotic nodules.

BACKGROUND/AIMS: Deep infiltrating endometriosis is a very painful condition and the mechanism of pain is still poorly understood. Pain and hyperalgesia can partly be explained by an increased number of nerve structures in the painful lesion. In order to clarify this issue, we assessed the nerve density in deep infiltrating endometriotic nodules of the posterior vagina and in the adjacent healthy vaginal tissue of the same patient. METHODS: A prospective clinical and pathological study of 31 cases of deep infiltrating vaginal endometriotic nodules was conducted. Fifteen patients were in the proliferative phase and 16 in the secretory phase. The nerve density was studied by immunohistochemistry with the monoclonal antibody NF against neurofilaments in deep infiltrating endometriosis and in the adjacent unaffected vaginal tissue in the proliferative and in the secretory phases. Neurofilaments constitute the main structural elements of neuronal axons and dendrites. RESULTS: The nerve density was significantly different in the endometriotic nodule than in the adjacent unaffected vaginal tissue (p = 0.0013). The same significant difference was found between endometriotic nodules and the unaffected vagina in the proliferative phase (p = 0.009) and in the secretory phase (p = 0.04). This difference was not significant between the proliferative and the secretory phases in the endometriotic lesions and in the controls. CONCLUSIONS: We hypothesize that the significantly increased number of nerve structures in the endometriotic nodules may contribute to the occurrence of severe and neuropathic pain that characterizes these lesions.

Small cell carcinoma of the esophagus: a multicentre Rare Cancer Network study.

Small cell carcinoma of the esophagus (SCCE) is a rare and aggressive malignant tumor with a poor prognosis. The aims of this retrospective study were to analyze the epidemiology, clinical characteristics, and treatment outcomes of these patients. Between 1994 and 2004, 24 patients with SCCE from several centers were reviewed for data on demographics, presenting symptoms, diagnosis, disease stage, type of treatment, and outcome. SCCE occurs in the sixth decade: median age (interquartile range [IQR]): 65 (59-69) years with a male predominance (63%). The most common complaining symptoms were rapidly progressive dysphagia (79%), weight loss (54%), and retrosternal/epigastric pain (46%). The tumor arises primarily in the middle (52%) or in the lower (35%) third of the esophagus. History of tobacco and alcohol exposure was present in 90% and 70% of case, respectively. Extensive disease was present in 13 cases (54%) at initial diagnosis. The overall median survival (IQR) was 11 (8-20) months for all 24 patients, and the 2-year overall survival was 25.1%. Four patients were alive more than 2 years after treatment. Chemotherapy increased the survival compared with symptomatic management in extensive disease (median survival [IQR]: 9.5 [6-14] vs. 6 [4-7] months, P= 0.05). In limited disease, concurrent chemo-radiotherapy was more effective than non-concurrent treatment (median survival [IQR]: 36 [14-93] vs. 11 [9-15] months, P= 0.04). Two patients were treated by surgery and chemoradiation therapy with a survival of 35 and 66 months. Chemotherapy is the cornerstone of treatment of SCCE in all stage. For limited disease SCCE, concurrent chemo-radiotherapy is the primary choice compared with sequential approach. The role of surgery was not assessable in our study.

How should we treat mucinous appendiceal neoplasm? By laparoscopy or laparotomy? A case report.

Appendicular mucocele (AM) usually denotes a dilatation of the appendiceal lumen as a result of mucus accumulation that may be related to various neoplastic and non-neoplastic processes. Most of them are discovered incidentally. Treatment consists in complete resection avoiding rupture of the cyst in the peritoneal cavity. Indeed, rupture of such a cystic lesion in the peritoneal cavity can induce a catastrophic complication such as 'pseudomyxoma peritonei' (PMP). Therefore, some authors recommend an open surgical treatment. Currently, the debate concerning the best surgical technique to adopt for AM remains controversial. We report a case of AM found incidentally and treated by laparoscopy. The macroscopic aspect of the appendix suggested the diagnosis intra-operatively and every effort was made to avoid cystic rupture during appendicular resection. The histopathological diagnosis was mucinous cystadenoma. The patient is doing well at 2-year follow-up. The reported case and literature review show us that AM is not a contra-indication for laparoscopic surgery, but major concern resides in the early recognition of such a lesion at laparoscopy and in taking appropriate precautionary measures to avoid rupture in the peritoneal cavity.

