Postgraduate Medical Journal (June 1980) 56, 431-434

A case of leiomyoma of the oesophagus complicated bysuperior vena cava obstruction and associated eosinophiliaP. HICKLING K. BUKSHB.Sc., M.R.C.P. M.B. B.S.

P. BECKM.D., M.R.C.P.

Departments of Medicine and Thoracic Surgery, Llandough Hospital, and University Hospital of Wales,Cardiff

SummarySuperior vena cava obstruction occurring as a com-plication of leiomyoma of the oesophagus has notbeen reported before. Such a case is recorded whichwas associated with striking eosinophilia, a featurepreviously noted in cases of uterine leiomyomas.

Case reportA 35-year-old merchant seaman had complained

of intermittent epigastric pain, water brash andheartburn for approximately a year. These symptomswere usually associated with drinking bouts in whichhe frequently and freely indulged, but which he hadrecently curtailed. He had 3 episodes of haematemesisduring the year, each being associated with drinking.For 6 months he had noticed increasing dysphagiafor solids and he occasionally regurgitated solid food.In March 1978 he had a barium meal examinationwhich showed a smooth compression of the oeso-phagus measuring 7 cm x 5 cm posteriorly and to theright side of the aortic arch (Fig. 1). This investiga-tion was repeated with tomography and confirmeda large extrinsic impression on the right side of theoesophagus. No hilar gland enlargement wasidentified. At this time his white cell count was8-2 x 109/1, with a normal differential. He was await-ing further investigation when in May he presentedas an emergency with a 6-day history of increasingswelling of his left arm, left hand and his face.On examination, there was pitting oedema of the

dorsum of his left hand and a firm swelling of thewhole of his left arm. Both supra-clavicular fossaeand the suprasternal notch were obliterated. Therewas swelling over the neck, face and anterior chestwall, and moderate conjunctival suffusion. Thejugular veins were distended to the level of the earlobes and were non-pulsatile. The retinal veins were

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FIG. 1. Barium meal examination-the leiomyoma isseen compressing the right side of the oesophagus.

0032-5473/80/0600-0431 $02.00 ( 1980 The Fellowship of Postgraduate Medicine

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432 Case reports

engorged but there was no papilloedema. Generalexamination was normal and in particular there wasno lymphadenopathy. His peripheral pulses werenormal, blood pressure 120/70 mmHg in both arms,and there were no abnormal signs in the chest orabdomen.

Investigations at that time were as follows: Hb,16-6 g/dl; white cell count, 23-1 x 109/1; (15% eosino-phils, 66% neutrophils, 15% lymphocytes, 1 %monocytes and 3% myelocytes).Bone marrow cytology showed a highly cellular

marrow with a striking degree of eosinophilia,constituting 65% of the total cells of which eosinophilmyelocytes constituted 20%, eosinophil metamyelo-cytes 40% and mature eosinophils 40% of the totaleosinophil count. His Mantoux test was negative at adilution of 1/10000.

Management and progressIt was decided to treat his superior vena caval

obstruction with steroids. He was given prednisone40 mg/day, and the signs in his face, neck and leftarm resolved completely over the next 48 hr. Theeosinophilia increased over this period with theWCC rising to 365 x 109/1, of which 28% wereeosinophils. Upper gastro-intestinal endoscopy wasperformed after the superior vena caval obstructionhad settled. This revealed a smooth swelling approxi-mately 3 cm in diameter, impinging on the oeso-phageal lumen at 25 cm. The mucosa overlying itwas normal and there was no pulsation visible. At35 cm there was a sliding hiatus hernia. The endo-

scope passed easily into the stomach which wasnormal in appearance.A right thoracotomy was performed (K.B.).

There was no mediastinal lymphadenopathy butthere was a round, shiny tumour, 6-5 cm in diameter,within the right antero-lateral wall of the oesophagus.The trachea was deviated to the right and the tumourwas bulging forwards closely adjacent to the junctionof the innominate veins, the azygos vein and thesuperior vena cava (Fig. 2). The tumour was easilyenucleated from the oesophageal muscle leaving themucosa intact. Treatment with prednisone wasstopped after 2 weeks.

Histology of tumourMacroscopic appearance - a rounded encapsulated

tumour, rubbery in consistency. The cut surface wasglistening and white with areas of degeneration atboth poles.

Microscopic appearance - the appearance was thatof a leiomyoma with areas of necrosis. There weremany focal and perivascular collections of eosino-phils (Fig. 3).The patient made an uneventful recovery. Post-

operative barium swallow showed normal oesopha-geal motility with no evidence of leakage. The patientwas soon swallowing normally. His differentialblood count returned to normal. A superior venacavagram with injection of contrast into both armswas performed 2 weeks after surgery and this demon-strated patent central veins with no evidence ofinvasion, displacement or thrombus. He remains

Oesophagus

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Ascending aorta

FIG. 2. Diagram showing position of leiomyoma as seen at right thoracotomy.

