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Reticulum cell sarcoma of the small bowel
Reticulum Cell Sarcoma of the Small Bowel ORVILLEF. GRIMES, M.D., Sun Francisco,
From
CuliJornia
tbe Department of Surgery, University of Calijorornia had been treated with a modified uIcer diet between Medicine,San Francisco, California. hospita1 admissions.
School of
HE management of malignant tumors of
the small intestine is frequently troublesome. First, diagnosis is often difficult, delayed or impossible because of the probIems inherent in the radiologic interpretation of lesions of the small bowel. Because of the Iength, tortuous loops and constant motion of the intestine, lesions within it are often concealed. Second, symptoms of malignancy of the small bowel, save for those which produce obstruction, are likely to be interpreted as being the result of diseases elsewhere in the intestinal tract. From a statistica standpoint alone, hemorrhage, anemia and vague abdominal stress in the absence of obstruction are usually attributed to lesions in other areas. Third, even when gross hemorrhage is a major factor in the history, one usually thinks of hemorrhagic processes in the Iower esophagus, stomach or duodenum, even if the resubs of repeated radiologic studies of these areas are negative or inconclusive. The case presented herein iIIustrates the latter point. Multiple roentgenologic studies of the upper gastrointestinal tract and Iarge intestine were performed during a period of several months in an effort to diagnose the cause of severe intestinal bleeding. Even though a malignant tumor of the small intestine was in a reIativeIy far advanced state, its presence was nearly overlooked in the last studies made of the upper gastrointestinal tract. T
CASE REPORT A seventy-six year oId man was admitted to the University of Cahfornia HospitaI on January 22, rg58. He had been a chronic invaIid for nine months, mainIy from the effects of massive repeated intestinal hemorrhages. Each of the episodes of bleeding had been treated by muItipIe transfusions of whoIe bIood and conservative management during the most severe periods of iIIness which required hospitaIization. The patient American
Jvurnvl of Surgery. Vvluma rvv, Octvber 1960
Three roentgenoIogic studies of the upper intestinal tract were performed eIsewhere in June, September and December of rg57. None of these showed evidence of disease. Barium enemas performed in September and December 1957 reveaIed no abnormaIity. Other studies had aIso been made in an effort to discover the source of the massive intestina1 breeding, which was manifest at a11times by the passage of tarry stooIs and on two occasions by tarry stools mixed with bright red bIood. At the time of admission to this hospita1 the patient was extremeIy weak, and he showed the effects of a prolonged iIIness. The main findings were Iimited to the abdomen. A suggestion of an iII-defined fuIIness was present in the Ieft portion of the abdomen at the IeveI of the umbiIicus. A barium enema performed as the initia1 radiologic study was normal. Results of a study of the stomach, duodenum and smaI1 bowel were normal except for an apparent irreguIarity in the smaI1 bowe1. The change in contour was noted on onIy one fiIm of the many obtained in the radioIogic series. (Fig. IA.) A repeated study directed toward this area verified the presence of a mass involving a rather long segment of smaI1 bowe1. (Fig. IB.) It was quite possible that the discovery of this irreguIarity on the initia1 roentgenoIogic study here was a fortunate coincidence since no obstruction of the area existed, nor was the fIow of barium significantly altered in speed or course at this juncture. After suitabIe preparation with transfusions of whole bIood and genera1 supportive care, Iaparotomy was performed on January 28, 1958. A mass was found in the smaI1 bowe1 at the junction of the jejunum and iIeum. The tumor had progressed to such a state that severa Ioops of smaI1 bowe1 were drawn into the substance of the tumor, and a fistuIa was present between adjacent loops of bowe1 at one point. The mass measured 12 cm. in Iength, 8 cm. in width and 6 cm. in thickness. Part of its volume was composed of adherent IOOPSof boweI. (Fig. 2.) Numerous Iymph nodes were found in the mesentery. These varied in size from a few miIIimeters up to 4 cm. The metastatic masses in the mesentery extended throughout its entire sur602
Reticuhm
CeII Sarcoma
of SmaII BoweI
1A
I3
FIG. I. A, note narrowed Iinear segment of smaIl intestine, IateraI to descending coIon. B, second study with special emphasis on Ieft lower quadrant of abdomen, which demonstrated extensively involved area of smaI1 intestine.
