A twenty-five-year follow-up of ninety-three resected typical carcinoid tumors of the lung.

From 1965 to 1990, 93 patients (57 women and 36 men) with typical bronchopulmonary carcinoids were operated upon. Patient ages ranged from 17 to 78 years, the mean age being 45.5 years. Central carcinoids were symptomatic in 80% of the patients. A correct preoperative diagnosis was made in 54 of 64 (84%) patients. Peripheral carcinoids were usually asymptomatic and a correct diagnosis was established in 4 of 29 patients (14%). The prognosis in the group of patients with bronchopulmonary carcinoids treated surgically was excellent. Seven patients died from nonrelated causes. The 5-, 10-, and 15-year survival rates for the remaining 86 patients are 100%. Only one patient died as a result of the tumor after 17 years and another patient is known to have had distant metastasis 9 years after resection. There was no hospital mortality. In the last decade a lung parenchyma-preserving attitude was adopted. Whenever possible, bronchoplastic surgery was applied for central carcinoids and resection of one segment or less was used for peripheral carcinoids. This approach was possible in 30 of 50 patients (60%). Nine patients were treated with preoperative endobronchial neodymium:yttrium-aluminum-garnet laser resection to facilitate a lung-preserving surgical resection. The prognosis of patients for whom a lung-preserving approach was adopted was as good as that of those with conventional resections. Involvement of regional lymph nodes (nine patients, 9.7%) or positive resection margins (two patients, 2.1%) had no influence on prognosis. We conclude that lung-preserving resections are often facilitated by preoperative neodymium:yttrium-aluminum-garnet laser treatment in central obstructing carcinoids.     

Synchronous tumors consisted of bronchial carcinoid and adenocarcinoma of the lung

We report a case of synchronous tumors consisted of bronchial carcinoid and adenocarcinoma of the lung. A 58-year-old female was referred to our hospital after screening, because an abnormal shadow was noted in the right lung on her computed tomography (CT) of the chest. CT scans showed a peripheral pulmonary mass in the right middle lobe and a nodule around the right lower lobe bronchus. The nodular lesion like swollen lymph node was diagnosed as bronchial carcinoid originated in B6 by bronchoscopy. The pulmonary mass was diagnosed as adenocarcinoma by using core needle biopsy during operation. Right middle and lower bilobectomy and mediastinal lymph node dissection were performed. Coincidence of a bronchial carcinoid and an adenocarcinoma of the same side of the lung is a rare occurrence.     

Spindle-cell carcinoid tumors of the lung: a clinicopathologic study of 35 cases

Thirty-five cases of spindle-cell carcinoid tumors of the lung were studied. Fifteen patients were male and 20 female, and they ranged in age from 33 to 78 years, with a mean of 57.6 years. Eleven neoplasms were located in the left lung and 23 in the right lung; a disproportionately large number of neoplasms were present in the right middle lobe. The tumors were most commonly encountered as an incidental finding on chest roentgenogram. None of the patients had unequivocal evidence of the carcinoid or any other endocrine syndrome. All but two of the lesions were located n the periphery of the lung and most were subpleural. They ranged in size from 0.7 to 4 cm, with 82% of the neoplasms having a maximum dimension of 2 cm or less. Microscopically, the neoplasms were composed predominantly or entirely of spindle cells which were fairly uniform in length in any single lesion, but showed moderate variation from case to case. The shorter spindle cells were often arranged in an "organoid" pattern, while the neoplasms composed of larger spindle cells were more "mesenchymal" in appearance. Silver stains were performed on 12 cases and all were argyrophil-positive. Cytoplasmic dense-core granules were identified in all four neoplasms examined ultrastructurally. Twelve cases were treated by wedge resection or segmental resection, and 18 cases by lobectomy. Hilar lymph nodes were removed from seven patients, and in two there were microscopic lymph node metastases. One other patient had a single microscopic bony metastasis. Follow-up information was available for 22 patients and ranged from 1 to 13 years with a mean of 5.4 years. None of the patients have had metastases other than those described above and none of the patients had died of their disease.     

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.     

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.     

Bronchial carcinoid tumors in children and adolescents.

