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• W2076012369 abstract "Thymic carcinoid (TC) is a rare entity: since the first description by Rosai and Higa1Rosai J. Higa E. Mediastinal endocrine neoplasm, of probable thymic origin, related to carcinoid tumor.Cancer. 1972; 29: 1061-1074Crossref PubMed Scopus (280) Google Scholar in 1972, approximately 150 cases have been reported in the literature. These tumors are frequently associated with endocrinopathies (Cushing syndrome or multiple endocrine neoplasia type 1; MEN-1) and carry a poor prognosis.2Teh B.T. Zedenius J. Kytola S. et al.Thymic carcinoids in multiple endocrine neoplasia type 1.Ann Surg. 1998; 228: 99-105Crossref PubMed Scopus (146) Google Scholar, 3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar TCs present an aggressive biological behavior with a tendency to invade adjacent structures (mediastinal fatty tissue, lung, pericardium, and great vessels) and with a tendency for local recurrences or distant hematogenous metastases. Surgery, when feasible, represents the treatment of choice3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar, 4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar, 5Sakuragi T. Rikitake K. Nastuaki M. Itoh T. Complete resection of recurrent thymic carcinoid using cardiopulmonary bypass.Eur J Cardiothorac Surg. 2002; 21: 152-154Crossref PubMed Scopus (10) Google Scholar even if, in some cases, radical resection is not achieved. The role of neoadjuvant or adjuvant therapy has not yet been assessed. We describe the successful multidisciplinary management of a giant TC treated with neoadjuvant radiotherapy/chemotherapy, radical surgical resection, and preoperative, intraoperative, and postoperative therapy with a somatostatin analog. In addition, we provide a review of the recent literature about the management of these tumors. A 39-year-old man underwent a bioptic left anterior mediastinotomy for a large mediastinal mass, discovered after he presented with cough and dyspnea. Histology revealed grade 2 neuroendocrine carcinoma (atypical carcinoid) of the thymus. Neoplastic tissue strongly expressed somatostatin receptors (sst2), and chromogranin immunostaining was positive. Total body computed tomographic (CT) scan showed involvement of the anterior mediastinum, of the thoracic inlet, and of the left lung (Figure 1). No distant metastases were seen. Indium 111 diethylenetriamine pentaacetic acid pentetreotide scintigraphy (Octreoscan; Mallincrodt Medical BV, Zaltbommel, The Netherlands) confirmed an elective uptake only in correspondence of the tumor (Figure 2). Figure 2Octreoscan shows an elective uptake only in correspondence with the thoracic lesion: no other pathologic images are evident.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Serum chromogranin A and neural specific enolase (NSE) levels were 143.3 ng/mL (reference range, 20-100 ng/mL) and 83 ng/mL (reference value, < 12.5 ng/mL), respectively. The patient's clinical status was good, and no associate diseases were evident. Neoadjuvant chemotherapy/radiotherapy was started—4 courses of cisplatin (100 mg/m2 on day 1) and etoposide (100 mg/m2 on days 1-3 and 5) repeated every 3 weeks—followed by irradiation (42 Gy in 2-Gy daily fractions) with a 10-MeV linear accelerator. Because of the presence of sst2 in the biopsy specimen, Octreotide (Sandostatina Novartis Pharma, Milan, Italy) was administered at 1500 mg/d subcutaneously for 15 days, shifting to the long-acting form (20 mg every 28 days; Sandostatina LAR Novartis Pharma, Milan, Italy). Radiologic and radionuclide restaging (CT scan, magnetic resonance imaging, and Octreoscan) showed a moderate reduction of the mediastinal lesion (Figure 3), without evidence of distant metastases. Blood chromogranin A and NSE levels were 109 and 20.7 ng/mL, respectively. Surgery was thus planned. To prevent the occurrence of refractory hypotension caused by the release of vasoactive peptides during surgical manipulation of the tumor, 100 mg of Octreotide was administered subcutaneously before the operation and continuously at 50 mg/h intravenously during surgery. Femoral vessels were prepared in case cardiopulmonary bypass was needed. A left lateral cervicotomy (medial to sternocleidomastoid muscle) and a median sternotomy were performed. The tumor was easily dissected free from the right hemithorax, but the involvement of the thoracic inlet necessitated the resection of the left brachiocephalic vein and some centimeters of the subclavian and internal jugular veins. A large tract of the involved pericardium, from the superior vena cava to the left hilum, was resected, but no heart infiltration was observed (Figure 4). The left main pulmonary artery was involved by the tumor, and left pneumonectomy was necessary. A transverse left sternotomy prolonged in an anterior thoracotomy in the fourth intercostal space was performed, and en bloc resection of the mediastinum along with the left lung was performed (Figure 5). Sampling mediastinal lymphadenectomy was performed. A Prolene mesh (Ethicon, Inc, Somerville, NJ) was used to close the large pericardial defect.Figure 5Surgical specimen of the radically resected thymic carcinoid.