title: Undifferentiated carcinoma of the esophagus: a clinicopathological study of 16 cases.
abstract: Undifferentiated carcinoma of the esophagus is a rare histologic variant of esophageal carcinoma. Using criteria based on studies of undifferentiated carcinomas arising at other sites, we have collected 16 cases of resected esophageal undifferentiated carcinomas. Patients ranged in age from 39 to 84 years (mean, 65.5 years) and were predominantly male (94%). The tumors were characterized by an expansile growth pattern of neoplastic cells organized in solid sheets and without significant glandular, squamous, or neuroendocrine differentiation. The neoplastic cells had a syncytial-like appearance, little intervening stroma, and patchy tumor necrosis. In a subset of cases, the tumor cells adopted a sarcomatoid (n = 2), rhabdoid (n = 1), or minor component (<5%) of glandular morphology (n = 3). In 1 case, reactive osteoclast-like giant cells were found interspersed among the neoplastic cells. Lymphovascular invasion, perineural invasion, and lymph node metastases were identified in 88%, 56%, and 81% of cases, respectively. In 12 (75%) specimens, the background esophageal mucosa was notable for Barrett esophagus. Consistent with the epithelial nature of these neoplasms, cytokeratin positivity was identified in all cases. In addition, SALL4 expression was present in 8 (67%) of 12 cases. Follow-up information was available for 15 (94%) of 16 patients, all of whom were deceased. Survival after surgery ranged from 1 to 50 months (mean, 11.9 months). Before death, 67% patients had documented locoregional recurrence and/or distant organ metastases. In summary, esophageal undifferentiated carcinomas are aggressive neoplasms and associated with a high incidence of recurrence and/or metastases and a dismal prognosis.
authors.affiliation: Department of Pathology, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213. Electronic address: singhiad@upmc.edu.
authors.forename: Aatur D
authors.initials: AD
authors.lastname: Singhi
authors.affiliation: Department of Pathology, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Raja R
authors.initials: RR
authors.lastname: Seethala
authors.affiliation: Department of Surgery, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Katie
authors.initials: K
authors.lastname: Nason
authors.affiliation: Department of Pathology, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Tyler J
authors.initials: TJ
authors.lastname: Foxwell
authors.affiliation: Department of Pathology, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Robyn L
authors.initials: RL
authors.lastname: Roche
authors.affiliation: Department of Medicine, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Kevin M
authors.initials: KM
authors.lastname: McGrath
authors.affiliation: Department of Surgery, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Ryan M
authors.initials: RM
authors.lastname: Levy
authors.affiliation: Department of Surgery, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: James D
authors.initials: JD
authors.lastname: Luketich
authors.affiliation: Department of Pathology, the University of Pittsburgh Medical Center, Pittsburgh, PA 15213.
authors.forename: Jon M
authors.initials: JM
authors.lastname: Davison
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000428:Alcohol Drinking / Q000453:epidemiology; D000740:Anemia / Q000453:epidemiology; D001471:Barrett Esophagus / Q000453:epidemiology; D014408:Biomarkers, Tumor / Q000032:analysis; D002277:Carcinoma / Q000737:chemistry / Q000453:epidemiology / Q000473:pathology* / Q000556:secondary; D002294:Carcinoma, Squamous Cell / Q000737:chemistry / Q000453:epidemiology / Q000473:pathology* / Q000556:secondary; D015897:Comorbidity; D003680:Deglutition Disorders / Q000453:epidemiology; D004938:Esophageal Neoplasms / Q000737:chemistry / Q000453:epidemiology / Q000473:pathology* / Q000556:secondary; D000077277:Esophageal Squamous Cell Carcinoma; D005260:Female; D005500:Follow-Up Studies; D005764:Gastroesophageal Reflux / Q000453:epidemiology; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007633:Keratins / Q000032:analysis; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D009367:Neoplasm Staging; D016609:Neoplasms, Second Primary / Q000473:pathology; D011379:Prognosis; D012907:Smoking / Q000453:epidemiology; D015996:Survival Rate; D014157:Transcription Factors / Q000032:analysis; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; C472575:SALL4 protein, human; D014157:Transcription Factors; D007633:Keratins
keywords: Carcinoma; Esophagogastric; Esophagus; Gastroesophageal; SALL4; Undifferentiated