Thirteen histologically proven cases of bronchial carcinoid from the literature and eight cases from the Armed Forces Tumor Registry, all under 20 yr of age, are presented. Hemoptysis, cough, and pneumonia were the most frequent presenting complaints. Bronchoscopy defined the lesion in all 16 cases so studied. Gross and microscopic pathology is briefly described, and treatment in these cases outlined. A good long-term prognosis can be expected, except in those cases with a long delay in diagnosis.     

Oncocytic carcinoid of the lung

A 49-year-old male was hospitalized after chest roentgenogram revealed a coin lesion in the right of middle lung field. Right upper lobectomy with lymphadenectomy was performed. The diagnosis on pathological examination was of carcinoid tumor. However, electron-microscopic examination revealed hyperplasia of numerous mitochondria and neurosecretory granules in the cytoplasm of the tumor cell, thus the diagnosis of oncocytic carcinoid was made. The patient has been well and experienced neither recurrences nor relapse 2 years after the surgery.     

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.     

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.

     

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.     

Treatment of rectal carcinoid tumors

We investigated the treatment of 24 rectal carcinoid tumors from both the clinicopathologic and prognostic viewpoints. All tumors less than 2 cm in diameter had neither muscle layer invasion nor lymph node metastasis, except for an atypical carcinoid tumor that had both lymphatic permeation and intramural metastasis. One typical carcinoid tumor larger than 2 cm had both several lymphatic permeations and urinary bladder invasion. All cases had a good prognosis with no recurrence and no new metastases. For rectal carcinoid tumors less than 2 cm in diameter, local resection is sufficient, whereas radical operation is required for tumors larger than 2 cm in diameter. For atypical rectal carcinoid tumors, radical operation should be considered even if the diameter is less than 2 cm.     

Bronchoplastic procedures in the treatment of carcinoid tumors of the tracheobronchial tree.

Sixteen patients, aged 10 to 70 years, had carcinoid tumors of the lower respiratory tract treated by various resective tracheobronchoplastic procedures. These represent 8.8 percent of 181 patients with carcinoid lesions treated during a recent 20 year period. All 16 patients had respiratory symptoms, and one patient also had the carcinoid syndrome. Roentgenographic changes ranged from a mass or atelectasis (or both) through unilateral lung hyperinflation to clear lungs with subtle filling defects in major airways. All tumors were visualized endoscopically, and 13 patients had biopsies. Histopathologically, all tumors were "typical" carcinoids . Before operation, the patients had minimal or no respiratory insufficiency, although flow-volume and ventilation-perfusion abnormalities were noted when major airways were affected. Surgical management at thoracotomy was as follows: (1) simple wedge tracheobronchotomy without lung resection (five patients); (2) bronchial sleeve resection without lung resection (three patients); and (3) bronchial sleeve with upper lobe resection (eight patients). These 16 operations were performed with eight technical anatomic variations. No early or late deaths occurred. One patient had early transient atelectasis, and three patients required late endoscopic removal of suture granulation tissue. All patients were alive without recurrence of tumor or carcinoid syndrome or other respiratory complications 6 months to 19 years postoperatively. Pulmonary resection should be avoided unless there is histologic evidence of tumor extension into lung parenchyma or irreversible pulmonary suppuration distal to the obstructive tumor.     

Extra-appendicular carcinoid tumors of the gastrointestinal tract

During the years 1958-1978, seventeen patients were treated for extra-appendicular carcinoid tumors of the gastrointestinal tract in Tel-Hashomer and Belinson hospitals. Most of the tumors were located in the colon (eight cases) and in the rectum (four cases). A direct correlation between the tumor size and invasiveness has been noted, with distant metastases in eight of 12 patients with tumors more than 2 cm in size. Gastrointestinal bleeding was the most frequent clinical symptom, whereas the "carcinoid syndrome" appeared only in three patients. Curative surgery was performed in six patients, palliative procedure in two cases, explorative laparotomy was performed in four cases, and in two patients diagnosis was made in postmortem examination. Eight of the 17 patients (47%) had distant metastases at the time of the diagnosis, and six of the 15 operated patients (40%) died during the first year after the operation. The malignant nature of these tumors, together with the high incidence (23%) of associated malignant neoplasms is stressed. Radical resection is advocated in the cases where the tumor's size is more than 2 cm or in the presence of muscular invasion.