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.     

Surgical Attitude towards Carcinoid Tumour of the Appendix Review of the Literature

Carcinoid tumours of the gastrointestinal tract are most frequently located at the appendix. We report two cases: In the first case, we realized a simple appendicectomy.

In the second case, two weeks after the appendicectomy was performed, according to the histological characteristics of the tumoral specimen, the patient underwent a laparoscopic right hemicolectomy with regional lymphadenectomy. In both cases, 5-HIAA (5 HydroxyIndolAceticAcid) is assayed regularly and remains normal.     

阑尾黏液囊肿和腹膜假性黏液瘤临床疗效分析

目的探讨阑尾黏液囊肿及腹膜假性黏液瘤的诊断和治疗方法.方法#例阑尾黏液囊肿患者,行单纯阑尾切除术>例;行回盲部切除$例;行右半结肠切除术!例; !例急诊行阑尾切除术后,再次行右半结肠切除术; !例术中发现阑尾囊肿破裂合并腹膜假性黏液瘤,行右半结肠切除术,吸净腹腔内腹水,生理盐水冲洗腹腔,术后全身化疗!次.结果所有患者均手术成功,无明显手术并发症.经随访,均无复发.结论本病临床不多见,但以右下腹痛及包块为主诉的患者应警惕本病.?型超声检查及消化道造影可能是较为简便经济的诊断方法.严格掌握手术适应证,对疑有本病的患者认真探查腹腔会得到最佳的治疗效果.对腹膜假性黏液瘤患者术后化疗或放射治疗可取得较好的疗效.     

Postoperative surveillance of small appendiceal carcinoid tumors

Background

The necessity and frequency of postoperative surveillance for appendiceal carcinoid tumors ≤1 cm are undetermined.

Methods

A retrospective review was conducted of all patients with appendiceal carcinoid tumors ≤1 cm managed at an academic, tertiary referral center. Clinicopathologic characteristics, treatment, surveillance, recurrence, and survival were assessed and analyzed.

Results

Over a 16-year period, 31 patients met the inclusion criteria. Appendicitis (n = 17) and pelvic mass (n = 5) were the most common presentations. Median tumor diameter was 5 mm (range, 1–10 mm). Two patients had mesoappendiceal involvement. No patients had regional lymph node involvement or distant metastasis. Postoperatively, 14 patients (45%) received follow-up recommendations, including ≥1 of the following: imaging (n = 9), medical oncology referral (n = 7), colonoscopy (n = 5), and laboratory studies (n = 5). There were no recurrences or disease-specific deaths during a median follow-up period of 5 years (range, 0–15 years).

Conclusions

Appendiceal carcinoids ≤1 cm are unlikely to recur. Therefore, postoperative surveillance may be unnecessary.     

Small intestinal angiosarcoma masquerading as an appendiceal abscess

Angiosarcomas of the small intestine are rare and present non-specifically. They usually manifest with abdominal discomfort, altered bowel habits, anaemia and gastrointestinal bleeding. Diagnosis is often challenging and occurs at an advanced tumour stage. We describe a case of a terminal ileum angiosarcoma masquerading as an appendiceal abscess, and discuss salient clinicopathological features in diagnosing and managing this disease.     

Unusual presentation of an appendiceal malignancy

Primary malignant epithelial tumors of the appendix are uncommon. The most common presentation of appendiceal malignancy is right lower abdominal pain suggestive of acute appendicitis. Presentation caused by loco-regional spread with involvement of neighboring organs is rare. We present the case of a 48-year-old woman with an appendiceal malignancy who presented with symptoms and signs suggestive of complicated diverticular disease with an enterovaginal fistula. From a review of the literature, this is the first report of an appendiceal malignancy presenting in this manner.     

Colonoscopic diagnosis of appendiceal intussusception: case report and review of the literature.

Intussusception of the appendix is an extremely rare condition. Although approximately 200 cases of appendiceal intussusception have been reported in the literature, very few have ever been diagnosed preoperatively. We report a case of appendiceal intussusception secondary to endometriosis in an otherwise healthy female. The case was diagnosed preoperatively by colonoscopy and treated surgically at laparoscopy. We review the literature of appendiceal intussusception and discuss the associated conditions, diagnosis, and a classification scheme for this unusual finding.     