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FIG. 3. Photomicrograph of leiomyoma showing intense infiltrate of eosinophils ( x 80).

perfectly fit, with no recurrence of his superior venacaval obstruction and his peripheral blood countis entirely normal, 6 months after removal of theoesophageal leiomyoma.

DiscussionLeiomyomas are the commonest benign tumours

of the oesophagus (Seremetis et al., 1975; Mansour,Hatcher and Huan, 1977). Symptoms, if any, areusually confined to the local effects of pressure onthe oesophageal lumen, such as dysphagia andchest pain. Acid reflux, usually seen in cases withassociated hiatus hernia, as in this case, is also acommon complaint. Weight loss, presumably due todysphagia, is a less common symptom. Complica-tions of oesophageal leiomyomas are rare, butmalignant change, haemorrhage and asphyxiahave all been reported (Seremetis et al., 1975).So far as the authors know, this is the first case inwhich potentially lethal obstruction of the superiorvena cava has been reported. No other lesion couldbe found to account for the obstruction eitherradiologically, endoscopically or at surgery.

It was initially suspected that the patient had amediastinal malignancy, possibly a lymphoma, in

view of the peripheral blood eosinophilia. Theprompt response of the obstruction to steroidsstrengthened these suspicions, but whether thisresponse was coincidental or due to a direct localeffect on the tumour and/or associated oedema ofthe surrounding tissues, is not known. No othercause for the eosinophilia was discovered despitean exhaustive search. He was not taking any drugsknown to cause eosinophilia.The most common haematological abnormality

associated with leiomyomas, iron-deficiency anae-mia excepted, is polycythaemia (Payne, Woods andWrigley, 1969). A leucocytosis in the presence of aninfected and/or necrotic leiomyoma sometimesoccurs (Buka, 1965). Although well recognized inassociation with malignancy, eosinophilia is onlyrarely associated with benign tumours (Isaacson andRapaport, 1946; Murray, 1953). Buka (1965)reported 2 cases of uterine leiomyomas associatedwith eosinophilia which resolved after hysterectomy.In neither case was the eosinophilia as marked as inthe present case. The extensive eosinophilic in-filtration of the tumour itself is also interesting in thiscase with respect to the peripheral blood and bonemarrow eosinophilia. In the cases reported by Buka,

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no areas of acute or chronic inflammation werepresent in the uteri, nor was there any collection ofeosinophils in the leiomyomas themselves, and noreport is made of areas of necrosis, in contra-distinction to the histological findings in the presentcase. The stimulus for the eosinophilia in all 3 casesmust remain conjectural but, like the polycythaemiasometimes associated with leiomyomas, the eosino-philia has promptly resolved when the leiomyomaswere removed (Rothman and Rennard, 1963).Eosinophilic infiltration of leiomyomas is not afeature which has hitherto been noted but eosino-philic infiltration of necrotic malignant tumours hasbeen reported (Isaacson and Rapaport, 1946). It isthought unlikely, however, that in the case ofleiomyomas, the necrosis alone is responsible for theeosinophilia, as necrotic degeneration, often as-sociated with haemorrhage, is a common event inleiomyomas of the uterus and eosinophilia is notusually seen in these cases. Buka speculates that inhis cases the eosinophilia is due to an auto-immuneresponse to the leiomyomas which is manifested bythe eosinophilia. It is not clear how this auto-immune response may be mediated but it is anattractive hypothesis to explain the puzzling associa-tion between leiomyomas and eosinophilia. Thepresent authors can only conclude, as Buka does,

that leiomyomas should be included in the differen-tial diagnosis of any unexplained eosinophilia.

It appears, therefore, that the present case is oneof leiomyoma of the oesophagus complicated bysuperior vena cava obstruction and associated withmassive eosinophilia involving the tumour itself,the bone marrow and the peripheral blood.

ReferencesBUKA, N.J. (1965) Eosinophilia associated with uterine

leiomyomas. Canadian Medical Association Journal, 93,163.

ISAACSON, N.H. & RAPAPORT, P. (1946) Eosinophilia inmalignant tumors: its significance. Annals of InternalMedicine, 25, 893.

MANSOUR, K.A., HATCHER, C.R. & HUAN, C.L. (1977)Benign tumors of the esophagus: Experience with 20 cases.Southern M.edical Journal, 70, 461.

MURRAY, R.C. (1953) The use of the absolute eosinophilcount in the diagnosis of neoplasms. New England Journalof Medicine, 248, 848.

PAYNE, P., WOODS, H.F. & WRIGLEY, P.F.M. (1969) Uterinefibromyomata and secondary polycythaemia. Journal ofObstetrics and Gynaecology of the British Commonwealth,76, 845.

ROTHMAN, D. & RENNARD, M. (1963) Myoma-erythrocytosissyndrome. Obstetrics and Gynecology, 21, 210.

SEREMETIS, M.G., LYONS, W.S., DEGUZMAN, V.C. & PEABODY,J.W. (1976) Leiomyomata of the esophagus. An analysisof 838 cases. Cancer. New York, Philadelphia, etc., 38,2166.

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