Atypical Carcinoid Tumor Research Articles

Hypercalcemia in Atypical Bronchial Carcinoid Tumors

Atypical carcinoid tumors of the bronchial tree are uncommon. Their tendency to metastasize is well recognized, characteristically producing osteoblastic bone deposits without disturbance of calcium homeostasis. We report two patients who presented with hypercalcemia and osteolytic bone metastases following surgical removal of atypical bronchial carcinoid tumors. In one of the patients, chemotherapy induced remission and controlled the hypercalcemia.

Review of Neuroendocrine Carcinomas of the Larynx

Neuroendocrine carcinomas of the larynx are uncommon tumors of considerable scientific interest and clinical importance. They include typical carcinoid tumors, atypical carcinoid tumors, and small cell neuroendocrine carcinomas. This paper considers these neoplasms from a personal experience and reviews the relevant medical literature. About 200 cases of neuroendocrine carcinomas of the larynx have been reported. The diagnosis is based on light microscopy and is confirmed by ultrastructural evidence of neurosecretory granules. Histochemical and immunocytochemical investigations may support it. Paraneoplastic syndromes associated with laryngeal neuroendocrine carcinomas have been reported occasionally. The histogenesis, treatment, and prognosis of these lesions also are discussed.

Atypical Carcinoid Tumor of the Lung: A Case Report and Discussion of the Literature

Carcinoid tumors comprise 1% to 2% of all tumors of the lung. Although carcinoid tumors are generally felt to be of low grade malignancy, an atypical variant exists that exhibits more virulent behavior and a less favorable prognosis. The treatment of carcinoid tumors is complete surgical removal and a more aggressive approach is required in the treatment of atypical carcinoids. A case is presented with a review of the literature.

Immunoreactivity of various peptides in typical and atypical bronchopulmonary carcinoid tumours.

The presence of a number of regulatory peptides (bombesin, gastrin, glucagon, somatostatin, calcitonin and ACTH) was compared in 30 typical carcinoid tumours and 27 well differentiated neuroendocrine carcinomas (atypical carcinoids) using conventional immunocytochemistry. Strong immunostaining for one or more peptide was observed in 97% of the typical carcinoids (29/30) whereas only 67% of the neuroendocrine carcinomas showed immunoreactivity. The peptide most frequently detected in typical carcinoids was bombesin (67%), while gastrin was more common in neuroendocrine carcinomas (44%). Immunoreactivity for more than one peptide was present in 33 tumours and in three cases, six different peptides were detected. The study shows that immunoreactivity to various peptides is more common in typical carcinoids than well differentiated neuroendocrine carcinomas. The significance of these findings is discussed.

Pulmonary Atypical Carcinoid Tumor with Marked Alphafetoprotein Production and Features of an Adenocarcinoma Differentiation

A 67-year-old man presented with a pulmonary atypical carcinoid tumor with marked elevation of the serum alpha-fetoprotein (AFP) level to 181,000 ng/ml and no hepatic metastases. Immunohistochemistry revealed AFP-positive fine granules, sparsely distributed in some cells. The proportion of the concanavalin A nonbinding subfraction was 33.7%. Light microscopy revealed hyaline globules within or outside the clear and reticular cytoplasm of a few cells. These were ultrastructurally electron-dense materials similar to the hyaline bodies observed in yolk sac tumors. The Grimelius silver method stained only a few cells and very few cells showed a positive Masson-Fontana reaction. Electron microscopy revealed secretory granules measuring 220 nm on the average in scattered cells. Immunohistochemical studies showed 5-hydroxytryptophan in many cells and 5-hydroxytriptamine or serotonin in only a few cells. As for polypeptide hormones, gastrin was detected and in autopsy specimens carcinoembryonic antigen (CEA) immunoreactive cells were observed. Past case reports on the coexistence of carcinoid tumors and adenocarcinomas in the digestive tract suggest that the tumor cells in our case are also derived from primitive or stem cells of endodermal origin and expressed unusual differentiation in the course of treatment.

Small Cell Undifferentiated Carcinoma of the Urinary Bladder

The clinicopathologic, immunocytochemical, and ultrastructural features of 12 small cell undifferentiated carcinomas (SCUCs) of the urinary bladder are herein reported. The patients ranged in age from 50 to 82 years (median, 76 years). Ten patients were male and two were female. Gross hematuria was the most frequent complaint (67%). Five patients died of disease (median survival, 4 months), three patients are still living with unresectable disease after 6, 10, and 12 months, and four patients were only recently diagnosed. Grossly, most of the tumors were large polypoid masses. On light-microscopic examination, they resembled the intermediate and oat cell variants of pulmonary SCUC. Eight SCUC were associated with other forms of in situ or invasive carcinoma, including transitional cell carcinoma (seven cases), adenocarcinoma (three cases), squamous cell carcinoma (three cases), spindle cell carcinoma (one case), and atypical carcinoid tumor (one case). Immunocytochemically, 11 of the 12 tumors expressed one or more epithelial cell markers (epithelial membrane antigen, human milk fat globule protein-2, cytokeratin). Eleven of the 12 SCUCs were also positive for one or more neuroendocrine markers (neuron specific enolase, chromogranin, Leu-7, vasoactive intestinal polypeptide, serotonin). Electron microscopy performed on seven tumors demonstrated dense-core granules (150-250 nm) in all cases, with tonofilaments in four cases, dendrite-like processes in two cases, and intercellular lumina in one case. The clinicopathologic features of 18 previously reported SCUC of the urinary bladder are also reviewed. SCUC arising in this location is an aggressive neoplasm that often demonstrates multidirectional differentiation, including the frequent but not invariable expression of neuroendocrine features.