Peritoneal carcinomatosis from appendiceal cancer: A paradigm for treatment of abdomino-pelvic dissemination of gastrointestinal malignancy

Summary Background Peritoneal carcinomatosis from appendix cancer is a rare disease which has generally not been studied. 104 patients treated by a uniform treatment strategy have been followed for up to 10 years. Methods The clinical features that correlate with improved survival included low vs. high histologic grade, complete vs. incomplete cytoreduction, lymph nodes negative rather than positive for metastasis and minimal vs. extensive prior surgery. By the Cox semi-parametric model the independent variables were histologic grade and completeness of cytoreduction. From these data prognostic groups 1, 2 and 3 were formulated. Results Patients in prognostic group 1 had a complete cytoreduction and a grade 1 histopathology with a 5 year survival of 90%. Patients in prognostic group 2 had a complete cytoreduction with grade 2 or 3 histopathology and a 70% 5 year survival. Patients in group 3 had an incomplete cytoreduction, and a 5 year survival of 22%. Conclusions These data accumulated on patients with appendix cancer may have relevance to the management of patients with peritoneal carcinomatosis from other gastrointestinal cancers.      

Hepatic resection for primary liver malignancy

The results of hepatic resection for patients with primary liver malignancy seen at our clinic during the past 21 years are reported. Of 92 patients, 57 had cirrhosis in addition to hepatocellular carcinoma, and 49 (53 percent) underwent hepatic resection of various degrees from partial resection to trisegmentectomy. Resectability rates of the liver were 52 percent in 77 patients with hepatocellular carcinoma, including 19 in whom the tumor was less than 5 cm in diameter, and 60 percent in 15 patients with other malignant tumors; operative mortality rates were 15 percent in the former and 0 percent in the latter. Cumulative survival rates of all patients who underwent hepatic resection, excluding death within one month, were 55 percent at one year, 29 at 3 and 5 years. In patients with hepatocellularcarcinoma, survival rates of 15 those who had a curative resection of the tumor were 87 percent at one year and 47 percent at 3 or 5 years, there was a significant difference in survival curves between those with tumors less than 5 cm and more than 5 cm (p<0.05). On the other hand, survival rates of all patients with other malignant tumors were 78 percent at one year and 37 percent at 5 years. These results indicate the importance of performing hepatic resection for patients with small hepatocellular carcinoma associated with cirrhosis and those with other malignant tumors.     

Surgical treatment of appendiceal mucocele

HYPOTHESIS: Clinical presentation of appendiceal mucocele is related to malignancy and can influence surgical approach. DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: All cases of primary appendiceal mucoceles (simple mucocele, cystadenoma, cystadenocarcinoma) diagnosed between 1976 and 2000 were reviewed. There were 135 patients, 74 of whom were female. Mean age at diagnosis was 59 years. Mean follow-up was more than 6 years. INTERVENTIONS: A total of 129 patients underwent surgery, consisting of appendectomy (22 patients), right hemicolectomy (25 patients), or more extensive procedures (82 patients). MAIN OUTCOME MEASURES: Clinical, diagnostic, and surgical variables were statistically compared with postoperative morbidity and mortality and the presence of malignancy. P<.05 was considered significant. RESULTS: The presence of symptoms was associated with malignancy (58% vs 15%, P<.001), particularly abdominal pain (56% vs 29%, P =.005) and weight loss (77% vs 31%, P =.002). Abdominal mass was also associated with malignancy (86% vs 25%, P<.001). Moreover, pseudomyxoma peritonei and mucocele extravasation were associated with malignancy (95% vs 13%, P<.001, and 83% vs 15%, P<.001, respectively). The lesion size was not associated with malignancy; however, cystadenomas were significantly larger than simple mucoceles (8.1 cm vs 4.1 cm, P<.001), and no cystadenoma was less than 2 cm in largest diameter. CONCLUSIONS: A number of clinical, diagnostic, and intraoperative findings are associated with malignant mucoceles. All mucoceles greater than 2 cm should be excised to remove premalignant lesions.     

