Surgery Today Jpn. J. Surg. (1992) 22:561-564
~
SURGERYTODAY
© Springer-Verlag 1992
Primary Squamous Cell Carcinoma of the Stomach In a Seventeen-Year-Old Boy GERHARD SCHWAB,| GEROLDWETSCHER,1 OTTO DIETZE,2 KURT SCHMID,2 and RUDOLF POINTNER1 1The SecondDepartment of Surgeryand 2the Department of Pathology,Universityof Innsbruck, Anichstrage 35, A-6060Innsbruck, Austria
Abstract: Primary squamous cell carcinoma (SCC) of the stomach is extremely rare and thought to arise from ectopic squamous epithelium, which in turn could either result from the squamous metaplasia of the gastric mucosa or be congenital in origin. We report herein a case of SCC of the stomach in a 17-year-old male, who died 1 year after undergoing a gastrectomy. To our knowledge, this is the youngest case of primary SCC of the stomach reported in the literature. Further speculative possibilities of the development of primary gastric SCC are discussed following the presentation of this case. Key Words: primary SCC of the stomach, squamous
metaplasia of the gastric mucosa
Introduction
Primary squamous cell carcinoma (SCC) of the stomach is extremely rare with less than 80 cases having been reported in the literature worldwide. 1-3 The incidence of this tumor peaks in patients about 1 decade earlier than adenocarcinoma of the stomach 2'4 and several controversial theories have been discussed regarding the pathogenesis of this rare neoplasm. 2'5-7 The youngest reported case of primary SCC of the stomach thus far documented in the literature was that of a 26-year-old woman. 8 However, we present herein the case of a 17-year-old male, who was admitted to our hospital with a stenosis of the oesophagogastric junction caused by a true primary SCC of the stomach.
Reprint requests to: G. Schwab (Received for publication on Mar. 12, 1991; accepted on Mar. 13, 1992)
Case Report
A 17-year-old boy with a history of having lost 10 kg in weight over the previous 2 months was admitted to our hospital with increasing dysphagia. An epigastric tumor mass was palpable and the lymph nodes were enlarged in both axillae. All laboratory examinations, however, were found to be within the normal range. X-ray examination revealed a cardial stenosis of the stomach and delayed passage but no certain signs of malignancy. Ultrasonographically the cardial wall of the stomach appeared thickened to about 3 cm (Fig. 1) and several enlarged epigastric and parapancreatic lymph nodes were identified. Endoscopically the esophagus was inconspicious and the cardial stenosis was dilated under general anesthesia to allow the endoscope to pass. The mucosa of the cardia was dark reddish in colour and extremely vulnerable but no unequivocal tumorous or ulcerated tissue was found. The only suspicious finding was a thickened and slightly protuberant mucosa in the subcardial parts of the stomach being strongly indicative of a submucosal tumor (Fig. 2). Histologic examination of biopsies taken from this lesion showed an angioinvasive tumor composed of small cells with roundish or oval nuclei infiltrating the lamina propria of the stomach wall. Immunohistochemically, the tumor cells were positive for cytokeratin antibodies and the tumor was diagnosed as a non-keratinizing primary SCC of the small cell type. Subsequently a total gastrectomy was performed. Macroscopically, the tumor invaded both the anterior and posterior walls of the stomach with the main tumor mass being located in the lesser curvature. The tumor was particularly sharply demarcated from the esophagus (Fig. 3). Numerous lymph nodes were found both in the greater and lesser omentum. The histologic appearance of the tumor was quite similar to that found in the endoscopically obtained
562
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
Stomach with thickened wall
Liver
•~
S ~ - ~
.--
~f
S
biopsies, although in addition to the non-keratinizing small cell type of SCC, areas of minimal keratinizing SCC of the large cell type were also demonstrated. In most parts of the carcinoma, tumor cells had infiltrated the lamina propria underneath the normal gastric mucosa (Fig. 4). The esophagus was histologically tumor free. Postoperative follow-up was uneventful for 6 months, however, extensive metastatic tumor growth in the mediastinum resulted in increasing esophageal stenosis. The patient died 1 year after the diagnosis of primary SCC of the stomach had been made. Post
Fig. 1. Sonographic appearance of the thickened wall of the stomach (arrows) (top) and schema of sonograph (bottom)
mortem examination showed extensive mediastinal lymph node metastasis, arising from the gastric cancer. There was no tumor involvement of the esophagus during autopsy.
