Acta Oto-Laryngologica

ISSN: 0001-6489 (Print) 1651-2251 (Online) Journal homepage: http://www.tandfonline.com/loi/ioto20

Pedunculated Intraluminal Fibro-Lipoma of the Oesophagus Case Report Å. Elner & N. G. Palm To cite this article: Å. Elner & N. G. Palm (1976) Pedunculated Intraluminal FibroLipoma of the Oesophagus Case Report, Acta Oto-Laryngologica, 82:1-6, 457-462, DOI: 10.3109/00016487609120934 To link to this article: http://dx.doi.org/10.3109/00016487609120934

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Date: 10 May 2016, At: 07:56

Acta Otolaryngol82: 457-462, 1976

PEDUNCULATED INTRALUMINAL FIBRO-LIPOMA OF THE OESOPHAGUS Case Report

A. Elner and N. G. Palm From the Departments of Otolaryngology and Roentgenology, University Hospital of Lund, Lund, Sweden

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(Received January 17, 1976)

Abstract. Only few cases of pedunculated, benign, intraluminal lipomas and fibrolipomas of the oesophagus have been reported. A further case is now added. The diagnosis is often delayed since it is difficult both for the radiologist and the endoscopist to detect the tumour. Symptoms and signs are few and uncharacteristic. The diagnosis could be made earlier and without sophisticated methods if the possibility of a tumour is kept in mind, especially in patients with a dilated oesophagus and a normal opening of the cardia. The value of fiberoptic endoscopy as a valuable tool for early diagnosis is stressed. The therapy is in most cases extirpation through cervical oesophagotomy.

Benign intraluminal pedunculated tumours of the oesophagus have been regarded as comparatively rare. During the last few decades, however, the number of cases reported in the literature has increased, though they are far kom common and are by far outnumbered by other tumours, above all the malignant tumours of the oesophagus. Up to the latest report in 1974 by Liljequist & Wiberg only 134 cases of benign, pedunculated intraluminal tumours had been reported and two more were added by these authors. Among the reported cases there are many so-called polyps, which constitute about one-third of all the tumours, and also myomas which are more or less pedunculated. True lipomas or fibrolipomas seem to be very rare and constitute only about 20% of all reported cases of benign pedunculated intraluminal tumours. A few malignant pedunculated tumours have been reported (Serfling & Bauditz, 1953)-mostly sarcomas

and carcinosarcomas-but these are very few indeed. Thus, as a working hypothesis, genuine pedunculated intraluminal tumours of the oesophagus can be regarded as benign. The stalk of the tumour is in most cases in the upper part of the oesophagus on the level with or just below the cricopharyngeal muscle (Moersch & Harrington, 1944). Liljequist & Wiberg (1974) give the figure 88% from a comprehensive perusal of the literature on all pedunculated tumours, but it is even higher for the true lipomas and fibrolipomas. Practically all of these tumours originate in the upper part of the oesophagus but can extend down to the cardia or even further down into the ventricle. The lipomas and fibrolipomas arise from the submucosal layer of the oesophageal wall and are covered by normal mucosa. They might start as small mucosal tumours which, when growing, are gradually “gripped” by the peristalsis of the oesophagus and become pedunculated by that mechanism. The tumours are usually lobulated or club-like in shape, but the stalk is often comparatively thin. The symptoms of these benign, pedunculated intraluminal tumours are often surprisingly few. The patients complain of a feeling of a lump in the throat or of dysphagia to a moderate degree and in some cases of loss of weight due to some difficulty in swallowing solid food. But dysphagia can also be absent Actu Ofolaryngol82

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and the first sign of tumour is the appearance of a mass in the mouth in a considerable number of cases. Thus, apart from the latter striking symptom, the signs are few and the tumours can often go undetected for a long time and grow to enormous size before the diagnosis is clear. The diagnostic means are X-ray and endoscopy. An ordinary barium meal often fails to give adequate information but examination under Buscopanem hypotonia (Uthgenannt et al., 1973), cinkfluorography and the new technique with computerized tomography are more refined methods, which should reveal the true nature of the disease. It is striking, however, that in many cases reported in the literature, repeated examinations have failed to establish the correct diagnosis. This reflects the difficulty of detecting these tumours, but also the fact that they are “rare birds” and therefore are mostly overlooked as a diagnostic possibility. Endoscopy is also difficult in these cases. As in the case of X-ray, repeated examinations are often necessary and the endoscopic picture is confusing and difficult to interpret. The tumour is in most cases covered by normal mucosa and the endoscopic picture can resemble that of a wide and folded oesophagus. In fact, the most common differential diagnosis has been achalasia cardiae or cardiospasm both in X-ray and endoscopy and a few cases have even been reported on which cardioplasty was performed before the correct diagnosis was established (McBride, 1951). CASE REPORT

