Carcinoid Tumour of the Appendix: An Analysis of 1,485 Consecutive Emergency Appendectomies

Aim  The aim of this study is to conduct a retrospective analysis of the incidence and long-term results of carcinoid tumours of the appendix in emergency appendectomies. Methods  A retrospective review of 1,485 appendectomies was performed in two centres from January 2000 until January 2006. Demographic data, clinical presentation, histopathology, operative reports and survival were scored and compared with the literature. Results  In three women and four men, carcinoid tumours were identified (0.47%). The mean age was 32.7 years (range, 20–59 years). The clinical presentation was resembling the symptoms of acute appendicitis in all cases. Laparoscopic appendectomy was the treatment of choice in five patients; in one of these patients, a conversion to laparotomy was necessary. The other two patients underwent primary open appendectomy. Five patients underwent additional surgery after the pathology report became available. Four patients underwent ileocecal resection; one other patient underwent right hemicolectomy. In none of the re-operation specimens was residual carcinoid tumour detected. After a mean follow-up of 65 months (range, 25–92), all patients were alive and disease- and symptom-free. Conclusion  Carcinoid tumours of the appendix most often present as acute appendicitis. It also emphasises the value of histopathological analysis of every removed appendix. The long-term prognosis of incidentally found carcinoids of the appendix is good.     

Management of complex appendicitis

Acute appendicitis is the commonest abdominal surgical emergency with a lifetime incidence of just under 7.6%. It is most common in patients under the age of 30 years. Appendicitis is thought to occur after occlusion of the appendix orifice. The diagnosis is usually made clinically but the clinical findings vary widely with only half the patients presenting with classical clinical findings. The diagnosis is more difficult at extremes of age and where there is uncertainty, laboratory investigations, radiological investigations or diagnostic laparoscopy are of use. If the presentation is delayed, patients may present with a perforated appendix, an appendix mass or abscess. Appendicectomy may be performed via the open or laparoscopic techniques. Laparoscopic appendicectomy is increasing in popularity with increasing use of diagnostic laparoscopy and increasing surgeon skills. The most common complication following appendicectomy is wound infection with an overall incidence of about 15%.     

Laparoscopic Treatment of Cystadenocarcinoma of the Appendix Penetrating in the Sigmoid Colon

Adenocarcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract that most commonly presents as right lower abdominal pain, mimicking acute appendicitis. Presentation caused by loco-regional spread with involvement of adjacent structures is rare. An accurate and complete preoperative diagnosis has been rare in the past; however, modern imaging techniques allow recognition of most complications and associated conditions. The diagnosis is confirmed postoperatively. Aggressive surgical management is the treatment of choice in appendicular adenocarcinoma. We report the case of appendicular mucinous cystadenocarcinoma in a 55-year-old lady with penetration of the sigmoid colon treated with laparoscopic-assisted sigmoid and en block right hemicolectomy.It was possible to manage this complex case by using a laparoscopic procedure with all the known benefits of minimally invasive surgery.     

Surgical and Chemotherapy Treatment Outcomes of Goblet Cell Carcinoid: A Tertiary Cancer Center Experience

Background Goblet cell carcinoid (GCC) is a rare malignant tumor with distinct histological and clinical features. Our goals were to review the surgical and chemotherapy outcomes of patients with GCC. Methods We performed a retrospective review of the Mayo Clinic database from 1984 to 2004 with a prospective follow-up of 57 patients with GCC. Results The age at diagnosis (mean ± SE) was 55 ± 13 years. The most common presentations were right lower quadrant pain mimicking appendicitis (70%) and right lower quadrant or pelvic mass (25%). Only patients with T4 lesions had positive mesenteric nodes, with a frequency of 28%. Fifty percent of female patients had metastasis to the ovaries. The disease-specific 5-year survivals for stages I, II, III, and IV were 100%, 76%, 22%, and 14%, respectively; the overall mean survival was 47 ± 3 months. All stage I patients had simple appendectomy. The overall 5-year survival rates for patients with combined stages II to IV who underwent appendectomy versus right hemicolectomy were 43% and 34%, respectively (P = .604). The corresponding survival rates for adjuvant chemotherapy versus no chemotherapy were 32% and 27%, respectively (P = .151). Conclusions The prognosis for patients with GCC tumors correlates well with the American Joint Committee on Cancer stage at initial presentation. Appendectomy alone seems adequate for stage I disease. For staging purposes, right hemicolectomy is appropriate for T4 tumors or stage II to III disease provided that it can be performed with minimal risk. Surgical debulking is a consideration but is controversial. Adjuvant chemotherapy with 5-fluorouracil and leucovorin regimen is minimally effective against GCC.     

