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LEIOMYOSARCOMA OF THE RECTUM

BY GWENDOLINE SMITH SOUTH LONDON HOSPITAL FOR WOMEN AND CHILDREN

SARCOMA of the large bowel is seen infrequently, unlike carcinoma which Occurs commonly. Sarco- mats in th is region of the gastro-intestinal tract behave differently from carcinomata, and study of the literature on the subject makes it Seem worth while to put on record a single case. This report confirms the views of many surgeons of the extreme malignancy of this tumour and illustrates the clinical characteristics and methods of treatment.

CASE REPORT

Operation was performed on 9 Nov., an attempt being made to carry out synchronous combined abdomino- perineal resection. The growth was found to surround the rectum and to be attached to the lateral wall of the pelvis; it was obvious that radical extirpation was impossible, but the main tumour mass was removed and colostomy performed.

The case of leiomyosarcoma to be described was a female patient of 72 years. Mrs. B. first attended hospital in May, 1961, complaining of haemorrhoids and a ‘lump coming out of the back passage’. She had bleeding, occurring usually when this lump was replaced. The duration of these symptoms was 4-5 months. There was nothing of significance in family or past history, but she suffered also from arthritis of the hips due to Paget’s disease.

O N ExAMINATIoN.-The patient was obese, flabby, and pale. No abnormality was found on general or abdominal examination. Rectal examination revealed a large, soft, bilobed, pedunculated polyp covered by intact mucosa and situated just inside the anal canal on the right lateral wall. Barium-enema examination showed a filling defect at this site, but no other disease in the colon. Haemoglobin estimation result was only 55 per cent (9.1 gm.).

AT OPERATION.-On 7 June, 1961, the polyp was excised. It was found that there was no true stalk and the mass had stretched the mucous membrane which had become ulcerated.

Mrs. B. made a good recovery and her anaemia was soon corrected with intravenous and oral iron therapy. In view of the histology report she was advised to undergo further operation, but she had an invalid husband whom she could not leave, and as she felt well and symptom- free she refused to return to hospital. When she reported in August there was a tender indurated ridge at the site of excision of the polyp. In October this area had increased in size and bled easily on examination. By November there was a large palpable mass, which was bleeding, and Mrs. B. agreed to come into hospital for another operation. At this time radiography of the chest was clear and no enlarged inguinal glands were detected. The possibility of deep X-ray therapy, as an alternative to surgery, was considered and rejected.

FIG. 700.-Polyp of rectum.

PROGRESS.-After a stormy convalescence Mrs. B. was discharged on 23 Dec., to spend Christmas with her family. At this time no growth could be felt per vaginam and no glands were palpable in the groins. Two weeks later, on 9 Jan., 1962, she reported to our clinic complaining of a swelling in the right groin which she had just noticed. There was an undoubted malignant gland here and pelvic examination revealed a mass on the right side. On I Feb. she had to be readmitted with subacute intestinal ob- struction. She had also a cough and vaginal bleeding. As it was obvious that she had diffuse metastases in the abdomen and pelvis and radiography revealed malignant deposits in the lungs, palliative treatment only was possible. Chemotherapy was not discussed but would probably have been ineffective, for the patient’s condition deteriorated rapidly and she died on 20 Feb., just 9 months from the time of her first artendance at hospital. It is interesting to speculate whether the prognosis could have been altered had the patient agreed to radical operation immediately after the biopsy. This seems unlikely in view of the rapid recurrence and spread of the tumour. If the diagnosis had been possible without biopsy, and radical extirpation performed when the patient was first seen, her chances might have been better.

PATHOLOGY (Dr. Anne Gibson).- I. PoZyp (Fig. 700) 13 June, 1961.-The polyp is

bilobed and measures 8 cm. in its longest axis. The distal

634 T H E B R I T I S H J O U R N A L O F S U R G E R Y

pole is haemorrhagic and covered with fibrino-purulent (Figs. 701, 702): This shows a leiomyosarcoma. Parts exudate. The sides are covered with normal mucosa. of the tumour are well differentiated but pleomorphic. The cut surface is firm and homogenous. Microscopy Giant cells are often multinucleated and there are

FIG. 701.--Photomicrograph of leiomyosarcoma. H. and E. FIG. 702.-High-power photomicrograph of tumour. H. and E. ( x 120.) ( X 300.)

many other cells showing mitotic figures throughout the section.

2. Operation Specimen (Fig. 703).-This is 30 cm. in length and consists of anus and rectum and part of colon. Just inside the anal canal a hard swelling protrudes 3 3 an. and is almost entirely covered with mucous membrane. The growth extends 4 cm. laterally into the perirectal fat and here measures 7 cm. in length.

There are many hard white enlarged lymph-nodes in fat and mesocolon.

Microscopy: Sections of growth, perirectal tissues, and lymph-nodes all show the same structure as the POlYP.

Post-mortem Examination (22 Feb., 1962).-External inspection showed masses in both groins.

