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Neuroendocrine Neoplasia

Short summary

60-year-old male was diagnosed with a symptomatic metastatic well differentiated rectal neuroendocrine tumor, with multiple liver metastases. This tumor is over-expressing somastatin receptors, which was demonstrated by an otreoscan. He was started on Sandostatin LAR 30 once a month.

Patient's questions

1. Can you confirm the therapy?

2. Can you suggest any further therapies?

3. What is the prognosis?

4. Any recommended centres in Italy and abroad?

Medical Background

Sex:M, Age:60 years

Diagnosis: Neuroendocrine Neoplasia
1963-Pneumothorax for specific process.
1974-Surgical repair of right hernia
Current history:
Approximately 8 months prior to this report, an ultrasound exam identified hepatic hypo echoic lesions at the hepatic segment VI – VIII; An abdominal CAT scan showed evidence of hypo dense lesions at segment IV and VI and at the margin between segment VII and segment VIII, of ambiguous diagnostic interpretation.
The patient was admitted into the hospital 2 months prior to this report and remained for 10 days for clinical-instrumental re-evaluation concerning the above mentioned ultrasound and the CAT scan findings of hepatic lesions of undefined nature.

Exams performed in the hospital showed the following:
  • Objective examination -> normal, except for a “hepatic margin at 8 cm from the costal arch and at 5 cm from the irregular, ensiform apophysis (not on deep inspiration).……”
  • Blood tests -> mostly normal except for the electrophoresis: “increment of alpha 1 globulins (5.3) monoclonal lgG-lambda component + small lgG-lambda component in gamma area". The neuroendocrine markers are: VIP 25.3 pmol/l; Somatostatin 6.6 pmol/l; Pancreatic polypeptide 12.2 pmol/l; Motilin 160 pg/ml; Gastrin 48.2 pg/ml; Neurotensin 37.7 pg/dl; Cromogranin A 28 U/L; Glucagon 266 pg/ml; Urinary 5-HIAA 5.9 mg/24h; Urinary 5HT+5HTP 4.8 mg/24 h; Urinary concentration of 5-HIAA at another lab: negative.
  • Diagnostic tests ->
    • Abdominal ultrasound: Evidence of multiple hepatic lesions on the right lobe, suggesting secondary lesions, therefore the following tests were performed:
    • Hepatic Biopsy: the result was positive showing well differentiated neuroendocrine neoplasia in multiple locations (minimum cellular pleomorphism, no evidence of necrosis, number of mitosis <2 in 10 HPF); assessment of semi-quantitative activity and proliferation activity through ki67 (MIB-1): 5-10%; immunohistochemical colorations performed:
    • Cytokeratin 7 (OV-TL12/30) (-/+, rare cells), cytokeratin 20 (K20.8), cromogranin A (LK2H10)(-), synaptophysin (SY38)(+).
    • Chest X-Ray: normal, except for a retracting left fibro thorax as a result of the pneumothorax .
    • Colonoscopy: the exam was interrupted due to inadequate preparation.
    • EGDS: normal.
    • Colonoscopy: identified a polypoid sessile formation of 1.5 cm of irregular and hemorrhagic surface; hystological biopsy found a well differentiated neuroendocrine neoplasia of morphological appearance and immunophenotype profile comparable to the hepatic one, in addition to two polyps of 5 mm at 18 and 15 cm from the anal margin; an histological examination, through biopsy, found adenomas with mild displasia.
For a complete diagnosis, the following was performed: a total body bone scintigraphy, with negative results, and tests on the concentration of neuroendocrine markers in blood or urine which showed only a modest increment of the Glucagon value, 266 pg/ml.
After hospitalisation, the following tests were performed: thorax CAT scan, showing no evidence of a current pathological thickening, and Octreoscan, confirming the presence of hepatic lesions expressing receptors for the Somatostatin in absence of additional areas of pathologic accumulation of the medication.
During the hospitalisation, a tolerance test was performed with medications similar to Somatostatin (octreotide 0.2 mg S.C.) which were well tolerated by the patient.
One month prior to this report the patient was discharged with the following diagnosis: “Neuroendocrine neoplasia with hepatic secondary lesions and carcinoid syndrome” and with the
following therapy:
  • Sandostatin LAR 30 (intramuscular injection, using a vial, every 28 days).


Medical opinion

It seems that the diagnostic procedure and treatment have been state of the art for the treatment of this disease. I would further evaluate the dynamic of both the clinical and radiological progression of the disease, and perhaps consider, in the future, a chemoembolization for the right lobe metastasis of the liver (according to the radiological picture of an actual CAT scan). I would also consider, in the future, a PRRT treatment with 177 Lu/ 90Y- DOTATOC. There are a few centres in Europe that perform this procedure. I have personal experience with Professor Mullerat the University Hospital in Basel, Switzerland as well as some experience with the Center in Rotterdam, Netherland.

One can expect a prognosis of a few years, and if a good objective response to the above treatment it may even be longer. At this point, it doesn't seem that chemotherapy should be considered for the treatment of the current situation. There are a few centres in Italy which have considerable experience in the treatment of this disease, among them Professor Gianfranco Delle Favefrom Rome, the center in Verona and the center in Milano.