Classification of primary and incisional abdominal wall hernias.

PURPOSE: A classification for primary and incisional abdominal wall hernias is needed to allow comparison of publications and future studies on these hernias. It is important to know whether the populations described in different studies are comparable. METHODS: Several members of the EHS board and some invitees gathered for 2 days to discuss the development of an EHS classification for primary and incisional abdominal wall hernias. RESULTS: To distinguish primary and incisional abdominal wall hernias, a separate classification based on localisation and size as the major risk factors was proposed. Further data are needed to define the optimal size variable for classification of incisional hernias in order to distinguish subgroups with differences in outcome. CONCLUSIONS: A classification for primary abdominal wall hernias and a division into subgroups for incisional abdominal wall hernias, concerning the localisation of the hernia, was formulated.

Actinomycotic abscess of the anterior abdominal wall: a case report and literature review.

Actinomycosis is a rare, chronic, suppurative, pseudotumoral illness caused by an anaerobic gram positive organism usually Actinomyces israelii which can mimick a tumoral pathology leading to a mutilating surgical resection. We report a case of abdominal actinomycosis and a literature review.

Nissen versus Toupet fundoplication: results of a randomized and multicenter trial.

BACKGROUND: Laparoscopic Toupet fundoplication (TF) is reported to be as effective as Nissen (NF), but to be associated with fewer unfavorable postoperative side-effects. This study evaluates the one- and three-year clinical outcome of 140 randomized patients after a laparoscopic NF or TF. PATIENTS AND METHODS: Inclusion criteria included patients over 16 years old with complications of gastro-oesophageal reflux disease (GORD) and persistence or recurrence of symptoms after three months of treatment. Subjects with a previous history of gastric surgery or repeated fundoplication, brachy-oesophagus or severe abnormal manometry results were excluded. Seventy-seven NF and 63 TF were performed. The severity of symptoms was assessed before and after the procedure. RESULTS: One hundred and twenty-one of the 140 patients after one year, and 118 after three years, were evaluated and no statistically significant clinical difference was observed. The level of satisfaction concerning the outcome of the operation remained high after one or three years regardless of the type of fundoplication performed. CONCLUSIONS: Functional complications after NF are not avoided with TF.

Amelanotic malignant melanoma of the esophagus. Report of a case.

Primary malignant melanomas of the esophagus are rare neoplasms with aggressive behavior and tendency to lymphatic and hematogenous spread. We report on a patient affected with a primary amelanotic melanoma of the esophagus synchronous to a squamous cell carcinoma of the epiglottis and treated by subtotal esophagectomy. A 58-year-old woman presented with a three-month history of dysphagia, retrosternal pain and weight loss. An upper gastrointestinal endoscopy revealed a 3 cm, non pigmented, polypoid mass of the middle esophagus and biopsy was consistent with undifferentiated carcinoma. Fiberoptic bronchoscopy revealed a 0.5 cm, polypoid lesion of the right aspect of the epiglottis consistent with a squamous cell carcinoma. The esophageal neoplasm was treated by subtotal esophagectomy while the squamous cell carcinoma of the epiglottis was referred to radiotherapy 1 month after surgery. Microscopy showed diffuse proliferation of anaplastic cells with multiple mitotic figures, marked nuclei and multilobulated nucleoli. The neoplasm stained positive for HBM-45 antigen and S-100 protein. Tumor stage was pT1N0M0. Review of patient's medical history and exploration of skin and mucous membranes failed to reveal malignant lesions and definitive diagnosis was primary amelanotic malignant melanoma of the esophagus. The patient died 16 months after surgery of disseminated disease. In conclusion the present case confirms that primary malignant melanomas of the esophagus have a dismal outcome even in cases of early-stage lesions amenable to surgical resection.

