Abstract
BackgroundCarcinoids at atypical sites are rare and mostly asymptomatic. In the majority, they show normal levels of serotonin and its metabolites in plasma and urine, resulting in a relatively late diagnosis and hence guarded prognosis. The relatively large size and repeated local recurrences are features of carcinoids at atypical locations and may direct the attention to its possible existence.Patients and methodsA report of six cases of carcinoid tumors in the breast, abdominal paravertebral region, duodenum, ovary, and stomach is given. Blood serotonin, plasma, and urine 5-hydroxyindole acetic acid were determined for cases suspected to have carcinoid syndrome. Those presented with a mass were radiologically studied by CT or MRI, and if a biopsy was taken (or the mass removed), keratin, CEA, and chromogranin for argentaffin reaction were studied. Follow-up was for 15 months, and a second surgery was done for the two cases (1 and 5).ResultsExcept for case 4, all patients were females. The symptoms and clinical findings in the six cases were vague and did not raise the possibility of a carcinoid. Repeated local recurrence was the clue for the diagnosis. The enrolled six cases had no common features that may point or suggest the presence of neither carcinoid tumor nor syndrome. Also, there was no relation between the site and size of the tumor and the detection of the manifestations of carcinoid syndrome. Additionally, in one case (case 1), the originally non-functioning lesion turned active when recurrence happened.ConclusionCarcinoid tumors at atypical sites are low-grade malignant neoplasms with a good long-term prognosis if correctly managed. Routine aggressive resection or even debulking is the treatment of choice whenever possible, even in recurrent cases. The survival of patients depends basically on the tumor size and/or the presence of distant metastases. Treatment by somatostatin analogy is usually mandatory if carcinoid syndrome is present.
Highlights
Carcinoid tumors at atypical sites are low-grade malignant neoplasms with a good long-term prognosis if correctly managed
Treatment by somatostatin analogy is usually mandatory if carcinoid syndrome is present
Rare (0.15%) (Robertson et al 2006), slowly growing with a high potential for local recurrences and metastasis, carcinoid tumors are of neuroendocrine origin (NETs)
Summary
Rare (0.15%) (Robertson et al 2006), slowly growing with a high potential for local recurrences and metastasis, carcinoid tumors are of neuroendocrine origin (NETs). Survival has improved over the last decades due to the introduction of treatment by octreotide (Sandostatin) in metastatic disease (Modlin et al 2003) They have five patterns: solid, trabecular, glandular, tubular, poorly differentiated, or mixed. A common finding is elastosis and fibrosis that surround the nests of the tumor cells and result in matting of the involved tissues and lymph nodes They have at least five somatostatin receptors frequently used for diagnosing the disease by octreotidereceptor scintigraphy and in its treatment. A substantial number of carcinoid tumors originate in less common anatomical sites and can range from indolent, unrecognized entities to highly active, metastatic secretory tumors. Their presentation within unfamiliar locations often results in clinical confusion. The relatively large size and repeated local recurrences are features of carcinoids at atypical locations and may direct the attention to its possible existence
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