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Orbital metastatic small cell carcinoma of the pancreas with optic nerve compression

Published:August 07, 2017DOI:https://doi.org/10.1016/j.jcjo.2017.06.017
      Small-cell carcinoma accounts for 18%–20% of all primary lung malignancies but has also been described as arising from the bladder, prostate, stomach, colon, and pancreas.
      • Wang D.
      • Rong Y.
      • Wu W.
      • Jin D.
      Primary small cell carcinoma of the pancreas: rare type of pancreatic cancer and review of the literatures.
      Small-cell cancer of the pancreas (SCCP) is an extremely rare malignancy and represents only 0.2%–1% of all pancreatic malignancies.
      • Wang D.
      • Rong Y.
      • Wu W.
      • Jin D.
      Primary small cell carcinoma of the pancreas: rare type of pancreatic cancer and review of the literatures.
      • Ivanics T.
      • Bergquist J.R.
      • Shubert C.R.
      • Smoot R.L.
      • Habermann E.B.
      • Truty M.J.
      Small cell carcinoma of the pancreas: a surgical disease.
      Here, we describe a unique case of an SCCP presenting as a metastatic lesion to an extraocular muscle causing optic nerve compression.

      Case Report

      A 51-year-old male presented with a 9-day history of progressively decreasing vision in the left eye and binocular diplopia in left gaze. His medical history was significant for HIV infection with a recent CD4 count of 850 cells/mm3 on antiretroviral therapy. His medical examination was otherwise negative.
      Clinical examination showed a best-corrected visual acuity of 20/25 OD and 20/40 OS. Intraocular pressures were normal, and there was no significant axial proptosis by exophthalmometry. Examination of the left eye was notable for a relative afferent pupillary defect, dyschromatopsia with Ishihara colour plates, and limitation of extraocular motility in abduction. There was no resistance to retropulsion, and the posterior pole examination was otherwise unremarkable.
      Magnetic resonance imaging with gadolinium showed an ill-defined, enhancing mass within the left lateral orbital apex causing optic nerve compression. The lesion appeared to arise from the lateral rectus muscle and was hypointense on T1-weighted imaging and isointense to brain parenchyma on T2-weighted imaging (Fig. 1).
      Fig. 1
      Fig. 1Left: T1-weighted MRI showing an ill-defined, low-signal-intensity mass that appears to arise from the lateral rectus muscle. Right: T2-weighted MRI showing an isointense lesion arising from the extraocular muscles with mass effect on the optic nerve. MRI, magnetic resonance imaging.
      Given the findings of an orbital lesion with compressive optic neuropathy, a lateral orbitotomy was performed. Intraoperatively, a friable lesion with ill-defined margins was found to be adherent to the apical portion of the belly of the left lateral rectus muscle. The remainder of the lesion was excised piecemeal, and histologic evaluation disclosed a tumour composed of basophilic cells and smudge nuclei that stained positively for cytokeratin, synaptophysin, TTF-1, and CK20 (Fig. 2). The findings were concerning for a diagnosis of metastatic small-cell cancer.
      Fig. 2
      Fig. 2Histopathologic examination disclosing small-cell carcinoma. Hematoxylin–eosin 40× (A) and 400× (B) original magnification revealing involvement of the lateral rectus muscle. (C) The tumour stains positively for TTF-1, (D) cytokeratin, and (E) synaptophysin immunostaining (all 100× original magnification). (F) The tumour stains variably positive for CK20 (100× original magnification).
      Postoperatively, the patient’s vision improved to 20/20 with resolution of compressive optic neuropathy. He continued to have limitation in abduction in his left eye. The patient was referred to the medical oncology service, where a systemic work-up revealed multiple liver lesions and a large pancreatic tail mass, suggestive of primary small-cell carcinoma of pancreatic origin. Notably, no lung foci were found on dedicated computed tomography imaging of the chest.

