title: Primary undifferentiated large cell carcinoma of the lacrimal gland.
abstract: OBJECTIVE
  To report a unique case of primary undifferentiated large cell carcinoma (LCCA) of the lacrimal gland, a tumor not previously described in the ophthalmic literature.
  
  
  METHODS
  Single interventional case report.
  
  
  METHODS
  A patient affected by undifferentiated LCCA of the lacrimal gland.
  
  
  METHODS
  A 65-year-old white man with a 3-month history of a painful mass in the left lacrimal gland fossa underwent an incisional biopsy that revealed a "high-grade" epithelial malignancy. Systemic workup revealed enlargement of the regional lymph nodes, and subsequently the patient underwent extended exenteration with clear histologic margins and radical neck lymphadenectomy followed by adjunctive radiotherapy. Fifteen months postoperatively, the patient is alive and well without evidence of local recurrence or metastatic disease.
  
  
  METHODS
  Treatment result, evidence of local recurrences or distant metastasis, and follow-up.
  
  
  RESULTS
  Histologic examination revealed a poorly circumscribed tumor composed of large cells invading orbital fat, lateral rectus muscle, and peripheral nerves. The surrounding orbital bone was infiltrated, but the surgical margins were clear. The cell population was composed of large cells (>30 μm) with eosinophilic cytoplasm and ovoid and irregular nuclei containing a prominent nucleoli and coarse chromatin. The cell borders were well defined. Mitosis figures were abundant, and Ki-67 was positive in more than 60% of the cells. The cells were arranged in cords and trabeculae or irregular sheets of discohesive cells. The immunophenotype analysis showed positivity for cytokeratin but negative cytokeratin 20 stains, which is considered a distinctive feature of LCCA.
  
  
  CONCLUSIONS
  Undifferentiated LCCAs are rare tumors of the major salivary glands, especially the parotid gland. Primary undifferentiated LCCA of the lacrimal gland has never been reported in the literature. Differential diagnosis must include a primary source in another organ. Given the aggressive nature of the tumor, radical surgery followed by radiotherapy is recommended, but evidence-based indications regarding the preferred line of treatment are lacking and the prognosis remains guarded.
  
  
  BACKGROUND
  The author(s) have no proprietary or commercial interest in any materials discussed in this article.
authors.affiliation: Department of Ophthalmology, Ospedale Evangelico Internazionale, Genova, Italy. info@oculoplasticabernardini.it
authors.forename: Francesco P
authors.initials: FP
authors.lastname: Bernardini
authors.forename: J Oscar
authors.initials: JO
authors.lastname: Croxatto
authors.forename: Roberto
authors.initials: R
authors.lastname: Bandelloni
mesh_terms: D000368:Aged; D001706:Biopsy; D018287:Carcinoma, Large Cell / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007765:Lacrimal Apparatus; D007766:Lacrimal Apparatus Diseases / Q000175:diagnosis* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D013508:Ophthalmologic Surgical Procedures / Q000379:methods