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Pancreatic squamous carcinoma mimicking a bleeding duodenal ulcer
Pancreatic squamous carcinoma mimicking a bleeding duodenal ulcer Jorge da Fonseca, MD, Maria José Brito, MD, Clara Castro, MD, Lygia Lopes, MD, António Folgado, MD, Francisco Murinello, MD, Cunha Leal, MD
Pancreatic squamous cell carcinoma is a rare malignancy with some similarities to the more common pancreatic adenocarcinoma. Both occur in individuals older than 50 years and early diagnosis is infrequent. These tumors seldom respond to radiotherapy or to chemotherapy and are associated with poor survival. We report a case of a pure squamous cell pancreatic carcinoma presenting as a bleeding duodenal ulcer. In addition to this unusual presentation of a rare tumor, we emphasize the role of standard endoscopic sclerotherapy in the control of the bleeding episode.
A
CASE REPORT A 52-year-old man was admitted after an episode of melena. He suffered from long-standing diabetes and consumed alcohol heavily with an average daily intake exceeding 150 gm. He had a history of duodenal ulcer that had been treated with ranitidine. Physical examination showed an alert cooperative man with normal mental status and vital signs. He was icteric and a large painful liver was palpable. No stigmata of chronic liver disease were found and his physical examination was otherwise unremarkable. Blood was present in the gastric aspirate. Laboratory tests (normal range in parentheses) were significant for a hemoglobin value of 9.5 gm/dL (12-16), total bilirubin 17.3 mg/dL (<1.2), aspartate aminotransferase 54UI/L (<25), alanine aminotransferase 49UI/L (<29), and alkaline phosphatase 273 UI/L (< 96). Urine analysis revealed bilirubin and glucose. Ultrasonography was highly suggestive of multiple liver metastasis, with dilated intrahepatic bile ducts. Urgent endoscopic examination identified a large ulcer oozing bright red blood located on the anterior wall of the duodenal bulb. Hemostasis was achieved with injection of 10 mL of 1:10.000 adrenaline solution and 6 mL of polidocanol. Endoscopy was performed again on the second day. Although simulating an ulcer (Fig. 1A), the lesion was in fact an elevated tumor (Fig. 1B). CT disclosed a large pancreatic malignancy involving the stomach and the liver hilum. Despite adequate control of the bleeding, the patient died 12 days later. The endoscopic biopsies and the autopsy identified the tumor as a pure epidermoid carcinoma aris-
From the Division of Gastroenterology, Departments of Pathology and Surgery, Hospital Garcia de Orta, Almada, Portugal. Reprint requests: Jorge da Fonseca, MD, Division of Gastroenterology, Hospital Garcia de Orta, Almada, Portugal; fax: 351-1-295-7004. Copyright © 2000 by the American Society for Gastrointestinal Endoscopy 0016-5107/2000/$12.00 + 0 37/54/104009 362
GASTROINTESTINAL ENDOSCOPY
B Figure 1. A, Endoscopic view of the duodenal bulb seen through the pylorus; the appearance of the lesion mimics a duodenal ulcer on the anterior wall. B, Closer view demonstrating the elevated tumor.
Figure 2. Photomicrograph of tissue obtained at autopsy showing the squamous carcinoma invading normal pancreatic tissue. There was no evidence of glandular differentiation (H&E, orig. mag. ×400). VOLUME 51, NO. 3, 2000
ing from the pancreas (Fig. 2). Adenomatous tissue was not present in the primary tumor or in the metastases.