A man, aged 75 years, was referred to the outpatient department in 1971 complaining of a lump in the throat and abundant mucous secretion. He had no difficulty in eating and no dysphagia and had been sent by a general practitioner who had interpreted his symptoms as being due to pharyngitis and prescribed a course of antibiotics. Physical examination was normal. X-ray examination of the hypoActa Otolaryngol82

pharynx, oesophagus, ventricle and duodenum showed sliding hernia with some regurgitation, but the other findings were normal. Motility studies showed normal coordination of the upper sphincter with a normal tonus. The peristalsis was irregular but propelling. There was no sign of gastric reflux but increased abdominal pressure showed a slightly incompetent lower sphincter. The patient was told to reduce weight and Gaviscon@and antacida were prescribed. He returned in November 1974, still complaining of a lump in the throat and for 2 months increasing difficulties in swallowing with a feeling of obstruction in the hypopharynx. He also sometimes vomited after meals. He had only been able to take fluids for the last few weeks. X-ray examination showed a 7 cm wide dilatation of the oesophagus. The cardia region was normal and the sphincter opened in a normal way. The picture was that of achalasia but the normal sphincter opening made this diagnosis questionable. As in 1971 there was a hiatal hernia of a moderate size and slight regurgitation. The findings were contradictory and though the fluoroscopist consulted two senior colleagues and actually suggested that there might be a tumour the findings were finally interpreted as achalasia. This called for a new examination, however, and this time an intraluminal tumour was found. It originated in the upper parts of the oesophagus and reached the cardia region (Fig. 1). Endoscopy was performed but did not reveal any tumour or dilatation of the oesophagus and the mucosa was normal in all parts. A hernia was found but no signs of oesophagitis. A biopsy was taken proximal to the cardia. At repeated endoscopy a week later by a more experienced examiner a tumour was found originating in the cricopharyngeal region and reaching the cardia. This was partly possible by fiberoptic examination during which the instrument could be rotated around the intraluminal tumour. A few days later cervical oesophagotomy on the left side was made and the tumour was extirpated. There was a fairly thin stalk

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Oesophageal pedunculated intraluminal fibro-liporna

Fig. 1 . Preoperative X-ray with the tumour entirely filling and dilating the esophagus. Note the multilobulated appearance and the sharply defined lower pole. The left

part of the picture shows the upper segment of the esophagus. Acta Otolaryngol82

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Fig. 2. The specimen.

emerging from the right side just below the level of the cricopharyngeal muscle. The stalk was clamped, stitched and resected. There was some difficulty in extracting the tumour through the oesophagotomy because of its size and club-like shape (Fig. 2). The specimen measured 25 x 5 X 6 cm and the histological picture was that of a fibrolipoma without signs of malignancy. The postoperative course and healing were uneventful. Postoperative X-rays showed a slight irregularity on the right side of the cricopharyngeal region but otherwise normal findings (Fig. 3). The patient has no dysphagia and eats without difficulty one year after the operation.

DISCUSSION It is obviously very difficult to establish a correct diagnosis in cases with pedunculated Actu Otolaq~ngol82

intraluminal tumours of the oesophagus. The symptoms are late and uncharacteristic and there can be a considerable doctor’s delay before the true cause of the patient’s complaints is revealed. Most reports in the literature show that X-ray examinations are often negative or misinterpreted. Fluoroscopy is often done by residents and the picture might be similar to that of achalasia with retained food in the oesophagus. A pedunculated tumour in the upper part of the oesophagus can also sometimes resemble swallowed air (Stein & Finkelstein, 1973). Thus, repeated examinations are often performed before an intraluminal tumour is even suspected. According to Liljequist & Wiberg (1974), however, there are some signs in an ordinary examination with barium contrast that can lead the examiner on towards the right conclusion.

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Oesophageal pedunculated intraluminal.)?bro-lipoma

Fig. 3. Postoperative X-ray with some irregularity at the level of the stalk of the tumour.