Surgical management of primary hyperhidrosis

Primary hyperhidrosis, although lacking a precise definition and of unknown aetiology, disrupts professional and social life and may lead to emotional problems. A variety of treatment methods are used to control or reduce the profuse sweating which involves mainly the palms, soles and axillae. The simplest method, the application of topical agents, is usually attempted first for axillary and plantar sweating. Iontophoresis may provide relief especially in patients with plantar or palmar involvement. In severe cases operative intervention is necessary. Excision of sweat glands is successful in patients with axillary hyperhidrosis but the role of suction-assisted removal of axillary sweat glands remains to be determined. Sympathectomy remains the standard by which other treatments must be judged. For upper thoracic sympathectomy a variety of surgical approaches are used with satisfactory relief of hyperhidrosis. Complications related to the surgical approach, such as Horner's syndrome, brachial plexus injuries, pneumothorax and painful scars may occur, while following sympathectomy compensatory hyperhidrosis is usual and hyperhidrosis may recur. Plantar hyperhidrosis which may be exacerbated or ameliorated by upper thoracic sympathectomy and which fails to respond to non-operative intervention is relieved by lumbar sympathectomy.     

阑尾黏液性肿瘤病理学特征及预后分析

目的探讨基于2010版WHO分类的各类阑尾黏液性肿瘤的病理学特征及预后。方法收集并复习2003年1月至2012年6月间在南京大学医学院附属鼓楼医院接受手术切除的70例阑尾黏液性肿瘤术后病理切片,按照2010年消化系统肿瘤WHO新分类,将阑尾黏液性肿瘤分为5种类型：阑尾黏液性腺瘤／囊腺瘤（MA）、低级别黏液性肿瘤（LAMN）、起源于阑尾的低级别腹膜假黏液瘤（PMP．L）、浸润性黏液腺癌（MAC）以及起源于阑尾的高级别腹膜假黏液瘤（PMP．H）。结果11例MA肿瘤上皮和黏液仅局限于黏膜肌层以内;术后无复发或死亡病例。41例LAMN黏液均可出现于黏膜肌层外阑尾壁或阑尾表面,其中39黏液湖内没有或仅见少数黏液上皮,上皮具有轻度异型性;有3例复发或进展,元死亡病例。7例PMP—L上皮具有轻度异型性,其中有4例黏液湖内上皮数量稀少或缺乏,其余3例上皮数量中等至较多：术后复发和死亡各1例。7例MAC和4例PMP—H均为浸润性肿瘤,至少局部出现高级别细胞学特征;术后复发4例,死亡3例（含2例复发后死亡）。MA和LAMN在组织学上表现为非浸润性肿瘤。而PMP-L、MAC和PMP-H则在生物学行为上表现为腺癌特征。结论阑尾黏液性肿瘤是一系列肿瘤谱系,其生物学行为取决于肿瘤性质和类型,统一、规范而准确的病理诊断对于治疗至关重要。     

Surgical management for peritoneal carcinomatosis of appendiceal origin with a high-tumor burden

Surgery Today - Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS + HIPEC) is the active treatment for peritoneal carcinomatosis of appendiceal origin. However,...     

Surgical management of primary hyperparathyroidism

We have experienced 186 patients with proven primary hyperparathyroidism operated on during past 16 years. In this series, 147 patients (79%) had adenoma, 24 patients (12.9%) had carcinoma and only 15 (8.1%) had hyperplasia. Because of a 92% of single gland involvement, the removal of an enlarged gland is adequate, if the remaining gland are grossly normal. Besides, interestingly enough, an incidence of parathyroid carcinoma is rather high in Japan, so that surgeons should be alert to recognize the parathyroid carcinoma on the basis of clinical and operative findings. In an effort to minimize recurrence, en bloc resection of the parathyroid carcinoma is important. When a patient with parathyroid carcinoma has a local recurrence or distant metastases, an aggressive surgical approach is recommended to alleviate hypercalcemic symptoms. The majority of patients with hyperplasia belong to multiple endocrine neoplasia, type 1. Recent development of the non-invasive diagnostic methods is remarkable. The last consecutive 31 patients underwent preoperative localization study by 201Thallium (201TI-CI) scanning and ultrasonography. 201TI-CI scanning gave the most accurate results and 100% correct localization was obtained when the parathyroid tumor was more than 1 g. However, it is noteworthy that the parathyroid tumor was less than 1 g in 64% of patients who had no evidence of generalized fibrous osteitis.     

The evolving management of the appendix mass in the era of laparoscopy and interventional radiology

Aim

An appendix mass is the result of a walled-off perforation of the appendix which localises, resulting in a mass and it is encountered in up to 7% of patients presenting with acute appendicitis. However, its management is controversial due to the lack of high level evidence. This review article sets out a rationale diagnostic and therapeutic strategy for the appendix mass based upon up-to-date available evidence.