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The procedure and the postoperative course were uneventful. The patient was extubated 18 hours after the operation, transferred to the ward on postoperative day 3, and discharged from the hospital in good clinical condition on postoperative day 14. Definitive histology showed a T2 N2 grade 2 neuroendocrine carcinoma (atypical carcinoid) of the thymus with mediastinal lymph nodal metastases and invasion of the lung. The surgical margins were tumor free. Because of the radicality of the intervention, oncologists decided that adjuvant chemotherapy or radiotherapy was not indicated and put the patient under hormonal therapy with long-acting Octreotide (20 mg). Follow-up was scheduled with blood marker tests (chromogranin A and NSE) every 3 months and total body CT scan and Octreoscan every 6 months. At the 18-month follow-up, the patient was in good clinical condition and had returned to his previous job (brick layer). Total body CT scan and Octreoscan showed an absence of local recurrence or distant metastases. Serum chromogranin A and NSE levels were 33 ng/mL (reference range, 20-100 ng/mL) and 9.2 ng/mL (reference value, <12.5 ng/mL), respectively. Medical therapy was well tolerated, and no important side effects were reported. TC is a distinct clinicopathologic entity that has been confused with thymoma since Rosai and Higa1Rosai J. Higa E. Mediastinal endocrine neoplasm, of probable thymic origin, related to carcinoid tumor.Cancer. 1972; 29: 1061-1074Crossref PubMed Scopus (280) Google Scholar first described this tumor as a separate entity in 1972. TC represents only 4% of all anterior mediastinal tumors6Moran C.A. Suster S. Spindle-cell neuroendocrine carcinoma of the thymus a clinicopathologic and immunohistochemical study of seven cases.Mod Pathol. 1999; 12: 587-591PubMed Google Scholar and is a rare entity: no more than 150 cases have been described in the literature. TCs are 3 times more frequent in men than in women4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar, 7Economopoulos G.C. Lewis J.W. Lee M.W. Silverman N.A. Carcinoid tumors of the thymus.Ann Thorac Surg. 1990; 50: 58-61Abstract Full Text PDF PubMed Scopus (87) Google Scholar; tumors have been described in patients from 8 years8Lin K.L. Chen C.Y. Hsu H.H. Ectopic ACTH syndrome due to thymic carcinoid tumor in a girl.J Pediatr Endocrinol Metab. 1999; 12: 573-575Crossref PubMed Scopus (18) Google Scholar to 87 years old,9Rosado de Christenson M.L. Abbott G.F. Kirejczyk W.M. Thoracic carcinoids radiologic-pathologic correlation.Radiographics. 1999; 19: 707-711Crossref PubMed Scopus (113) Google Scholar but more frequently they occur between the fourth and the sixth decades of life. Most TCs present an aggressive biological behavior from the time of presentation. Symptoms are usually strictly related to the neoplastic mass effects: chest pain, cough, and dyspnea are frequently observed. Superior vena cava syndrome is described in approximately 20% of cases.3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar A clinically apparent endocrinopathy develops in approximately half of patients: Cushing syndrome is the most frequent of these. de Perrot and associates10de Perrot M. Spiliopoulos A. Fisher S. Totsch M. Keshavjiee S. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome.Ann Thorac Surg. 2002; 73: 675-681Abstract Full Text Full Text PDF PubMed Scopus (58) Google Scholar reported that approximately 40 TCs associated with Cushing syndrome had been described in the literature since 1972 and that there was no difference in sex distribution in cases of TC and Cushing syndrome. TC can occur in association with other endocrine neoplasms (eg, MEN-1), as Rosai and Higa11Rosai J. Higa E. Davie J. Mediastinal endocrine neoplasm in patients with multiple endocrine adenomatosis. A previously unrecognised association.Cancer. 