Recurrent Carcinoid Research Articles

Resistant gastroenteropancreatic neuroendocrine tumors: a definition and guideline to medical and surgical management

Gastroenteropancreatic neuroendocrine tumors (NETs), also historically known as carcinoids, are tumors derived of hormone-secreting enteroendocrine cells. Carcinoids may be found in the esophagus, stomach, small intestine, appendix, colon, rectum, or pancreas. The biologic behavior of carcinoids differs based on their location, with gastric and appendiceal NETs among the least aggressive and small intestinal and pancreatic NETs among the most aggressive. Ultimately, however, biologic behavior is most heavily influenced by tumor grade. The incidence of NETs has increased by 6.4 times over the past 40 years. Surgery remains the mainstay for management of most carcinoids. Medical management, however, is a useful adjunct and/or definitive therapy in patients with symptomatic functional carcinoids, in patients with unresectable or incompletely resected carcinoids, in some cases of recurrent carcinoid, and in postoperative patients to prevent recurrence. Functional tumors with persistent symptoms or progressive metastatic carcinoids despite therapy are called “resistant” tumors. In patients with unresectable disease and/or carcinoid syndrome, an array of medical therapies is available, mainly including somatostatin analogues, molecular-targeted therapy, and peptide receptor radionuclide therapy. Active research is ongoing to identify additional targeted therapies for patients with resistant carcinoids.

再発とリンパ節転移をきたした中耳カルチノイド症例

再発とリンパ節転移をきたした中耳カルチノイド症例

Umbilical Hernia Simulating Recurrent Carcinoid on Octreoscan

Physiologic bowel activity on In-111 octreotide imaging is commonly seen. However, on occasion, normal bowel activity may mimic lesions in the abdomen, which can be very difficult to differentiate, particularly after surgical intervention. We report a case of focal bowel activity simulating a lesion in a patient who had an In-111 octreotide scan (Octreoscan), postoperatively, looking for recurrent carcinoid. SPECT/CT demonstrated that the uptake was localized in the anterior abdomen, and corresponded to a loop of bowel within a ventral hernia at the site of surgical incision. The correlation with CT as well as the precise localization made possible by the fusion images helped avoid misinterpretation of this finding as possible recurrent carcinoid.

Tricuspid and Mitral Valve Carcinoid Disease in the Setting of a Patent Foramen Ovale

59-yr-old-woman with an 8-yr history of carci-noid syndrome with hepatic metastasis presentedwith progressive shortness of breath. Transesophagealechocardiographic examination (TEE) revealed severetricuspid regurgitation, tricuspid stenosis, mild mitralstenosis, moderate mitral regurgitation, and a patentforamen ovale (PFO). She was referred for a tricuspidand mitral valve replacement.Intraoperative TEE confirmed the preoperative diag-nosis of mild tricuspid stenosis with leaflet and chordalthickening, severe tricuspid regurgitation by extent ofcolor flow jet area and hepatic vein flow reversal bypulse wave Doppler, severe right atrial and ventricularenlargement Video Clip 1; please see video clips avail-able at www.anesthesia-analgesia.org), mild mitral ste-nosis, and moderate mitral regurgitation with mitralleaflet thickening and posterior leaflet restriction (Figs. 1and 2; Video Clip 1). Agitated saline contrast injectionconfirmed a PFO. Mild pulmonary regurgitation wasalso noted but the aortic valve was uninvolved. Both themitral and tricuspid valves were replaced with pericar-dial bioprosthetic valves, and the PFO was repaired(Video Clip 2). The excised tricuspid valve was diffuselythickened and fibrotic and the mitral valve leafletsappeared white and smooth, consistent with carcinoidvalvular heart disease. The patient’s hospital course wascomplicated by recurrent carcinoid crises occurringwithin the first postoperative day. Her subsequent hos-pital course was uneventful and she was discharged onpostoperative day 10.Carcinoid tumors arise from the gastrointestinaltract. Malignant carcinoid syndrome occurs whenserotonin and other vasoactive substances are releasedfromcarcinoidtumorcellsandresultinoneormoreofa constellation of symptoms which include flushing,hypotension, diarrhea and bronchospasm. More thanhalf of patients with carcinoid syndrome develop carci-noid heart disease as a late complication and it isassociated with progressive disease.

Facial Flushing Due to Recurrent Bronchial Carcinoid

Carcinoid syndrome is quite a rare presentation in bronchial carcinoid. A review of the literature suggests a figure of 2% to 7% in various series. This is usually associated with recurrent carcinoid tumor in the presence of hepatic metastasis. We discuss a patient who presented with flushing attacks 13 years after a left pneumonectomy. Further investigation found that the patient had recurrence at the pneumonectomy stump and in subcarinal lymph nodes.

RECURRENT CARCINOID TUMOR OF THE COLON.

Carcinoid tumors are among the most unusual tumors occurring in man. This lesion was probably first described by Lubarsch in 1888 1 and was named and differentiated from carcinoma in 1907 by Oberndorfer. 2 These tumors arise from the chromaffin of the intestine which were first described in 1897 by Nicholas Kultschitzky. 3 Masson 4 demonstrated cytoplasmic granules in these which are capable of reducing silver compounds, hence the names argentaffine cells and argentaffine tumors. During the ensuing years there have been many reports in the literature of these tumors occurring in various parts of the intestinal tract. In the past decade they have been found occasionally to be associated with a bizarre symptom complex which has been named the syndrome or functioning carcinoid tumor. This discovery by Biorck et al 5 and Thorson et al 6 has added greatly to the interest and importance of these