FIG. 2. In gross specimen, Iinear nature of Iesion is evident. UIceration had occurred in severa areas of tumor but most of lesion was covered with intact mucosa.
face and involved a11 segments of the mesentery of the entire smaII boweI. There were no identifiabIe metastases to the Iiver. In order to prevent further massive hemorrhages from the Iesion, resection of the smaI1 bowe1 was performed. As much of the invoIved mesentery was removed as was compatibIe with the blood suppIy of the remaining bowe1. This was considered at the time to be a paIIiative resection. It was hoped that the patient’s genera1 condition wouId be improved by preventing further Ioss of bIood, and that the growth of the remaining metastatic
deposits wouId be inhibited. A course of nitrogen mustard therapy was administered Iate in the postoperative period. Pathologic examination of the specimen showed numerous metastases to the mesenteric lymph nodes. Cross sections of the tumor showed an intact mucosa in most areas. (Fig. 3.) Histologic studies demonstrated the typica picture of a reticuIum ceI1 sarcoma. (Figs. 4 and 5.) The patient’s immediate postoperative course was satisfactory, and he was quite we11 for six months. About the seventh month after operation
603
Grimes his condition began to deteriorate rapidIy. He was admitted to the hospita1 for termina1 care. At this time he had abdomina1 ascites, generalized weakness and evidence of spread of maIignant disease throughout the abdomina1 cavity. An autopsy showed extensive mesenteric Iymphatic metastases as we11 as deposits on the peritoneum, within the prostatic gtand, and in both kidneys and the perinephric fat. The Iiver was free from metastases as were the Iungs. COMMENTS
Reticulum ceI1 sarcoma, a form of Iymphosarcoma, is probably the rarest mahgnant Iesion of the smal1 bowe1. The Iesion is highIy maIignant. Although its behavior pattern is simiIar to that of Iymphosarcoma, it occasionaIIy may be IocaIized, which justifies its radical remova [I]. Lymphoid tissue, consisting of a connective tissue reticuIum aIong with aggregates of Iymphoid ceIIs contained within it, is Iocated throughout the intestine as isoIated areas in the mucosa, and is also arranged in patches in various areas. Most
FIG. 3. Cross section of portion of tumor. Note that mucosa is intact in some areas ovedying tumor (arrows) and eroded in others2(semicircle).
FIG. 5. Pathognomic reticuIum is shown in siIver-stain preparation. Amount of reticulum is increased and intimateIy reiated to ceIIs of tumor.
FIG. 4. Microscopic section of portion of tumor in area of erosion of mucous membrane. Cytoplasm is abundant and demonstrates more pIeomorphism than does Iymphosarcoma. Note Iarge nucIei. Hematoxylin and eosin stain.
604
ReticuIum
CeII Sarcoma
observers beheve that malignancies of this tissue arise in the Iymph folhcles in the submucosa [f]. From this point of origin the tumor spreads by direct extension in the long axis of the bowel. Since it arises in the deeper layers, ukeration is not common. When ulceration does occur, it results from necrosis of the overIying mucosa, IargeIy by virtue of the bulk of the mass rather than by malignant invasion of the mucosa, aIthough both mechanisms can occur. ProbabIy because of the greater accumulation of lymphoid tissue in the cecum and ascending colon, Iymphomas in this area tend to be polypoid, whiIe those in the smal1 intestine are more 1ikeIy to be diffusely infihrative, possibIy because the aggregates of Iymphoid tissue are more scattered here than elsewhere. Intussusception is not uncommon even in those Iesions arising in the small bowel [2,4]. The origin of these lesions in the submucosal regions and the Iack of intraluminal involvement until Iate in their deveIopment suggests that obstruction is the exception rather than the rule. For this reason the predominant symptom in lesions of this type is caused by ulceration produced by the buIk of the tumor eroding through the mucosa of the bowel at a Iate stage in the development of the tumor. This symptom is, of course, hemorrhage which is usually massive. Vague abdomina1 distress bordering on true but incompIete symptoms of obstruction is often dismissed from consideration when the patient’s history is obtained. This was indeed true in a recently reported series [j]. In a study of thirty-two patients, the symptoms produced by these tumors were of two main types: (I) obstruction, which was usually incomplete, causing vague but crampy abdominal pain; and (2) occuh or severe Ioss of blood which was intermittent. Reticulum cell sarcoma apparently has no special prediIection to the smaI1 bowel for its development. The presence of reticuIum throughout the body permits the development of malignancy in any Iocation in about the same statistical order of magnitude, except in bone. RadioIogic diagnosis of tumors of the small bowe1 is diffrcuh. The tortuosity of the considerabIe Iength of smaII bowe1 aIong with its constant movement aIIows 0verIapping of 100~s of intestine and Ieads to diffrcu1ty in the diagnosis.
