A rare case of poorly differentiated mixed neuroendocrine-nonneuroendocrine tumor of the caecum with long term survival: A case report.

Abstract

A 59-year-old woman presented with flushing attacks accompanied by tachycardia and hypotension, which lasted approximately 30 to 60 minutes, underwent 18 years ago a gastrointestinal tumor resection. The histologic examination revealed a poorly differentiated mixed neuroendocrine/adenocarcinoma located in the caecum with regional metastases. Postoperatively, the patient received combined chemotherapy of 5-fluorouracil with interferon for six months and since has remained asymptomatic. Her examination revealed positivity for chromogranin A (CgA) and a-Fetoprotein (aFP) (580 ng/24 h, normal range 27-94, and 10 IU/mL, normal range 0-6, respectively). Urinary 5-hydroxy indole acetic acid excretion was remarkably high (41.8 mg/24 h, normal range 2-10 mg/24 h). An abdominal Magnetic Resonance Imaging scan revealed multiple focal loci in the liver whose histological examination revealed a carcinoid tumor confirmed by an Octreoscan. Additional uptake was noted on the right shoulder and the right sternum-clavicle joint confirmed by Tc-99m MDP scan. The patient received somatostatin analogue therapy followed by long-acting release octreotide analogue therapy (30 mg/month) showing a partial improvement of relevant biomarkers. Two years later, carcinoid syndrome symptoms reappeared and due to the tumors expression of somatostatin receptors the patient received peptide receptor radionuclide therapy with 177Lu-DOTATATE that resulted in both clinical and biochemical improvements.

• Authors: Antonis Polymeris, Christina Kogia, Paraskevi Kazakou, Stavroula Psachna, Dimitrios Lilis, Maria Drakou, Konstantinos Michalakis, Dimitrios Ioannidis
• Journal: Endocrine regulations (Endocr Regul) Vol. 56 Issue 4 Pg. 249-253 (Oct 01 2022) ISSN: 1336-0329 [Electronic] Germany
• PMID: 36270345 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2022 Antonis Polymeris et al., published by Sciendo.

Chemical References

• Octreotide
• Receptors, Somatostatin
• Chromogranin A
• alpha-Fetoproteins
• Technetium Tc 99m Medronate
• copper dotatate CU-64
• Somatostatin
• Fluorouracil
• Interferons
• Radioisotopes

Topics

• Humans
• Female
• Middle Aged
• Octreotide (therapeutic use)
• Receptors, Somatostatin (metabolism)
• Chromogranin A
• alpha-Fetoproteins
• Technetium Tc 99m Medronate
• Neuroendocrine Tumors (diagnostic imaging, drug therapy)
• Somatostatin
• Fluorouracil (therapeutic use)
• Cecum (metabolism, pathology)
• Interferons
• Radioisotopes