The abdomen revealed extensive malignant disease. The mesentery and omentum were thick and rigid. The parietal and visceral peritoneum were studded with hard white secondary nodules so that the coils of small intestine were all firmly adherent to each other. The liver and colon were covered with deposits. In the pelvis there was a hard mass very adherent to the wall of the pelvis, involving a small area of the posterior wall of bladder and surrounding the lower end of the right ureter.

There were deposits in the lungs, liver, and inguinal lymph-nodes, and sections confirmed that these metastases were of the same type of leiomyosarcoma seen in the Polyp.

DISCUSSION Incidence.-Although only 50 or 60 cases of

leiomyosarcoma of the rectum have been reported, it is possible to formulate a reasonably clear description

perirectal invasion. of this condition. The age-distribution corresponds FIG. 703.-Operation specimen showing tumour in rectum and

L E I O M Y O S A R C O M A

to that of carcinoma of the rectum; the sex incidence is of no significance in a small series.

Clinical History.-This neoplasm is nearly always found in the lower third of the rectum, so symptoms occur early but may be ascribed to haemor- rhoids. The most significant complaints are of bleeding, a swelling within the anal canal which may prolapse, and, less frequently, obstruction. Examina- tion may reveal a polyp, a mass inside the rectum under intact mucosa, or an ulcerated haemorrhagic swelling. It is unlikely that the regional lymph- nodes will be enlarged, but, at a relatively early stage, some perirectal thickening will be palpable.

Diagnosis.-When a polyp is removed, it may well be considered to be just a simple condition and only careful histological examination will reveal that it is a leiomyoma and of grave significance. If a submucosal swelling with perirectal thickening is palpated, there is the possibility of a sarcoma-either lymphomatous (when other evidence of recticulosis must be sought) or a fibro- or leiomyosarcoma. The relationship between simple and malignant leio- myomata is by no means well defined and it is probably safer to regard the condition as serious, even if not reported as malignant.

Treatment.-It is generally agreed that radio- therapy is of no value. Surgery offers the only rational method of treatment, and the extent of resection depends on the local condition and pathologist’s report. When a small and apparently simple polyp or submucosal tumour has been excised, radical removal should be considered if any evidence of pre-malignant or malignant changes are seen in the sections. Each case warrants careful review by surgeon and pathologist.

Prognosis.-This is difficult to determine. In a series of 14 cases reported from St. Mark’s Hospital (Morson, 1960) the survival rate was reasonably good, but the tumours were well differentiated and of a low grade of malignancy. The same extensive local spread was noted in these cases and considered to be characteristic of these tumours. Most other authors report a very poor prognosis and death within a year (Thorlakson and Ross, 1961; Jay, 1958). Undoubtedly the factors of early diagnosis, radical resection with wide removal of perirectal tissues, and a growth presenting a highly differ- entiated cell pattern will contribute to a longer survival, but the prospect of 10-year ‘cure’ is remote.

Pathology.-The interest in these tumours of plain muscle is partly in their rarity in the rectum compared with the stomach. In the rectum the majority are malignant and in the stomach most are simple. The surgeon who has seen one of these cases may make a diagnosis on clinical evidence, but more usually histology will reveal the serious nature of the disease. Simple leiomyomata and anaplastic sarco- mata may be easily identified, but between these two groups will be turnours of doubtful malignancy. Sanger and Leckie (1959) reviewed the literature on this aspect of the condition and their report is the

O F T H E R E C T U M 635

latest on the subject in the BRITISH JOURNAL OF SURGERY. The most recent article appears to be one by Sanders (1961) in the Frederick A. Coller Com- memorative Issue of the Annals of Surgery. Sanders states that only 36 cases have been reported, but this number does not, it seems, include the 14 cases of Morson (1960). Sanders comments on the variable microscopic picture, and confirms that irradiation is of no value and that wide surgical excision is the only available treatment. He states that lymphogenous metastases have never been demonstrated, an aspect of this condition which the case presented here dis- proves, but he agrees with the conclusions as to the rarity of the tumour and its poor prognosis.

Fibrosarcomata present the same clinical fea- tures and histological interest as leiomyosarcomata (Stoller and Weinstein, 1956)~ whereas lympho- sarcomata demand quite different consideration clinically and histologically (Ray, Hines, and Hanley, 1957). All these sarcomata of the rectum form a small but important group of rectal malignancies.

SUMMARY I. A case is presented of leiomyosarcoma of the

rectum occurring in a woman of 72 years. 2. The clinical and pathological features of this

rare tumour are discussed. 3. The difference in the behaviour of this neo-

plasm from that of carcinoma is noted, particularly the high grade of malignancy and the rapid local spread round the rectum rather than metastases to glands and liver.

Acknowledgements.-I am very grateful to Sir Cecil Wakeley for his encouragement and help.

I am greatly indebted to my colleague, Dr. Anne Gibson, not only for pathological reports, but also for her assistance in the preparation of specimens and sections for photography and her constant interest in the care of this patient and the preparation of this paper.

Dr. T. M. Robb kindly saw the patient at Lam- beth Hospital and advised me that the condition would not respond to radiotherapy.

The photographs were taken by Mr. E. V. Will- mott, Imperial Cancer Research Fund Photographic unit.

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