Journal of Surgical Oncology 50:274-277 (1992)
Adenocarcinoma Arising Within a Gastric Duplication Cyst DANIEL G.COIT, MD, FACS, AND CAROLYN MIES, MD From the Departments of Surgery (D.C.C.) and Pathology (C.M.), Memorial Sloan-Kettering Cancer Center, New York, New York
A rare case of carcinoma arising within a gastric duplication cyst in a 72 year old woman is presented. This is the oldest patient in whom neoplastic transformation of this usually benign developmental abnormality has been reported. The clinical and pathologic features of this patient are demonstrated and reviewed in the context of prior reported cases. 0 1992 Wiley-Liss, Inc.
KEY WORDS:gastric neoplasm, enteric duplication cyst, congenital cysts, stomach abnormalities
INTRODUCTION Duplication of the alimentary tract is an unusual congenital anomaly which may occur at any level, from oral cavity to rectum. Most often presenting as a benign partially obstructing mass in the neonatal or pediatric age group, the primary surgical treatment is directed towards relief of the symptoms while preserving as much normal gastrointestinal length and physiology as possible [ 1-31. Enteric duplications presenting in the adult population are much less common, and the stomach is the site least often affected regardless of age [4-71. Malignancy arising within a duplication cyst is extremely rare, unreported in children, with only nine cases reported in adults to date [8,9]. Two of these malignancies arose within gastric duplication cysts [lo,]11, and to this experience we add a third case report.
was no succussion splash and no mass was appreciated. Complete blood count and liver function tests were normal. An upper GI series revealed a vague but consistent filling defect in the gastric antrum with some associated gastric antral deformity (Fig. 1). Computerized tomography (CT) scan revealed an antral soft tissue mass, but no regional lymphadenopathy, no ascites, and no liver metastases (Fig. 2). Upper GI endoscopy showed a 2 X 2 cm submucosal mass in the antrum with normal overlying mucosa; biopsies showed only mild inflammatory changes. At operation the antral mass was found to be mobile with a small area of serosal puckering; there was a separate small omental nodule noted within 3 cm of the mass. No gross regional lymphadenopathy was seen. The patient underwent a radical distal subtotal gastrectomy with Billroth I reconstruction. Postoperatively she had a prolonged gastric ileus which eventually resolved. She is CASE REPORT A 72 year old Hispanic woman presented in October currently well and free of disease 72 months after her 1985 with a 1 year history of vague postprandial epigas- operation. tric pain and fullness. This was associated with a 10 PATHOLOGY pound weight loss, but she reported no vomiting or The operative specimen was a segment of distal symptoms related to gastrointestinal (GI) bleeding. She stomach bearing a 3.2 cm anterior intramural cyst had no history of peptic ulcer disease. adjacent to the greater curvature. The cyst contained Her medical history was significant only for a bilateral mucoid fluid and did not communicate with the gastric tubal ligation after a single pregnancy. She had no other significant medical illnesses, and was a nonsmoker and lumen. The cyst lining had a granular region with nondrinker. Her family history was significant in that her father had died of gastric cancer, and her sister had died Accepted for publication August 6, 1991. of breast cancer. Address reprint requests to Dr. Daniel G. Coit, Department of Surgery, Physical examination was essentially unremarkable, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New with only minimal epigastric tenderness elicited. There York, NY. 10021.
0 1992 Wfley-Lm, Inc.
Malignant Gastric Duplication Cyst
275
Fig. 2. Abdominal CT scan demonstrating cystic antral soft tissue mass (arrows).
Fig. I . defect.
Upper GI X-ray series demonstrating persistent antral filling
thickening of the subjacent muscle wall and retraction of the overlying serosa. A distinctly separate ill defined 0.3 cm submucosal nodule was identified in the wall of the lesser curvature. The gastric mucosa was intact; no other lesions were identified. The attached omental fat contained two firm 1 cm nodules. Microscopic examination revealed an in situ and infiltrating papillary mucinous adenocarcinoma arising within a gastric duplication cyst. The duplication consisted of the large grossly observed cyst and several microscopic locules scattered within the muscularis propria (Fig. 3). The duplication mucosa was largely of small intestinal type with a few small foci of gastric antral mucosa. Parietal and chief cells were not seen. Much of the mucosa had a villous architecture and an exuberant papillary proliferation with a transition to in situ carcinoma corresponding to the grossly observed granular mucosal focus (Fig. 4,5).A well defined submucosa was lacking and the duplication mucosa rested on a thick smooth muscle and fibrous tissue layer which merged with the muscularis propria of the stomach. Infiltrating mucin producing carcinoma showing a spectrum of differentiation involved the full thickness of the gastric wall in the observed region of induration and serosal puckering. The carcinoma had no special features, resembling those arising de novo in the intestinal tract. Two nodules of metastatic carcinoma were demonstrated in the omental fat. The proximal and distal specimen
Fig. 3. Stomach wall with intact normal antral mucosa and duplication lumen lined by small intestinal type mucosa (large arrow). Shared incomplete smooth muscle wall (small arrow). (Hematoxylin and eosin.)