DISCUSSION Adenocarcinoma is the most common form of pancreatic malignancy of duct cell origin. Adenosquamous carcinoma is an unusual ductal cell cancer with a mixture of neoplastic glandular and squamous components, with the squamous component comprising at least 30% of the tumor tissue. The relative frequency is 3% to 4%.4 Pure squamous (epidermoid) carcinoma of the pancreas is extremely rare.1-3 It seems to be a special form of adenosquamous carcinoma according to the World Health Organization (WHO) classification.4 In most cases, extensive sampling may reveal the presence of neoplastic glands in the squamous tissue.5,6 However, in some cases only pure squamous cell tumor can be demonstrated. Despite their pathologic differences, these types of pancreatic carcinoma have similar clinical patterns of presentation and biologic behavior. Epidermoid carcinoma may be associated with malignant hypercalcemia.5 Some investigators report a better response to chemotherapy,1 and some patients seem to have a more “benign” course.7 Although it may occur as a complication of a pancreatic cancer,8 upper GI hemorrhage is an unusual presentation of this malignancy.6 In our patient, the history of ulcer disease coupled with the initial
VOLUME 51, NO. 3, 2000
endoscopic appearance of the lesion made the diagnosis more difficult. To the best of our knowledge, there is no relationship between the histologic type and a presentation with bleeding. REFERENCES 1. Sears HF, Kim Y, Strawitz J. Squamous cell carcinoma of the pancreas. J Surg Oncol 1980;14:261-5. 2. Nakashima H, Hayakawa T, Hoshino M, Kamiya Y, Ohara H, Yamada T, et al. Squamous cell carcinoma of the pancreas with massive invasion of the retroperitoneum. Intern Med 1995;34:61-4. 3. Serafini F, Rosemurgy AS, Carey LC. Squamous cell carcinoma of the pancreas. Am J Gastroenterol 1996;91:2621-2. 4. Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumors of the exocrine pancreas. (WHO International Histological Classification of Tumors). Berlin: Springer; 1996. p. 17. 5. Pedrajas JM, Valverde JJ, Téllez MJ, Castaño A, Pieltain R, Fernández-Cruz A. Carcinoma epidermoide de páncreas asociado a hipercalcémia humoral maligna. Rev Clín Esp 1993;193:73-5. 6. Bringel RWA, Souza CPM, Araújo SEA, Lopassa FP, GamaRodrigues J, Pinotti HW, et al. Squamous cell carcinoma of the pancreas with gastric metastasis. Case report. Rev Hosp Clín Fac Med S Paulo 1996;51:195-7. 7. Squamous cell carcinoma of the pancreas. Report of an unusual case and review of the literature. Dig Dis Sci 1992; 37:312-8. 8. Angelescu N, Jitea N, Cristian D, Mircea N. Cancer de Cap de Pancreas cu Evolutie Particulara (Cancer of the Head of the Pancreas with a Unique Evolution). Chirurgia (Bucur) 1996; 45:129-32.
GASTROINTESTINAL ENDOSCOPY
363
Pancreatic squamous cell carcinoma is a rare malignancy with some similarities to the more common pancreatic adenocarcinoma. Both occur in individuals older than 50 years and early diagnosis is infrequent. These tumors seldom respond to radiotherapy or to chemotherapy and are associated with poor survival. We report a case of a pure squamous cell pancreatic carcinoma presenting as a bleeding duodenal ulcer. In addition to this unusual presentation of a rare tumor, we emphasize the role of standard endoscopic sclerotherapy in the control of the bleeding episode.
A
CASE REPORT A 52-year-old man was admitted after an episode of melena. He suffered from long-standing diabetes and consumed alcohol heavily with an average daily intake exceeding 150 gm. He had a history of duodenal ulcer that had been treated with ranitidine. Physical examination showed an alert cooperative man with normal mental status and vital signs. He was icteric and a large painful liver was palpable. No stigmata of chronic liver disease were found and his physical examination was otherwise unremarkable. Blood was present in the gastric aspirate. Laboratory tests (normal range in parentheses) were significant for a hemoglobin value of 9.5 gm/dL (12-16), total bilirubin 17.3 mg/dL (<1.2), aspartate aminotransferase 54UI/L (<25), alanine aminotransferase 49UI/L (<29), and alkaline phosphatase 273 UI/L (< 96). Urine analysis revealed bilirubin and glucose. Ultrasonography was highly suggestive of multiple liver metastasis, with dilated intrahepatic bile ducts. Urgent endoscopic examination identified a large ulcer oozing bright red blood located on the anterior wall of the duodenal bulb. Hemostasis was achieved with injection of 10 mL of 1:10.000 adrenaline solution and 6 mL of polidocanol. Endoscopy was performed again on the second day. Although simulating an ulcer (Fig. 1A), the lesion was in fact an elevated tumor (Fig. 1B). CT disclosed a large pancreatic malignancy involving the stomach and the liver hilum. Despite adequate control of the bleeding, the patient died 12 days later. The endoscopic biopsies and the autopsy identified the tumor as a pure epidermoid carcinoma aris-
From the Division of Gastroenterology, Departments of Pathology and Surgery, Hospital Garcia de Orta, Almada, Portugal. Reprint requests: Jorge da Fonseca, MD, Division of Gastroenterology, Hospital Garcia de Orta, Almada, Portugal; fax: 351-1-295-7004. Copyright © 2000 by the American Society for Gastrointestinal Endoscopy 0016-5107/2000/$12.00 + 0 37/54/104009 362
GASTROINTESTINAL ENDOSCOPY
B Figure 1. A, Endoscopic view of the duodenal bulb seen through the pylorus; the appearance of the lesion mimics a duodenal ulcer on the anterior wall. B, Closer view demonstrating the elevated tumor.