Oesophageal perforation by an anterior cervical fixation device: management in debilitated patients.

Oesophageal perforation following anterior cervical fixation has been reported in the neurosurgical and orthopaedic literature as a rare complication of such procedure. The complications associated with oesophageal perforation may range from minor symptoms to mediastinitis and death. We report two oesophageal perforations following cervical fixation device migration in patients with poor prognosis, managed successfully with conservative surgical and endoscopic techniques.

[Verrucous carcinoma of the esophagus. A case report]. / Su di un caso di carcinoma verrucoso dell'esofago.

Verrucous carcinoma (VC) of the esophagus is a rare variant of squamous cell carcinoma and only 20 cases have so far been reported in the international literature. The neoplasm is usually highly differentiated, presents a slow growth pattern with invasion of surrounding organs rather than blood-borne metastases. Recently, a causative role of human papillomavirus (HPV) has been hypothesized. The case of a patient affected with locally advanced VC of the esophagus and treated by means of local antiviral therapy is reported. A 41-year-old male patient was admitted to our institution for persistent atypical thoracic pain. The imaging techniques (thoracic and abdominal CT scans; upper GI endoscopy; hydrosoluble contrast swallow; endoscopic US) revealed a cauliflower-like protruding esophageal mass, active mucosal mycosis, multiple ulcerations of the distal esophagus, as well as 2 broncho-esophageal fistulas. The neoplasm extended beyond the esophageal wall, infiltrating surrounding cervical and mediastinal organs and the patient presented with secondary esophageal achalasia and right bilobar pneumonia. The histologic specimen was consistent with VC of the esophagus and the presence of HPV infection was detected by means of qualitative PCR assay. The patient was deemed not fit for surgery and a local antiviral treatment with hydroxy-phosphonyl-methoxypropyl-cytosine 5 mg/kg a week was started. After initial response to treatment, the patient presented with sudden progression leading to further broncho-esophageal fistula treated with endoscopic stent placement and ultimate death 6 months after referral to our center. In keeping with international data, our case confirms that esophageal VC has a highly unfavorable outcome, despite its high degree of differentiation and slow growth pattern. The long natural history, the lack of specific symptoms and the presence of coexisting esophageal diseases delay the diagnosis and account for the local advancement of this malignancy. Surgery is the option of choice for early stage lesions and advanced VC does not seem to benefit from current chemotherapeutic regimens. The causative role of HPV and the advancements of molecular pharmacology might allow for effective treatment in high-risk patients.

The role of PET scan in the preoperative management of oesophageal cancer.

AIM: The aim of this study was to evaluate the role of the 18F-FDG-PET in the preoperative evaluation of patients with oesophageal or gastro-oesophageal junction (GEJ) cancer and to define its impact on therapeutic management. PATIENTS AND METHODS: This study included 58 patients with biopsy proven oesophageal or GEJ cancer who underwent PET in addition to the conventional diagnostic work-up. The sensitivity, specificity and accuracy of CT and PET were calculated for detection of tumour and distant metastases. RESULTS: The sensitivity, specificity and accuracy of tumour detection were, respectively, 84, 100 and 84% for CT and 87, 100 and 87% for PET (p=ns). PET permitted detection of distant metastases, which were not seen on CT in seven patients. In two patients, a second primary tumour was detected on PET. CONCLUSIONS: The sensitivity of PET for loco regional lymph nodes detection is low. The major clinical impact is the detection of distant metastases.

Idiopathic eosinophilic oesophagitis: atypical presentation of a rare disease.