margins were free of tumor as were twelve regional lymph nodes. The observed lesser curvature nodule consisted of heterotopic pancreatic acinar tissue without islets of Langerhans.
276
Coit and Mies
Fig. 4. Benign small intestinal type mucosa lining duplication. (Hematoxylin and eosin.)
DISCUSSION The pathogenesis of alimentary tract duplications is controversial, but abnormal recanalization after the solid epithelial stage of embryonic bowel development is thought by most to underlie these lesions [ 121. This case highlights a number of the more unusual aspects of this rare congenital anomaly. Firstly, the stomach is the site of only 2 4 % of all alimentary tract duplication cysts, the least common site after the ileum, esophagus, and colon. Of reported gastric duplication cysts, most occur in females, 80% presenting in patients younger than 12 years of age. They are characteristically located on the distal greater curvature, and the majority are cystic and noncommunicating as in this case. Histologically they are usually lined with typical gastric mucosa, although ectopic pancreatic tissue has been reported present in about 9% of patients [5-71. The separate pancreatic ectopia located remote from the duplication in this patient was probably unrelated to the developmental anomaly. Secondly, it is unusual for enteric duplication cysts to remain asymptomatic until adulthood, with the majority of large series reported from the neonatal and pediatric age group [ 1,2]. Anderson et al. collected 63 cases of “adult” enteric duplication cysts from the literature in patients aged 14-67; only 14 patients in this series (23%) were aged 50 or older [4]. To our knowledge, the patient presented herein is the oldest to be reported with an antemorterndiagnosis Of an enteric cyst. Thirdly, enteric duplication cysts are always benign in
Fig. 5 . Transition to in situ carcinoma within the duplication mucosa (arrow). (Hematoxylin and eosin.)
Malignant Gastric Duplication Cyst
277
TABLE I. Clinical Characteristics of Patients With Adenocarcinoma Arising in a Gastric Duplication Cyst Reference
Ageisex
Symptoms
Physical examination
Size (cm)
Pathology 1.5 cm polypoid adenocarcinoma; one positive node Gastric ulcer with infiltrating “epithelial carcinoma” Mucinous papillary adenocarcinoma, transmural, negative nodes
Mayo et al., 1955 [lo]
64lF
Weakness, weight loss
Mass
6
Treiger et al., 1969 [ 111
50lM
Vomiting, weight loss
Mass
17
Current report (1991)
72lF
Abdominal pain, weight loss
Normal
4
FOIIOW-UP NEDa at 12 months None available
NED at 72 months
aNED. no evidence of disease.
the neonatal and pediatric age group. Including the present case, only 10 cases of malignancy arising in an enteric duplication have been reported [8-111. These have all been adults, with an age range of 37-72, and a fema1e:male ratio of 8:2. Eight of these tumors have been reported as adenocarcinomas, with one case of well differentiated squamous carcinoma and one case of “epithelial malignancy.” Seven of these cases occurred in the ileum, colon, or rectum, reflecting the more frequent occurrence of enteric duplication cysts in these sites. Only two prior cases of carcinoma in a gastric duplication cyst have been reported, with clinical details summarized in Table I. As can be seen, there is little in the clinical picture of these patients to direct the clinician to the correct preoperative diagnosis. In the case presented, the serosal puckering and omental nodule suggested a malignant process, prompting a radical distal subtotal gastrectomy with en bloc regional lymphadenectomy. The influence of the extent of the operation performed on the outcome to date is unknown. Finally, with respect to its pathologic features, the gastric duplication in this case was an organized structure comprised of an enteric mucosal lined cyst surrounded by a definite smooth muscle wall, all typical features of this entity. The pathologic differential diagnosis includes hamartoma and mature cystic teratoma. The well organized character of the duplication excludes the former diagnosis while the absence of heterologous tissues derived from more than one germ layer excludes the latter. The adenocarcinoma in this unusual case does not differ from those described in previous reports.