Atypical carcinoid tumor of the small bowel complicating celiac disease

An atypical carcinoid tumor of the small bowel in a patient with celiac disease is described. Although an increased incidence of malignancy has been observed in celiac disease, this seems to be the first report of a carcinoid tumor of the small intestine associated with this condition. Although the significance of this association is unclear, the implications with regard to gastrointestinal endocrine cell proliferation and tumor histogenesis are discussed.

Ovarian metastases from cancer of the lung: Problems in interpretation—A report of seven cases

Seven women, 26 to 66 (average 42) years of age, from whom ovarian tumors were removed before (three cases), synchronously with (three cases), or less than 1 year after (one case) the discovery of a pulmonary neoplasm are the subjects of this report. The ovarian tumor was unilateral in six cases and bilateral in one case and was unassociated with intraabdominal spread. The lung tumors, which were similar to the ovarian tumors on microscopic examination, were central in six cases and peripheral in one; three of them were small cell undifferentiated carcinomas, two large cell undifferentiated carcinomas, one a poorly differentiated adenocarcinoma, and one an atypical spindle cell carcinoid tumor. In each case the question of the primary site of the tumor had been raised clinically. The histological similarity of the ovarian and pulmonary tumors in all the cases to well-recognized forms of pulmonary neoplasia as well as additional clinical and pathological features of the cases led to an interpretation of a pulmonary origin of the tumors in all the cases.

Changing Times in Surgical Management of Bronchopulmonary Carcinoid Tumor

The bronchopulmonary carcinoid tumor occurs at all levels from trachea to lung periphery. It should be managed by conservatism in airway or lung resection. The long-term survival is excellent: 82% at 10 years in this series of 111 resected patients. The atypical carcinoid tumor (10% of this series) has a more ominous prognosis and requires special surgical attention. Six conceptual changes in the evolution of management of the tumor are presented (the “changing times”).

A reappraisal of limited-stage undifferentiated carcinoma of the lung: Does Stage I small cell undifferentiated carcinoma exist?

Patients with small cell undifferentiated carcinoma of the lung (SCUC) have a poor prognosis. Surgical excision is avoided if the diagnosis can be made with small biopsy specimens or cytologic preparations. We reviewed 323 consecutive patients with pulmonary neoplasms diagnosed as SCUC, oat cel carcinoma, and undifferentiated or poorly differentiated carcinoma. At the time of diagnosis, only 18 patients had neoplasms classified as clinical Stage I, and only one of these had SCUC after histologic review. Fifteen patients had atypical carcinoid, a tumor with features intermediate between ordinary bronchial carcinoid and SCUC. In two instances, there was insufficient tissue for definitive diagnosis. Cumulative survival of the 15 patients with Stage I atypical carcinoid tumor was 80% at 1 year and 60% at most recent follow-up (mean follow-up 20 months). Mean survival for the 305 remaining patients was 7.9 months. Atypical carcinoid may be misdiagnosed as SCUC or poorly differentiated carcinoma, particularly with limited tissue samples or cytologic preparations. Stage I SCUC exists but is exceedingly rare. Many examples of purported Stage I SCUC probably represent atypical carcinoid. Because atypical carcinoid has a far better prognosis than SCUC, precise diagnosis is important and surgical resection should be considered.

Atypical carcinoid tumor of the lung. A clinicopathologic study of 17 cases.

We describe 17 atypical carcinoid tumors of the lung that are histologically intermediate between small-cell undifferentiated carcinoma and typical carcinoid tumor. Atypical carcinoid tumors have a distinctive microscopic pattern of nests, trabeculae, and ribbons of intermediate-sized, moderately pleomorphic cells that lack the nuclear molding and dense hyperchromasia of small-cell undifferentiated carcinoma. Mucin was present in 15 cases and nine contained argyrophilic granules. The three primary tumors and one metastasis studied ultrastructurally contained dense-core granules and variable degrees of squamous and glandular differentiation. Eleven patients were men; six were women. Their mean age was 58 years. Sixteen patients were known smokers. Twelve tumors involved the upper lobes and three were located in the right middle lobe. Mean tumor size was 4.9 cm (median 4; range 2.5--20). Four of 13 patients having potentially curative resection died of tumor. The other nine patients were disease free with a mean follow-up of 20 months. Four additional patients treated palliatively died.

Primary carcinoid and carcinoma of the rectum occurring simultaneously: report of a case.

A case of atypical carcinoid tumor of the rectum associated with, and colliding with, adenocarcinoma is described. To our knowledge, no such collision tumor has been reported previously.

Atypical carcinoid tumors of the lung

Atypical carcinoid tumors of the lung

AN ATYPICAL CARCINOID TUMOUR SECRETING 5-HYDROXYTRYPTOPHAN

AN ATYPICAL CARCINOID TUMOUR SECRETING 5-HYDROXYTRYPTOPHAN