Gastrointestinal carcinoid tumors

Fifty-five patients were evaluated and treated for gastrointestinal (GI) carcinoid tumors at our institution from 1968 through 1988. Thirty patients had primary tumors smaller than 1 cm and were treated surgically or endoscopically by local excision, whereas 25 underwent resection with regional lymphadenectomy for larger primaries. At the time of initial treatment 17 patients demonstrated metastasis. Although 13 of these patients had primary tumors larger than 2 cm, in four patients with ileal carcinoids, the primary measured 0.8 to 1.5 cm. Serosal involvement was present in nine of 17 patients with metastasis. With a follow-up range of 3 months to 20 years, six patients have died with metastatic carcinoid tumor, two patients died without evidence of disease, 12 patients are alive with metastasis, and 35 patients are alive, free of disease. In this series three determinants for risk of metastasis in GI carcinoid tumors were evident: primary size, serosal penetration, and primary site in the small intestine. In patients with any of these risk factors, resection with regional lymphadenectomy is recommended.     

Gastrointestinal carcinoid tumors: diagnosis and treatment

Carcinoid tumors are malignancies originating from neuroendocrine cells, most commonly encountered in the gastrointestinal tract, in a rate of 64%, with the majority affecting the ileum. Certain genetic syndromes sometimes include carcinoid tumors in their phenotype. These malignancies may be rather asymptomatic, although occasionally they behave aggressively. They are typically diagnosed due to the effects of a locally advanced disease, or when producing symptoms because of hormone secretion (carcinoid syndrome). A better understanding of their molecular biology and behavior is demanded so that these tumors to be diagnosed precociously and managed effectively.     

Giant bronchial carcinoid tumors: a multidisciplinary approach

BACKGROUND: Bronchial carcinoid tumors account for approximately 2% of all lung tumors. Although they were considered benign lesions, they are now categorized malignant, occasionally with poor prognosis. The clinical symptoms can be highly variable and are often present for many years before diagnosis. Whereas some carcinoids are entirely asymptomatic, others are accompanied by carcinoid or paraneoplastic syndromes. METHODS: We describe the multidisciplinary management of a 34-year-old female patient with a massive actively secreting bronchial carcinoid tumor of the right lung. Furthermore, we provide a review of the literature regarding the operative treatment and the perioperative management of pulmonary carcinoid tumors with respect to surgical, anesthetic, radiologic, and pathologic considerations. RESULTS: In the reported case, the first symptoms were chronic watery diarrhea, skin flushing, progressive shortness of breath, and increasing right shoulder pain. When the patient initially presented at our institution, the tumor had already reached an enormous size and it involved the right and left atrium as well as the atrial septum. Using an evidence-based, multidisciplinary approach the patient was treated successfully with extended surgical resection. CONCLUSIONS: Carcinoid tumors are potentially curable even if they reach a significant size and thus an aggressive strategy is warranted. The management of such cases requires careful investigation, planning, and treatment with collaborative expertise provided by a multidisciplinary team. We demonstrated that this approach can lead to a favorable outcome in what first appeared to be a formidable and unresectable tumor.     

Gastrointestinal carcinoid tumors: 10-year experience of a general surgical department

Gastrointestinal (GI) carcinoids are rare neuroendocrine tumors of great interest because of the multitude of the peptide hormones they produce, their clinical manifestations, the carcinoid syndrome they cause, their biologic behavior, and the methods of their surgical and adjuvant treatment. In this retrospective study, 25 patients with carcinoid tumors admitted and surgically treated in our department during the last 10 years are presented. Most of the cases were located in the ileum (n = 8) and appendix (n = 12). The remaining cases were located in the rectum (n = 2), stomach (n = 1), duodenum (n = 1), and ileocecal valve (n = 1). After tumor resection and postoperative treatment, mainly with somatostatin analogs, our patients had a good recovery, and only five deaths were reported during follow-up. Carcinoids, despite the fact that they are usually silent and diagnosed with advanced stage, have good prognosis and offer, after effective treatment, long survival.     

