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Highlights • Transient jejunal intussusception is a rare cause of intestinal obstruction in adults. • There are eight recorded cases in the English literature and this is the first known case of a malignancy. • The usual presentation is in the 6th and 7th decades of life but the patient here is in her thirties., Introduction Transient jejunal intussusception in an adult is a rare clinical finding as reported in the English literature. The diagnosis is usually a matter of exclusion given the extremely rare nature of this medical condition. Presentation of the case A young female presented to our hospital with abdominal pain and distention of six months duration. The episodes were intermittent in nature and resolved with conservative management. The aetiology remained obscure until a computerized tomography(CT) scan diagnosed a small bowel intussusceptionIntraoperatively a small bowel tumour was identified and resected. Pathology confirmed an intestinal carcinoid of the small bowel with no evidence of metastatic disease. Discussion Transient jejunal intussusception is a rare finding with only eight reported cases in the English literature. All previously reported cases have been ascribed to benign aetiologies and to our knowledge this is the first case of a malignancy causing transient jejunal intussusception. The management is usually conservative unless an actual cause for the intussusception can be ascertained. The diagnosis is usually one of exclusion and CT scan remains the gold standard in eliciting a diagnosis. Conclusion Transient jejunal intussuception in adults is an extremely rare pathological condition and the diagnosis is usually entertained as a matter of exclusion.

Adenocarcinomas of the large and small bowel, as well as intestinal carcinoid tumors have been reported at increased rates in Crohn's disease. We herein report a rare case of concurrent adenocarcinoma and carcinoid tumor of the small bowel presenting as intestinal obstruction and found incidentally at laparotomy in a 55 year old male with longstanding ileal Crohn's disease. We performed a Medline Pubmed search for cases of synchronous or composite adenocarcinoma and carcinoid tumor in the setting of Crohn's disease and identified four similar cases. Concurrent adenocarcinoma and carcinoid tumor occurred both in newly diagnosed and longstanding Crohn's disease, most commonly involved the terminal ileum and presented with symptoms mimicking Crohn's disease. Diagnosis was made incidentally at laparotomy in all cases. Lymph node involvement was variable. Clinicians should be aware of this rare entity for expeditious surgical intervention.

We present a patient with clinical suspicion of intestinal carcinoid relapse confirmed by a somatostatin receptor scintigraphy. A very intense somatostatin avid abdominal lesion was located and radioguided surgery was proposed. Prior to the procedure, we performed a SPECT-CT that made it possible to detect and localize the lesion anatomically, thus facilitating the performance of the radioguided procedure. Furthermore, it modified the planning of the intervention with the adequate physical and human resources to prevent the possible surgical complications.

We present a patient with clinical suspicion of intestinal carcinoid relapse confirmed by somatostatin receptor scintigraphy. A very intense somatostatin avid abdominal lesion was located and radioguided surgery was proposed. Prior to the procedure, we performed a SPECT-CT allowing detection and localization of the lesion anatomically, thereby facilitating the performance of the radioguided procedure. Furthermore, SPECT-CT modified the planning of the intervention with adequate physical and human resources to prevent possible surgical complications.

A 59-year-old man with co-existing carcinoid syndrome and hyperinsulinism is described. The clinical examination revealed multiple tumour foci in an enlarged liver, and a subtotal hepatectomy (800 g) was performed. No primary tumour was revealed. The tumour had a light microscopical picture agreeing with a carcinoid or an insuloma; cytochemically and ultrastructurally most of the tumour cells presented characteristics of an intestinal carcinoid. The evidence of a presence of insulin producing β-cells in the tumour was rather vague. Biochemically, the tumour tissue showed only a faint insulin-like activity and a low content of radio-immunologically measurable insulin. Since the total tumour mass was large, it could, however, not be excluded that production of insulin by some tumour cells may have caused the hyperinsulinism. The possibility of insulin-releasing substances produced by the tumour cells was also considered possible. A co-existing islet β-cell tumour was improbable since both the carcinoid syndrome and the hyperinsulinism disappeared postoperatively.

In two patients with carcinoid syndrome, a reduced venoreactivity to serotonin (5-HT) was found by means of the venoconstriction test. Parachlorophenylalanine (PCPA) treatment increased 5-HT venoreactivity. A normal noradrenaline venoreactivity was detected before and during PCPA administration. The altered platelet monoamine oxidase (MAO) activity corrected by PCPA treatment. The abnormal 5-HT turnover may alter the synthesis of MAO and provoke the production of isoenzymes. Low plasma amine oxidase and high plasma histaminase activity were found in 1 of these 2 patients. A low level of plasma prekallikrein in the two patients remained unchanged during PCPA administration.

Familial cases of carcinoid tumours that are not associated with any known syndrome or disease are extremely rare. All cases reported in the world literature have involved carcinoid tumours of the gastrointestinal tract. Two cases of carcinoid tumours of the small intestine in a father and daughter are presented. Laboratory analyses did not support the hypothesis that the occurrence of carcinoid tumours in this family is a variant of the multiple endocrine neoplasia type 1 syndrome. A review of the literature on familial occurrence of intestinal carcinoid tumours in the absence of any other known carcinoid tumour-predisposing genetic syndrome is provided.

