carcinoma of the œsophagus with histological appearances simulating a ‘carcinosarcoma’

3
CARCINOMA OF THE ESOPHAGUS 487 CARCINOMA OF THE (ESOPHAGUS WITH HISTOLOGICAL APPEARANCES SIMULATING A ' CARCINOSARCOMA ' BY RODNEY SMITH AND N. F. C. GOWING, LONDON THE occurrence of a true carcinosarcoma is rare in human pathology and such a diagnosis should be made with caution. The possibility of the ' sarcomatous ' element being merely an anaplastic component of the epithelial tumour must always be carefully excluded. Even where more specialized mesenchymal tissues such as bone and cartilage are present, the possibility of these having resulted from metaplasia in the non-neoplastic stroma must be considered. These points have been stressed by several authors (Trudhope and Chisholm, 1934 ; Willis, 1948). Nevertheless, a few reported cases, such as the two tumours of the breast described by Govan (1945), rarer. The case to be described is one in which the latter tumour was simulated by a carcinoma present- ing unusual histological features. CASE REPORT Mrs. M. M., aged 53, had been in good health until three months previously, when she had begun to have difficulty in swallowing solids. The dysphagia increased progressively and she was now having difficulty even with liquids. She had lost z st. in weight and also complained of lassitude and a recent cough which kept her awake at night. Physical examination revealed a minute, wasted lady weighing 59 st. Apart from the obvious loss of weight, examination provided merely negative evidence and there FIG. ~57.-Pre-operative barium swallow. appear to be genuine examples of carcinosarcoma. In Govan's cases the sarcomatous moiety appeared to be derived from striated muscle. A few cases of true mixed tumours have also been reported in the thyroid, but more commonly in dogs than in man (Rudduck and Willis, 1938). Malignant obstruction of the oesophagus is now routinely treated when possible by oesophagectomy, and in examining the increasing numbers of excised tumours one should remain alive to the possibility of an epithelial tumour exhibiting, in this region as elsewhere, areas suggesting a tumour of mesenchymal origin. As in all surgery, it is useful to remember that unusual manifestations of common lesions are more often encountered than the classic manifesta- tions of rare lesions. Sarcoma of the oesophagus is a very rare lesion and carcinosarcoma must be even was no detectable secondary metastasis from the suspected carcinoma. Barium swallow (Fig. 557) showed a constant large filling defect, the esophagus being expanded by an intraluminal tumour, and an area of stenosis directly above it. Esophagoscopy confirmed the presence of a friable tumour and the report on a piece taken for biopsy was : " Histological examination s F w s a poorly differentiated squamous-celled carcinoma . Radiographs of the lungs and bronchoscopy revealed no abnormality. A mild secondary anremia was corrected before operation, during which a slow drip transfusion of whole blood was maintained. Anzsthesia was induced with pentothal and maintained with intratracheal nitrous oxide, oxygen, and cyclopro- pane, supplemented by curare. AT OPERATION.-A long incision was made following the sixth left interspace and posteriorly extending

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C A R C I N O M A O F T H E E S O P H A G U S 487

CARCINOMA OF THE (ESOPHAGUS WITH HISTOLOGICAL APPEARANCES SIMULATING A ' CARCINOSARCOMA '

BY RODNEY SMITH AND N. F. C . GOWING, LONDON

THE occurrence of a true carcinosarcoma is rare i n human pathology and such a diagnosis should be made with caution. T h e possibility of the ' sarcomatous ' element being merely a n anaplastic component of the epithelial tumour must always be carefully excluded. Even where more specialized mesenchymal tissues such as bone and cartilage are present, the possibility of these having resulted from metaplasia in the non-neoplastic stroma must be considered. These points have been stressed by several authors (Trudhope and Chisholm, 1934 ; Willis, 1948).

Nevertheless, a few reported cases, such as the two tumours of the breast described by Govan (1945),

rarer. The case to be described is one in which the latter tumour was simulated by a carcinoma present- ing unusual histological features.