胃肠道类癌

目的 探讨胃肠道类癌的诊断和治疗。方法 回顾性分析１０４例胃肠道类癌的资料。结果 ５４．８％的类癌浸及肌层,１６．３％的类癌有区域淋巴转移;３,５,１０年生存率分别为８１．７％,６６．７％,４４．４％。结论 胃肠道类癌临床表现不典型。肿瘤直径〈２ｃｍ者可行局部广泛切除术;〉２ｃｍ者应行根治术,结肠及胃部类癌多灶及混合性类癌切除范围应相对扩大,而阑尾及空回肠类癌手术范围应相对缩小。     

Ampullary carcinoid tumors: Rationale for an aggressive surgical approach

Two cases of ampullary carcinoid tumor are reported. These tumors are among the most rare of GI tract carcinoids and appear to have a distinct presentation and biological behavior from carcinoids arising in the duodenum. The existing literature is reviewed with attention to the implications for surgical management of this rare disease.     

Hypovascular renal tumors: an aggressive diagnostic approach required

The value of a fine needle aspiration biopsy for confirmation of malignancy is demonstrated in 3 cases of angiographically hypovascular renal tumors. In 2 cases ultrasound examination suggested solid lesions but in 1 case it was predominantly sonolucent. Reliable documentation in the literature indicates that the risk of spreading tumor cells and, hence, of decreasing the survival rate is negligible.     

Pulmonary carcinoid tumors

Carcinoid tumors of the lung are an uncommon group of neoplasms of neuroendocrine origin. Pulmonary carcinoid tumors are typically benign and slow growing. However, more aggressive subtypes may develop early nodal and distant metastases. Although several histologic classification strategies have been proposed to distinguish benign from more aggressive subtypes, the lack of uniformity in terminology has resulted in increased ambiguity and confusion. Because these tumors are generally resistant to chemotherapy, complete surgical resection is the primary form of therapy. Long-term survival for patients with typical carcinoid is excellent but is decreased in those with the atypical subtype. Complete tumor resection with preservation of uninvolved pulmonary parenchyma remains the fundamental goal in the surgical treatment of this unusual clinical entity.     

Bronchial carcinoid tumors

Forty-six patients with bronchial carcinoid tumors were operated on over a 37-year period. The results were reviewed with special reference to presenting complaint, histological diagnosis, location of the tumor, lymphatic involvement, and type of surgical resection. Age at operation ranged from 9 to 86 years (mean, 43.6 years). Presenting symptoms were hemoptysis in 21 instances, chronic cough in 17, and pneumonia in 15. The primary tumor was within the main bronchus in 17 patients. Twenty-one patients required pneumonectomy, and 20 had lobectomy or bilobectomy . Nine of the patients under-going pneumonectomy had severely damaged lung tissue distal to the lesion in the main bronchus. Six patients had metastases to hilar nodes. Four patients died of carcinoid tumor, but none with metastases died of carcinoid tumor. This series confirms the low malignancy potential of bronchial carcinoid tumors, even in the presence of lymphatic involvement. Although conservative resection is an attractive surgical option, only 10 of the 46 (22%) were potential candidates for such intervention. Standard surgical resection resulted in "cure" in 90% of the patients in the series.     

Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach

To identify specific markers for the diagnosis of choroid plexus tumors, gene expression profiles of choroid plexus epithelial cells (n = 8) and ependymal cells (n = 6) microdissected from human autopsy brains as well as choroid plexus papilloma tissue were investigated using DNA microarrays. Protein expression of genes overexpressed in choroid plexus was evaluated in normal choroid plexus, choroid plexus papilloma, choroid plexus carcinoma, other primary brain tumors, and cerebral metastases. Forty-six genes found to be overexpressed in normal choroid plexus epithelial cells were also present in choroid plexus papilloma. Among those, 11 were further analyzed by immunohistochemistry. Expression of inward rectifier potassium channel Kir7.1 was confirmed in normal choroid plexus (34 of 35), choroid plexus papilloma (12 of 18), and choroid plexus carcinoma (5 of 5) but was not found in 100 other primary brain tumors and cerebral metastases. Similarly, stanniocalcin-1 stained normal choroid plexus (32 of 35), choroid plexus papilloma (16 of 18), and choroid plexus carcinoma (3 of 5), whereas staining was seen in only 2 of 100 other primary brain tumors and cerebral metastases. Transthyretin stained choroid plexus (33 of 35), choroid plexus papilloma (14 of 18), and plexus carcinoma (2 of 5), but its specificity was significantly lower. Antibodies directed against coagulation factor V, glutathione peroxidase 3, pigment epithelium derived factor, serotonin receptor 5-HTR2C, lumican, fibulin-1, plastin-1, and cytokeratin 18 revealed varying degrees of specificity and sensitivity. Our data suggest that antibodies directed against Kir7.1 and stanniocalcin-1 might serve as sensitive and specific diagnostic markers for choroid plexus tumors.     