1972; 29: 1075-1082Crossref PubMed Scopus (128) Google Scholar first described in 1972. Since then, the association between TC and MEN-1 has been recognized in several cases: Teh and associates2Teh B.T. Zedenius J. Kytola S. et al.Thymic carcinoids in multiple endocrine neoplasia type 1.Ann Surg. 1998; 228: 99-105Crossref PubMed Scopus (146) Google Scholar concluded that approximately 20% of patients with TC presented with MEN-1. Patients with TC and MEN-1 are predominantly men and tend to be younger than those without any endocrine disease. The aggressive biological behavior is in contrast with that of generally benign carcinoids that arise elsewhere in the body, in particular, bronchial carcinoid (BC). In our recently published series,12Filosso P.L. Rena O. Donati G. Casadio C. Ruffini E. Papalia E. et al.Bronchial carcinoid tumors surgical management and long-term outcome.J Thorac Cardiovasc Surg. 2002; 123: 303-309Abstract Full Text Full Text PDF PubMed Scopus (181) Google Scholar we observed lymph node metastases in only 6 (8%) of 75 typical BCs and in 14 (36%) of 38 atypical ones. The 10- and 15-year survival was 93% and 84% for typical BC and 52% and 52% for atypical BC, respectively. TCs often present as locally invasive at the time of diagnosis7Economopoulos G.C. Lewis J.W. Lee M.W. Silverman N.A. Carcinoid tumors of the thymus.Ann Thorac Surg. 1990; 50: 58-61Abstract Full Text PDF PubMed Scopus (87) Google Scholar, 13Valli M. Fabris G.A. Dewar A. et al.Atypical carcinoid tumour of the thymus a study of eight cases.Histopathology. 1994; 24: 371-375Crossref PubMed Scopus (52) Google Scholar; Wang and colleagues14Wang D.Y. Chang D.B. Kuo S.H. et al.Carcinoid tumours of the thymus.Thorax. 1994; 49: 357-360Crossref PubMed Scopus (76) Google Scholar described locally invasive TC in 7 of 8 patients. In the series of Fukai and colleagues,4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar 12 of 15 TCs had invasive features. Metastatic spread occurs by the hematogenous and lymphatic route, as with thymic carcinomas. Mediastinal lymph node metastases are frequently observed at presentation: de Montpréville and associates3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar and Fukai and colleagues4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar reported metastatic TC in 7 of 14 and 9 of 15 patients, respectively. Distant metastasis sites include lung, bone, adrenal glands, liver, and spleen, in order of frequency. Distant metastases are observed in approximately 20% of patients with TC with a protracted clinical course.3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar To simplify the nomenclature and make it more uniform, TCs have been reclassified according to specific criteria (mitotic activity, presence of necrosis, and cytologic atypia) as grade 1 neuroendocrine carcinoma (previous typical carcinoid), grade 2 neuroendocrine carcinoma (previous atypical carcinoid), and grade 3 neuroendocrine carcinoma (previously large-cell neuroendocrine carcinoma and small-cell carcinoma).15Klemm K.M. Moran C.A. Suster S. Pigmented thymic carcinoids a clinicopathological and immunohistological study of two cases.Mod Pathol. 1999; 12: 946-948PubMed Google Scholar, 16Wick M.R. Neuroendocrine neoplasia current concepts.Am J Clin Pathol. 2000; 113: 331-335Crossref PubMed Google Scholar Histologic criteria of this classification are shown in Table 1. In Table 2, a proposed new TNM classification is shown.4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google ScholarTABLE 1Histologic criteria for neuroendocrine tumors of the thymus classification17Gal A.A. Kornstein M.J. Cohen C. Duarte I.G. Miller J.I. Mansour K.A. Neuroendocrine tumors of the thymus a clinicopathologic study.Ann Thorac Surg. 2001; 72: 1179-1182Abstract Full Text Full Text PDF PubMed Scopus (67) Google ScholarVariableTypical carcinoidAtypical carcinoidSmall-cell carcinomaMitoses per 100 high-power fields<10>10AnyNecrosisAbsentPresentPresentPleomorphismMinimalModerateModerateNuclear moldingNoneMinimalProminentCrush artifactNoneMinimalProminent Open table in a new tab TABLE 2TNM classification of thymic neuroendocrine tumors4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google ScholarT1Macroscopically completely encapsulated and microscopically no evidence of capsular invasionT2Macroscopic adhesion or invasion into surrounding fatty tissue or mediastinal pleura; microscopic invasion into the capsulaT3Invasion into neighboring organs (pericardium, lung, and great vessels)T4Pleural or pericardial disseminationN0No lymph node metastasisN1Metastasis to anterior mediastinal lymph nodesN2Metastasis to intrathoracic lymph nodesN3Metastasis to extrathoracic lymph