Mucocele of the Appendix: Case Report and Review of Literature

Appendiceal mucocele is a rare disease. Sometimes it is discovered accidentally and sometimes it resembles acute appendicitis. Correct diagnosis before surgery is very important for the selection of adequate surgical treatment to avoid intraoperative and postoperative complications. Ultrasonography, and particularly computed tomography, should be used extensively for this purpose. If mucocele is treated incorrectly pseudomyxoma peritonei, which is characterized by malignant process, may develop. We present a case of a 54-year-old man who was admitted to the emergency department with the signs of acute appendicitis. Open surgery was performed. At the time of surgery, a cystic mass of the appendix with dimensions 7 × 4 ×3 cm, with inflamed walls, but without perforation was discovered in the right iliac fossa. No discharge was found in the peritoneal cavity. Diagnosis of mucocele was suspected. Only appendectomy was performed because no pathologic process was found in the base of the appendix and lymph nodes were not increased in size. Hystopathologic diagnosis was mucinous cystadenoma. After 2 years, the patient is feeling well.     

原发性肾脏类癌2例报告并文献复习

目的：探讨原发性肾脏类癌的临床特征及诊治方法。方法：总结2例原发性肾脏类癌患者的临床资料结合文献复习讨论。例1,女,48岁。因右腰酸痛1个月入院。B超示右肾中上极中等回声占位,边界清楚,内有血流信号。CT示右肾中上极10．0cm×7．5cm实性占位,增强后病灶均匀轻度强化。例2,男,44岁。体检发现左肾占岱40天入院。B超示左肾门处中等回声占位,边界清楚,内有血流信号。CT示左肾中极肾门处3．7cm×3．5cm实性占位,增强后病灶均匀轻度强化;其内伴-钙化点。结果：2例均行根治性肾切除术。病理检查镜下肿瘤细胞形态为多边形,嗜酸性颗粒状细胞质,边界不清,呈柱状或缎带状排列混合有实性巢状排列;细胞核呈园形大小一致,核分裂像难见。免疫组化：突触素、波形蛋白均为阳性。病理诊断为肾脏类癌伴淋巴结转移。例1术后30个月死于肝骨转移;例2术后随访26个月,未见肿瘤复发转移。结论：原发性肾脏类癌临床罕见,易发生淋巴结转移,确诊依靠病理学检查,根治性肾切除应为治疗原发性肾类癌的首选方法。     

Appendiceal carcinoid tumor with lymph node metastasis in a child: case report and review of the literature

Most appendiceal carcinoids (ACs) in children present without lymph node metastasis. Lymph node metastasis is rarely present when primary tumor diameter exceeds 1 cm. We present the extraordinary case of an AC with a primary tumor diameter of 0.7 cm and infiltration of the mesentery, as well as 1 positive lymph node of the mesentery in a 14-year-old boy. Besides adding a rare case, we review the data published in the current literature on AC with lymph node metastasis in children and summarize up-to-date guidelines for diagnostic workup, therapy, and follow-up.     

Non-Functioning Malignant Neuro-Endocrine Tumour of the Pancreas: a Case Report with a Review of the Literature

Cystic abnormalities of the pancreas encompass a wide variety of lesions ranging from the non-malignant pseudo-cyst to neoplastic lesions. Although cystic neoplasms of the pancreas are rare, differentiation is important in determining the proper treatment.

