Pathologic Quiz Case: Large Retroperitoneal Mass in an Asymptomatic 43-Year-Old Woman.
The tumor was a well-circumscribed lobulated mass, measuring 15 x 10 x 10 cm, with a variegated cut surface (hemorrhage, necrosis, and yellow fatty to firm areas). Microscopic examination revealed pseudostratified columnar tumor cells with eosinophilic granular cytoplasm and round nuclei. These cells surrounded stalks of stroma that contained blood vessels with foamy histiocytes, extensive hemorrhage, ischemic necrosis, and foreign body giant cell reaction to cholesterol (Figures 2 and 3). The mitotic count was low.
What is your diagnosis?
Pathologic Diagnosis: Solid and Papillary Epithelial Neoplasm of Pancreas
Solid and papillary epithelial neoplasm of pancreas (SPENP; also described as papillary cystic neoplasm) is a rare but distinctive tumor of low malignant potential with a predilection for female patients, largely young nonwhite women. The pancreatic cystic neoplasm was described in 1959 by Frantz. Clinically, the patients are often asymptomatic with a gradually enlarging abdominal mass or have complaints of vague abdominal pain or discomfort.
The SPENPs are large, averaging 9 to 10 cm in diameter, and are well circumscribed with a pseudocapsule. The variegated gross and microscopic appearance of pseudopapillary and cystic components within the tumor seem to evolve by degenerative changes, probably by vascular ischemia. The foamy degeneration of tumor and presence of cholesterol clefts are retrogressive changes. In a study done on 56 cases, more than half of the cases were located in the body of the pancreas, followed by the tail and head of the pancreas. The treatment is surgical and has a very good prognosis. However, cases have been reported with local recurrences and liver metastasis. The results of a study done by Nishihara et al on papillary cystic neoplasm indicated that venous invasion, high nuclear grade, and prominent "necrobiotic nests" are useful parameters to detect the malignant potential of papillary cystic tumors.
In a study done by Stommer et al on 10 cases, endocrine as well as exocrine differentiation was suggested, owing to immunoreactivity with [[Alpha].sub.1]-antitrypsin, [[Alpha].sub.1]-chymotrypsin, and neuroendocrine markers. A karyotypic abnormality in SPENP has been described in a 43-year-old black woman; the abnormality consisted of an unbalanced translocation between chromosomes 13 and 17: der(17)t(13:17)(q14:p11). This translocation together with the genes flanking the breakpoints may prove to be markers of this neoplasm.
The definite increase of estrogen and progesterone receptors in SPENP relative to levels in normal pancreas explains the preponderance of the tumor in female patients and suggests it as a hormone-dependent neoplasm. High levels of progesterone receptors seen in the tumor studied by Ladanyi et al support the hypothesis that SPENP is estrogen responsive, since expression of progesterone is induced by estrogen.
The treatment of resectable lesions should be wide resection, that is, pancreatoduodenectomy for lesions in the head of the pancreas and distal/subtotal pancreatectomy for lesions in the body and tail.
[1.] Frantz VK. Tumors of the Pancreas. Blumberg CW, ed. Washington, DC: Armed Forces Institute of Pathology; 1959:32-33. Atlas of Tumor Pathology; Vol VII, fascicles 27 and 28.
[2.] Lieber MB, Lack EE, Roberts JR Jr, et al. Solid and papillary epithelial neoplasm of the pancreas: an ultrastructural and immunocytochemical study of six cases. Am J Surg Pathol. 1987;11:85-93.
[3.] Buetow PC, Buck JL, Pantongrag-Brown L, Beck KG, Ros PR, Adair CF. Solid and papillary epithelial neoplasm of the pancreas: imaging-pathologic correlation in 56 cases. Radiology: 1996;199:707-711.
[4.] Cappellari J, Geisinger K, Albertson D, Wolfman N, Kute T. Malignant papillary cystic tumor of the pancreas. Cancer. 1990;66:193-198.
[5.] Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayashi I. Papillary cystic tumors of the pancreas: assessment of their malignant potential. Cancer. 1993;71:82-92.
[6.] Stommer P, Kraus J, Stolte M, Giedl J. Solid and cystic pancreatic tumors: clinical, histochemical and electron microscopic features in ten cases. Cancer. 1991;67:1635-1641.
[7.] Grant LD, Lauwers GV, Meloni AM, et al. Unbalanced chromosomal translocation, der(17)t(13;17)(q14;p11) in a solid and cystic papillary epithelial neoplasm of the pancreas. Am J Surg Pathol. 1996;20:339-345.
[8.] Ladanyi M, Mulay S, Arseneau J, Bettez P. Estrogen and progesterone receptor determination in the papillary cystic neoplasm of the pancreas. Cancer. 1987;60:1604-1611.
[9.] Sclafani LM, Reuter VE, Coit DG, Brenner MF. The malignant nature of papillary and cystic neoplasm of the pancreas. Cancer. 1991;68:153-158.
Accepted for publication December 6, 2000.
From the Department of Pathology, St Luke's Roosevelt Hospital Center, New York, NY.
Reprints not available from the authors.
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|Title Annotation:||solid and papillary epithelial neoplasm of pancreas|
|Author:||Eapen, Elizabeth; Wiriosuparto, Sony|
|Publication:||Archives of Pathology & Laboratory Medicine|
|Date:||Jul 1, 2001|
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