      Discussion

      Metastases to the orbit represent 3%–7% of all orbital tumours, with breast, lung, and prostate carcinoma being the most prevalent.
      • Char D.H.
      • Miller T.
      • Kroll S.
      Orbital metastases: diagnosis and course.
      In up to 35% of all cases, no known primary malignancy is found at the time of diagnosis.
      • Char D.H.
      • Miller T.
      • Kroll S.
      Orbital metastases: diagnosis and course.
      • Shields J.A.
      • Shields C.L.
      • Brotman H.K.
      • Carvalho C.
      • Perez N.
      • Eagle Jr, R.C.
      Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture.
      Involvement of the extraocular muscles is equally uncommon, representing only 5% of all orbital metastatic lesions.
      • Char D.H.
      • Miller T.
      • Kroll S.
      Orbital metastases: diagnosis and course.
      Review of prior reports of orbital pancreatic metastasis shows that the majority of cases are due to pancreatic adenocarcinoma, with 1 case of pancreatic islet cell carcinoma.
      • Geetha N.
      • Chandralekha B.
      • Kumar A.
      • Ittiyavirah A.K.
      • Ramadas K.
      • Joseph F.
      Carcinoma of the pancreas presenting as an orbital tumor: a case report.
      • Goldberg R.A.
      • Rootman J.
      • Cline R.A.
      Tumors metastatic to the orbit: a changing picture.
      • Foo F.Y.
      • Lee M.
      • Looi A.
      Asymptomatic pancreatic adenocarcinoma presenting as an orbital metastatic tumor.
      Recently, a retrospective analysis of over 30 million records in the National Cancer Database from 1998 to 2011 yielded a total of 541 cases of SCCP compared to 156 733 cases of primary ductal adenocarcinoma, with SCCP representing only 0.2% of all pancreatic tumours.
      • Ivanics T.
      • Bergquist J.R.
      • Shubert C.R.
      • Smoot R.L.
      • Habermann E.B.
      • Truty M.J.
      Small cell carcinoma of the pancreas: a surgical disease.
      Of those SCCP cases, 91% are found to have metastases at the time of diagnosis, with the most frequent sites of involvement being the liver, peripancreatic lymph node, lungs, bone marrow, and colon.
      • Wang D.
      • Rong Y.
      • Wu W.
      • Jin D.
      Primary small cell carcinoma of the pancreas: rare type of pancreatic cancer and review of the literatures.
      In our case, the one primary pancreatic focus with numerous hepatic lesions presented similarly to previously reported cases of SCCP.
      • Corrin B.
      • Gilby E.D.
      • Jones N.F.
      • Patrick J.
      Oat cell carcinoma of the pancreas with ectopic ACTH secretion.
      No lesions were found on dedicated imaging to suggest a primary lung cancer. With these findings, the pancreas was deemed the primary origin, with metastases to the liver and the lateral rectus muscle causing the presenting symptoms of diplopia and decreased vision.
      In cases in which there is pathologic evidence of small-cell carcinoma, prompt referral to an oncologist for systemic work-up is essential given the propensity for numerous distant metastases. It is interesting to speculate what effect our patient’s HIV status played as a predisposing factor for the development of SCCP, and indeed there has been a reported case of small-cell carcinoma of the anus in an HIV-positive patient.
      • Brenner B.
      • Shah M.A.
      • Gonen M.
      • Klimstra D.S.
      • Shia J.
      • Kelsen D.P.
      Small-cell carcinoma of the gastrointestinal tract: a retrospective study of 64 cases.
      Extensive discussion regarding the treatment of SCCP is beyond the scope of this case report, although recent evidence recommends combination chemotherapy with carboplatin and etoposide, with consideration of surgical resection in stage I/II disease.
      • Ivanics T.
      • Bergquist J.R.
      • Shubert C.R.
      • Smoot R.L.
      • Habermann E.B.
      • Truty M.J.
      Small cell carcinoma of the pancreas: a surgical disease.
      Despite treatment, prognosis is poor, with a median survival of 3 months.
      • Wang D.
      • Rong Y.
      • Wu W.
      • Jin D.
      Primary small cell carcinoma of the pancreas: rare type of pancreatic cancer and review of the literatures.

      Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

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