A 55-year-old female presented with a cystic abdominal mass. Her computed tomography scan showed a cystic mass of 102 χ 99 χ 97 mm which was well-circumscribed and homogeneous with few thin septations and mild contrast enhancement of the fibrous wall located in the body of the pancreas. Percutaneous diagnostic aspiration of the cyst fluid was performed under ultrasonic guidance for proper diagnosis and management, which revealed a CEA greater than 200 ng/ml (0–3) and amylase within normal limits 30U/L (< 100). High CEA and normal amylase values supported the diagnosis of mucinous cystadenoma rather than pseudo-cyst. With these findings, the patient underwent distal pancrea-tectomy with splenectomy. Pathological analysis revealed a mucinous cystadenoma of the pancreas. This report is a confirmation that cyst fluid analysis can provide a pre-operative classification of these diagnostically difficult lesions.     

Clinicopathological features and the outcome of surgical management for adenocarcinoma of the appendix

AIM:To present a comprehensive analysis of incidence,clinicopathological features,appropriateness of surgical procedures,and survival for adenocarcinoma of the appendix.METHODS:A retrospective case analysis was conducted for the 10-year period 1998-2008.All patients diagnosed with adenocarcinoma of the appendix were analyzed for their demographics details,clinical features,tumor incidence and characteristics,tumor stage,surgical procedures performed,and their survival.RESULTS:Nine thousand three hundred and twentythree patients underwent appendectomies during the study period,and of these,10 (0.1%:8 men and 2 women with a mean age of 53.1 years,age range 21-83 years) were found to have primary adenocarcinoma of the appendix.Appendicular neoplasia was not suspected pre-operatively in any of the patients.Six (60%) patients underwent secondary right hemicolectomy.Four (40%) cases had appendectomy alone,and two of them died,whereas all those who underwent right hemicolectomy are alive and disease free.Five (50%) were reported to have grade 1 disease,three (30%) grade 2,and two (20%) grade 3 with mean survival of 34,48,and 22 mo,respectively.Six (60%) patients presented with advanced disease (Duke’s C and D).At the end of follow up (mean period:37.9 mo),eight patients are alive and disease free at the end of follow up.Overall mean survival was 36.3 mo (conf idence interval;16%-56%) with 41.3 and 16 mo for men and women,respectively.Mean survival for those with and without lymph node involvement was 33.6 and 40.2 mo,respectively.Right hemicolectomy gave better results than appendectomy alone,although the difference was not statistically signif icant due to the small number of cases.CONCLUSION:Adenocarcinoma of the appendix is extremely rare neoplasm with varied presentations,and is usually advanced when diagnosed.Right hemicolectomy is the treatment of choice for such tumors.     

Intraspinal Primitive Neuroectodermal Tumour: Report of Two Cases and Review of the Literature

Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.     

Implementation and usefulness of single‐access laparoscopic segmental and total colectomy

Aim Single‐access laparoscopic surgery is a recent vogue in the field of minimally invasive colorectal surgery. While selected series have indicated feasibility, we prospectively examined its usefulness for resectional surgery in routine practice. Method All patients undergoing laparoscopic colorectal resection over a 12‐month period were considered for a single‐access approach by a single surgical team in a university hospital. This utilized a ‘glove’ port via a 3–5 cm periumbilical or stomal site incision, with standard rigid laparoscopic instruments then being used. Results Of 76 planned laparoscopic colorectal resections, 35 (47%) were performed by this single‐incision laparoscopic modality without disruption of theatre list efficiency or surgical training obligations. The mean (range) age and body mass index of these 25 consecutive right‐sided resections, eight total colectomies (seven urgent operations) and two anterior resections was 58 (22–82) years and 23.9 (18.6–36.2) kg/m², respectively. The modal postoperative day of discharge was 4. For right‐sided resections, the mean (range) postoperative stay in those undergoing surgery for benign disease was 4.0 days, while for those undergoing operation for neoplasia (n = 18, mean age 71 years) it was 5.8 days and the average lymph node harvest was 13. Use of the glove port reduced trocar cost by 58% (€60/£53) by allowing the use of trocar sleeves alone without obturators. Conclusion Single‐incision laparoscopic surgery is an effective option for abdominal surgery and seems especially suited for laparoscopic‐assisted right‐sided colonic resections. The glove port technique facilitates procedural frequency and familiarity and proves economically favourable.     