of SmaII
BoweI
Tumors of the small bowe1 unfortunately are not always recognized even at the time of Iaparotomy. WiIkie [6] reported three instances in which the gallbIadder, appendix and adhesions were removed as the most likely cause of the patient’s symptoms, even though a tumor of the smaI1 bowe1 existed at the time. It is also important to realize that even in the presence of a known reticuIum cell sarcoma of the small intestine, the presence of enIarged mesenteric Iymph nodes does not necessarily indicate that they are involved by metastatic cancer. Mesenteric Iymph nodes in one-half the bowel resections performed in a recent series [7] did not demonstrate malignant metastatic involvement. The present case report indicates that constant vigilance and awareness of the possibility of a tumor of the small bowel as a cause of symptoms will perhaps do more to unany other singIe cover early Iesions than method. The prognosis (which is poor at best) can be improved onIy by discovering these Iesions at an early stage of their development since treatment at a Iate date is so ineffective. SUMMARY
A case of reticulum ceII sarcoma of the small intestine is presented. Repeated radiologic studies failed to demonstrate the Iesion, aIthough it was in an advanced stage of development at Iaparotomy. The possibility of a tumor of the smah bowel shouId always be kept in mind even if roentgenoIogic studies of the upper gastrointestinal tract appear normal in patients with vague abdomina1 distress and hemorrhage. REFERENCES I. CLIFFORD,
W. J. ReticuIum cell sarcoma of the
jejunum. Am. Surgeon, 18: I 164, 1952. 2. MACKENZIE, J. and ROBERTSON, R. F. ReticuIumcell sarcoma of the gastrointestinal tract. Gastroenterology, 26: 70, 1934. 3. PATTERSON,J. F., CALLOW, A. D. and E~TINC;ER,A. The chnical patterns of small-bowel tumors; a study of thirty-two cases. Ann. Int. Med., 48: 123, ‘958. 4. SPENCER, J. G. C. MuItipIe reticulosarcoma of the duodenum and jejunum. Brit. J. Surg., 41: 75, 1953. 5. USHER, F. C. and DIXON, C. F. Lymphosarcoma of the intestines. Gastroenterology, I: 160, 1943. 6. WILKIE, D. ReticuIum-celI sarcoma of the smaI1 intestine with perforation. hit. J. Surg., 41: 50, 1953. 7. Case records of the Massachusetts Genera1 HospitaI, weeMy cIinicopathoIogica1 exercises; case no. 41281. New England J. Med.. 253: 107, 1955.
From
CuliJornia
tbe Department of Surgery, University of Calijorornia had been treated with a modified uIcer diet between Medicine,San Francisco, California. hospita1 admissions.
School of
HE management of malignant tumors of
the small intestine is frequently troublesome. First, diagnosis is often difficult, delayed or impossible because of the probIems inherent in the radiologic interpretation of lesions of the small bowel. Because of the Iength, tortuous loops and constant motion of the intestine, lesions within it are often concealed. Second, symptoms of malignancy of the small bowel, save for those which produce obstruction, are likely to be interpreted as being the result of diseases elsewhere in the intestinal tract. From a statistica standpoint alone, hemorrhage, anemia and vague abdominal stress in the absence of obstruction are usually attributed to lesions in other areas. Third, even when gross hemorrhage is a major factor in the history, one usually thinks of hemorrhagic processes in the Iower esophagus, stomach or duodenum, even if the resubs of repeated radiologic studies of these areas are negative or inconclusive. The case presented herein iIIustrates the latter point. Multiple roentgenologic studies of the upper gastrointestinal tract and Iarge intestine were performed during a period of several months in an effort to diagnose the cause of severe intestinal bleeding. Even though a malignant tumor of the small intestine was in a reIativeIy far advanced state, its presence was nearly overlooked in the last studies made of the upper gastrointestinal tract. T
CASE REPORT A seventy-six year oId man was admitted to the University of Cahfornia HospitaI on January 22, rg58. He had been a chronic invaIid for nine months, mainIy from the effects of massive repeated intestinal hemorrhages. Each of the episodes of bleeding had been treated by muItipIe transfusions of whoIe bIood and conservative management during the most severe periods of iIIness which required hospitaIization. The patient American
Jvurnvl of Surgery. Vvluma rvv, Octvber 1960
