Rectal carcinoids: a systematic review

Background

Rectal carcinoids are increasing in incidence worldwide. Frequently thought of as a relatively benign condition, there are limited data regarding optimal treatment strategies for both localized and more advanced disease. The aim of this study was to summarize published experiences with rectal carcinoids and to present the most current data.

Methods

Following PRISMA guidelines, an electronic literature search performed of PubMed, Medline, Embase, and the Cochrane Library using the terms “rectum” or “rectal” AND “carcinoid” over a 20-year study period from January 1993 to May 2013. Non-English-language studies, animal studies, and studies of fewer than 100 patients were excluded. Study end points included demographic information, tumor features, intervention and outcomes. All included articles were quality assessed.

Results

Using the search parameters and exclusions as outlined above, a total of 14 articles were identified for detailed analysis. The quality of articles was low/moderate for all included scoring 9 to 17 of 27. The articles included 4,575 patients diagnosed with a rectal carcinoid. Approximately 80 % of tumors were <10 mm, 15 % 11–20 mm, and 5 % >20 mm. Eight percent of patients presented with regional lymph node metastases, and 4 % presented with distant metastases. Tumor size >10 mm, and muscular and lymphovascular invasion are independently associated with an increased risk of metastases. The 5-year survival was 93 % in patients presenting with localized disease and 86 % overall.

Conclusions

Small tumors up to 10 mm without any adverse features can be treated with endoscopic or local excision. The treatment of carcinoids between 10 and 20 mm is still contentious, but those up to 16 mm without adverse feature are suitable for local/endoscopic excision followed by careful histopathological assessment. Those >20 mm or with adverse features require radical surgery with mesorectal clearance in suitable patients.     

Bronchial carcinoids: a review of 60 patients

Sixty patients with a bronchial carcinoid underwent surgical treatment. Preoperative fiberoptic bronchoscopy revealed a characteristic pink, smooth, bleeding tumor in 71.4% of the patients with a typical carcinoid and 16.7% of those with an atypical carcinoid (p less than 0.05). Eight pneumonectomies, seven bilobectomies, 34 lobectomies, three lobectomies with bronchoplasty, six bronchotomies with bronchoplasty, and two segmental resections were performed. All patients entered follow-up, and 47 were followed for more than 5 years. Ten-year survival was 89.6% for patients with a typical carcinoid and 60% for those with an atypical carcinoid. Ten-year survival was 88.1% for patients with carcinoids without lymph node involvement. All patients with lymph node involvement died within 5 years. Overall, 5 of the 8 patients having pneumonectomy died of acute cardiorespiratory failure. We conclude that a limited surgical resection with or without bronchoplasty and systematic lymphadenectomy is the procedure of choice in patients with typical carcinoids. On the other hand, atypical carcinoids are comparable to well-differentiated malignancies of the lung. Whenever possible, pneumonectomy should be avoided in favor of bronchial sleeve resection.     

Primary liver leiomyoma: a review of this unusual tumour

Primary liver leiomyoma (PLL) should be considered in the differential diagnosis of liver lesions. A literature review has been completed and two cases are reported. The first is a 45‐year‐old white woman complaining of vague abdominal pain. She was initially evaluated with abdominal ultrasonography (US) that revealed a heterogeneous liver mass measuring 18 cm in greatest diameter. The tumour demonstrated hypointensity on T1‐weighted and hyperintensity on T2‐weighted magnetic resonance imaging. The second case is a 45‐year‐old Asian male who had undergone kidney transplantation 16 years ago for IgA glomerulonephritis and who developed mild, self‐limiting epigastric pain. US showed a 4.3‐cm‐diameter lesion that was predominantly hypoechoic and was either compressing or arising from segment 2 of the liver. Computed tomography showed a well‐circumscribed 4‐cm‐diameter mass that appeared to be arising from segments 2/3 of the liver and was adjacent to the anterior gastric wall. He underwent an uneventful laparoscopic left lateral sectionectomy and discharged on post‐operative day 3. Pathological examination of the resection specimen confirmed the lesion as a PLL in each case. Herein, we report two cases of PLL and review the literature regarding this uncommon disorder.     

