Carcinoid tumors of the rectum

CARCINOID TUMORS OF THE RECTUM EDMOND F . COHEN, M .D. Denver, Colorado

HE subject of carcinoid or argentaffm tumors of the gastrointestinal tract has been well reviewed in a number of excellent monographs .' -4 Carcinoids involving the large bowel and particularly the rectum have received somewhat less attention . The fact that rectal carcinoid tumors are being found with increasing regularity, coupled with controversy regarding etiology and the very nature of the tumor makes the subject interesting and opens the way for further research . The tumor usually occurs in the gastrointestinal tract, most commonly in the appendix and the small intestine, but has also been reported as occurring in the stomach, gallbladder, ovarian teratomas, Meckel's diverticulum and the mesentery . It is interesting to note that the majority of cases of rectal carcinoids have been detected during the last four years . The findings of Ehrlich and Hunter, 5 who in a survey of 813 gastrointestinal tumors in patients of military age determined ten of these to be carcinoids of the rectum, further substantiates the fact that these tumors are probably more commonplace than was formerly believed . To further demonstrate this point, Jackman at the Mayo Clinic reported four carcinoid tumors in a series of eighty-seven consecutive patients presenting submucosal rectal masses . The subject has in the past been exploited mainly by the pathologist whose interest in the morphologic and histologic aspect has somewhat overshadowed the clinical aspect of the disease. Controversy still exists over several phases. The purpose of this article is an over-all summary of both the pathologic and clinical phases of the disease. It is generally accepted that the tumor arises from the argentaffin cells of the gastrointestinal tract described by Kultschitzky in 1897 as specialized epithelial cells with a characteristic structure . Schmidt in 19o5 called attention to their yellow color and believed they were part of the chromaffin systern . In 1914 Gossett and

T

Masson noticed that certain cytoplasmic granules reduced silver compounds producing blackened particles thus giving us the common name argentaffin cells and tumors . These cells are concentrated at the bases of the crypts of Lieberkuhn . Some tumors have been demonstrated histologically as arising from this region . 6 Clara in 1934 demonstrated that these basigranular cells are derived in the human embryo from undifferentiated intestinal epithelium and later Popoff showed evidence that their formation continued in adult life from aberrant mucous-secreting glands . Stout' concludes from work done by Erspamer that the histologic and morphologic variations characteristic of the tumor may be explained on the premise that the basigranular cells are formed from the goblet cells, and acquisition of their granules and assumption of their pyramidal and wedge shapes are secondary . The festooning effect may well be effected by large scale early differentiation of the basigranular cells as they approximate the shapes of the columnar crypt cells of Kultschitzky . In the final consideration of the pathologic evolution of the tumor one must conclude that there are rather widespread controversial concepts, each well substantiated, but none acceptable in toto . Clinically perhaps the most noteworthy characteristic of rectal carcinoids is the negative fact that they generally do not give rise to local symptoms, metastatic lesions notwithstanding. Pearson and Fitzgerald 8 in their evaluations of thirty-two cases state that in nine cases no symptoms were mentioned or that the tumors were found on routine proctoscopic examination or necropsy . It should be noted that of these thirty-two cases clinical data were unavailable in thirteen cases . In the remaining ten cases, four evidenced degrees of melena ranging from tarry stools to frank bleeding . Five had change of bowel function, either constipation or actual sensations of obstruction, but without bleeding. Those with metastasis showed the typical general picture

*This paper was read by title .

November, 1952

617

618

Cohen-Carcinoid Tumors of the Rectum

Photomicrograph (low power) showing typical argentiffin cells and normal acini .