CONCLUSIONS A case of gastric adenocarcinoma arising within a gastric duplication cyst in a 72 year old woman is presented. This is the third reported case of a carcinoma arising within a gastric duplication cyst, and the oldest
known patient to be reported as presenting with an enteric duplication cyst. This rare evolution of an unusual developmental abnormality should be included in the differential diagnosis of partially obstructing cystic masses of the GI tract.
ACKNOWLEDGMENTS The authors would like to acknowledge the assistance of Mr. Kin Kong and Ms. Lisa Hollis for their help in preparing the photomicrographs. Supported in part by NIH grant CA 01151.
REFERENCES 1. Ildstad ST, Tollerud DJ, Weiss RG, Ryan DP, McGowan MA,
2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
12.
Martin LW: Duplications of the alimentary tract, clinical characteristics, preferred treatment, and associated malformations. Ann Surg 208:18&189, 1988. Holcomb GW, Gheissari A, O’Neill JA, Shorter NA, Bishop HC: Surgical management of alimentary tract duplications. Ann Surg 209: 167-1 74, 1989. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107:434-442, 1964. Anderson MC, Silberman WW, Shields TW: Duplications of the alimentary tract in the adult. Arch Surg 85:110-124, 1962. Bartels RJ: Duplication of the stomach, a case report and review of the literature. Am Surg 33:747-752, 1967. Lewis PL, Holder T, Feldman M: Duplication of the stomach, report of a case and review of the English literature. Arch Surg 82:18&186, 1961. Abrami G, Dennison WM: Duplication of the stomach. Surgery 49:79&801, 1961. Orr MM, Edwards AJ: Neoplastic change in duplications of the alimentary tract. Br J Surg 62:269-274, 1975. Weitzel RA, Breed JR: Carcinoma arising in a rectal duplication (enterocystoma). Ann Surg 157:476480, 1963. Mayo HW, McKee EE, Anderson RM: Carcinoma arising in reduplication of the stomach (gastrogenous cyst): a case report. Ann Surg 141:550-555, 1955. Treiger M, Rubens J, Chindler J, Lobao M, Keiserman I, Martins J, San Juan F: Duplicao gastrica, apresentacao do segundo caso da literatura complicado por ulcera peptica e neoplasia maligna. 0 Hospital 75: 1-10, 1969. Gross RE, Holcomb JW, Farber S: Duplications of the alimentary tract. Pediatrics 9449-467, 1952.
Adenocarcinoma Arising Within a Gastric Duplication Cyst DANIEL G.COIT, MD, FACS, AND CAROLYN MIES, MD From the Departments of Surgery (D.C.C.) and Pathology (C.M.), Memorial Sloan-Kettering Cancer Center, New York, New York
A rare case of carcinoma arising within a gastric duplication cyst in a 72 year old woman is presented. This is the oldest patient in whom neoplastic transformation of this usually benign developmental abnormality has been reported. The clinical and pathologic features of this patient are demonstrated and reviewed in the context of prior reported cases. 0 1992 Wiley-Liss, Inc.
KEY WORDS:gastric neoplasm, enteric duplication cyst, congenital cysts, stomach abnormalities
INTRODUCTION Duplication of the alimentary tract is an unusual congenital anomaly which may occur at any level, from oral cavity to rectum. Most often presenting as a benign partially obstructing mass in the neonatal or pediatric age group, the primary surgical treatment is directed towards relief of the symptoms while preserving as much normal gastrointestinal length and physiology as possible [ 1-31. Enteric duplications presenting in the adult population are much less common, and the stomach is the site least often affected regardless of age [4-71. Malignancy arising within a duplication cyst is extremely rare, unreported in children, with only nine cases reported in adults to date [8,9]. Two of these malignancies arose within gastric duplication cysts [lo,]11, and to this experience we add a third case report.
