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Topic: Neuroendocrine Tumors

Orbital lesions, an exceedingly rare site of neuroendocrine tumor
metastasis

Sara Pusceddu , Massimo Milione , Silvia Ortolani , Alessio Pellegrinelli , Marco Brugia , Filippo de Braud ,
1
3
4
1
2
2
Lorenzo Antonuzzo 4,5
1 Department of Medical Oncology, Fondazione IRCCS “Istituto Nazionale dei Tumori”, 20133 Milan, Italy.
2 Department of Phatology, Fondazione IRCCS “Istituto Nazionale dei Tumori”, 20133 Milan, Italy.
3 Department of Medical Oncology, Azienda Ospedaliera Universitaria Integrata, University of Verona, 37134 Verona, Italy.
4 Department of Medical Oncology, Azienda Ospedaliera Universitaria Careggi, 50134 Firenze, Italy.
5 Medical Genetics, University of Siena, 53100 Siena, Italy.
Correspondence to: Dr. Sara Pusceddu, Department of Medical Oncology, Fondazione IRCCS “Istituto Nazionale dei Tumori”, Via G. Venezian
1, 20133 Milan, Italy. E-mail: sara.pusceddu@istitutotumori.mi.it
A B S T R AC T
Neuroendocrine tumors are rare neoplasms arising primarily in the gastrointestinal tract and lung. The liver is the most common
site of metastases, but these tumors can rarely metastasize to atypical sites. Surgery is the only curative approach while the
optimal medical treatment is debated. From this perspective, a multidisciplinary approach for each single case becomes very
important. In this report we describe the case of a male affected by a single intraorbital metastasis from a midgut well differentiated
neuroendocrine tumor. The patient refused surgical removal and therefore he was at first treated with stereotactic radiotherapy
and systemic treatment with a somatostatin analog (SSA). After achieving a stable disease for four months he underwent primary
tumor resection. Six years after the initial diagnosis, the patient is currently stable and receiving SSA at standard dose.

Key words: Neuroendocrine tumors; orbital metastases; somatostatin analogues

INTRODUCTION somatostatin analog (SSA) therapy alone may constitute
valid treatment options towards the goal of long-term
Neuroendocrine tumors are rare neoplasms derived from disease stabilization and improved quality of life.
enterochromaffin cells, which are primarily found in the
gastrointestinal tract and lung. [1,2] Liver is the most common CASE REPORT
site of metastasis, however as survival is increasing by
improved treatment options, new metastatic patterns We report the case of a 65-year-old male patient, in
have emerged. Ocular metastases, considered “a rarity good general conditions, with a past medical history of
[3]
in the rare,” have now been described in neuroendocrine hypertension, diabetes mellitus and ischemic heart disease.
tumors. [4,5] Considering the rarity of these tumors, it is clear
that a multidisciplinary approach is necessary in order to He presented in September 2009 complaining the recent
obtain the best therapeutic outcome for each single patient. onset of right exophthalmos.
Here, we present a case where the integrated use of local- A computed tomography (CT) scan of the head and
regional and systemic treatments resulted in long-term neck documented the presence of a retroocular lesion
disease stabilization, preserving the quality of life. with a maximum diameter of 28 mm, invading both the

intraconal and the extraconal space and causing optic
This case raises important issues. Considering the favorable nerve impingement. A biopsy of the lesion was performed
general prognosis despite the advanced stage, treatments in November 2009 through endoscopic endonasal
that maintain a good quality of life are the fundamental approach and the pathologic examination diagnosed well-
issues for these patients. Therefore, the alternative loco- differentiated neuroendocrine tumour cells.
regional treatments alone (stereotactic radiotherapy)
or in combination with systemic therapy, or systemic This is an open access article distributed under the terms of the Creative
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How to cite this article: Pusceddu S, Milione M, Ortolani S,
Pellegrinelli A, Brugia M, de Braud F, Antonuzzo L. Orbital lesions, an
exceedingly rare site of neuroendocrine tumor metastasis. J Cancer
DOI: Metasta Treat 2016;2:341-4.
10.20517/2394-4722.2016.41
Received: 20-06-2016; Accepted: 18-07-2016
©2016 Journal of Cancer Metastasis and Treatment ¦ Published by OAE Publishing Inc. 341