Neuroendocrine Tumors of Meckel's Diverticulum: Lessons from a Single Institution Study of Eight Cases.
Summary of "Neuroendocrine Tumors of Meckel's Diverticulum: Lessons from a Single Institution Study of Eight Cases."
INTRODUCTION:
Endocrine tumors of Meckel's diverticulum are rare. Their clinical and pathological characteristics are not well known, making it difficult to assess the best strategy for therapeutic management. MATERIALS AND
METHODS:
Eight cases of endocrine tumors of Meckel's diverticulum, submitted to surgical resection in our institution between 1977 and 2009, were studied. Clinical charts were reviewed; classification, grading, and staging were performed according to recent international recommendations. Five cases, including two associated with the carcinoid syndrome, were revealed by mesenteric mass or liver metastases; three cases were diagnosed incidentally at laparotomy or laparoscopy.
RESULTS:
All cases presented as typical well-differentiated midgut endocrine tumors. Five cases were associated with mesenteric lymph node metastases; three presented with liver metastases. Seven cases were classified as well-differentiated endocrine carcinomas, one as well-differentiated endocrine tumor of benign behavior.
DISCUSSION:
All tumors >1 cm, but one, had regional or distant disease. All patients had complete surgical resection of the primary. One patient deceased after 25 months; the others were alive after 12-101 months.
CONCLUSION:
In conclusion, endocrine tumors of Meckel's diverticulum are rarely symptomatic and often diagnosed at an advanced stage. All tumors measuring more than 1 cm in diameter must be resected according to oncological principles.
Affiliation
Hospices Civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités Digestives, Lyon, France.
Journal Details
This article was published in the following journal.
Name: Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
ISSN: 1873-4626
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20824383
- DOI: http://dx.doi.org/10.1007/s11605-010-1312-x
Medical and Biotech [MESH] Definitions
Neuroendocrine Tumors
Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition.
Meckel Diverticulum
A congenital abnormality characterized by the outpouching or sac formation in the ILEUM. It is a remnant of the embryonic YOLK SAC in which the VITELLINE DUCT failed to close.
Vitelline Duct
The narrow tube connecting the YOLK SAC with the midgut of the EMBRYO; persistence of all or part of it in post-fetal life produces abnormalities, of which the commonest is MECKEL DIVERTICULUM.
Carcinoma, Neuroendocrine
A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)
Synaptophysin
A 38-kDa integral membrane glycoprotein of the presynaptic vesicles in neuron and neuroendocrine cells. It is expressed by a variety of normal and neoplastic neuroendocrine cells and is therefore used as an immunocytochemical marker for neuroendocrine differentiation in various tumors. In ALZHEIMER DISEASE and other dementing disorders, there is an important synapse loss due in part to a decrease of synaptophysin in the presynaptic vesicles.
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