CASE REPORT Mrs. M. M., aged 53, had been in good health until

three months previously, when she had begun to have difficulty in swallowing solids. The dysphagia increased progressively and she was now having difficulty even with liquids. She had lost z st. in weight and also complained of lassitude and a recent cough which kept her awake at night.

Physical examination revealed a minute, wasted lady weighing 59 st. Apart from the obvious loss of weight, examination provided merely negative evidence and there

FIG. ~57.-Pre-operative barium swallow.

appear to be genuine examples of carcinosarcoma. In Govan's cases the sarcomatous moiety appeared to be derived from striated muscle. A few cases of true mixed tumours have also been reported in the thyroid, but more commonly in dogs than in man (Rudduck and Willis, 1938).

Malignant obstruction of the oesophagus is now routinely treated when possible by oesophagectomy, and in examining the increasing numbers of excised tumours one should remain alive to the possibility of an epithelial tumour exhibiting, in this region as elsewhere, areas suggesting a tumour of mesenchymal origin. As in all surgery, it is useful to remember that unusual manifestations of common lesions are more often encountered than the classic manifesta- tions of rare lesions. Sarcoma of the oesophagus is a very rare lesion and carcinosarcoma must be even

was no detectable secondary metastasis from the suspected carcinoma.

Barium swallow (Fig. 557) showed a constant large filling defect, the esophagus being expanded by an intraluminal tumour, and an area of stenosis directly above it.

Esophagoscopy confirmed the presence of a friable tumour and the report on a piece taken for biopsy was : " Histological examination s F w s a poorly differentiated squamous-celled carcinoma .

Radiographs of the lungs and bronchoscopy revealed no abnormality. A mild secondary anremia was corrected before operation, during which a slow drip transfusion of whole blood was maintained.

Anzsthesia was induced with pentothal and maintained with intratracheal nitrous oxide, oxygen, and cyclopro- pane, supplemented by curare.

AT OPERATION.-A long incision was made following the sixth left interspace and posteriorly extending

488 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

upwards to the level of the third rib. The sixth inter- and the exposed mediastinal cellular tissue were dusted space was opened into the pleural cavity and posteriorly, with penicillin-sulphathiazole powder and the thorax in order to gain adequate access, I in. of each of the fourth closed with a rubber-tube drain inserted through a stab to the eighth ribs was excised. When the mediastinum incision in the eighth interspace. This was later connected

FIG. 5sg.--Gross appearance of the tumour. (Natural size.)

to an under-water seal. The condition of the patient remained good throughout the operation.

PROGRESS.-~OSt-OperatiVely, fluids were allowed by mouth after the first 24 hours and feeds increased until

FIG. 558.-E'ost-operative barium swallow.

was entered and the tumour palpated, it was found to extend in its upper part to the lower margin of the arch of the aorta. It was, however, mobile, and it was con- sidered that it could be removed in its entirety.

The diaphragm was now split radially from the hiatus and the peritoneal cavity opened. No intra-abdominal secondary deposits could be found. The stomach was extensively mobilized, dividing the lesser omentum, gastro-splenic ligament, and gastro-colic omentum, but preserving the right gastric and right gastro-epiploic vessels and the gastro-epiploic arcade.

The oesophagus was mobilized up to the arch of the aorta and, after incision of the pleura above the arch, completely mobilized behind the arch and above it to the level of the manubrium sterni. It was now divided just above the aortic arch and the whole of the oesophagus distal to this point removed together with the cardia of the stomach and adjacent lesser curvature with the left gastric group of lymph-glands.