Granular Cell Tumour of the Perianal Region: which Therapeutic Attitude

Granular cell tumours are uncommon soft tissue tumours that could be located anywhere throughout the body. Most of time they are benign but the diagnosis of malignancy is difficult to establish on histology: this is why the therapeutic decision is not easy to take in particular for some locations. We report the case of a 18-year old man: he presented, in the perianal region, a granular cell tumour of uncertain malignant potential (so far the first case described in the literature) for which we decided a conservative surgical treatment that failed.     

Current practice in the United Kingdom for the use of diagnostic laparoscopy in suspected acute appendicitis

Objective  Diagnostic laparoscopy is advocated in the management of patients with acute right iliac fossa pain. We asked consultant surgeons in the UK about their current use of this technique.
Method  A short anonymous questionnaire was sent to consultant surgeons from the ASGBI database. Information was sought on general surgical specialty, participation in the emergency surgical on-call rota, current practice regarding the use of diagnostic laparoscopy in patients with suspected acute appendicitis and on the management of an inflamed or noninflamed appendix. Statistical analysis was by means of χ² test.
Results  There were 161 eligible returns from 250 questionnaires (64%) and the proportion of consultants replying from each subspecialty was similar to membership numbers of subspecialty organizations. Most consultants (68%) performed diagnostic laparoscopy in patients with suspected acute appendicitis. The majority (69%) reserved its use for women of reproductive age and 14% of respondents laparoscoped all patients with suspected appendicitis. Compared to nongastrointestinal (GI), GI surgeons were significantly more likely to perform diagnostic laparoscopy (75 vs 52%, P  = 0.008). In the case of an overtly inflamed appendix, 81% of respondents would remove it laparoscopically with significantly more GI surgeons following this course than nonGI surgeons ( P  = 0.04).
Conclusion  Despite good evidence on the benefits of diagnostic laparoscopy in certain patients with suspected acute appendicitis, there is significant variation in its use. This difference appears to be based upon subspecialty and may be as a result of increasing subspecialization.     

Surgical Resection of Phyllodes Tumour: a Radical Approach as a Safeguard Against Local Recurrence

Phyllodes tumour is a rare benign neoplasm of the breast. It is a mixed tumour of epithelial and mesenchymal origin. The epithelial element is characterized by proliferation of ductolobular units. The fibrous tissue and collagen bundles represent the mesenchymal element. It is also known as “cystosarcoma” phyllodes to characterize some important features, viz. cyst-like or cleft-like spaces within the mass along with a leaf- or frond-like pattern of the stromal element. The tumour is well known for its high potential for local recurrence. Most patients in developing countries present with very large breast tumours with close proximity to the skin and pectoralis major. In these cases, there is a need to perform a three-dimensional en bloc removal of the mass with overlying skin and underlying muscle(s). If a skin flap is raised in the vicinity of the tumour, there is a risk of cutting close to the tumour, increasing risk of local recurrence. Here, we describe a surgical technique that permits a three-dimensional en bloc removal of phyllodes tumour.     

Solitary Fibrous Tumour of Thyroid: Report of Two Cases with Immunohistochemical Features and Literature Review

Solitary fibrous tumour (SFT) is a rare tumour principally found in adults in the pleural cavity. Extrapleural occurrences are rare. Two cases of SFT of the thyroid gland are described in this paper showing their distinctive microscopical architecture, namely “patternless growth pattern”. It is characterized by a bland spindle-cell proliferation alternating hyper- and hypo-cellular areas, keloid-like hyalinization and a focal hemangiopericytoma-like vascular pattern. Tumour cells revealed a diffuse strong positivity for CD34, CD99, bcl-2 and Vimentin, but negativity for Desmin, EMA, AE1/AE3, SMA, S-100 and CD31 antibodies. The differential diagnosis of thyroid SFT includes different types of spindle cell proliferation, benign and malignant mesenchymal tumours, medullary thyroid carcinoma, fasciitis-like papillary carcinoma, and undifferentiated (anaplastic) carcinoma. However, the morphologic and immunohistochemical findings of SFT are so characteristic that this diagnosis seldom represent a difficulty.     

