Thymic neuroendocrine carcinoma (carcinoid): A clinicopathologic study of fourteen cases

Abstract

The medical records and histologic documents of 14 patients treated at our institution for a thymic carcinoid tumor were reviewed. There were 3 women and 11 men with an age range from 35 to 71 years. One patient had a multiple endocrine neoplasia syndrome; another had a neurofibromatosis. Twelve tumors were revealed by local symptoms and two were asymptomatic. One patient had Cushing's syndrome that appeared secondarily and was related to metastases. Tumors ranged from 6 to 20 cm and had the characteristic histologic appearance of atypical carcinoid tumor. Immunohistochemical evaluations were done. Tumors were positive for cytokeratin (92%), neuroendocrine markers (100%), and p53 oncoprotein (29%). S-100 protein antibody revealed numerous sustentacular cells in one case. Overall survival was 46% and 31% at 3 and 5 years, respectively. However, all patients died of the disease within 109 months as a result of local progression (n = 5), local relapse (n = 3), distant metastases (n = 8), or a combination of these reasons. Median survival was 71, 30, and 5 months for patients who had total resection (n = 4), partial resection (n = 5), or simple biopsy (n = 4), respectively (p = 0.023). In conclusion, thymic carcinoid tumors can be considered thymic neuroendocrine carcinomas because of their malignant behavior and histologic appearance of atypical carcinoid tumors. Complete surgical resection offers the best hope for long-term survival.

Primary Neuroendocrine Carcinoma (Thymic Carcinoid) of the Thymus with Prominent Oncocytic Features: A Clinicopathologic Study of 22 Cases

“…Therefore, it may be argued that thymic carcinoids should be coded as "atypical" following the designation given by Arrigoni et al (58,59) to similar tumors in the lung. Conversely, there are those who consider the term carcinoid anachronistic and would rather classify such tumors within the spectrum of neuroendocrine carcinomas (60). We fully support the latter philosophy and believe that thymic carcinoids should be regarded as belonging within the spectrum of neuroendocrine carcinomas and that they should be further categorized on the basis of their cytologic features into well-, moderately, and poorly differentiated tumors.…”

Durable response in a thymic neuroendocrine tumor: A case report and review of the literature

“…[12] Distant metastases are common, with various case series reporting frequencies of between fifty and ninety-two percent. [13] Given this, adjuvant chemotherapy is often administered and Cisplatin-based regimens are the favored approach. In addition, the European Society for Medical Oncology (ESMO) notes that temozolomide-based treatments may offer some benefit.…”

p53, cellular proliferation, and apoptosis-related factors in thymic neuroendocrine tumors

“…16,[19][20][21][22][23][24][25] Although many of the previous studies have focused on non-neuroendocrine thymic neoplasms, there has been very little attention regarding these factors in thymic neuroendocrine tumors, and there has not been a systematic study of these markers with standardized immunohistochemical scoring and rigorous statistical analysis for a large series of cases (Table 3). 14,[26][27][28][29] This prompted us to focus on some of these factors, namely, p53 expression, cellular proliferation, the antiapoptosis markers Bcl-2 and Bcl-x, and the proapoptosis marker Bax.…”