Liposarcoma most commonly arises in the retroperitoneum and lower extremities. Liposarcoma of the head and neck region is a rare and potentially life-threatening malignancy. Those originating in the right retrocervical space cause special diagnostic and therapeutic difficulties. In present report, a case of differentiated liposarcoma of the right cervical region with intestinal carcinoid is reported. The tumor continued to grow slowly over 3 years before a definitive diagnosis was established. Extended extirpation of the tumor and modified radical neck dissection, postoperative radiotherapy followed by chemotherapy were performed and proved to be efficacious in that no recurrence was observed for 2 years. Recommendations for earlier and correct diagnosis and treatment of this rare neoplasm are discussed.

Summary Lichen planus is not usually associated with malignancy and an association with carcinoid tumour has not been previously described. We now describe a patient in whom lichen planus and a carcinoid tumour presented simultaneously. In this case report, gastrointestinal symptoms of a small intestinal carcinoid tumour coincided with the appearance of a rash which progressed from a generalized exfoliative dermatitis to extensive lichen planus.

Carcinoid with rare presentation, and a literature review. A 58-year-old woman died in our intensive care unit (ICU) where she had been admitted for urgent ventriculotomy for a bleeding gastric ulcer. Chest radiography showed bilateral pulmonary infiltration, and respiratory support was introduced. Peristalsis was silent. Coma, tachycardia and high temperature were present, which did not respond to therapy. Hyperthyreosis therapy was started empirically. Melaena, as a sign of gastrointestinal bleeding, was observed. Control chest radiography showed infiltrate regression and normalised temperature, but despite these features the patient died. Clinical diagnosis included status, post-operation, of gastric ulcer, haemorrhagic shock, pneumonia, sepsis. Autopsy revealed massive intestinal bleeding due to primary exulcerated small intestine carcinoid. Primary condition was aggravated by bilateral consolidated suppurative bronchopneumonia, sepsis and nodular goitre. Since the patient was operated on in emergency, insufficient analyses were made. Melaena 5 days after the operation misled clinicians and pointed towards possible suture rupture due to sepsis. Tachycardia and hypertonia were recognised as untreated hyperthyreosis symptoms. Intestinal carcinoid was not suspected.

Metastatic neoplasm to the breast is a rare and heterogeneous event which can mimic primary breast carcinoma or benign neoplasm. Distinguishing metastasis from primary breast carcinoma is very important because of the difference in treatment options. This study aimed to investigate the diagnostic utility of fine-needle aspiration biopsy (FNAB) in differentiating breast metastases from primary breast neoplasm. FNAB of 50 metastases to the breast were evaluated for the following: cytomorphology, influence of clinical history and adjunctive methods on the final diagnosis. Reliable cytological criteria of malignancy were found in all 50 smears. These criteria, correlated with clinical data or complemented by ancillary techniques in selected cases, allowed correct diagnosis in 48 patients. Of the remaining two cases, wrongly diagnosed as primary carcinoma, one patient presented with a breast mass and the other had a history of intestinal carcinoid. FNAB plays an important role in the separation of metastatic neoplasm in the breast from primary breast carcinoma. Recognition of unusual cytomorphologic patterns, awareness of patient history and, in selected cases, ancillary techniques, are necessary in rendering a correct diagnosis of breast metastasis in FNAB smears.

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e14579 Background: Octreotide LAR is indicated for treatment of the malignant carcinoid syndrome, and has been studied at doses of 10-30mg intramuscularly every 4 weeks. It has also been proven to delay time to progression of metastatic midgut carcinoid tumors at a dose of 30mg every 4 weeks. In clinical practice, higher doses are often prescribed for patients who experience refractory carcinoid syndrome (flushing and/or diarrhea) or tumor growth while on the maximal labeled dose. We performed a retrospective, longitudinal review of octreotide LAR administration at a tertiary institution to determine the frequency of ‘above-label’ dosing and outcomes. Methods: A retrospective chart-review was performed using a database of patients with metastatic small-bowel carcinoid tumors treated at the Moffitt Cancer Center between the years 2000 and 2010. Data included the maximal dose of octreotide LAR administered, reasons for change in dose or frequency (above the labeled dose of 30mg every 4 weeks), and clinical responses to dose change. Results: 337 patients were considered evaluable, among whom 99 patients (27%) underwent at least one increase in dose or frequency of octreotide-LAR above the standard labeled dose. The most common maximal doses were 40mg every 4 weeks (37 patients), 60mg every 4 weeks (34 patients), and 30mg every 3 weeks (17 patients). The indications for dose increase were worsening carcinoid syndrome (60 patients), radiographic progression (33 patients) and rising urine 5-HIAA (6 patients). Among patients whose doses were increased for refractory carcinoid syndrome, 62% experienced improvement in diarrhea and 56% experienced improvement in flushing. Conclusions: In clinical practice, octreotide LAR is commonly prescribed in doses or schedules above the labled dose and frequency. Patients with refractory carcinoid syndrome appear to experience a clinical benefit from the change. Prospective data is needed to evaluate this strategy.

