Indian Journal of Thoracic and Cardiovascular Surgery 1985-86; 4:92-94

C l i n i c o - p a t h o l o g i c a l C o n f e r e n c e - 4

A 9-year-old boy was hospitalised with complaints of progressive dysphagia, recurrent bouts of regurgitation and occasional retrostemal pain over a period of two years. He also had bilateral congenital cataract and partial deafness. His chest x-ray had a smooth- edged homogenous opacity in the right hemithorax (Fig. I a) and barium swallow examination showed an enormously dilated proximal oesophagus with distal narrowing (Fig.lb).

Fig. 1 (a) X-ray chest: posteroanterior view and (b) barium oesophagogram

93

Oesophageal Leiomyoma in a Child AMIT BANERJEE ~ USHA KIRAN +, VEENA MALHOTRA ~', BK GUPTA*, DALJEET GHAMIIEER*, S GAIND*, e s NARAYANAN ~"

KEY WORDS : esophagus, esophageal neoplasms, leiomyoma

OPERATIVE FINDINGS

Left thoracotomy revealed a well-encapsulated spherical tumour about 10 cm in diameter arising

from the lower oesophagus. It was eccentrically located extending between the pulmonary hilum and the cardia just across the oesophageal hiatus. We had to incise the diaphragm to facilitate delivery of the tumour into the chest (Fig. 2). A submucosal enucleation could be performed without difficulty and the patient made an uneventful recovery. He had complete relief from symptoms though the oesophageai dilation persisted. Histopathologically the tumour turned out to be leiomyoma.

Fig. 2 Operative photograph showing the oesophageal tumour extending across the hiatus

"Cardiothoracic Surgeon. +Senior Resident. "***Associate Professor, "Professor and Head of the Department.

From the Department of Cardiothoracic Surgery'*", Anaesthesiology +*" and Pathology'-, GB Pant Hospital, New Delhi, India.

Address for correspondence : Dr. Amit Banerjee, Department of Cardiothoracic Surgery, JIPMER, Pondicherry 605 006, India.

DISCUSSION

Leiomy.oma is the most common benign tumour of the oesophagus *'2. Its histologic characteristics were first enumerated by Virchow * in 1863. It is a rarity in children, the average age of presenta- tion being 44 years ~.2. We could not find any instance of oesophageal leiomyoma in a patient under 12 and possibly ours is the youngest patient till date.

The majority of cases arise in the lower third of the oesophagus with simultaneous involvement of the stomach in about 10 per cent. Less commonly, they may be located in the proximal oesophagus or present as multiple lesions 35.

The commonest presenting symptoms are related to oesophageal obstruction and stasis. However, nearly half the cases with even large tumours remain a symp- tomatic H. This could be 1 of the reasons accoun- ting for their late presentation. Upto 55 per cent of these tumours may not be visualised radiographically 6.

94 Banerjee et al

The characteristic barium swallow demonstrates a smooth defect with no proximal dilatation 2-7. Occa- sional unexpected degree of oesophageai dialatation has been attributed to a very slow progression of the tumour 4 or rarely due to encircling of the lower end of oesophagus by the tumour producing an achalasia- like picture 4-8. In our patient, dilatation of the proxi- mal oesophagus was associated with an eccentrically located lesion. Slow progression of tumour is unlikely to be responsible for such enormous proximal dilatation. It is probable that location of the tumour across the hiatus led to obstruction of the oesophageal lumen resulting in proximal dilation.

Congenital cataract with partial deafness seen in the present case are found in rubella syndrome. They may be coincidental.

The first surgeon to remove an oesophageal leio- myoma was Sauerbruch (1932) 6. Transthoracic sub- mucosal enucleation is preferred and usually possible in 9 out of 10 cases. Oesophageal resection is usually reserved for annular tumours or those exceeding 8 cm in size. It may also be required in the event of mucosal tethering or accidental injury to the oeso- phageal wall 1,2. In our patient, submucosal removal of the tumour was possible inspite of its large size.

The present case is reported in view of its unusual presentation in possibly the youngest patient reported so far.

References

1. SEREMETIS MG, LYONS SW, DE GUZMAN VC, PEABODY JW. Leiomyomata of the esophagus: an analysis of 838 cases. Cancer 1976; 38: 2166-77.

2. GALLINGER S, STEINHARDT MI, GOLDBERG M. Giant leiomyoma of the esophagus. Am J Gastroenterol 1983; 78:708-11.

3. SCHABEL SI, RrrrENBERG GM. Esophageal perforation secondary to benign leiomyoma. Southern Med J 1980; 70: 84-5.

4. BARREmO F, SECO JL, MOLINA J, VILLAMOR J. Giant esophageal leiomyoma with secondary megaeso- phagus. Surgery 1976; 79: 436-9.

5. HICKLXNG P, BUKSH K, BECK P. A case of leiomyoma of the oesophagus complicated by SVC obstruction and associated eosinophilia. Postgrad Med J 1980; 56:431-4.

6. Kos'rlAINEN S, VIRKKULA L, TEPPO L. Smooth muscle tumours of the esophagus. Cardiovasc Surg 1973; 7: 98-103.

7. SCHAZZKI R, HAWES LE. Tumors of the oesophagus below the mucosa and their roentgenological differ- ential diagnosis. Rev Gastroenterol 1950; 17: 991-1014.

8. Tsuzuga T, KAKEGAWA T, ARIMORI M et al. Giant leiomyoma of the esophagus and cardia weighing more than 1000 grams. Chest 1971; 60: 396-9.