The incision into the lumen of the stomach so made was closed and the fully mobilized viscus drawn up into the chest in front of the aortic arch, the fundus reaching

A

B

C

beyond the cut proximal oesophagus without difficulty. FIG. ~6o.-Microscopical appearance of the tumour. Low I t was anchored in this situation with a number of power. (H. and 8.) A, Anaplastic carcinoma cells lying in interrupted silk sutures to the parietal pleura, and the SfrOma and producing a 'sarcomatous' appearance; 6, C, esophagus, lying upon its anterior surface, was anasto- Groups Of

mosed to it by end-to-side anastomosis, the redundant stomach being wrapped around the anastomosis to soft solids were taken after ten days and a normal diet enclose it completely. Interrupted silk sutures on after three weeks. There was little drainage from the eyeless needles were used throughout. The junction intercostal tube, which was removed after 48 hours.

cancer

C A R C I N O M A O F T H E C E S O P H A G U S 489 Some patchy atelectasis on both sides resolved spon- taneously without recourse to bronchoscopy. Conval- escence was otherwise uneventful and the patient was discharged to a convalescent home after three and a half weeks. A post-operative barium swallow (Fig. 558) showed a normally functioning stoma.

She remained in good health fot a little under three years, when recently renewed dysphagia occurred.

A A

FIG. 561.-Microscopical appearance of the tumour. High power. (H. and E.) A, Compact group of well-differentiated cells ; 6, Loosely arranged anaplastic cells lying in stroma. .

a ' carcinosarcoma '. This suggestion is maintained in sections stained by Van Giesen's method and in those impregnated for reticulin. The reticulin outlines the well-differentiated groups within which there are very few argyrophil fibrils. By contrast the stroma containing rhe anaplastic cells shows a close meshwork of reticulin as seen in many cases of sarcoma. Nevertheless, careful examination reveals the identity and continuity of the

Investigation has now revealed recurrence of an anaplastic carcinoma in the oesophageal stump.

PATHOLOGICAL REPORT.-The report on the excised oesophagus was as follows :-

Macroscopical Appearance.-The tumour is roughly ovoid in shape, measuring 5.5 cm. in length and 2 3 cm. in its greatest diameter, tapering towards its lower extremity. It is attached to the esophageal wall by a short narrow pedicle around the base of which malignant infiltration of the wall can be seen. This pedunculated variety of oesophageal carcinoma, although well recog- nized, is distinctly rare. (Fig. 559.)

Histology.-Examination of sections from the opera- tion specimen confirm the diagnosis by biopsy of squamous-celled carcinoma. There are, however, one or two additional features of interest.

Firstly, the degree of differentiation in the tumour varies greatly, not merely from one block of tissue to the next but even in different parts of the same histological section, well-differentiated and anaplastic components of the carcinoma being seen side by side in the same micro- scopical field. The differentiated areas form circum- scribed islands, some of which show keratinization at their centres. The anaplastic cells are distributed singly or in small clumps in the fibrovycular stroma, thus giving the latter a sarcomatous appearance. This combination of well-differentiated and poorly differen- tiated elements produces a histological pattern which suggests a mixed epithelial and mesenchymal twnour-

B

FIG. c6z.-Microsco~ical anoearance of the tumour. Hinh ~~ . .. ~ ~~

power. (Reticulin impregnation.) A, Anaplastic carcinoka lying in a network of argyrophil fibres ; 6, lpithelial groups of well-differentiated carcinoma sharply outlined by reticulin.

tumour elements in the two types of tissue, for several sections show clear transition betyeen the epithelial ' islands ' and the anaplastic ' stroma . At the periphery of the differentiated groups the cells can be seen to become spindle-shaped, to break away from the main mass and pass in amongst the fibroblasts and vessels of the support- ing tissue. There would thus seem to be no doubt regarding the true histological interpretation. (Figs.

A second point of interest is the remarkable absence of necrosis even at the very tip of the pedunculated tumour despite the very narrow stalk.

Sections through the oesophagus at the base of the pedicle show invasion of the wall and surrounding connective tissue.

In view of the pathological report, the length of survival must be considered unusual.

560-562.)

REFERENCES GOVAN, A. D. T. (1945), J . Path. Bact., 57, 397. RUDDUCK, H. B., and WILLIS, R. A. (I938), Amer. J .

Cancer, 33, 205. TRUDHOPE, G. R., and CHISHOLM, A. E. (1934), 3.

Obstet. Gynrec., Brit. Emp., 41, 708. WILLIS, R. A, (1948), Pathology of Turnours. London :

Butterworth.