Primary carcinoma of the liver: Report of a case and review of the literature

• 1.1. Primary carcinoma of the liver is comparatively rare.
• 2.2. There is very little written on this subject in the surgical literature.
• 3.3. An exhaustive review of the literature on the subject was made, and 1,125 cases were found to have been reported.
• 4.4. The pathology and the clinical course of the disease are discussed.
• 5.5. A plea for more surgical interference in primary carcinoma of the liver is made, through the medium of men in the past who are more qualified to speak on the subject.
• 6.6. A table is included containing a summary of fourteen cases that have been operated on for primary carcinoma of the liver.
• 7.7. An additional case of primary carcinoma of the liver complicated by miliary tuberculosis is reported.
• 8.8. References are included, comprising practically everything that has been written on the subject.
     

Primary neuroendocrine carcinoma of the liver: difficult diagnosis of a rare neoplasm

Primary neuroendocrine neoplasms of the liver are extremely rare: about 30 cases only have been described in the literature. We report the case of a 42-year-old woman with a ten-year evolution. According to the previously reported cases, primary neuroendocrine carcinoma of the liver is usually multicentric, often mimicking liver metastases. The demonstration of the hepatic origin of a neuroendocrine carcinoma is often arduous. A careful surgical exploration and a prolonged follow-up are mandatory. The treatment of choice is surgical resection when possible. For progressive and unresectable disease, hepatic arterial chemoembolization may be considered. However, the prognosis of liver neuroendocrine tumours is much more favorable than that of hepatocellular carcinoma and progression has to be demonstrated before instauration of potentially harmful therapies.     

Primary sarcoma of the inferior vena cava: review of diagnosis, treatment, and outcomes in a case series

Leiomyosarcoma of the inferior vena cava is a rare mesenchymal tumor. The diagnostic approach, based on general guidelines of oncologic surgery, seems to be relatively routine; specific aspects of treatment, including vascular reconstruction, depend on tumor stage, grade, and location. In this report, the management of this disease in 5 patients is summarized and the literature is reviewed. A thorough diagnostic assessment includes sonography, computed tomography, angiography or duplex ultrasonography, perioperative pathohistologic examination, and appropriate differential diagnosis. Radical resection is associated with the best outcome and long-term survival. In this series, 4 of 5 patients underwent tumor resection. In 2 patients, the disease was classified as R0. Another patient had R1 status found at resection and underwent postoperative radiation after the tumor bed was marked intraoperatively. She has remained stable since treatment. One patient died of pulmonary metastases 32 months after primary R1 tumor resection. The 5th patient has been stable since diagnosis; resection was not possible because of severe accompanying diseases and because consent for surgical intervention could not be obtained from the patient. There is reasonable hope that leiomyosarcoma of the inferior vena cava can be treated successfully, even in advanced stages, with novel antineoplastic drugs and radiotherapeutic protocols. However, general treatment recommendations have not yet been compiled.     

Primary aortoenteric fistulae: the challenges in diagnosis and review of treatment

Primary aortoenteric fistula (PAEF) is a potentially fatal condition which poses a considerable diagnostic challenge because of its infrequency and the nonspecific presentation. We report the case of a 61-year old woman who presented to her general practitioner with nonspecific and intermittent rectal bleeding, hematemesis, and weight loss. Four days later, she presented to the emergency department with worsening symptoms. The investigations revealed a new small 3.8-cm abdominal aortic aneurysm on computed axial tomography (CT), however, no evidence of a fistula was observed either on CT scan or endoscopy. Two days later, she became unstable requiring an emergency laparotomy and was taken to the operation theater for an en bloc resection of a segment of the aorta and duodenum with exclusion of the duodenum with an inline reconstruction of the aorta using a Dacron graft for a PAEF. The published data were reviewed to address the issues of optimal diagnostic methods and management of PAEF.     

Primary palmar hyperhidrosis presenting with unilateral symptoms: a report of two cases and review of the literature

Two cases of primary palmar hyperhidrosis are presented. T₂-T₃ sympathetic ganglionectomy of the affected side completely alleviated perspiration of the palms, but oversweating of the contralateral palms appeared a few weeks later. A similar sympathetic ganglionectomy of the second side, 1 month and 1 year later, resulted in renewed oversweating of the palm on the first operated side within 3 months of the second operation. During the same period, 127 other patients with primary palmar hyperhidrosis underwent a bilateral upper dorsal sympathectomy, though the condition did not recur in any of these patients. The possible mechanism(s) of why overperspiration of the second hand developed after the first sympathectomy in these two patients, and why it recurred in the first hand after the second operation are examined, but remain obscure.     