nodesM0No distant metastasisM1Distant metastasis (hematogenous) Open table in a new tab Most TCs are atypical carcinoids (or grade 2 neuroendocrine carcinoma) because of an increased mitotic count, nuclear pleomorphism, and the presence of necrosis. Immunohistochemical staining is usually strongly positive with chromogranin A and synaptophysin; this is a useful tool for a correct diagnosis of neuroendocrine carcinoma. Adrenocorticotrophic hormone staining is usually (but not necessarily) positive for TC associated with Cushing syndrome. Incomplete resections are frequent. In this case, the local recurrence rate is high and survival is poor. Incomplete resections and local recurrences are the factors that influence the outcome in TC.3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar, 17Gal A.A. Kornstein M.J. Cohen C. Duarte I.G. Miller J.I. Mansour K.A. Neuroendocrine tumors of the thymus a clinicopathologic study.Ann Thorac Surg. 2001; 72: 1179-1182Abstract Full Text Full Text PDF PubMed Scopus (67) Google Scholar de Montpréville and associates,3de Montpréville V.T. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid) a clinicopathologic study of fourteen cases.J Thorac Cardiovasc Surg. 1996; 111: 134-141Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar on the basis of follow-up of 13 patients, observed that the median survival for complete resections (n = 4), incomplete resections (n = 5), and biopsy alone (n = 4) was 71, 30, and 5 months, respectively. Surgery, when feasible, is the treatment of choice. An aggressive approach often includes en-bloc resection of the tumor with the pericardium, lung, great vessels, or all of these.18Spaggiari L. Pastorino U. Double transmanubrial approach and sternotomy for resection of a giant thymic carcinoid tumor.Ann Thorac Surg. 2001; 72: 629-631Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar Median sternotomy is often indicated as a surgical approach, but occasionally, in case of large and invasive TC, the addition of an anterior (or posterolateral) thoracotomy should be considered to obtain better exposure of the involved hemithorax. Sampling lymphadenectomy is mandatory for staging and oncologic planning. Economopoulos and colleagues7Economopoulos G.C. Lewis J.W. Lee M.W. Silverman N.A. Carcinoid tumors of the thymus.Ann Thorac Surg. 1990; 50: 58-61Abstract Full Text PDF PubMed Scopus (87) Google Scholar and Fukai and associates4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar concluded that long-term survival in TC can be achieved only by resection of both the primary tumor and the subsequent recurrences and metastases. Sakuragi and colleagues5Sakuragi T. Rikitake K. Nastuaki M. Itoh T. Complete resection of recurrent thymic carcinoid using cardiopulmonary bypass.Eur J Cardiothorac Surg. 2002; 21: 152-154Crossref PubMed Scopus (10) Google Scholar reported a successful radical resection of a recurrent TC in which cardiopulmonary bypass was used to control the massive bleeding caused by dense adhesions between the sternum and the great vessels due to 3 previous interventions (2 sternotomies and 1 lateral thoracotomy). The role of neoadjuvant/adjuvant therapy (chemotherapy or radiotherapy alone or in combination) has not been adequately assessed because of the low number of cases. Fukai and associates4Fukai I. Masaoka A. Fujii Y. Yamakawa Y. Yokoyama T. Murase T. et al.Thymic neuroendocrine tumor (thymic carcinoid) a clinicopathologic study in 15 patients.Ann Thorac Surg. 1999; 67: 208-211Abstract Full Text Full Text PDF PubMed Scopus (105) Google Scholar and de Perrot and colleagues10de Perrot M. Spiliopoulos A. Fisher S. Totsch M. Keshavjiee S. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome.Ann Thorac Surg. 2002; 73: 675-681Abstract Full Text Full Text PDF PubMed Scopus (58) Google Scholar reported the utility of postoperative radiotherapy to prevent local recurrences of invasive TC; 45 to 60 Gy were administered. Sakuragi and colleagues5Sakuragi T. Rikitake K. Nastuaki M. Itoh T. Complete resection of recurrent thymic carcinoid using cardiopulmonary bypass.Eur J Cardiothorac Surg. 2002; 21: 152-154Crossref PubMed Scopus (10) Google Scholar reported that 1 patient who underwent 4 interventions received 39 Gy of adjuvant radiotherapy after the third operation. Spaggiari and Pastorino18Spaggiari L. Pastorino U. Double transmanubrial approach and sternotomy for resection of a giant thymic carcinoid tumor.Ann Thorac Surg. 2001; 72: 629-631Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar reported use of adjuvant radiotherapy in a patient they operated on