Impotence: a diagnostic approach

A diagnostic approach to erectile impotence using a sexual function questionnaire, nocturnal penile plethysmography, penile blood pressure measurement, cavernosograms, cystometrograms and plasma testosterone levels is presented. Case examples demonstrate the clinical value of these diagnostic tools. Psychogenic impotence should be diagnosed only after nocturnal penile plethysmography has demonstrated the presence of complete erections.     

Primary mediastinal carcinoid tumors

A study of 15 cases of carcinoid tumor of the thymus, diagnosed and treated at the Mayo Clinic, revealed histopathologic features of the neoplasm that might cause it to be confused with other mediastinal tumors, both primary and secondary. The tumor was associated with ectopic ACTH production in six patients, of whom five had Cushing's syndrome. Metastasis, which occurred in 11 patients (73%), was delayed for as long as 8 years after initial diagnosis. Eleven patients underwent surgical resection of their primary thymic neoplasms, and nine of these received postoperative radiotherapy or chemotherapy or both. One patient was treated with chemotherapy alone, and three had no treatment for their neoplasms, which were discovered at autopsy. Eight patients are still alive. Of these, five have developed metastases, and only one is free of metastatic disease more than 5 years after diagnosis. In the two remaining cases, the discovery of the thymic tumor was recent and follow-up is not yet meaningful. Overall, four of the seven patients who died had proven metastatic disease. The mean survival after the appearance of extrathymic tumor in this group was 3 years. Of the five patients who are still alive with metastasis, all have survived at least 2 years since that spread of their disease.     

Small bowel carcinoid tumors

During the last 7 years we have managed 48 cases of carcinoid tumor. Of these 15 have been tumors of the small bowel. In this article we describe and discuss the presentation of small bowel carcinoid tumors, the carcinoid syndrome, and its treatment. The content reflects our clinical experience.

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Resumen Los tumores del yeyuno y del ileon constituyen hasta un 5% de todos los tumores del tracto gastrointestinal. Los tumores malignos más frecuentes del intestino delgado son los adenocarcinomas y los tumores carcinoides, con una ligera preponderancia de los adenocarcinomas. El ileon es el lugar de más frecuente localización de los carcinoides del intestino delgado, con una incidencia global que sólo la sobrepasa el apéndice. Cerca de 10% de los carcinoides de intestino delgado ocurren en el yeyuno. En los Últimos siete años hemos manejado 48 casos de tumores carcinoides. De estos, 15 han sido tumores del intestino delgado.Los tumores carcinoides poseen una apariencia histológica similar a la de otros tumores neuroendocrinos. Contienen y secretan 5-hidroxitriptamina; excesivas cantidades de ácido-5-hidroxi indolacético aparecen en la orina. Sinembargo, no todos los tumores producen 5-hidroxitriptamina y algunos secretan dopamina y noradrenalina. Tales hallazgos son consistentes con el concepto de que las células de un tumor carcinoide se derivan de la serie APUD. Los componentes principales del síndrome carcinoide son flushing (rubor), diarrea, lesiones valvulares cardiacas y asma.El primer objetivo del tratamiento quirÚrgico es corregir la obstrucción, lo cual implica la resección del tumor y de las asas intestinales afectadas por la masa tumoral. En algunos casos es necesario realizar hemicolectomía derecha. Cuando el tumor se extiende por fuera del intestino, la resección debe incluir tanto tumor como sea posible, incluyendo ganglios mesentéricos y paraaórticos y depósitos secundarios en el hígado. En aquellos casos en que haya mÚltiples depósitos, la resección parcial de hígado puede ser beneficiosa. La ligadura de la arteria hepática ha sido utilizada en casos de depósitos mÚltiples, pero este procedimiento ha sido reemplazado por la embolización; muchos patientes pueden sobrevivir por periodos largos de tiempo con metástasis hepáticas. Se ha utilizado la quimioterapia citotóxica para enfermedad difundida, así como la irradiación para el manejo de dolor por metástasis óseas.