Discussion
The diagnosis of primary SCC of the stomach is only correct if an esophageal origin of the tumor can be excluded. 9 Following this criterion there are only about 80 cases of true SCC of the stomach reported in the
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
563
Fig. 2. Endoscopic view of the cardiac part of the stomach in inversion. C, carcinoma
literature. According to the California Cancer Register this tumor comprises only 0.04% of all gastric cancers. 3 H o e r r et al. 1° found one case in their series of 244 gastric carcinomas and Scheffler and Falk 11 found 2 out of 273 cases. In our hospital, the present case was the first seen from a total of 800 carcinomas of the stomach diagnosed in the last 10 years. According to a study published by Altshuler and Shaha in 19655 primary SCC of the stomach should have a better prognosis than the more " c o m m o n " adenocarcinoma. However, since the 5-year survival rate of adenocarcinoma of the stomach has increased consistently over the last 20 years, this statement has to be reviewed. In recently puplished case reports dealing with primary SCC of the stomach, all patients with one exception 1 survived gastrectomy by only a few months. Our patient died 12 months after his operation. Endoscopy was performed preoperatively in only five of the cases reported in the literature and a correct histologic diagnosis was obtained on biopsy material in only two of these cases. Although there was no doubt about the malignant nature of this tumor, either endoscopically or histologically, it was difficult to establish the exact histologic diagnosis with regard to tumor type. Considering the age of the patient in particular, a non-keratinizing SCC of the small cell type had to be distinguished from other rare primary gastric and secondary tumors. Furthermore, gastric carcinoids can exhibit a similar histologic pattern and, according to Grould, 12 a neuroendocrine "carcinoid" differentiation can be found in SCC. In such cases an exact diagnosis is only possible by means of immunohistochemistry using epithelial and neuroendocrine markers such as cytokeratine, neuron specific enolase or chromogranins. There are several speculative theories about the development of SCC in the stomach. According to Talerman and Woo-Ming 7 SCC of the stomach located
Fig. 3. Macroscopic view of the totally-resected stomach. C, carcinoma; E, distal esophagus
at the esophagogastric junction arises from squamous cells of the distal esophagus, regardless of whether or not a connection of the tumor with the esophagus can be histologically demonstrated.
564
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
Fig. 4. Microphotograph of the muscularis propria of the resected stomach, showing small tumor foci with keratinization
Small islands of congenitally misplaced esophageal squamous mucosa might give rise to a gastric SCC. However, no such island nor any squamous metaplasia of the gastric mucosa was demonstrated in our case, although if an island was responsible for the development of SCC, it could have been destroyed during the growth of the tumor. Dixon in 1954 reported a case of rectal carcinoma resulting from squamous metaplasia 6 and the development of SCC in the stomach from squamous metaplasia in our case cannot be excluded. Theoretically, the occurrence of a precursor cell from squamous as well as glandular differentiated cells in the stomach must be considered. It is presumed that gastric chorionic carcinoma may arise from such a precursor cell 13 and squamous differentiation can also be found in the socalled adeno-acanthoma type of adenocarcinoma, 14 although this can be excluded in our case, because no glandular differentiation was demonstrated in the serial sections. Finally, it might be of interest that Huvos and Marcove suggested a vascular origin in their report of a case of squamous carcinoma. 15 Nevertheless, all these theories remain speculative until irrefutably proven.
