Gastrointest Radiol 15:69-71 (1990)
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc.1990
Linitis Plastica of the Colon: Computed Tomography Findings Eric J. Howell, 1 Eduard E. de Lange, 1 and Henry F. Frierson, J r . 2 Departments of x Radiology and 2 Pathology, University of Virginia Health Sciences Center, Charlottesville, Virginia, USA
Abstract. Primary linitis plastica of the colon is
a rare entity and its radiographic findings have been described previously in the literature. We present the computed tomography findings of this unusual lesion and briefly review its pathogenesis. Key words: Colon cancer, computed tomography - Colon, linitis plastica.
Primary linitis plastica is an uncommon manifestation of carcinoma of the large bowel. The entity was first described by Laufman and Saphir in 1951, and approximately 100 cases have subsequently been reported [1, 2]. There are few descriptions of the plain radiographic findings of this entity [3]. To our knowledge, its features as seen by other imaging techniques have not been described. We present the CT findings of a case of linitis plastica of the colon. Case Report A 47-year-old male, who had been in good health, presented with intermittent diarrhea of four months' duration. He was treated with antibiotics for two weeks, but the diarrhea continued. One month later he developed abdominal pain in the left lower quadrant and passed frequent bloody stools. Flexible sigmoidoscopy revealed a narrowed sigmoid colon 25 cm from the anal verge with edematous and friable mucosa. A biopsy was performed at another institution and was reported to show marked edema and chronic inflammation with eosinophils. Treatment was initiated with Sulfasalazine, but the drug was discontinued due to upper gastrointestinal discomfort. The patient was then given corticosteroids. He continued to have intermittent diarrhea for another month. Approximately three weeks later he developed wheezing, increased abdominal girth, severe scrotal pain, and edema of the left lower extremity.
Address reprint requests to: Eduard E. de Lange, M.D., Department of Radiology, Box 170, University of Virginia Health Sciences Center, Charlottesville, VA 22908, USA
The patient was admitted to the University of Virginia Health Sciences Center for further evaluation. Physical examination revealed diffuse abdominal tenderness, chiefly in the left upper quadrant. The liver was palpable, but abdominal masses could not be palpated. The scrotum was enlarged, but no masses were felt. Digital rectal examination was normal, but blood was noted on the glove. There was edema of the left lower extremity. Laboratory studies on admission revealed normal liver enzyme levels and coagulation parameters. Stool studies revealed white blood cells with negative cultures and Clostridium difficile titers. Ova and parasites were absent. A chest x-ray showed bilateral hilar and mediastinal adenopathy. Interstitial infiltrates were seen predominantly in the lower lobes of the lungs. A bone marrow biopsy was performed and yielded a normal specimen. A computed tomography (CT) examination of the abdomen showed uniform thickening of the wall of the sigmoid colon (Fig. 1). Mediastinoscopy was performed, and a biopsy of a right paratracheal lymph node revealed poorly differentiated adenocarcinoma. Colonoscopy showed a constricting lesion of the sigmoid colon, beginning approximately 20-35 cm from the anal verge. Biopsy of the lesion revealed poorly differentiated adenocarcinoma, consistent with a diagnosis of linitis plastica of the colon. Microscopically, the diffusely invasive tumor formed clusters, small nests with occasional glandular lumens, and single neoplastic cells. Cells had copious eosinophilic granular cytoplasm with occasional mucin-filled vacuoles. The pleomorphic nuclei had coarse, clumped chromatin and occasional prominent nucleoli. Signet ring cells were not conspicuous. The metastasis to a paratracheal lymph node had a microscopic appearance similar to that of the primary neoplasm. The patient underwent palliative treatment with laser surgery of the colon and chemotherapy, after which his condition improved. Eight weeks following therapy the patient returned to work and is currently under clinical observation.