The diagnosis and treatment of bronchial carcinoids]

The results of diagnosis and treatment of bronchial carcinoids in 37 patients are discussed. Establishment of the diagnosis is difficult because the clinico-radiological pictures of these tumours are similar to those of unspecific diseases of the lungs as well as tuberculosis. Twenty-six patients received antituberculotic treatment for a long period of time (2 to 5.5 years). Bronchoscopy with biopsy revealed the true cause of the disease in all of the 37 patients, in 9 of them coexistence of bronchial carcinoid and tuberculosis of the lung was found. Eight operations for pulmonectomy, 4 for bilobectomy, and 25 for lobectomy were performed. The volume of the surgical intervention was determined by the location of the bronchial carcinoids and extent of the secondary changes in the pulmonary tissue. Retrospective analysis showed that in timely diagnosis of bronchial carcinoids treatment of some of the patients could be limited to reconstructive-plastic operations with preservation of the whole lung or part of it.     

Primary carcinosarcoma of the liver: clinicopathologic features of 5 cases and a review of the literature

Carcinosarcoma of the liver is very rare worldwide. The terminology and pathogenesis of hepatic carcinosarcoma remain controversial issues. In this article, we studied the clinicopathologic features of 5 cases of hepatic carcinosarcomas (matching the World Health Organization definition), analyzed the clinical data, histologic and immunohistochemical (IHC) results, and discussed the terminology, pathologic differential diagnoses, pathogenesis, and prognosis. The patients were 40 to 68 years old, and included 4 males and 1 female. All patients were Hepatitis B surface antigen positive with para-tumorous cirrhosis. The largest dimensions of the neoplasms ranged from 6.0 to 14.0 cm. Satellite nodules, portal vein tumor thrombi, direct invasion into local tissues (right diaphragm, right adrenal gland, and gastric wall) as well as metastatic foci in lungs and abdominal lymph nodes were identified. Pathologically, the neoplasms consisted of carcinomatous and sarcomatous components. The carcinomatous components were exclusively conventional hepatocellular carcinomas in all 5 cases, whereas the sarcomatous components exhibited complex features. Confirmed by IHC studies, the sarcomatous elements in different cases included rhabdomyosarcomas, malignant fibrous histiocytomas, fibrosarcoma, and poorly differentiated spindle cells without distinctive differentiation. Furthermore, the sarcomatous elements in these 5 neoplasms stained negative for all the epithelial markers we applied for IHC staining, which support the pathologic diagnosis of carcinosarcoma rather than sarcomatoid carcinoma. The presence of transitional zones between carcinomatous and sarcomatous components may support the transformation theory. Four patients with palliative hepatectomy died within 6 months, whereas 1 patient is still alive 21 months after radical resection. The poor prognosis of hepatic carcinosarcoma may be due to their highly invasive and metastatic features. Radical resection of early stage hepatic carcinosarcoma may contribute to a relatively optimistic prognosis.     

Talus fractures: a current concepts review of diagnoses, treatments, and outcomes

The talus is the key articular segment linking the leg and foot, and as such, is subject to complex loads and may occasionally fracture. Fracture patterns provide clues to the underlying pathomechanics and energy of the injury, both of which can help guide treatment and suggest prognosis. Talus fractures have a wide variety of presentation from low-energy avulsion fractures of the lateral or posterior processes, to high-energy comminuted talar body fractures. Appropriate, expedient treatment provides the patient the best chance of obtaining a good functional outcome. Treatment relies on appropriate diagnosis, which hinges on clinical suspicion provided by the patient's account of pathomechanics, clinical examination, and radiological workup. This current concepts review discusses the pathomechanics, presentation, workup, treatment, and prognosis of fractures of the talar head, neck, body, lateral process, posterior process, and talar extrusions. Key words: talus, fracture, talar neck, talar head, talar body, lateral process, posterior process, talar extrusion, orthopaedic surgery, review.     