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Résumé Au cours des 7 dernières années les auteurs ont observé 48 cas de tumeurs carcinoÏdes dont 15 siégeaient au niveau de l'intestin grÊle. Dans cet article ils décrivent les différents aspects revÊtus par ces tumeurs, le syndrome carcinoÏde et leur traitement adéquat. Le pronostic de ces lésions est très variable et donc difficile à prévoir pour chaque malade. Leur croissance et leur dissémination est souvent lente, de ce fait les malades soumis à un traitement médical et chirurgical convenables peuvent survivre de 15 à 20 ans au-delà des premières manifestations de la maladie. La qualité de vie peut Être améliorée soit par l'exérèse chirurgicale du plus grand nombre possible de métastases hépatiques, soit par embolisation hepatique.

     

Bronchial typical carcinoid tumors

The current WHO classification of lung tumors recognizes bronchial typical carcinoid as low-grade neuroendocrine tumors. These tumors grow slowly but can metastasize to regional nodes (4 to 20%) and more rarely to extrathoracic sites. Symptoms are usually related to local compression and obstruction of the bronchial tree. Paraneoplastic syndrome can be present (carcinoid syndrome, Cushing's syndrome, acromegaly). Preoperative diagnosis is usually obtained with bronchoscopic biopsy. Computed tomography and somatostatin receptor scintigraphy are useful in the preoperative staging. Only selected cases can be treated endoscopically with laser resection. The complete surgical resection remains the only therapy with curative intent in the majority of patients. Parenchyma-sparing resections are indicated whenever possible. Overall survival after surgery is excellent (5-year rate, 87 to 100%) with low recurrence rate (2 to 11%). N-status and type of resection seem not to affect prognosis. Local relapse can be treated successfully with surgery, whereas distant metastases have a poor prognosis even after chemotherapy.     

Bronchial carcinoid tumors: a retrospective analysis of 126 patients.

From 1970 until 1990, 8,958 cases of primary carcinoma of the lung were diagnosed at the Duke University Medical Center. During the same period, 126 patients (mean age, 53 +/- 13 years) were diagnosed with bronchial carcinoid. The overall survival was 78% for 5 years and 71% for 10 years. Surgical treatment in 106 patients included pneumonectomy (15), lobectomy (63 with 9 bronchoplastic procedures), stapled wedge resection (22), and bronchoscopic laser resection (6). The method of diagnosis was chest roentgenography (121), chest computed tomography (77), mediastinal tomography (31), bronchoscopy (81), bronchoscopic brushing and washing (50), bronchoscopic biopsy (40), transthoracic needle biopsy (27), thoracotomy (100), and autopsy (5). Univariate analysis of the medical history, presenting signs and symptoms, diagnostic test results, and pathologic data predicted improved survival (p less than 0.001) for: female sex (n = 58), asymptomatic presentation (n = 47), normal serum serotonin or urinary hydroxyindoleacetic acid levels (n = 76), peripheral location of the primary tumor (n = 50), pathologic stage I or II (n = 91), negative lymph nodes (n = 80), primary tumor 2 cm or less in diameter (n = 67), and typical histology (n = 80). No significance (p greater than 0.1) was observed for age, smoking history, race, family history of carcinoid, environmental exposure, or hemoptysis. The most important factors affecting survival defined by multivariate analysis were (p less than 0.01) pathologic stage, atypical histology, and asymptomatic presentation. Bronchial carcinoid tumors are unique, making up 1% to 2% of primary lung neoplasms and having an excellent prognosis after resection with a 95% 5-year and 93% 10-year survival for pathologic stage I disease.     

Pulmonary carcinoid tumors: report on 19 cases

Nineteen cases of pulmonary carcinoid tumors, three of which showed clinically malignant behavior, are reported. The diagnostic and therapeutic possibilities are discussed. Diagnosis is sometimes possible on radiologic examination, when the clinical behavior is not yet malignant. However, these tumors are frequently mistaken for malignant heteroplasias, during the diagnostic work-up so that the postoperative histologic examination results are sometimes surprising. The clinical postoperative course is favorable in the majority of cases, and in the eight cases, we were able to follow up for more than five years, there was only one recurrence. Although pulmonary carcinoids are histologically similar to intestinal carcinoids, and like the latter are abundantly vascularized, they are only rarely accompanied by the carcinoid syndrome, which is often observed in the intestinal form.