References 1. Bonnheim DC, Sarac OK, Fett W (1985) Primary squamous cell carcinoma of the stomach. Am J Gastroenterol 80:91-94
2. Boswell JT, Helwig EB (1965) Squamous cell carcinoma and adenoacanthoma of the stomach. A clinico-pathologic study. Cancer 18:181-192 3. Strauss R, Heschel S, Fortmann DJ (1969) Primary adenosquamous carcinoma of the stomach. Cancer 24:985995 4. Hee Won OK, Farman J, Krishnan MN, Iyer SK, Vuletin JC (1978) Squamous cell carcinoma of the stomach. Am J Gastroenterol 69:594-589 5. Altshuler HJ, Shaka JA (1965) Squamous cell carcinoma of the stomach. Cancer 19:831-838 6. Dixon CF (1954) Symposium on unusual surgical lesions of the colon. Squamous cell carcinoma of the mid rectum: Report of a case. Proc Staff Meet Mayo Clin 29:420 7. Talerman A, Woo-Ming MO (1968) The origin of squamous cell carcinoma of the gastric cardia. Cancer 22:1226-1232 8. Eaton H, Tennekoon GE (1972) Squamous carcinoma of the stomach following corrosive acid burns. Br J Surg 59:382387 9. Parks RE (1967) Squamous neoplasms of the stomach. AJR 101:447-449 10. Hoerr SO, Hazard JB, Bailey D (1966) Prognosis in carcinoma of the stomach in relation to the microscopic type. Surg Gynecol Obstet 122:485 11. Scheffler MM, Falk AB (1940) Epidermoid carcinoma of the stomach. Am J Cancer 38:359-363 12. Grould VE (1981) Multidirectional differentiation in human epithelial cancers. J Submicrosc Cytol 13:79-81 13. Regan JF, Cremin JH (1960) Chorionepithelioma of the stomach. Am J Surg 10:224-228 14. Donald KJ (1967) Adenocarcinoma of the pyloric antrum with extensive squamous differentiation. J Clin Pathol 20:136-139 15. Huvos AG, Marcove RC (1975) Adamantinoma of the long bones - a clinicopathological study of cases with vascular origin suggested. J Bone Joint Surg [Am] 57:148-154
~
SURGERYTODAY
© Springer-Verlag 1992
Primary Squamous Cell Carcinoma of the Stomach In a Seventeen-Year-Old Boy GERHARD SCHWAB,| GEROLDWETSCHER,1 OTTO DIETZE,2 KURT SCHMID,2 and RUDOLF POINTNER1 1The SecondDepartment of Surgeryand 2the Department of Pathology,Universityof Innsbruck, Anichstrage 35, A-6060Innsbruck, Austria
Abstract: Primary squamous cell carcinoma (SCC) of the stomach is extremely rare and thought to arise from ectopic squamous epithelium, which in turn could either result from the squamous metaplasia of the gastric mucosa or be congenital in origin. We report herein a case of SCC of the stomach in a 17-year-old male, who died 1 year after undergoing a gastrectomy. To our knowledge, this is the youngest case of primary SCC of the stomach reported in the literature. Further speculative possibilities of the development of primary gastric SCC are discussed following the presentation of this case. Key Words: primary SCC of the stomach, squamous
metaplasia of the gastric mucosa
Introduction
Primary squamous cell carcinoma (SCC) of the stomach is extremely rare with less than 80 cases having been reported in the literature worldwide. 1-3 The incidence of this tumor peaks in patients about 1 decade earlier than adenocarcinoma of the stomach 2'4 and several controversial theories have been discussed regarding the pathogenesis of this rare neoplasm. 2'5-7 The youngest reported case of primary SCC of the stomach thus far documented in the literature was that of a 26-year-old woman. 8 However, we present herein the case of a 17-year-old male, who was admitted to our hospital with a stenosis of the oesophagogastric junction caused by a true primary SCC of the stomach.
