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JBR­BTR, 2002, 85: 254-256.

FLUSH SYMPTOMS CAUSED BY A MESENTERIC CARCINOID WITHOUT LIVER METASTASES

S. Sonnet, W. Wiesner1 Flushing is a known symptom in intestinal carcinoid tumors which usually occurs only in the presence of liver metastases. A 62-year-old women presented with abdominal pain, nausea and flush symptoms. US, CT, octreotide scintigraphy and biopsy revealed a primary mesenteric carcinoid with retroperitoneal lymph node metastases and a solitary leftsided supraclavicular lymph node metastasis proving lymphatic spread over the thoracic duct, but liver metastases were excluded. This is a report on a mesenteric carcinoid which lead to flush symptoms despite absence of liver metastases, since retroperitoneal lymph node metastases enabled a direct hormone release into the systemic circulation.

Key-word: Carcinoid.

Carcinoid tumors are the most common gastrointestinal neuroendocrine tumors, accounting for approximately 75% of all such neoplasms. Carcinoid tumors may pre-

sent with abdominal pain, gastrointestinal bleeding, or intestinal obstruction and the latter may be caused by the tumor itself or by retractile mesenteritis which typical-

ly may derive from peritumoral stimulation of fibrous proliferation. Another manifestation of these tumors is the carcinoid syndrome which consists of a triad of symp-

A

B

Fig. 1. -- Contrast enhanced CT of the lower abdomen (A) shows a contrast enhancing tumor in the root of the mesentery (arrows) which is located anteriorly to the branches of the superior mesenteric artery and which contacts the wall of the horizontalis part of the duodenum. Note the enlarged retroperitoneal lymph node (arrowhead). Contrast enhanced CT of the lower abdomen (B) shows an irregularly shaped contrast enhancing tumor in the root of the mesentery (arrow) which shows a streaky infiltration of the surrounding mesenterial fat indicating retractile mesenteritis. Contrast enhanced computed tomography of the upper abdomen at a higher level than A shows several enlarged retroperitoneal lymph nodes sorrounding the abdominal aorta at the level of the renal veins (arrowheads).

C

From: Institute of Diagnostic Radiology, University Hospital Basel, Switzerland. Address for correspondence: Dr. W. Wiesner, Institute of Diagnostic Radiology, University Hospital Basel, Petersgraben 4, CH-4031 Basel, Switzerland.

FLUSH SYMPTOMS IN MESENTERIC CARCINOID -- SONNET et al

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toms including cutaneous flushing, diarrhea, and valvular heart disease. Overall, flushing is found in approximately 85 percent of patients with carcinoid tumors whereas the carcinoid syndrome occurs only in approximately 40 percent of these patients. However, it is generally accepted that intestinal carcinoids lead to flushing only in the presence of metastatic liver disease since otherwise the hormones produced by these tumors are cleared by the liver. Contrarily, bronchial and extraintestinal carcinoids may occasionally lead to flush symptoms even in the absence of liver metastases if they are large enough as depending on their location their hormones may be directly released into the systemic circulation. We report on a 62-year-old woman with a mesenteric carcinoid who developed flush symptoms despite absence of liver metastases and in whom several retroperitoneal lymph node metastases enabled a direct hormone release into the systemic circulation showing that flush symptoms may occur in abdominal carcinoids even despite absence of liver metastases. Case report A 62 year-old woman was admitted because of diffuse abdominal pain. Symptoms had started several weeks ago and were pronounced especially during sexual intercourse. The patient suffered from nausea but diarrhea was absent. The patient also suffered from cutaneous flushing as well as from minor dyspnea during the flush attacks. A gynecological examination including ultrasound of the small pelvis showed no abnormalities. However, an abdominal ultrasound examination revealed a 5 x 2 x 2 cm large tumor in the mesentery and enlarged retroperitoneal lymph nodes above the level of the renal arteries with a diameter of 2 cm. These findings were confirmed later on by contrast enhanced computed tomography of the abdomen which showed a contrast enhancing tumor in the root of the mesentery, contacting the inferior wall of the horizontal part of the duodenum, but endoscopy excluded a duodenal carcinoid (fig. 1). The primary tumor was shaped irregular and surrounded by retractile mesenteritis, which suggested already the correct radiological diagnosis of a carcinoid tumor in context with the clinical findings but there were no focal