Usually there is a dilatation of the oesophagus, especially in the case of larger tumours. The mucosal folds are flattened while the tumour surface is irregular and lobulated, giving the impression of multiple intraluminal masses. In most cases, however, there is only one tumour even though multiple ones have been reported. The contrast surrounds the growth as a thin 30-162951

film observable when the patient is rotated and examined in different positions, and the lower pole of the tumour is usually well delimited. Furthermore, a discrepancy between a dilated oesophagus giving the impression of achalasia and a normally wide cardia with a normal passage of contrast to the ventricle-as was found in the case reported here-is highly Actu Otolaryngol82

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indicative of an intraluminal pedunculated tumour. Endoscopic examination also frequently fails to establish the diagnosis. In many reports in the literature repeated examinations are made before the tumour is diagnosed endoscopically, and quite often this is not done until the roentgenological diagnosis is clear. It is remarkable that even cardia surgery has been performed after a false diagnosis of achalasia. The case reported here provides the same picture of diagnostic difficulties. Presumably the patient had a small tumour even when he was first examined as an outpatient 3 years before the diagnosis was crbvious. If the possibility of a pedunculated tumour is kept in mind by the roentgenologist as well as the otolaryngologist, diagnostic mistakes could be avoided. The use of fiberoptic endoscopy is valuable. The flexibility of the instrument allows the examiner to “circle around’ the tumour or twist the instrument around it. Perhaps the diagnosis could have been made earlier in the case reported here if fiberoptic examination had been performed already when the patient was examined for the first time as an outpatient. Fiberoptic endoscopy cannot replace a roentgenological examination but is a very valuable complement and can perhaps prevent time-consuming and more expensive and sophisticated examinations than the routine barium there is no

ones and the stalk of the tumour can be reached that way in cases-a majority-where it originates in the upper part of the oesophagus. Only rarely is thoracotomy necessary. If the tumour is club-shaped and of considerable size, as in our case, there might be some difficulty in removing it from the oesophagus. But by widening the incision in the esophageal wall and by gentle handling of the tumour this can be done safely. Acta Otolaryngol82

Finally, it must be stressed that the prognosis of these histologically benign tumours-if they are left undiagnosed and untreatedmight be grave owing to progressive emaciation of the patient, complications from the airways, and sometimes lethal bleeding from erosions and ulcerations (McBride, 1951; Burrel & Toffler, 1973). The prognosis is excellent, however, when proper treatment is instituted in good time. ZUSAMMENFASSUNG Uber gesticlte, gutartige, i ~ Lumen i gelegene Lipome und Fibrolipome des Oesophagus ist nur selten berichtet worden. Ein eigener Fall wird vorgestellt. Schwierigkeiten den Tumor bei der Rontgenuntersuchung und der Endoskopie aufzufinden, verzogern mehrmals die Diagnosestellung. Charakteristische Symptome und Beschwerden finden sich nicht. Die Diagnose konnte friiher und ohne aufwendige Untersuchungsmethoden gestellt werden, wenn an die Moglichkeit des Vorliegens eines Tumors besonders in Fdlen mit Oesophagusdilatation und normaler Offnung der Cardia gedacht wird. Die Bedeutung der Endoskopie mit Fiberoptik fur die friihe Diagnosestellung wird betont. Die Therapie besteht in den meisten Fallen in der Exstirpation durch eine zervikale Oesophagotomie.

REFERENCES Burrel, M . & Toffler, R. 1973. Fibrovascular polyp of the esophagus. Digestive Diseases 18, 714. Liljequist, B. & Wiberg, A. 1974. Pedunculated tumours of the esophagus. Acta Radio1 (Diagn) (Stockh) 15, 383. McBride, Andrew F. Jr. 1951. Benign polypoid tumours Of the esophagus. Cancer 4~ ’08. Moersch, H. J . & Harrington, S. W. 1944. Benign turnours of the esophagus. Ann Otol Rhino1 Larynpol

Stein, G. N. & Finkelstein, A . K. 1973. The Esophagus and Stomach. Year Book Medical Publishers Inc., Chicago. Uthgenannt, H., Stromlid, A . & Zwad, H.-D. 1973. Die Rontgenuntersuchung des Osophagus in BuscopanHypotonie. Fortschr G e b Rontgenstr Nuklearmed 119, 10.

A. Elner, M.D. Dept. of Otolaryngology University Hospital S-22185 Lund Sweden

Pedunculated intraluminal fibro-lipoma of the oesophagus. Case report.

Acta Oto-Laryngologica ISSN: 0001-6489 (Print) 1651-2251 (Online) Journal homepage: http://www.tandfonline.com/loi/ioto20 Pedunculated Intraluminal...
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