Discussion
Linitis plastica of the colon is a descriptive term referring to the gross pathologic appearance of the involved bowel. The colon has a uniformly thickened and rigid wall, similar to that of linitis plastica of the stomach. Histologically, the adenocarcinoma is poorly differentiated. As is often found in
70
E.J. Howell et al. : Linitis Plastica of the Colon
Fig. 1. A A C T image through the sigmoid colon demonstrates diffuse thickening of the bowel wall (arrows). B Similar findings are seen in the rectum
(arrows').
linitis plastica of the stomach, colonic linitis plastica may contain numerous signet ring cells; these tumors have also been referred to as signet ring carcinomas. Signet ring cells were, however, absent in our case. Metastatic disease from primary linitis plastica of the colon is usually present at the initial diagnosis [3]. Linitis plastica of the colon can also be seen secondary to metastatic spread from other malignant neoplasms, such as adenocarcinoma of the breast, gallbladder, and prostate. Colonic linitis plastica due to metastatic tumor is more common than the primary form of the disease.
Linitis plastica of the large bowel represents less than 2% of colorectal carcinomas [2]. The age range of the patients is 17-84 years, and the mean age is approximately 54 years [4, 5]. Males and females are almost equally affected, with a slight predominance for males. Clinical symptoms are usually related to colonic wall thickening by the tumor and narrowing of the lumen. These symptoms include abdominal pain, changes in bowel habits, blood in the stool, and weight loss. Occasionally, tumor invades ureteral walls and periureteral lymphatics, resulting in intermittent back pain and hematuria [6].
E.J. Howell et al. : Linitis Plastica of the Colon
The frequency of occurrence of the neoplasm in the particular segments of the large bowel is similar to that for the common form of adenocarcinoma of the colon; the rectosigmoid is involved in 78% of cases, the left colon in 6%, the right colon in 14%, and the entire colon in 2% [3]. This distribution differs from secondary linitis plastica due to metastatic gastric cancer; in this situation, the transverse colon is most commonly involved by tumor because of lymphatic spread of the malignant cells through the gastrocolic ligament. There is an association between linitis plastica of the colon and long-standing ulcerative colitis, and a 20-30% incidence of preexisting chronic ulcerative colitis has been reported [3, 6, 7]. The prognosis of primary colonic linitis plastica is poor. In one study, the mean survival was only 8.3 months after a diagnosis was established [8]. However, one reported patient was disease free after 79 months [3]. Other reports have indicated that survival is not significantly different from the more common form of adenocarcinoma of the colon [9]. Several factors contribute to the poor survival of most patients. For instance, the diagnosis may be difficult because the clinical gross appearance of the bowel wall may mimic an inflammatory process, such as Crohn's disease or ulcerative colitis. Also, as the mucosa may not contain tumor (the bulk of the neoplasm may remain in the submucosa), the lesion may remain undetected on endoscopic examination. Furthermore, clinical symptoms are usually not manifest until the tumor is at an advanced stage [10]. The neoplasm most commonly metastasizes to pericolic lymph nodes, mesentery, peritoneum, and ovaries. Metastases to the liver and lungs are rare [4, 6, 8, i 1, 12]. When the lesion is localized, treatment is radical surgical resection. In females, hysterosalpingo-oophorectomy is also recommended [13]. Radiation therapy and chemotherapy have been used for treatment of the more advanced lesions [6, 14]. The radiographic diagnosis of the tumor may be difficult. Barium enema findings include narrowing and abnormal peristalsis of the involved segment [6, 7, 8]. The mucosa may be intact, or may be coarse and nodular with irregular folds [6, 1 i]. Marked obstruction is u n c o m m o n [11]. On CT there is diffuse thickening of a large segment of the bowel wall, as demonstrated in this case.