A 72 year-old man presented severe dysphagia and weight loss of recent onset. Repeated oesophageal endoscopy and biopsies with macroforceps were normal. Oesophageal manometry disclosed features compatible with achalasia. Oesophageal EUS endoscopy localized an infiltrating process between muscular layers of the oesophageal wall and CT scan delimited a circular thickening in the inferior part of the oesophagus. Because of severe clinical presentation mimicking a possible oesophageal neoplasm like a lymphoma, partial oesophagectomy was performed and revealed eosinophilic oesophagitis. This unusual presentation emphasizes that idiopathic eosinophilic oesophagitis must be proposed in the differential diagnosis of dysphagia, even in old patient without apparent oesophageal lesion at endoscopy.

Laparoscopic Nissen fundoplication after failure of Enteryx injection into the lower esophageal sphincter.

BACKGROUND: We investigated the feasibility, safety, and efficacy of laparoscopic antireflux surgery (LARS) after failure of Enteryx injection in the lower esophageal sphincter for the treatment of gastroesophageal reflux disease (GERD). METHODS: Four patients underwent LARS after failure of Enteryx injection. Particular care was taken during the procedure to identify unusual material or fibrosis. RESULTS: All patients underwent LARS successfully. In three patients, tight adhesions with fibrous tissues and black foreign material were observed around the esophagus. No complications occurred during the procedures. The postoperative period was uneventful and functional results were excellent. CONCLUSION: LARS following Enteryx injection is feasible. Careful dissection is mandatory to avoid operative complications. Both techniques may be considered as options for treating GERD.

Preferential infiltration of large bowel endometriosis along the nerves of the colon.

BACKGROUND: Little is known about the mode and the extent of infiltration of endometriotic lesions in the large bowel. METHODS: In 31 patients undergoing large bowel resection for severe deep-infiltrating endometriosis of the sigmoid and rectum with severe digestive symptoms, we performed a prospective morphological, histological and immunohistological study (using the monoclonal antibodies S100 for the detection of the nerves and CD10 for the detection of the endometriotic stromal cells) on the large bowel resection specimen. The evaluation of invasion of the large bowel by endometriosis was performed by studying the presence, localization and mean number of lesions in the different layers of the colon, the relationship between endometriosis and the nerves of the colon, the nerve density in the respective layers of the large bowel and the presence of endometriosis on the resection margins. RESULTS: The most richly innervated layers of the large bowel are the most intensely involved by endometriosis. We found that 53 +/- 15% of endometriotic lesions were in direct contact the nerves of the colon by means of perineurial or endoneurial invasion. The mean largest diameter of the lesion does not seem to be correlated with the depth of infiltration. The margins were positive in 9.7% of cases. In cases of positive margins, the endometriotic lesions were in close histological relationship with the nerves. CONCLUSIONS: There is a close histological relationship between endometriotic lesions of the large bowel and the nerves of the large bowel wall. Endometriotic lesions seem to infiltrate the large bowel wall preferentially along the nerves, even at distance from the palpated lesion, while the mucosa is rarely and only focally involved.

Newly developed Barrett's esophagus after subtotal esophagectomy.

BACKGROUND AND STUDY AIMS: More detailed information regarding the early mucosal events that lead to intestinal metaplasia would be very beneficial for understanding the pathogenesis of Barrett's esophagus (BE). Gastroesophageal reflux and duodenogastroesophageal reflux play a major role in the pathogenesis of Barrett's esophagus. The aim of this study was to investigate the prevalence of newly developed BE in patients who had previously undergone a subtotal esophagectomy - a clinical condition characterized by the absence of a lower esophageal sphincter and massive gastroesophageal reflux. PATIENTS AND METHODS: A retrospective examination was carried out on all patients who underwent subtotal esophagectomy (n = 87) listed in our institution's computer files from 1995 to 2000. Twenty-one patients were excluded due to missing data or no upper gastrointestinal endoscopy after surgery. RESULTS: Based on the Savary-Miller classification, 47 patients developed either type I (n = 2), II (n = 8), III (n = 11) or IV (n = 26) esophagitis after surgery. Newly developed BE was observed in nine patients (13.5 %) after subtotal esophagectomy (median time to diagnosis: 489 days, range 43 - 1172). None of the patients had persistent BE immediately after surgery, and two of the patients with newly developed BE had had no history of BE before surgery or at the time of surgery. Proton-pump inhibitor therapy after surgery and neoadjuvant chemotherapy did not appear to influence the development of BE after subtotal esophagectomy. CONCLUSIONS: Newly developed BE after subtotal esophagectomy may provide further insights into the early mucosal events that lead to intestinal metaplasia and into the roles of gastroesophageal and duodenoesophageal reflux in the pathogenesis of BE.