Three roentgenoIogic studies of the upper intestinal tract were performed eIsewhere in June, September and December of rg57. None of these showed evidence of disease. Barium enemas performed in September and December 1957 reveaIed no abnormaIity. Other studies had aIso been made in an effort to discover the source of the massive intestina1 breeding, which was manifest at a11times by the passage of tarry stooIs and on two occasions by tarry stools mixed with bright red bIood. At the time of admission to this hospita1 the patient was extremeIy weak, and he showed the effects of a prolonged iIIness. The main findings were Iimited to the abdomen. A suggestion of an iII-defined fuIIness was present in the Ieft portion of the abdomen at the IeveI of the umbiIicus. A barium enema performed as the initia1 radiologic study was normal. Results of a study of the stomach, duodenum and smaI1 bowel were normal except for an apparent irreguIarity in the smaI1 bowe1. The change in contour was noted on onIy one fiIm of the many obtained in the radioIogic series. (Fig. IA.) A repeated study directed toward this area verified the presence of a mass involving a rather long segment of smaI1 bowe1. (Fig. IB.) It was quite possible that the discovery of this irreguIarity on the initia1 roentgenoIogic study here was a fortunate coincidence since no obstruction of the area existed, nor was the fIow of barium significantly altered in speed or course at this juncture. After suitabIe preparation with transfusions of whole bIood and genera1 supportive care, Iaparotomy was performed on January 28, 1958. A mass was found in the smaI1 bowe1 at the junction of the jejunum and iIeum. The tumor had progressed to such a state that severa Ioops of smaI1 bowe1 were drawn into the substance of the tumor, and a fistuIa was present between adjacent loops of bowe1 at one point. The mass measured 12 cm. in Iength, 8 cm. in width and 6 cm. in thickness. Part of its volume was composed of adherent IOOPSof boweI. (Fig. 2.) Numerous Iymph nodes were found in the mesentery. These varied in size from a few miIIimeters up to 4 cm. The metastatic masses in the mesentery extended throughout its entire sur602
Reticuhm
CeII Sarcoma
of SmaII BoweI
1A
I3
FIG. I. A, note narrowed Iinear segment of smaIl intestine, IateraI to descending coIon. B, second study with special emphasis on Ieft lower quadrant of abdomen, which demonstrated extensively involved area of smaI1 intestine.
FIG. 2. In gross specimen, Iinear nature of Iesion is evident. UIceration had occurred in severa areas of tumor but most of lesion was covered with intact mucosa.
face and involved a11 segments of the mesentery of the entire smaII boweI. There were no identifiabIe metastases to the Iiver. In order to prevent further massive hemorrhages from the Iesion, resection of the smaI1 bowe1 was performed. As much of the invoIved mesentery was removed as was compatibIe with the blood suppIy of the remaining bowe1. This was considered at the time to be a paIIiative resection. It was hoped that the patient’s genera1 condition wouId be improved by preventing further Ioss of bIood, and that the growth of the remaining metastatic
deposits wouId be inhibited. A course of nitrogen mustard therapy was administered Iate in the postoperative period. Pathologic examination of the specimen showed numerous metastases to the mesenteric lymph nodes. Cross sections of the tumor showed an intact mucosa in most areas. (Fig. 3.) Histologic studies demonstrated the typica picture of a reticuIum ceI1 sarcoma. (Figs. 4 and 5.) The patient’s immediate postoperative course was satisfactory, and he was quite we11 for six months. About the seventh month after operation
603
Grimes his condition began to deteriorate rapidIy. He was admitted to the hospita1 for termina1 care. At this time he had abdomina1 ascites, generalized weakness and evidence of spread of maIignant disease throughout the abdomina1 cavity. An autopsy showed extensive mesenteric Iymphatic metastases as we11 as deposits on the peritoneum, within the prostatic gtand, and in both kidneys and the perinephric fat. The Iiver was free from metastases as were the Iungs. COMMENTS
Reticulum ceI1 sarcoma, a form of Iymphosarcoma, is probably the rarest mahgnant Iesion of the smal1 bowe1. The Iesion is highIy maIignant. Although its behavior pattern is simiIar to that of Iymphosarcoma, it occasionaIIy may be IocaIized, which justifies its radical remova [I]. Lymphoid tissue, consisting of a connective tissue reticuIum aIong with aggregates of Iymphoid ceIIs contained within it, is Iocated throughout the intestine as isoIated areas in the mucosa, and is also arranged in patches in various areas. Most
FIG. 3. Cross section of portion of tumor. Note that mucosa is intact in some areas ovedying tumor (arrows) and eroded in others2(semicircle).
FIG. 5. Pathognomic reticuIum is shown in siIver-stain preparation. Amount of reticulum is increased and intimateIy reiated to ceIIs of tumor.
FIG. 4. Microscopic section of portion of tumor in area of erosion of mucous membrane. Cytoplasm is abundant and demonstrates more pIeomorphism than does Iymphosarcoma. Note Iarge nucIei. Hematoxylin and eosin stain.