Primary hyperparathyroidism--experiences with concomitant diseases, symptoms, preoperative diagnosis and surgical procedure over a period of 14 years

In a 14-years period (1970-1984) eight-three patients were operated on for primary hyperparathyroidism (pHPT) at the University Hospital of Erlangen. Special attention was paid to associated diseases, symptoms, preoperative diagnostic parameters were serum calcium, parathyroid hormone in venous blood, cyclic AMP in 24 h-urine. In 15.7% only borderline increased serum calcium values were measured. Ulcer incidence in pHPT was 19% as compared with approx. 7% in the general population. There was no increased incidence of pancreatitis in pHPT. The possibility of an association of pHPT with the Multiple Endocrine Neoplasia (MEN) syndrome was emphasized and measures of early diagnosis proposed.     

直肠类癌的诊治现状

直肠类癌属潜在恶性肿瘤,长期以来,一直认为直肠类癌罕见,近年来发现其发病率有增高趋势,临床并不少见,可能与结肠镜技术的进步与人们认识水平的提高有关.由于直肠类癌具有恶变倾向,临床表现无特异性,因此必须重视对它的早期诊断、治疗与随访.直肠类癌的诊断有较高的误诊率,良恶性的判断有时较难把握.目前外科手术是唯一有效的治疗方法,对于手术方式的选择,国内外尚无统一标准,因此总结直肠类癌的诊断及治疗近况具有重要的意义.     

Primary leiomyosarcoma of the breast: diagnosis, management and outcome. A report of a new case and review of literature

Primary leiomyosarcoma of the breast is very rare and represents a diagnostic challenge. Only 16 cases have been reported in the English language literature. We report another new case and have analysed reports of the previous cases aiming to present a simple evidence-based approach to the clinical, radiological and pathological diagnosis of this rare tumour. The average age of presentation is 56 years. All neoplasms have been limited to the breast at the time of diagnosis. The usual presentation is a slowly growing mass, but in the current case report the patient present with mastalgia. There is always a possibility of local recurrence or distant spread, which can occur many years after primary surgery. Leiomyosarcoma must be histologically distinguished from leiomyoma and the presence of >3 mitoses per 10 high-power fields is usually indicative of malignancy. Immunohistochemistry is helpful to confirm diagnosis. As this is a slow-growing malignant tumour with a propensity for local recurrence, total mastectomy is the treatment of choice. The tumour is not hormone-dependent and hormone manipulation is not a treatment option.     

Primary bone tumors of the femur presenting with spinal symptoms: a report of two cases and review of the literature

Two case reports of femoral bone lesions simulating lumbar spinal disease are presented. Physical examination and case history were strongly suggestive of lumbar spinal pathology. In case 1, surgical resection of a venous hemangioma in the lumbar epidural space was performed but did not relieve pain. In case 2, conservative treatments for a protruded disk were performed for 3 months before an accurate diagnosis was made. After correct diagnoses were made, excision of the femoral tumors brought rapid relief of all abnormal findings in both cases. Compared with other causes of sciatica, femoral bone tumors are rare. However, careful attention should be paid to rule out these lesions if the diagnosis of a lumbar spinal disease is uncertain. Bone scintigraphy seems to be a sensitive diagnostic method to detect extraspinal osseous lesions.     

Descending perineum syndrome: a review of the presentation,diagnosis, and management

Introduction and hypothesis

Defecatory dysfunction is a relatively common and challenging problem among women and one that practicing pelvic reconstructive surgeons and gynecologists deal with frequently. A subset of defecatory dysfunction includes obstructed defecation, which can have multiple causes, one of which is descending perineum syndrome (DPS).

Methods

A literature search was performed to identify the pathophysiology, diagnosis, and management of DPS.

Results

Although DPS has been described in the literature for many decades, it is still uncommonly diagnosed and difficult to manage. A high index of suspicion combined with physical examination consistent with excess perineal descent, patient symptom assessment, and imaging in the form of defecography are required for the diagnosis to be accurately made. Primary management options of DPS include conservative measures consisting of bowel regimens and biofeedback. Although various surgical approaches have been described in limited case series, no compelling evidence can be demonstrated at this point to support surgical intervention.

Conclusions

Knowledge of DPS is essential for the practicing pelvic reconstructive surgeon to make a timely diagnosis, avoid harmful treatments, and initiate therapy early on.