A

B

Fig. 2. -- Octreotide scintigraphy shows (A) a strong focal uptake in the primary tumor (arrowheads) and in retroperitoneal lymph nodes (arrow). Note absence of liver metastases. It also shows (B) a small focal uptake (small arrow) in a solitary left sided supraclavicular lymph node, suggesting lymphatic spread over the thoracic duct into the Virchov's lymph node.

liver lesions detected by sonography or computed tomography. Laboratory findings of an elevated urinary 5-HIAA/creatinin quotient of 16.4 umol/mmol were consistent with a carcinoid tumor and a CT guided biopsy of one of the enlarged retroperitoneal lymph nodes confirmed this diagnosis. An octreotide scintigrapy with 111 Mbq In - marked somatostatin analogon showed a strong focal uptake in the primary tumor, in the retroperitoneal lymph node metastases, and additionally in a solitary leftsided supra-clavicular lymph node (Virchov's lymph node) but no focal activity in the liver suggesting liver metastastases (fig. 2). According to the lack of any intestinal obstruction and the presence of distant lymph node metastases the patient was not operated and underwent metabolic therapy with five cycles of 160 mCi 90 YDOTATOC which led to an impressive regression of all tumor manifestations. Discussion Carcinoid tumors are the most common tumors of neuroendocrine

origin. The total incidence of these tumors is about 15 per 1 million / year. Although carcinoid tumors may occur at any location, 73% of these tumors arise in the ileum, 4% in the jejunum and 7% in the tracheobronchial tree whereas other locations such as ovaries or testes are very rare. At the time of diagnosis around 90% of carcinoids present with liver metastases and this is also the reason why flushing may be observed in up to 85% of these patients (1). Intestinal carcinoid tumors may cause abdominal pain or gastrointestinal bleeding and even intestinal obstruction may result from tumor growth or from retractile mesenteritis (2, 3). However, although retractile mesenteritis may often lead to the correct radiological diagnosis in context with suggestive clinical findings such as flushing or diarrhea, it is not specific for carcinoid tumors as it may also be associated with leiomyomas, leiomyosarcomas, segmental ileitis, and ischemic enteritis (4, 5). Primary tumors in gastrointestinal carcinoids may be missed by conventional imaging methods, especially if they are small and the sensitivity of CT and MR for detec-

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JBR­BTR, 2002, 85 (5)

tion of the primary tumor in such cases is significantly lower (25%) than for detection of lymph node metastases (62%) and organ metastases (97%) respectively (6). Although somatostatin receptor scintigraphy (Octereotide-scintigraphy) has been reported to have an overall sensitivity of 72-100% for detection of intestinal carcinoids and their metastases a recent study showed that the sensitivity of somatostatin receptor scintigraphy drops to 57% if those lymph node metastases, that are not detected by CT or MR but solely by F18 DOPA PET, are not ignored (6). In this particular study the overall sensitivity of F18 DOPA PET and CT/MR for detection of gastrointestinal carcinoids and metastases was 65% and 73% respectively, whereas FDG PET showed only a sensitivity of 29%. Although CT/MR are more sensitive for detection of organ metastases they are less sensitive for detection of lymph node metastases and especially for detection of the pimary tumor itself in such cases. Therefore, the highest overall sensitivity for detection of gastrointestinal carcinoids and metastases will be achieved by a combination of a cross sectional imaging procedure and a scintigraphic method, and especially the combination of CT / MR and F18 DOPA PET has been reported to result in an overall sensitivity of 99% (6). The carcinoid syndrome is a well known manifestation of carcinoid tumors which constists of a triad of cutaneous flushing, diarrhea, and endocardial fibrosis. Endocardial fibrosis usually affects the tricuspid and pulmonary valves leading to insufficiency and/or stenosis and is known as Hedinger Syndrome (7). Various hormones including 5hydroxytryptamin, 5-hydroxytryptophan, kallikrein, histamine, tachykinins (neurokinin A, neuropeptide K, substance P) and prostaglandins are supposed to be responsible for the carcinoid syndrome (8, 9, 10). It is interesting that tracheobronchial carcinoids present with a