Management and outcome of neuroendocrine tumours of the appendix—a two centre UK experience

Background

Neuroendocrine tumours (NET) of the appendix are rare histopathological neoplasms detected following operation for appendicitis in childhood. The role (if any) for radical surgery notably right hemicolectomy (RHC) has often reflected the ‘expert opinion’ of adult general surgeons with wider experience of managing NET lesions of the gastrointestinal tract. Critical decisions have focused on (a) tumour size, (b) histology, (c) tumour location/invasion and (d) positive lymph nodes. Against this background we report the clinical outcome of children with ‘incidental’ appendix carcinoid tumours managed at two regional UK paediatric surgery centres. A critical review of the literature is additionally provided in an effort to define contemporary patterns of care in paediatric surgical practice.

Methods

Hospital records and pathology database(s) identified 27 patients at two UK centres with a confirmed histological diagnosis of appendix NET lesions during January 1997–January 2013. A PUBMED and EMBASE search strategy—(English language publications only), 1975–present, was performed to gather information on all patients younger than 20 years at primary diagnosis with NET appendix tumours to review their management and outcomes.

Results

All 27 patients treated at the two institutions had acute appendicitis including 3 cases presenting with an appendix mass. Twenty-five underwent appendicectomy with two having interval operations. Tumours had a maximum diameter of 2–18 mm (median 9 mm) with 73% of lesions located at the appendix tip. Fourteen (52%) had tumour invading the mesoappendix. All patients underwent appendicectomy only with no single case having RHC or additional surgery. Surveillance studies (5HIAA, chromogranin-A) and imaging including ultrasound or CT were deployed in a minority of patients revealing no abnormality. All 27 cases are alive and well—(mean follow up 5 years; range: 9 months–16 years). The literature highlights varied management strategies and no recorded fatalities with radical surgery in children largely evolving from adult surgical practice.

Conclusions

This study confirms that paediatric patients with ‘incidental’ NET tumours of the appendix have an excellent prognosis. Consensus guidelines should ideally be developed by paediatric oncology surgeons to avoid unnecessary radical surgery in many otherwise healthy children.     

Carcinoid of the Ampulla of Vater-Report of two Cases

Carcinoid of the ampulla of Vater is extremely rare, accounting for less than 0.3% of all gastro-intestinal carcinoids. To our knowledge, only 80 cases of ampullary carcinoid have been reported in the literature to date. Ampullary carcinoid is more commonly presented with jaundice or upper abdominal discomfort and diagnosis is more often made postoperatively due to submucosal spread of the tumour. As metastatic potential cannot be predicted by tumour size, Whipple pancreatoduodenectomy rather than local excision is considered to be the treatment of choice. We report here two cases of ampullary carcinoid treated in our department.     

Surgical Reintervention After Failed Antireflux Surgery: A Systematic Review of the Literature

Background  Outcome and morbidity of redo antireflux surgery are suggested to be less satisfactory than those of primary surgery. Studies reporting on redo surgery, however, are usually much smaller than those of primary surgery. The aim of this study was to summarize the currently available literature on redo antireflux surgery. Material and Methods  A structured literature search was performed in the electronic databases of MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials. Results  A total of 81 studies met the inclusion criteria. The study design was prospective in 29, retrospective in 15, and not reported in 37 studies. In these studies, 4,584 reoperations in 4,509 patients are reported. Recurrent reflux and dysphagia were the most frequent indications; intraoperative complications occurred in 21.4% and postoperative complications in 15.6%, with an overall mortality rate of 0.9%. The conversion rate in laparoscopic surgery was 8.7%. Mean(±SEM) duration of surgery was 177.4 ± 10.3 min and mean hospital stay was 5.5 ± 0.5 days. Symptomatic outcome was successful in 81.1% and was equal in the laparoscopic and conventional approach. Objective outcome was obtained in 24 studies (29.6%) and success was reported in 78.3%, with a slightly higher success rate in case of laparoscopy than with open surgery (85.8% vs. 78.0%). Conclusion  This systematic review on redo antireflux surgery has confirmed that morbidity and mortality after redo surgery is higher than after primary surgery and symptomatic and objective outcome are less satisfactory. Data on objective results were scarce and consistency with regard to reporting outcome is necessary.