71
Both the fluoroscopic and CT findings are not specific, and the abnormalities may also be seen in cases of inflammatory bowel disease, lymphoma, ischemic colitis, radiation colitis, endometriosis, hemorrhage, metastatic carcinoma, and appendicitis [6, 15, 16]. Although barium enema may be more useful for demonstrating early mucosal and peristaltic irregularities, CT may be more effective in determining the depth of bowel wM1 penetration and the extent of extracotonic disease. References i 1. Lanfman H, Saphir O : Primary linitis plastica type of carcinoma of the colon. Arch Surg 62:79-91, 1951 2. Soybel D, Bliss D, Wells S: Colon and rectal carcinoma. Curr Probl Cancer 11 .257-356, 1987 3. Ojeda V, Mitchell KM, Waiters MN, Gibson M J: Primary colo-rectal linitis plastica type of carcinoma: report of two cases and review of the literature. Pathology 14:181-189, 1982 4. Nadel L, Mori K, Shinya H: Primary linitis plastica of the colon and rectum. Dis Colon Rectum 26." 736-740, 1983 5. Lui IO, Kung ITM, Lee JMH, Boey JH: Primary colorectal signet-ring cell carcinoma in young patients: report of 3 cases. Pathology 17: 31-35, 1985 6. Greenfield SM, Seedor JW, Nack SL, Sohn M: Obstructive uropathy. An unusual presentation of primary linitis plastica of the colon. Dig Dis Sci 30:68%696, 1985 7. Balthazar EJ, Rosenberg HD, Davidian MM: Primary and metastatic scirrhous carcinoma of the rectum. A JR 132:711-715, 1979 8. Amorn Y, Knight W: Primary linitis plastica of the colon: report of two cases and review of the literature. Cancer 41.'2420-2425, 1978 9. Giacchero A, Aste H, Baracchini P, et al: Primary signetring carcinoma of the large bowel. Report of nine cases. Cancer 56." 2723-2726, 1985 10. Bonello JC, Quan SHO, Sternberg SS: Primary linitis plastica of the rectum. Dis Colon Rectum 23:337-342, 1980 11. Fayemi AO, Ali M, Braun E: Metastatic carcinoma simulating linitis plastica of the colon. A case report. Am J Gastroenterol 71 : 311-314, 1979 12. Kirkham N: Editorial. Colorectal signet ring cell carcinoma in young PeOple. J Pathol 155:93-94, 1988 13. Almagro U: Primary signet-ring carcinoma of the colon. Cancer 52." 1453-1457, 1983 14. Sizer JS, Frederick PL, Osborne MP: Primary linitis plastica of the colon: report of a case and review of the literature. Dis' Colon Rectum 10: 339-343, 1967 15. Gore RM: Cross sectional imaging of inflammatory bowel disease. Radiol Clin North Am 25:115-131, 1987 16. Seltzer SE, Jochelson M, Balikiun JP: Organ envelopment in lymphoma visualized by computed tomography. Clin Radio137: 525-529, 1986 Received: March 28, 1989; accepted." May 4, 1989
Gastrointestinal
Radiology 9 Springer-VerlagNewYorkInc.1990
Linitis Plastica of the Colon: Computed Tomography Findings Eric J. Howell, 1 Eduard E. de Lange, 1 and Henry F. Frierson, J r . 2 Departments of x Radiology and 2 Pathology, University of Virginia Health Sciences Center, Charlottesville, Virginia, USA
Abstract. Primary linitis plastica of the colon is
a rare entity and its radiographic findings have been described previously in the literature. We present the computed tomography findings of this unusual lesion and briefly review its pathogenesis. Key words: Colon cancer, computed tomography - Colon, linitis plastica.
Primary linitis plastica is an uncommon manifestation of carcinoma of the large bowel. The entity was first described by Laufman and Saphir in 1951, and approximately 100 cases have subsequently been reported [1, 2]. There are few descriptions of the plain radiographic findings of this entity [3]. To our knowledge, its features as seen by other imaging techniques have not been described. We present the CT findings of a case of linitis plastica of the colon. Case Report A 47-year-old male, who had been in good health, presented with intermittent diarrhea of four months' duration. He was treated with antibiotics for two weeks, but the diarrhea continued. One month later he developed abdominal pain in the left lower quadrant and passed frequent bloody stools. Flexible sigmoidoscopy revealed a narrowed sigmoid colon 25 cm from the anal verge with edematous and friable mucosa. A biopsy was performed at another institution and was reported to show marked edema and chronic inflammation with eosinophils. Treatment was initiated with Sulfasalazine, but the drug was discontinued due to upper gastrointestinal discomfort. The patient was then given corticosteroids. He continued to have intermittent diarrhea for another month. Approximately three weeks later he developed wheezing, increased abdominal girth, severe scrotal pain, and edema of the left lower extremity.