Reprint requests to: G. Schwab (Received for publication on Mar. 12, 1991; accepted on Mar. 13, 1992)
Case Report
A 17-year-old boy with a history of having lost 10 kg in weight over the previous 2 months was admitted to our hospital with increasing dysphagia. An epigastric tumor mass was palpable and the lymph nodes were enlarged in both axillae. All laboratory examinations, however, were found to be within the normal range. X-ray examination revealed a cardial stenosis of the stomach and delayed passage but no certain signs of malignancy. Ultrasonographically the cardial wall of the stomach appeared thickened to about 3 cm (Fig. 1) and several enlarged epigastric and parapancreatic lymph nodes were identified. Endoscopically the esophagus was inconspicious and the cardial stenosis was dilated under general anesthesia to allow the endoscope to pass. The mucosa of the cardia was dark reddish in colour and extremely vulnerable but no unequivocal tumorous or ulcerated tissue was found. The only suspicious finding was a thickened and slightly protuberant mucosa in the subcardial parts of the stomach being strongly indicative of a submucosal tumor (Fig. 2). Histologic examination of biopsies taken from this lesion showed an angioinvasive tumor composed of small cells with roundish or oval nuclei infiltrating the lamina propria of the stomach wall. Immunohistochemically, the tumor cells were positive for cytokeratin antibodies and the tumor was diagnosed as a non-keratinizing primary SCC of the small cell type. Subsequently a total gastrectomy was performed. Macroscopically, the tumor invaded both the anterior and posterior walls of the stomach with the main tumor mass being located in the lesser curvature. The tumor was particularly sharply demarcated from the esophagus (Fig. 3). Numerous lymph nodes were found both in the greater and lesser omentum. The histologic appearance of the tumor was quite similar to that found in the endoscopically obtained
562
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
Stomach with thickened wall
Liver
•~
S ~ - ~
.--
~f
S
biopsies, although in addition to the non-keratinizing small cell type of SCC, areas of minimal keratinizing SCC of the large cell type were also demonstrated. In most parts of the carcinoma, tumor cells had infiltrated the lamina propria underneath the normal gastric mucosa (Fig. 4). The esophagus was histologically tumor free. Postoperative follow-up was uneventful for 6 months, however, extensive metastatic tumor growth in the mediastinum resulted in increasing esophageal stenosis. The patient died 1 year after the diagnosis of primary SCC of the stomach had been made. Post
Fig. 1. Sonographic appearance of the thickened wall of the stomach (arrows) (top) and schema of sonograph (bottom)
mortem examination showed extensive mediastinal lymph node metastasis, arising from the gastric cancer. There was no tumor involvement of the esophagus during autopsy.
Discussion
The diagnosis of primary SCC of the stomach is only correct if an esophageal origin of the tumor can be excluded. 9 Following this criterion there are only about 80 cases of true SCC of the stomach reported in the
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
563
Fig. 2. Endoscopic view of the cardiac part of the stomach in inversion. C, carcinoma
literature. According to the California Cancer Register this tumor comprises only 0.04% of all gastric cancers. 3 H o e r r et al. 1° found one case in their series of 244 gastric carcinomas and Scheffler and Falk 11 found 2 out of 273 cases. In our hospital, the present case was the first seen from a total of 800 carcinomas of the stomach diagnosed in the last 10 years. According to a study published by Altshuler and Shaha in 19655 primary SCC of the stomach should have a better prognosis than the more " c o m m o n " adenocarcinoma. However, since the 5-year survival rate of adenocarcinoma of the stomach has increased consistently over the last 20 years, this statement has to be reviewed. In recently puplished case reports dealing with primary SCC of the stomach, all patients with one exception 1 survived gastrectomy by only a few months. Our patient died 12 months after his operation. Endoscopy was performed preoperatively in only five of the cases reported in the literature and a correct histologic diagnosis was obtained on biopsy material in only two of these cases. Although there was no doubt about the malignant nature of this tumor, either endoscopically or histologically, it was difficult to establish the exact histologic diagnosis with regard to tumor type. Considering the age of the patient in particular, a non-keratinizing SCC of the small cell type had to be distinguished from other rare primary gastric and secondary tumors. Furthermore, gastric carcinoids can exhibit a similar histologic pattern and, according to Grould, 12 a neuroendocrine "carcinoid" differentiation can be found in SCC. In such cases an exact diagnosis is only possible by means of immunohistochemistry using epithelial and neuroendocrine markers such as cytokeratine, neuron specific enolase or chromogranins. There are several speculative theories about the development of SCC in the stomach. According to Talerman and Woo-Ming 7 SCC of the stomach located
Fig. 3. Macroscopic view of the totally-resected stomach. C, carcinoma; E, distal esophagus
at the esophagogastric junction arises from squamous cells of the distal esophagus, regardless of whether or not a connection of the tumor with the esophagus can be histologically demonstrated.
