Elsevier

Human Pathology

Volume 30, Issue 2, February 1999, Pages 216-221
Human Pathology

Original contribution
Primary undifferentiated small cell carcinoma of the esophagus

https://doi.org/10.1016/S0046-8177(99)90279-4Get rights and content

Abstract

We histologically examined undifferentiated small cell carcinoma of the esophagus from 21 patients and used immunohistochemical methods for detection of chromogranin A and p53, bc1–2, and Rb oncoproteins. Nine (43%) of the 21 carcinomas consisted solely of undifferentiated cells, but heterogeneous components of in situ or invasive squamous cell carcinoma or mucoepidermoid carcinoma were observed in the other 12 (57%) tumors. Squamous cell carcinoma in situ was observed in the mucosa adjacent to the main tumor in 7 (50%) of the 14 resected esophageal specimens. An admixture of invasive squamous cell carcinoma and undifferentiated carcinoma was observed in 4 (19%) of the 21 tumors, and mucoepidermoid carcinoma was noted in one case. Chromogranin A staining yielded a positive reaction in two (10%) undifferentiated components but was negative in all heterogeneous components. Multiple sites of p53 immunopositivity were seen in the undifferentiated component of 17 (81%) of the 21 tumors, as well as in the in situ or invasive squamous cell carcinoma or mucoepidermoid carcinoma components of 9 (75%) of 12 tumors. Seven (33%) of the 21 tumors showed positive bc1–2 immunoreactivity in the small cell component, but all of the heterogeneous components were negative. Rb protein immunoreactivity was observed in the small cell component of one (5%) case and in 9 (75%) of the 12 heterogeneous components. Six (86%) of the seven in situ squamous cell carcinoma components were positive for Rb protein. Eighteen (86%) of the 21 patients died within 24 months of diagnosis. Two patients (10%) who survived for more than 24 months had received chemotherapy.

References (18)

  • CB Caldwell et al.

    Unusual malignant neoplasms of the esophagus: Oat cell carcinoma, melanoma, and sarcoma

    J Thorac Cardiovasc Surg

    (1991)
  • K-J Ho et al.

    Small cell carcinoma of the esophagus: Evidence for a unified histogenesis

    Hum Pathol

    (1984)
  • MF Fer et al.

    Extrapulmonary small cell carcinoma

  • RL Richardson et al.

    Undifferentiated small cell carcinomas in extrapulmonary sites

    Semin Oncol

    (1982)
  • F McKeown

    Oat cell carcinoma of the esophagus

    J Pathol Bacteriol

    (1952)
  • K Takubo

    Undifferentiated carcinoma of the esophagus

  • SY-K Law et al.

    Small cell carcinoma of the esophagus

    Cancer

    (1994)
  • GL Nichols et al.

    Small cell carcinoma of the esophagus: The Memorial Hospital experience 1970 to 1986

    Cancer

    (1989)
  • KL Beyer et al.

    Primary small-cell carcinoma of the esophagus: Report of 11 cases and review of the literature

    J Clin Gastroenterol

    (1991)
There are more references available in the full text version of this article.

Cited by (87)

  • Clinicopathological features and outcome of esophageal neuroendocrine tumor: A retrospective multicenter survey by the digestive endoscopy society of Taiwan

    2021, Journal of the Formosan Medical Association
    Citation Excerpt :

    The result was similar using Ki-67 55% as the cut-off level according to the NORDIC study15 (P = 0.09, Table 4). Similar to other GEP-NEC, esophageal NEC has diffusely positive immunostaining for synaptophysin but infrequent positive immunostaining to chromogranin A.4 Consistent with reports from Japan, India and China,5,6,10 our survey found esophageal NECs were commonly small cell type, had a high proportion of mixed NEC with SCC, and middle (43.6%) or upper (10.3%) esophagus sites were not uncommon. On the contrary, studies from the USA3 and Korea9 indicated the major locations of esophageal NET were distal esophagus and gastroesophageal junction, where neuroendocrine cells were mainly distributed.5

  • A rare rarity: Neuroendocrine tumor of the esophagus

    2019, Critical Reviews in Oncology/Hematology
    Citation Excerpt :

    Data pertaining to prognostic factors are unclear. While some studies found mixed esophageal NECs tended toward a better outcome than pure esophageal NECs without non-neuroendocrine components (Maru et al. 2008), this was not observed in other studies (Babu Kanakasetty et al., 2016; Takubo et al., 1999). Prognostic factors affecting survival are generally age (Nemoto et al., 2002), the extent of the disease (Ku et al., 2008; Maru et al., 2008), tumor-node-metastasis (TNM) classification (Wang et al., 2013) and local treatment versus local plus systemic treatment (Yun et al., 2007; Chen et al., 2014a; Ding et al., 2013) or chemotherapy (Chen et al., 2014b; Poynton et al., 1997; Lv et al., 2008).

  • Surgical resection of rare esophageal cancers

    2016, Annals of Thoracic Surgery
  • Upper Gastrointestinal Tract

    2022, Handbook of Practical Immunohistochemistry: Frequently Asked Questions
View all citing articles on Scopus

Supported by a Grant-in-Aid (7-7) for Cancer Research from the Ministry of Health and Welfare and a Grant-in-Aid for Scientific Research (09470264) from the Ministry of Education, Science and Culture of Japan.

View full text