Address reprint requests to: Eduard E. de Lange, M.D., Department of Radiology, Box 170, University of Virginia Health Sciences Center, Charlottesville, VA 22908, USA
The patient was admitted to the University of Virginia Health Sciences Center for further evaluation. Physical examination revealed diffuse abdominal tenderness, chiefly in the left upper quadrant. The liver was palpable, but abdominal masses could not be palpated. The scrotum was enlarged, but no masses were felt. Digital rectal examination was normal, but blood was noted on the glove. There was edema of the left lower extremity. Laboratory studies on admission revealed normal liver enzyme levels and coagulation parameters. Stool studies revealed white blood cells with negative cultures and Clostridium difficile titers. Ova and parasites were absent. A chest x-ray showed bilateral hilar and mediastinal adenopathy. Interstitial infiltrates were seen predominantly in the lower lobes of the lungs. A bone marrow biopsy was performed and yielded a normal specimen. A computed tomography (CT) examination of the abdomen showed uniform thickening of the wall of the sigmoid colon (Fig. 1). Mediastinoscopy was performed, and a biopsy of a right paratracheal lymph node revealed poorly differentiated adenocarcinoma. Colonoscopy showed a constricting lesion of the sigmoid colon, beginning approximately 20-35 cm from the anal verge. Biopsy of the lesion revealed poorly differentiated adenocarcinoma, consistent with a diagnosis of linitis plastica of the colon. Microscopically, the diffusely invasive tumor formed clusters, small nests with occasional glandular lumens, and single neoplastic cells. Cells had copious eosinophilic granular cytoplasm with occasional mucin-filled vacuoles. The pleomorphic nuclei had coarse, clumped chromatin and occasional prominent nucleoli. Signet ring cells were not conspicuous. The metastasis to a paratracheal lymph node had a microscopic appearance similar to that of the primary neoplasm. The patient underwent palliative treatment with laser surgery of the colon and chemotherapy, after which his condition improved. Eight weeks following therapy the patient returned to work and is currently under clinical observation.
Discussion
Linitis plastica of the colon is a descriptive term referring to the gross pathologic appearance of the involved bowel. The colon has a uniformly thickened and rigid wall, similar to that of linitis plastica of the stomach. Histologically, the adenocarcinoma is poorly differentiated. As is often found in
70
E.J. Howell et al. : Linitis Plastica of the Colon
Fig. 1. A A C T image through the sigmoid colon demonstrates diffuse thickening of the bowel wall (arrows). B Similar findings are seen in the rectum
(arrows').
linitis plastica of the stomach, colonic linitis plastica may contain numerous signet ring cells; these tumors have also been referred to as signet ring carcinomas. Signet ring cells were, however, absent in our case. Metastatic disease from primary linitis plastica of the colon is usually present at the initial diagnosis [3]. Linitis plastica of the colon can also be seen secondary to metastatic spread from other malignant neoplasms, such as adenocarcinoma of the breast, gallbladder, and prostate. Colonic linitis plastica due to metastatic tumor is more common than the primary form of the disease.
Linitis plastica of the large bowel represents less than 2% of colorectal carcinomas [2]. The age range of the patients is 17-84 years, and the mean age is approximately 54 years [4, 5]. Males and females are almost equally affected, with a slight predominance for males. Clinical symptoms are usually related to colonic wall thickening by the tumor and narrowing of the lumen. These symptoms include abdominal pain, changes in bowel habits, blood in the stool, and weight loss. Occasionally, tumor invades ureteral walls and periureteral lymphatics, resulting in intermittent back pain and hematuria [6].