Figure 2. Photomicrograph of tissue obtained at autopsy showing the squamous carcinoma invading normal pancreatic tissue. There was no evidence of glandular differentiation (H&E, orig. mag. ×400). VOLUME 51, NO. 3, 2000
ing from the pancreas (Fig. 2). Adenomatous tissue was not present in the primary tumor or in the metastases.
DISCUSSION Adenocarcinoma is the most common form of pancreatic malignancy of duct cell origin. Adenosquamous carcinoma is an unusual ductal cell cancer with a mixture of neoplastic glandular and squamous components, with the squamous component comprising at least 30% of the tumor tissue. The relative frequency is 3% to 4%.4 Pure squamous (epidermoid) carcinoma of the pancreas is extremely rare.1-3 It seems to be a special form of adenosquamous carcinoma according to the World Health Organization (WHO) classification.4 In most cases, extensive sampling may reveal the presence of neoplastic glands in the squamous tissue.5,6 However, in some cases only pure squamous cell tumor can be demonstrated. Despite their pathologic differences, these types of pancreatic carcinoma have similar clinical patterns of presentation and biologic behavior. Epidermoid carcinoma may be associated with malignant hypercalcemia.5 Some investigators report a better response to chemotherapy,1 and some patients seem to have a more “benign” course.7 Although it may occur as a complication of a pancreatic cancer,8 upper GI hemorrhage is an unusual presentation of this malignancy.6 In our patient, the history of ulcer disease coupled with the initial
VOLUME 51, NO. 3, 2000
endoscopic appearance of the lesion made the diagnosis more difficult. To the best of our knowledge, there is no relationship between the histologic type and a presentation with bleeding. REFERENCES 1. Sears HF, Kim Y, Strawitz J. Squamous cell carcinoma of the pancreas. J Surg Oncol 1980;14:261-5. 2. Nakashima H, Hayakawa T, Hoshino M, Kamiya Y, Ohara H, Yamada T, et al. Squamous cell carcinoma of the pancreas with massive invasion of the retroperitoneum. Intern Med 1995;34:61-4. 3. Serafini F, Rosemurgy AS, Carey LC. Squamous cell carcinoma of the pancreas. Am J Gastroenterol 1996;91:2621-2. 4. Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumors of the exocrine pancreas. (WHO International Histological Classification of Tumors). Berlin: Springer; 1996. p. 17. 5. Pedrajas JM, Valverde JJ, Téllez MJ, Castaño A, Pieltain R, Fernández-Cruz A. Carcinoma epidermoide de páncreas asociado a hipercalcémia humoral maligna. Rev Clín Esp 1993;193:73-5. 6. Bringel RWA, Souza CPM, Araújo SEA, Lopassa FP, GamaRodrigues J, Pinotti HW, et al. Squamous cell carcinoma of the pancreas with gastric metastasis. Case report. Rev Hosp Clín Fac Med S Paulo 1996;51:195-7. 7. Squamous cell carcinoma of the pancreas. Report of an unusual case and review of the literature. Dig Dis Sci 1992; 37:312-8. 8. Angelescu N, Jitea N, Cristian D, Mircea N. Cancer de Cap de Pancreas cu Evolutie Particulara (Cancer of the Head of the Pancreas with a Unique Evolution). Chirurgia (Bucur) 1996; 45:129-32.
GASTROINTESTINAL ENDOSCOPY
363