Carcinoid tumors of the intestine may present with bowel obstruction or with the carcinoid syndrome. Confirmation of the diagnosis by biochemical means is easy. Management is often surgical and a number of different procedures have been advocated, e.g. resection of the primary, hemicolectomy and lately, partial hepatectomy. The angiographic features of two cases are presented and discussed; the usefulness of the information so gleaned in the choice of management is stressed.

The chest films of 44 patients with liver metastases from intestinal carcinoid tumors were reviewed in order to evaluate the frequency of significant carcinoid heart disease. Only two patients had obvious signs of cardiac involvement, which is contradictory to most other studies.

Coexisting carcinoid tumors within Meckel's diverticula have been described rarely. Our experience with an elderly patient with this unusual pathologic combination led to an extensive review of the world's literature. This review documents that carcinoid tumors are the most commonly reported tumors of Meckel's diverticula, with risks of symptoms and metastases similar to those described with primary small intestinal carcinoid tumors.

The diagnostic value of computed tomography (CT) and iodine-131 meta-iodo-benzylguanidine (MIBG) scintiscanning was studied in nine patients with histologically proved carcinoid tumors of intestinal (n = 4), bronchial (n = 3), or thymic (n = 2) origin. CT scans clearly depicted the tumors and metastases in relation to surrounding vital structures but did not provide findings specific for carcinoids. The appearance on CT of an abdominal soft-tissue mass with a radiating pattern of linear densities was found to be highly suggestive of intestinal carcinoid tumors. I-131 MIBG scintiscans disclosed intense tracer uptake in the tumors and metastases in five patients. MIBG studies correctly depicted nine of nine tumor manifestations in intestinal carcinoids and four of six tumor manifestations in bronchus carcinoids. No MIBG concentration was found in thymus carcinoids. Because of its selective uptake mechanism, I-131 MIBG scintigraphy can allow specific detection and localization of neuroendocrine tumor tissue in patients with suspected carcinoid tumors. MIBG scintigraphy has diagnostic potential as a screening procedure in carcinoid tumors, especially those of intestinal origin.

The roentgenologic features of small intestinal carcinoid tumors have received little attention in the literature compared to their clinical and potential pharmacologic manifestations. Eighteen proven cases were analyzed.A variety of roentgenologic appearances may be present, reflecting the growth patterns and tissue reaction incited by the tumor. Roentgenologic appearances vary from solitary or multiple submucosal nodules to extensive mesenteric desmoplasia without demonstration of a tumor mass. Some manifestation of the desmoplastic reaction is the most common roentgen finding.Because of the nonspecific clinical findings, the radiologist will often have the first opportunity to suggest the diagnosis.

Introduction Multiple endocrine neoplasia type 1 (MEN-1) patients are prone to develop carcinoid tumors. Few cases report the development of gastrointestinal carcinoid tumors in patients with MEN-1 syndrome related tumors. This is the first paper to report the occurrence of an intestinal carcinoid tumour in association with a pituitary adenoma. Case presentation A sixty eight year old female presented with intestinal obstruction four years after transphenoidal pituitary resection for pituitary adenoma. During surgical exploration and lysis of adhesions, we accidentally discovered an intestinal carcinoid tumour. Resection of the involved small bowel segment and the draining lymph nodes was undertaken. Postoperative follow up showed no biochemical or radiological evidence of residual tumor. Neuroendocrine tumors (NETs) may occur as part of familial endocrine cancer syndromes including MEN-1. It is recommended that clinicians search thoroughly for MEN-1 in patients presented with NETs, however, there is no current consensus for screening patients suspected to have MEN-1 to rule out NET. Conclusion We recommend screening patients suspected to have any familial type of endocrine tumors for the presence of NET.

IN 1927 Postma (1892-1960),1a Dutch dermatologist, described the first case of a flushing syndrome in a patient who 4 years later was proven to suffer from an intestinal carcinoid. Postma, who has also made other contributions to internal medicine,2designated the disease as "haemangioma planum extensum." The title of his article is important since it indicates that Postma was well aware of the nature of the cutaneous lesion: In his opinion, the ruddiness was due not to the flushing of normal skin but to a hyperemic reaction in a pre-existing angiomatosis of the skin. Report of a Case Postma's patient, a 45-year-old man, had had a ruddy face for as long as he could remember. Between 1924 and 1927, the red color of the face had become darker. In addition, the skin of his arms and trunk and ultimately also of the legs became red. The

Excerpt The details of the comparatively new and, indeed, bizarre clinical entity of argentaffinoma, pulmonary stenosis and cutaneous flush are not well documented. Patients with this triad present...