Pancreatic Tumor of Mesenchymal Origin Presenting as Obstructive Jaundice Richard M. Margules, MD, San Francisco, California Robert E. Allen, MD, San Francisco, California J. Englebert Dunphy, MD, San Francisco, California

Recently, a patient with an unusual pancreatic tumor of mesenchymal origin presented to the University of California, San Francisco Medical Center. The exact pathologic identification of this pancreatic lesion is in doubt. The lesion has features resembling both myxofibrosarcoma and fibrous histiocytoma. Many authors have attempted to summarize the literature on pancreatic sarcomata. As many as fifty cases have been reported in autopsy studies since 1882. Several of these on review appear to be secondary involvement of the pancreas by systemic lymphomas. Others are of such bulk and involving so many contiguous organs as to cast doubt on tissue of origin. One must conclude that the real incidence is extremely rare. Only five operated cases of pancreatic sarcomata could be found in surgical series and four of these were of such bulk and invasiveness as to cloud their exact site of origin. To our knowledge, a fibrous histiocytoma, a benign mesenchymal tumor, of the head of the pancreas has not previously been reported in either surgical or autopsy series. The following is a report of our case. Case Report A twenty-two year old white female was hospitalized at the University of California Medical Center, San Francisco (UCSF) on October 2, 1972, with a diagnosis of chronic active hepatitis. The patient had been well until April 1972 when she began complaining of postprandial pain in the right upper quadrant. Nausea and malaise were associated with her pain. In May 1972, dark urine, pruritus, and increasing malaise were noted. The liver was enlarged on physical examination and liver function tests were abnormal. A diagnosis of hepatitis was entertained. From the Department of Surgery, University of California School of Medicine. San Francisco. California. Reprint requests $hould be addressed to J. Englebert Dunphy, MD. Department of Surgery. University of California School of Medicine. San Francisco, California 94143.

votuma 131. Merch 1979

Despite medical treatment, her liver functions continued to deteriorate and in August 1972, a liver biopsy was interpreted as “postnecrotic cirrhosis with areas of chronic active hepatitis.” She was subsequently started on a course of steroids. Because of progression of her illness and a forty pound weight loss, the patient was transferred to UCSF on October 2, 1972. On admission, she was cathectic and jaundiced. Her liver was nontender and extended 10 cm below the right costal margin. Initial laboratory data included: total bilirubin, 5.7 mg/106 ml; direct bilirubin, 2.6 mg/106 ml; alkaline phosphatase, 810 international units/L; serum glutamic oxaloacetic transaminase (SGOT), 60 international units/L; and prothrombin time, 11.4 seconds. Our pathology department reviewed the liver biopsy done in August and reported “consistent with cholestasis with some inflammation and probably not hepatitis.” Upper gastrointestinal series showed extrinsic compression of the duodenal bulb and sweep by the head of the pancreas. Barium enema revealed a large right upper quadrant extrinsic filling defect impinging upon the transverse colon. Percutaneous cholangiography demonstrated a dilated intrahepatic biliary tree, a dilated tortuous common bile duct without filling of the distal third, and no filling of the gallbladder. (Figure 1.) On October 9, 1972, exploratory laparotomy demonstrated an enlarged liver, a 3 cm common bile duct, a tense thin walled gallbladder, and a 10 cm doughy mass in the head of the pancreas effacing the duodenum. Needle aspiration of the mass yielded a scant amount of gelatinous material. Multiple node biopsies were negative and transduodenal biopsy of the mass demonstrated a 1 cm thick fibrous capsule overlying a yellow myxomatous center. Frozen section was inconclusive. The pathologist thought the mass was a benign myxomatous cyst but a low grade myxosarcoma could not be ruled out. Permanent pathologic sections of the biopsy were interpreted as low grade myxofibrosarcoma and on October 16, 1972, a pancreaticoduodenectomy was performed. Final opinion on the histologic diagnosis was divided within our own institution and other pathologists’ opinions did not resolve the question. (Figures 2 and 3.) The diagnosis was divided between low grade myxofibrosarcoma and fibrous histiocytoma obstructing the duo-

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Figure 2. Gtvss photograph of the resected &nor and intestine. Circumscribed mass in head of pancreas. On cut surface the mass was bright yeiiow in co/or with a s~fl consistency.

