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Figure 3: Lymph node metastasis from well differentiated neuroendocrine tumour (G1) of the ileo-cecal valve. (A) Metastatic mesenteric lymph node (not microscope
photo); (B) evidence of nodal compression with serotonin stain (arrow, X4); (C) compression of the nodal parenchima by neoplastic cells (arrow, X4); (D) mindbomb
homolog 1/ki-67 proliferation index below 2% (arrow). Non-neoplastic lymphocytes (D, oval) as internal control (X40)
tumour. Serum tumour markers such as neuron specific Six years after the first diagnosis, the patient is continuing
enolase and chromogranin A (CgA) resulted in range. The maintenance medical treatment with Lanreotide ATG 120
diagnostic work-up was therefore suggestive for a single, mg every 28 days, since stabilization of the disease.
intraorbital metastasis from midgut well differentiated
neuroendocrine tumour. DISCUSSION

The patient was evaluated for radical resection of The therapeutic strategy for neuroendocrine neoplasms
the intraorbital lesion through a transcranial excision (NENs) needs to be diversified according to the clinical
(exenteratio orbitis), but the patient refused the orbitotomy. presentation of each single case, and moreover according
to its biological behaviour, due to the wide heterogeneity
He therefore underwent two 4-week courses of stereotactic of these tumors.
radiotherapy to the right orbital metastases (4,000 cGy
in 20 fractions) and he started a systemic treatment with Because of this, a multidisciplinary care team is critical
for patient management starting from the earliest steps of
Lanreotide Autogel (ATG) (fl 120 mg, 1 fl i.m. q 28 d).
the diagnostic workup.
The patient remained stable with persistent right Ocular metastases have been rarely described in
exophthalmos and conjunctivitis, but without any vision neuroendocrine tumours; the vast majority of metastases
deterioration after two months of radiation therapy and SSA. affect the uveal tract rather than orbital space, and
typically occur through haematogenous spread by carotid
He continued the SSA as maintenance treatment and and ophthalmic artery. [4,5] Data regarding survival after the
after four months he underwent primary tumour resection diagnosis of orbital metastases of NENs are exceedingly
with right hemicolectomy and lymphadenectomy. rare. Mehta et al. describe a series of 13 patients with
[6]
The pathology report confirmed a well-differentiated metastatic orbital carcinoid tumors with overall survival of
neuroendocrine tumour of ileocecal valve, G1, pT3, N1, 72% at 5 years and 38% at 10 years.
M1 according to the ENETS/UICC TNM classification.
The immunohistochemical analysis showed wide Considering the favorable general prognosis despite the
positivity for CgA, synaptophysin; serotonin and CDX2 advances stage, treatments that maintain a good quality
[Figures 2 and 3]. of life with the preservation of vision are the fundamental
Journal of Cancer Metastasis and Treatment ¦ Volume 2 ¦ August 31, 2016 ¦ 343