FIG . 2 . Photomicrograph (high power) showing argentiffin granules.

characteristic of malignant lesions, namely, fatigue, loss of weight and strength in addition to local symptoms . Duration of symptoms in this series was from one week to several months . The rectal lesion has a distinct predilection to occur in the anterior rectal wall . In fifteen cases in which localization is described, thirteen lesions were in the anterior or anterolateral quadrants, while one presented a diffusely infiltrating picture and the other was in both anterior and posterior quadrants . Distance above the anorectal varies from 1 to 14 cm . with the majority approximating the 5 to 8 cm . level . The type of lesions has been determined for twenty-nine of the reported cases . Breakdown of these shows fifteen as small submucosal or sessile nodules ; nine as polypoid lesions and the remainder as large constricting or infiltrating lesions . The solitary submucosal masses generally were of small caliber, ranging from 0 .2 to 2 cm . Polypoid lesions were likewise of small size. The tumors, with exception of the large infiltrating types, are freely moveable and are not fixed . The diffusely infiltrating carcinoid cannot be clinically differentiated from an infiltrating carcinoma . Similarly the polypoid carcinoid resembles the typical pedunculated adenoma grossly . The nodular or sessile carcinoid must be differentiated from the submucosal tumors of which they make up a very small percentage. Differential lesions to be considered include paraffinomas from previous injection therapy ; inflammatory lesions ; tumors whose origin is in the connective tissue such as leiomyomas, lipomas or lymphomas . From this it becomes obvious that positive diagnosis can be made only by histologic examination .

The treatment of carcinoid tumors of the rectum presents a somewhat complex problem . Solitary freely movable tumors may be excised locally and the patient carefully followed for signs of local recurrence or metastasis . Mayo and McKee 9 report that in thirty cases of rectal carcinoid in which local excision was performed no recurrences or metastasis have been reported in twenty-four, the follow-up periods varying from two months to nine years . No data were available on the other six cases . The more extensive lesions call for radical surgery such as is indicated in rectal adenocarcinoma . The low grade malignant nature of the lesion calls for careful evaluation of the surgical procedure of choice . Each case should be individualized as to treatment, since metastasis are generally uncommon but nevertheless must always be watched for .

FIG . I .

CASE REPORT

In August, 194,7, F . B ., age twenty-six, presented symptoms referable only to anorectal disorder. At the time of surgery a small, firm, freely moveable tumor was noticed . The mass lay in the anterior aspect of the rectal ampulla 4 cm . above the anorectal line . It was excised without difficulty and found to be a rectal carcinoid . (Figs . 1 and 2 .) The diagnosis was confirmed by Dr . Fred Stewart . Postoperative course was uneventful . The patient was well when last seen . SUMMARY

The histopathologic and clinical manifestations of carcinoid tumors of the rectum have been reviewed . The asymptomatic and benign

American Journal of Surgery

Cohen-Carcinoid Tumors of the Rectum character of the lesion is stressed . One new case in which there were no symptoms or apparent metastasis is reported . The patient is well four years after removal of the tumor.

5.

small intestine . Bull . Johns Hopkin4 Hosp ., 37 : 130-153, 1925 . EHLICH, J . C . and HUNTER, 0 . B ., JR . Tumors of the gastrointestinal tract . A survey of 813 in persons of military age during World War H . Surg ., Gynec. Obst ., 85 : 98-106 . 1947 . RAVEN, R. W. Carcinoid tumors of the rectum . Proc . Roy . Soc. Med ., 43 : 675-677, 1950 . STOUT, A . P. Carcinoid tumors of the rectum derived from erspamers pre-enterochrome cells . Am . J. Path ., 18 : 993-1004, 1942 . PEARSON, C. and FITZGERALD, P . J . Carcinoid tumors of the rectum . Ann . Surg ., 128 : 128- 143, 1948 . MAYO, H . W ., JR . and EDWARD E . McKEE . Carcinoid of the rectum . Arch . Surg., 62 :5o6 - 513, 1951

e

REFERENCES 1 . MASSON, P . Carcinoids (argentaffin-cell tumors) and nerve hyperplasia of the appendicular mucosa . Am . J. Path ., 4 : 181-212, 1928 . 2 . COOKE, H . H . Carcinoid tumors of the small intestine . Arch . Surg ., 22 : 568-597, 193 1 3- RITCHIE, G . and STAFFOR, W . T. Argentaffin tumors of the gastro-intestinal tract . Arch . Path ., 38 : 123 - 127,1944 . 4- FoaBus, W . Argentaffin tumors of the appendix and

November, 1952

6. 7-

8.

9.

619