Figure 1. Percutaneous cholangiogram reveaiing dilated intrahepatic ducts. The dilated structure resembling a gaiibiadder was shown to be dilated proximal common duct at exploration.

bilirubin, 1.0 mg/lOO ml; alkaline phosphatase, 350 international units/L; SGOT, 92 international unit& The patient was subsequently followed in clinic where she continued to do well and on March 1, 1974, seventeen months after resection, she was readmitted for reevaluation. At that time she weighed 54.5 kg and felt well except for a recent flu-like syndrome. Her liver was palpable 4 cm below the right costal margin and measured 12 cm in span. Laboratory data kere as follows: total bilirubin, 0.4 mg/lOO ml; alkaline phosphatase, 118 international units/L; SGOT, 30 international units/L; fasting blood sugar, 91 mg/lOO ml. Upper gastrointestinal series demonstrated all anastomoses to be patent, a normal caliber extrahepatic biliary tree, mild esophageal reflux, and some esophageal edema.

Comments

Figure 3. Representative photomicrography of the specimen. High power view of pancreatic tumor showing a ioose mesh of small spindie ceils which contaiq their fiiamentous cytoplasm/c projections and small round to oval nuclei. denum and common duct. Postoperatively, the patient developed a left subphrenic abscess which responded to drainage and antibiotics. She was discharged on a regular diet and gained weight without pancreatic supplements. Discharge laboratory data were as follows: total

Autopsy cases of sarcomata of the pancreas have on occasion been reported in the literature. Unfortunately, it has been difficult to rule out other sites of origin, such as stomach, duodenum, or bile duct because of the bulk of these lesions and the invasion of contiguous structures. Five operative cases have been reported in the surgical literature as pancreatic sarcomata. In one of these reports the tumor was adherent to the duodenal wall and could be shelled out of the pancreas, thus raising the question of duodenal wall origin [I]. A second case report describes a tumor which was very bulky and reportedly “adherent to but not invading the stomach” [2]. It required distal pancreatectomy and partial gastrectomy for resection. In a third case, the tumor surrounded and constricted the second portion of the duodenum and required total pancreatectomy and duodenec-

The American Journal of Surgery

Mesenchymal Pancreatic Tumor

tomy; exact site of origin could not be determined (3). In a fourth case, the tumor invaded the ampulla and common bile duct, again raising the possibility of nonpancreatic origin; it required hemipancreatectomy and duodenectomy [4]. A fifth case report described a tumor which was more clearly of pancreatic origin and was resected in a fashion similar to the case herein reported [5]. In the present case the tumor mass was limited to the head of the pancreas. It stretched the duodenum and the common bile duct, obstructing the latter, but without invasion of either. No other organs were adherent to the mass and the organ of origin was definitely pancreas. Fibrous histiocytomata may, of course, occur in the retroperitoneum, but in our experience, they have not to date been reported in the head of the pancreas necessitating pancreaticoduodenectomy. This lesion, if it indeed is a fibrous histiocytoma, would be classified as a benign tumor. However, the strategic location of the lesion in this patient would have necessitated resection even if the question of malignancy had not been raised by the possible diagnosis of myxofibrosarcoma. In the present case theinitial diagnosis of hepatitis compounded by the initial interpretation of the liver biopsy delayed surgical intervention. For-

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tunately, surgical exploration was performed before irreversible liver damage, and the subsequent course has been most gratifying. Summary A case of an unusual tumor occurring in the head of the pancreas is presented. Definitive diagnosis could not be made, the lesion being either a low grade myxofibrosarcoma or a benign fibrous histiocytoma. The literature is discussed and several cases believed to be pancreatic sarcomata are presented, only one of which was definitely of pancreatic origin. No other case of fibrous histiocytoma of the pancreas has been reported. References 1. Ariel F: Rare tumors: etiology and localization. J Int Co// Surg 12: 673, 1949. 2. Neibling H: Primary sarcoma of the pancreas. Am Surg 34: 690, 1968. 3. Feather H, Kuhn C: Total pancreatectomy for sarcoma of the pancreas. Ann Surg 134: 904, 1951. 4. Berman J, Levene N: Sarcoma of the pancreas. Arch Surg 73: 894. 1956. 5. Brooke W, Maxwell J: Primary sarcoma of the pancreas. Am J Surg 112: 657, 1966. 6. Ackerman L, Rosai J: Surgical Pathology. St. Louis, CV Mosby, 1974, p 1124.

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Pancreatic tumor of mesenchymal origin presenting as obstructive jaundice.

Pancreatic Tumor of Mesenchymal Origin Presenting as Obstructive Jaundice Richard M. Margules, MD, San Francisco, California Robert E. Allen, MD, San...
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