was no succussion splash and no mass was appreciated. Complete blood count and liver function tests were normal. An upper GI series revealed a vague but consistent filling defect in the gastric antrum with some associated gastric antral deformity (Fig. 1). Computerized tomography (CT) scan revealed an antral soft tissue mass, but no regional lymphadenopathy, no ascites, and no liver metastases (Fig. 2). Upper GI endoscopy showed a 2 X 2 cm submucosal mass in the antrum with normal overlying mucosa; biopsies showed only mild inflammatory changes. At operation the antral mass was found to be mobile with a small area of serosal puckering; there was a separate small omental nodule noted within 3 cm of the mass. No gross regional lymphadenopathy was seen. The patient underwent a radical distal subtotal gastrectomy with Billroth I reconstruction. Postoperatively she had a prolonged gastric ileus which eventually resolved. She is CASE REPORT A 72 year old Hispanic woman presented in October currently well and free of disease 72 months after her 1985 with a 1 year history of vague postprandial epigas- operation. tric pain and fullness. This was associated with a 10 PATHOLOGY pound weight loss, but she reported no vomiting or The operative specimen was a segment of distal symptoms related to gastrointestinal (GI) bleeding. She stomach bearing a 3.2 cm anterior intramural cyst had no history of peptic ulcer disease. adjacent to the greater curvature. The cyst contained Her medical history was significant only for a bilateral mucoid fluid and did not communicate with the gastric tubal ligation after a single pregnancy. She had no other significant medical illnesses, and was a nonsmoker and lumen. The cyst lining had a granular region with nondrinker. Her family history was significant in that her father had died of gastric cancer, and her sister had died Accepted for publication August 6, 1991. of breast cancer. Address reprint requests to Dr. Daniel G. Coit, Department of Surgery, Physical examination was essentially unremarkable, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New with only minimal epigastric tenderness elicited. There York, NY. 10021.
0 1992 Wfley-Lm, Inc.
Malignant Gastric Duplication Cyst
275
Fig. 2. Abdominal CT scan demonstrating cystic antral soft tissue mass (arrows).
Fig. I . defect.
Upper GI X-ray series demonstrating persistent antral filling
thickening of the subjacent muscle wall and retraction of the overlying serosa. A distinctly separate ill defined 0.3 cm submucosal nodule was identified in the wall of the lesser curvature. The gastric mucosa was intact; no other lesions were identified. The attached omental fat contained two firm 1 cm nodules. Microscopic examination revealed an in situ and infiltrating papillary mucinous adenocarcinoma arising within a gastric duplication cyst. The duplication consisted of the large grossly observed cyst and several microscopic locules scattered within the muscularis propria (Fig. 3). The duplication mucosa was largely of small intestinal type with a few small foci of gastric antral mucosa. Parietal and chief cells were not seen. Much of the mucosa had a villous architecture and an exuberant papillary proliferation with a transition to in situ carcinoma corresponding to the grossly observed granular mucosal focus (Fig. 4,5).A well defined submucosa was lacking and the duplication mucosa rested on a thick smooth muscle and fibrous tissue layer which merged with the muscularis propria of the stomach. Infiltrating mucin producing carcinoma showing a spectrum of differentiation involved the full thickness of the gastric wall in the observed region of induration and serosal puckering. The carcinoma had no special features, resembling those arising de novo in the intestinal tract. Two nodules of metastatic carcinoma were demonstrated in the omental fat. The proximal and distal specimen
Fig. 3. Stomach wall with intact normal antral mucosa and duplication lumen lined by small intestinal type mucosa (large arrow). Shared incomplete smooth muscle wall (small arrow). (Hematoxylin and eosin.)
margins were free of tumor as were twelve regional lymph nodes. The observed lesser curvature nodule consisted of heterotopic pancreatic acinar tissue without islets of Langerhans.
276
Coit and Mies
Fig. 4. Benign small intestinal type mucosa lining duplication. (Hematoxylin and eosin.)
DISCUSSION The pathogenesis of alimentary tract duplications is controversial, but abnormal recanalization after the solid epithelial stage of embryonic bowel development is thought by most to underlie these lesions [ 121. This case highlights a number of the more unusual aspects of this rare congenital anomaly. Firstly, the stomach is the site of only 2 4 % of all alimentary tract duplication cysts, the least common site after the ileum, esophagus, and colon. Of reported gastric duplication cysts, most occur in females, 80% presenting in patients younger than 12 years of age. They are characteristically located on the distal greater curvature, and the majority are cystic and noncommunicating as in this case. Histologically they are usually lined with typical gastric mucosa, although ectopic pancreatic tissue has been reported present in about 9% of patients [5-71. The separate pancreatic ectopia located remote from the duplication in this patient was probably unrelated to the developmental anomaly. Secondly, it is unusual for enteric duplication cysts to remain asymptomatic until adulthood, with the majority of large series reported from the neonatal and pediatric age group [ 1,2]. Anderson et al. collected 63 cases of “adult” enteric duplication cysts from the literature in patients aged 14-67; only 14 patients in this series (23%) were aged 50 or older [4]. To our knowledge, the patient presented herein is the oldest to be reported with an antemorterndiagnosis Of an enteric cyst. Thirdly, enteric duplication cysts are always benign in
Fig. 5 . Transition to in situ carcinoma within the duplication mucosa (arrow). (Hematoxylin and eosin.)