for an invasive atypical TC; this patient was free of disease 10 months after the intervention. There are no standard regimens of chemotherapy, and only occasional experiences have been reported in the literature. Spaggiari and Pastorino18Spaggiari L. Pastorino U. Double transmanubrial approach and sternotomy for resection of a giant thymic carcinoid tumor.Ann Thorac Surg. 2001; 72: 629-631Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar reported that their patient received 3 cycles of neoadjuvant chemotherapy with ifosfamide and etoposide, but no signs of response were observed. Single agents or combination drug therapies with 5-fluorouracil, streptozocin, etoposide, or cisplatin have been proposed by Wang and colleagues14Wang D.Y. Chang D.B. Kuo S.H. et al.Carcinoid tumours of the thymus.Thorax. 1994; 49: 357-360Crossref PubMed Scopus (76) Google Scholar and Takayama and colleagues19Takayama T. Kameya T. Inagaki K. et al.MEN type 1 associated with mediastinal carcinoid producing parathyroid hormone, calcitonin and chorionic gonadotropin.Pathol Res Pract. 1993; 189: 1090-1094Crossref PubMed Scopus (11) Google Scholar: these had no significant effect on survival or recurrence rates. Gal and colleagues17Gal A.A. Kornstein M.J. Cohen C. Duarte I.G. Miller J.I. Mansour K.A. Neuroendocrine tumors of the thymus a clinicopathologic study.Ann Thorac Surg. 2001; 72: 1179-1182Abstract Full Text Full Text PDF PubMed Scopus (67) Google Scholar concluded that neither chemotherapy nor radiotherapy leads to differences in survival. Our patient was a moderate responder to neoadjuvant chemotherapy/radiotherapy. This was enough to consider him for surgery and to start hormonal therapy with Octreotide. To our knowledge, this is the first case of successful multidisciplinary treatment of a TC with neoadjuvant chemoradiotherapy, radical resection, and hormonal therapy. Neuroendocrine tumors, including thymic tumors, express sst2 at immunohistochemistry. In vivo sst2 expression is provided by indium 111 diethylenetriamine pentaacetic acid pentetreotide scintigraphy (Octreoscan).20Cadigan D.G. Hollett P.D. Collingwood P.W. Ur E. Imaging of mediastinal thymic carcinoid tumor with radiolabelled somatostatin analogue.Clin Nucl Med. 1996; 21: 487-488Crossref PubMed Scopus (21) Google Scholar, 21Lastoria S. Vergara E. Palmieri G. et al.In vivo detection of malignant thymic masses by indium-111-DTPA-d-pentetreotide scintigraphy.J Nucl Med. 1998; 39: 634-639PubMed Google Scholar Recent generations of somatostatin analogs (Octreotide and lanreotide) bind with high affinity to sst2 receptors22Reisiene T. Bell G.I. Molecular biology of somatostatin receptors.Endocr Rev. 1995; 16: 427-442PubMed Google Scholar, 23Patel Y.C. Srikant C.B. Subtype selectivity of peptide analogs for all five cloned human somatostatin receptors (hsstr 1-5).Endocrinolgy. 1994; 135: 2814-2817Crossref PubMed Scopus (0) Google Scholar; Octreotide has demonstrated good antiproliferative activity on neuroendocrine tumors, even if they are metastatic, without important side effects.24Filosso P.L. Ruffini E. Oliaro A. Papalia E. Donati G. Rena O. Long-term survival of atypical bronchial carcinoids with liver metastases, treated with octreotide.Eur J Cardiothorac Surg. 2002; 21: 913-917Crossref PubMed Scopus (73) Google Scholar Serum chromogranin A seems very useful for monitoring the disease, both with regard to recurrences and the efficacy of the Octreotide therapy. However, any suspicion of relapse has to be confirmed with CT scan and Octreoscan. In conclusion, primary neuroendocrine thymic carcinomas are rare and aggressive tumors that are often characterized by local invasive behavior. An aggressive surgical approach remains the treatment of choice; preoperative chemotherapy/radiotherapy (cisplatin, etoposide, and 45 Gy of irradiation) and Octreotide therapy should improve the possibility of achieving a radical resection. Postoperative radiotherapy has been proposed to prevent local recurrences, with unpredictable results. Adjuvant therapy with Octreotide should be considered because of the well-recognized antiproliferative activity of this drug and its limited side effects. Because the in vivo sst2 expression is provided by Octreoscan, this procedure should be considered for follow-up along with CT scan." @default.
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• W2076012369 title "Multidisciplinary treatment of advanced thymic neuroendocrine carcinoma (carcinoid): Report of a successful case and review of the literature" @default.
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