604
ReticuIum
CeII Sarcoma
observers beheve that malignancies of this tissue arise in the Iymph folhcles in the submucosa [f]. From this point of origin the tumor spreads by direct extension in the long axis of the bowel. Since it arises in the deeper layers, ukeration is not common. When ulceration does occur, it results from necrosis of the overIying mucosa, IargeIy by virtue of the bulk of the mass rather than by malignant invasion of the mucosa, aIthough both mechanisms can occur. ProbabIy because of the greater accumulation of lymphoid tissue in the cecum and ascending colon, Iymphomas in this area tend to be polypoid, whiIe those in the smal1 intestine are more 1ikeIy to be diffusely infihrative, possibIy because the aggregates of Iymphoid tissue are more scattered here than elsewhere. Intussusception is not uncommon even in those Iesions arising in the small bowel [2,4]. The origin of these lesions in the submucosal regions and the Iack of intraluminal involvement until Iate in their deveIopment suggests that obstruction is the exception rather than the rule. For this reason the predominant symptom in lesions of this type is caused by ulceration produced by the buIk of the tumor eroding through the mucosa of the bowel at a Iate stage in the development of the tumor. This symptom is, of course, hemorrhage which is usually massive. Vague abdomina1 distress bordering on true but incompIete symptoms of obstruction is often dismissed from consideration when the patient’s history is obtained. This was indeed true in a recently reported series [j]. In a study of thirty-two patients, the symptoms produced by these tumors were of two main types: (I) obstruction, which was usually incomplete, causing vague but crampy abdominal pain; and (2) occuh or severe Ioss of blood which was intermittent. Reticulum cell sarcoma apparently has no special prediIection to the smaI1 bowel for its development. The presence of reticuIum throughout the body permits the development of malignancy in any Iocation in about the same statistical order of magnitude, except in bone. RadioIogic diagnosis of tumors of the small bowe1 is diffrcuh. The tortuosity of the considerabIe Iength of smaII bowe1 aIong with its constant movement aIIows 0verIapping of 100~s of intestine and Ieads to diffrcu1ty in the diagnosis.
of SmaII
BoweI
Tumors of the small bowe1 unfortunately are not always recognized even at the time of Iaparotomy. WiIkie [6] reported three instances in which the gallbIadder, appendix and adhesions were removed as the most likely cause of the patient’s symptoms, even though a tumor of the smaI1 bowe1 existed at the time. It is also important to realize that even in the presence of a known reticuIum cell sarcoma of the small intestine, the presence of enIarged mesenteric Iymph nodes does not necessarily indicate that they are involved by metastatic cancer. Mesenteric Iymph nodes in one-half the bowel resections performed in a recent series [7] did not demonstrate malignant metastatic involvement. The present case report indicates that constant vigilance and awareness of the possibility of a tumor of the small bowel as a cause of symptoms will perhaps do more to unany other singIe cover early Iesions than method. The prognosis (which is poor at best) can be improved onIy by discovering these Iesions at an early stage of their development since treatment at a Iate date is so ineffective. SUMMARY
A case of reticulum ceII sarcoma of the small intestine is presented. Repeated radiologic studies failed to demonstrate the Iesion, aIthough it was in an advanced stage of development at Iaparotomy. The possibility of a tumor of the smah bowel shouId always be kept in mind even if roentgenoIogic studies of the upper gastrointestinal tract appear normal in patients with vague abdomina1 distress and hemorrhage. REFERENCES I. CLIFFORD,
W. J. ReticuIum cell sarcoma of the
jejunum. Am. Surgeon, 18: I 164, 1952. 2. MACKENZIE, J. and ROBERTSON, R. F. ReticuIumcell sarcoma of the gastrointestinal tract. Gastroenterology, 26: 70, 1934. 3. PATTERSON,J. F., CALLOW, A. D. and E~TINC;ER,A. The chnical patterns of small-bowel tumors; a study of thirty-two cases. Ann. Int. Med., 48: 123, ‘958. 4. SPENCER, J. G. C. MuItipIe reticulosarcoma of the duodenum and jejunum. Brit. J. Surg., 41: 75, 1953. 5. USHER, F. C. and DIXON, C. F. Lymphosarcoma of the intestines. Gastroenterology, I: 160, 1943. 6. WILKIE, D. ReticuIum-celI sarcoma of the smaI1 intestine with perforation. hit. J. Surg., 41: 50, 1953. 7. Case records of the Massachusetts Genera1 HospitaI, weeMy cIinicopathoIogica1 exercises; case no. 41281. New England J. Med.. 253: 107, 1955.