Malignant Gastric Duplication Cyst
277
TABLE I. Clinical Characteristics of Patients With Adenocarcinoma Arising in a Gastric Duplication Cyst Reference
Ageisex
Symptoms
Physical examination
Size (cm)
Pathology 1.5 cm polypoid adenocarcinoma; one positive node Gastric ulcer with infiltrating “epithelial carcinoma” Mucinous papillary adenocarcinoma, transmural, negative nodes
Mayo et al., 1955 [lo]
64lF
Weakness, weight loss
Mass
6
Treiger et al., 1969 [ 111
50lM
Vomiting, weight loss
Mass
17
Current report (1991)
72lF
Abdominal pain, weight loss
Normal
4
FOIIOW-UP NEDa at 12 months None available
NED at 72 months
aNED. no evidence of disease.
the neonatal and pediatric age group. Including the present case, only 10 cases of malignancy arising in an enteric duplication have been reported [8-111. These have all been adults, with an age range of 37-72, and a fema1e:male ratio of 8:2. Eight of these tumors have been reported as adenocarcinomas, with one case of well differentiated squamous carcinoma and one case of “epithelial malignancy.” Seven of these cases occurred in the ileum, colon, or rectum, reflecting the more frequent occurrence of enteric duplication cysts in these sites. Only two prior cases of carcinoma in a gastric duplication cyst have been reported, with clinical details summarized in Table I. As can be seen, there is little in the clinical picture of these patients to direct the clinician to the correct preoperative diagnosis. In the case presented, the serosal puckering and omental nodule suggested a malignant process, prompting a radical distal subtotal gastrectomy with en bloc regional lymphadenectomy. The influence of the extent of the operation performed on the outcome to date is unknown. Finally, with respect to its pathologic features, the gastric duplication in this case was an organized structure comprised of an enteric mucosal lined cyst surrounded by a definite smooth muscle wall, all typical features of this entity. The pathologic differential diagnosis includes hamartoma and mature cystic teratoma. The well organized character of the duplication excludes the former diagnosis while the absence of heterologous tissues derived from more than one germ layer excludes the latter. The adenocarcinoma in this unusual case does not differ from those described in previous reports.
CONCLUSIONS A case of gastric adenocarcinoma arising within a gastric duplication cyst in a 72 year old woman is presented. This is the third reported case of a carcinoma arising within a gastric duplication cyst, and the oldest
known patient to be reported as presenting with an enteric duplication cyst. This rare evolution of an unusual developmental abnormality should be included in the differential diagnosis of partially obstructing cystic masses of the GI tract.
ACKNOWLEDGMENTS The authors would like to acknowledge the assistance of Mr. Kin Kong and Ms. Lisa Hollis for their help in preparing the photomicrographs. Supported in part by NIH grant CA 01151.
REFERENCES 1. Ildstad ST, Tollerud DJ, Weiss RG, Ryan DP, McGowan MA,
2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
12.
Martin LW: Duplications of the alimentary tract, clinical characteristics, preferred treatment, and associated malformations. Ann Surg 208:18&189, 1988. Holcomb GW, Gheissari A, O’Neill JA, Shorter NA, Bishop HC: Surgical management of alimentary tract duplications. Ann Surg 209: 167-1 74, 1989. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107:434-442, 1964. Anderson MC, Silberman WW, Shields TW: Duplications of the alimentary tract in the adult. Arch Surg 85:110-124, 1962. Bartels RJ: Duplication of the stomach, a case report and review of the literature. Am Surg 33:747-752, 1967. Lewis PL, Holder T, Feldman M: Duplication of the stomach, report of a case and review of the English literature. Arch Surg 82:18&186, 1961. Abrami G, Dennison WM: Duplication of the stomach. Surgery 49:79&801, 1961. Orr MM, Edwards AJ: Neoplastic change in duplications of the alimentary tract. Br J Surg 62:269-274, 1975. Weitzel RA, Breed JR: Carcinoma arising in a rectal duplication (enterocystoma). Ann Surg 157:476480, 1963. Mayo HW, McKee EE, Anderson RM: Carcinoma arising in reduplication of the stomach (gastrogenous cyst): a case report. Ann Surg 141:550-555, 1955. Treiger M, Rubens J, Chindler J, Lobao M, Keiserman I, Martins J, San Juan F: Duplicao gastrica, apresentacao do segundo caso da literatura complicado por ulcera peptica e neoplasia maligna. 0 Hospital 75: 1-10, 1969. Gross RE, Holcomb JW, Farber S: Duplications of the alimentary tract. Pediatrics 9449-467, 1952.