carcinoid syndrome only in 3.5% of cases. This could be explained by the fact that tracheobronchial carcinoid tumors are mostly detected in early stages secondary to tracheobronchial obstruction, at a time when the amount of hormones produced by the tumor is not yet sufficient to cause systemic symptoms. In contrast, intestinal carcinoids are often detected at advanced stages only ­ at a time when they have already grown to a size of several centimeters. In this context it is generally accepted that intestinal carcinoids lead to flushing or to the carcinoid syndrome only in the presence of liver metastases as otherwise the endocrine active hormones produced by these tumors are cleared by the liver (7-10). However, under certain circumstances flushing or even the carcinoid syndrome may occur in abdominal carcinoids despite absence of liver metastases. This constellation may occur for example if an intestinal carcinoid tumor is very large and if the inactivation of hormones by the liver is exceeded as it may be found in huge primary tumors with an immense secretion of metabolites, or if there are intraabdominal metastases that are drained by the systemic venous circulation (11, 12). In our experience, another rare constellation how intestinal carcinoids may lead to flushing or even to the carcinoid syndrome despite absence of liver metastases is their occurrence in patients with liver cirrhosis as under these circumstances the hormones released by the tumor occasionally may reach the systemic circulation over portosystemic shunts without being cleared by the liver. Our case documents another example of how abdominal carcinoids may lead to flushing despite absence of liver metastases and shows once again that neither the occurrence of flushing nor the diagnosis of a carcinoid syndrome should be regarded as a general evidence for the presence of metastatic liver disease under these conditions.

References

1. Norheim I., Oberg K., TheodorssonNorheim E., Lindgren P.G., Lundquist G., Magnusson A., Wide L., Wilander E.: Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production and survival. Med Pediatr Oncol, 1987, 20: 221-223. 2. Seigel R.S., Kuhns L.R., Borlaza G.S., McCormick T.L., Simmons J.L.: Computed tomography and angiography in ileal carcinoid tumor and retractile mesenteritis. Radiology, 1980, 134: 437-440. 3. Aspestrand F., Pollard L.: Carcinoid infiltration and fibroplastic changes of the mesentery as a cause of malabsorption. Radiologe, 1986, 26: 7981. 4. Rigaud C., Bogomoletz W.V.: Association of retractile mesenteritis and ischemic enteritis. Ann Pathol, 1983, 3: 171-173. 5. Koornstra J.J., van Olffen G.I.I., van Noort G.: Retractile mesenteritis. To treat or not to treat. Hepatogastroenterology, 1997, 44: 408-410. 6. Hoegerle St., Altehoefer C., Ghanem N., Koehler G., Waller C.F., Scheruebl H., Moser E., Nitzsche E.: Whole-Body 18 F DOPA PET for detection of gastrointestinal carcinoid tumors. Radiology, 2001, 220: 373380. 7. Kaplan L.E. Carcinoid. In: Fauci A.S. et al.: Harrison's Principles of Internal Medicine, 14th Edition. Mc Graw-Hill Health Professions Division, 1988, 95: 585-588. 8. Lucas K.J., Feldman J.M. Flushing in the carcinoid syndrome and plasma kallikrein. Cancer, 1986, 58: 22902293. 9. Conlon J.M., Deason C.F., Richter G., Stockman F., Creutzfeld W.: Circulating tachykinins (substance P, neurokinin A, neuropeptide K) and the carcinoid flush. Scand J Gastroenterol, 1987, 22: 97-105. 10. Ahlman H.: Serotonin and carcinoid tumors. J Cardiovasc Pharmacol, 1985, 7: 79-85. 11. Feldman J.M., Jones R.S.: Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis. Ann Surg, 1982, 196: 33-37. 12. Haq A.U., Yook C.R., Hiremath V., Kasimis B.S.: Carcinoid syndrome in the absence of liver metastasis: a case report and review of literature. Med Pediatr Oncol, 1992, 20: 221-223.

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