564
G. Schwab et al.: Primary Squamous Cell Carcinoma of the Stomach
Fig. 4. Microphotograph of the muscularis propria of the resected stomach, showing small tumor foci with keratinization
Small islands of congenitally misplaced esophageal squamous mucosa might give rise to a gastric SCC. However, no such island nor any squamous metaplasia of the gastric mucosa was demonstrated in our case, although if an island was responsible for the development of SCC, it could have been destroyed during the growth of the tumor. Dixon in 1954 reported a case of rectal carcinoma resulting from squamous metaplasia 6 and the development of SCC in the stomach from squamous metaplasia in our case cannot be excluded. Theoretically, the occurrence of a precursor cell from squamous as well as glandular differentiated cells in the stomach must be considered. It is presumed that gastric chorionic carcinoma may arise from such a precursor cell 13 and squamous differentiation can also be found in the socalled adeno-acanthoma type of adenocarcinoma, 14 although this can be excluded in our case, because no glandular differentiation was demonstrated in the serial sections. Finally, it might be of interest that Huvos and Marcove suggested a vascular origin in their report of a case of squamous carcinoma. 15 Nevertheless, all these theories remain speculative until irrefutably proven.
References 1. Bonnheim DC, Sarac OK, Fett W (1985) Primary squamous cell carcinoma of the stomach. Am J Gastroenterol 80:91-94
2. Boswell JT, Helwig EB (1965) Squamous cell carcinoma and adenoacanthoma of the stomach. A clinico-pathologic study. Cancer 18:181-192 3. Strauss R, Heschel S, Fortmann DJ (1969) Primary adenosquamous carcinoma of the stomach. Cancer 24:985995 4. Hee Won OK, Farman J, Krishnan MN, Iyer SK, Vuletin JC (1978) Squamous cell carcinoma of the stomach. Am J Gastroenterol 69:594-589 5. Altshuler HJ, Shaka JA (1965) Squamous cell carcinoma of the stomach. Cancer 19:831-838 6. Dixon CF (1954) Symposium on unusual surgical lesions of the colon. Squamous cell carcinoma of the mid rectum: Report of a case. Proc Staff Meet Mayo Clin 29:420 7. Talerman A, Woo-Ming MO (1968) The origin of squamous cell carcinoma of the gastric cardia. Cancer 22:1226-1232 8. Eaton H, Tennekoon GE (1972) Squamous carcinoma of the stomach following corrosive acid burns. Br J Surg 59:382387 9. Parks RE (1967) Squamous neoplasms of the stomach. AJR 101:447-449 10. Hoerr SO, Hazard JB, Bailey D (1966) Prognosis in carcinoma of the stomach in relation to the microscopic type. Surg Gynecol Obstet 122:485 11. Scheffler MM, Falk AB (1940) Epidermoid carcinoma of the stomach. Am J Cancer 38:359-363 12. Grould VE (1981) Multidirectional differentiation in human epithelial cancers. J Submicrosc Cytol 13:79-81 13. Regan JF, Cremin JH (1960) Chorionepithelioma of the stomach. Am J Surg 10:224-228 14. Donald KJ (1967) Adenocarcinoma of the pyloric antrum with extensive squamous differentiation. J Clin Pathol 20:136-139 15. Huvos AG, Marcove RC (1975) Adamantinoma of the long bones - a clinicopathological study of cases with vascular origin suggested. J Bone Joint Surg [Am] 57:148-154