Wilkie's syndrome.

A superior mesenteric artery syndrome (SMAS) was diagnosed in two young women with, respectively, a 2- and 1-year history of postprandial vomiting and epigastric pain. The patients underwent a laparoscopic duodenojejunal bypass, and resumed a normal diet on the fifth postoperative day. The patients are still symptom-free with patent anastomosis on gastrointestinal radiographic control at 24 and 6 months, respectively, following their operation. Herein we also describe the varying clinical presentation of this rare syndrome, as well as treatment options. We conclude that laparoscopic duodenojejunostomy offers a new therapeutic approach to SMAS. It is reliable and safe; the operating time is acceptable; and diet recovery and hospital stay are both short. However, these preliminary results still need to be confirmed by further observations.

Hyperalgesia, nerve infiltration and nerve growth factor expression in deep adenomyotic nodules, peritoneal and ovarian endometriosis.

BACKGROUND: The aim of this study was to investigate a possible role for nerve growth factor (NGF) in the mechanism of pain and hyperalgesia induced by deep adenomyotic nodules and other forms of endometriosis and to clarify the relationship between endometriotic lesions and the surrounding nerves. METHODS: Endometriotic lesions (deep adenomyotic nodules, peritoneal endometriosis, ovarian endometriosis) and eutopic endometrium were obtained from 51 patients presenting with pain. Patients were allocated to two groups (group 1: patients with a deep adenomyotic nodule (n = 23); group 2: patients with peritoneal and/or ovarian endometriosis but without deep adenomyotic nodule (n = 28). Immunohistochemistry with antibodies against NGF, NGF specific tyrosine-kinase receptor (Trk-A) and S-100 protein was performed. Results were expressed as mean H-scores +/- SD, and correlated with the presence of hyperalgesia. RESULTS: The percentage of patients presenting hyperalgesia at physical examination was significantly higher in group 1 (96%) than in group 2 (11%) (P < 0.001). NGF expression was significantly stronger in deep adenomyotic nodules (DAN) than in ovarian (OE) and peritoneal endometriosis (PE), both in the proliferative phase in the glands [DAN: 226 +/- 18; OE: 140 +/- 9 (P < 0.001); PE: 110 +/- 7 (P < 0.001)] and in the stroma [(DAN: 204 +/- 21; OE: 125 +/- 15 (P < 0.001); PE: 100 +/- 9 (P < 0.01)]. NGF expression in DAN is also significantly stronger than in OE and PE in the secretory phase in the glands [DAN:181 +/- 32; OE: 85 +/- 3.3 (P < 0.001); PE: 65 +/- 9 (P < 0.001)] and in the stroma [DAN: 173 +/- 28; OE: 85 +/- 3.7 (P < 0.001); PE: 35 +/- 13 (P < 0.001)]. Perineurial and intraneurial invasion by endometriotic lesions were found only in deep adenomyotic nodules and not in the other forms of endometriosis. The specific receptor for NGF (Trk-A) is expressed in all the nerves that were included in the biopsies. CONCLUSIONS: These results suggest a role of NGF in endometriotic pain and hyperalgesia in deep adenomyotic nodules. The strong expression of the NGF-TrkA pathway in deep adenomyotic nodules could explain why this type of lesion infiltrates in richly innervated anatomical sites.