E.J. Howell et al. : Linitis Plastica of the Colon
The frequency of occurrence of the neoplasm in the particular segments of the large bowel is similar to that for the common form of adenocarcinoma of the colon; the rectosigmoid is involved in 78% of cases, the left colon in 6%, the right colon in 14%, and the entire colon in 2% [3]. This distribution differs from secondary linitis plastica due to metastatic gastric cancer; in this situation, the transverse colon is most commonly involved by tumor because of lymphatic spread of the malignant cells through the gastrocolic ligament. There is an association between linitis plastica of the colon and long-standing ulcerative colitis, and a 20-30% incidence of preexisting chronic ulcerative colitis has been reported [3, 6, 7]. The prognosis of primary colonic linitis plastica is poor. In one study, the mean survival was only 8.3 months after a diagnosis was established [8]. However, one reported patient was disease free after 79 months [3]. Other reports have indicated that survival is not significantly different from the more common form of adenocarcinoma of the colon [9]. Several factors contribute to the poor survival of most patients. For instance, the diagnosis may be difficult because the clinical gross appearance of the bowel wall may mimic an inflammatory process, such as Crohn's disease or ulcerative colitis. Also, as the mucosa may not contain tumor (the bulk of the neoplasm may remain in the submucosa), the lesion may remain undetected on endoscopic examination. Furthermore, clinical symptoms are usually not manifest until the tumor is at an advanced stage [10]. The neoplasm most commonly metastasizes to pericolic lymph nodes, mesentery, peritoneum, and ovaries. Metastases to the liver and lungs are rare [4, 6, 8, i 1, 12]. When the lesion is localized, treatment is radical surgical resection. In females, hysterosalpingo-oophorectomy is also recommended [13]. Radiation therapy and chemotherapy have been used for treatment of the more advanced lesions [6, 14]. The radiographic diagnosis of the tumor may be difficult. Barium enema findings include narrowing and abnormal peristalsis of the involved segment [6, 7, 8]. The mucosa may be intact, or may be coarse and nodular with irregular folds [6, 1 i]. Marked obstruction is u n c o m m o n [11]. On CT there is diffuse thickening of a large segment of the bowel wall, as demonstrated in this case.
71
Both the fluoroscopic and CT findings are not specific, and the abnormalities may also be seen in cases of inflammatory bowel disease, lymphoma, ischemic colitis, radiation colitis, endometriosis, hemorrhage, metastatic carcinoma, and appendicitis [6, 15, 16]. Although barium enema may be more useful for demonstrating early mucosal and peristaltic irregularities, CT may be more effective in determining the depth of bowel wM1 penetration and the extent of extracotonic disease. References i 1. Lanfman H, Saphir O : Primary linitis plastica type of carcinoma of the colon. Arch Surg 62:79-91, 1951 2. Soybel D, Bliss D, Wells S: Colon and rectal carcinoma. Curr Probl Cancer 11 .257-356, 1987 3. Ojeda V, Mitchell KM, Waiters MN, Gibson M J: Primary colo-rectal linitis plastica type of carcinoma: report of two cases and review of the literature. Pathology 14:181-189, 1982 4. Nadel L, Mori K, Shinya H: Primary linitis plastica of the colon and rectum. Dis Colon Rectum 26." 736-740, 1983 5. Lui IO, Kung ITM, Lee JMH, Boey JH: Primary colorectal signet-ring cell carcinoma in young patients: report of 3 cases. Pathology 17: 31-35, 1985 6. Greenfield SM, Seedor JW, Nack SL, Sohn M: Obstructive uropathy. An unusual presentation of primary linitis plastica of the colon. Dig Dis Sci 30:68%696, 1985 7. Balthazar EJ, Rosenberg HD, Davidian MM: Primary and metastatic scirrhous carcinoma of the rectum. A JR 132:711-715, 1979 8. Amorn Y, Knight W: Primary linitis plastica of the colon: report of two cases and review of the literature. Cancer 41.'2420-2425, 1978 9. Giacchero A, Aste H, Baracchini P, et al: Primary signetring carcinoma of the large bowel. Report of nine cases. Cancer 56." 2723-2726, 1985 10. Bonello JC, Quan SHO, Sternberg SS: Primary linitis plastica of the rectum. Dis Colon Rectum 23:337-342, 1980 11. Fayemi AO, Ali M, Braun E: Metastatic carcinoma simulating linitis plastica of the colon. A case report. Am J Gastroenterol 71 : 311-314, 1979 12. Kirkham N: Editorial. Colorectal signet ring cell carcinoma in young PeOple. J Pathol 155:93-94, 1988 13. Almagro U: Primary signet-ring carcinoma of the colon. Cancer 52." 1453-1457, 1983 14. Sizer JS, Frederick PL, Osborne MP: Primary linitis plastica of the colon: report of a case and review of the literature. Dis' Colon Rectum 10: 339-343, 1967 15. Gore RM: Cross sectional imaging of inflammatory bowel disease. Radiol Clin North Am 25:115-131, 1987 16. Seltzer SE, Jochelson M, Balikiun JP: Organ envelopment in lymphoma visualized by computed tomography. Clin Radio137: 525-529, 1986 Received: March 28, 1989; accepted." May 4, 1989
