Abstract
This study is the second to report a pancreatic “sugar” tumor (ST) case. This ST was incidentally discovered in a 31-year-old woman using computed tomography scan (CT scan) for work-up of a hepatic focal nodular hyperplasia. Both CT scan and endoluminal ultrasonography (EUS) features evoked a 15-mm large benign endocrine tumor. Pathological examination of EUS-guided fine-needle aspiration biopsies could not confirm this diagnosis. Laparoscopic corporeo-caudal pancreatectomy was performed. The tumor was intrapancreatic, well circumscribed, and organized in sheets of epithelioid cells. The tumor cells expressed HMB-45 but did not express epithelial or endocrine immunohistochemical markers. These histophenotypic features are those of an extra pulmonary ST, which belong to the PEComa family of tumors. Retrospective examination of preoperative biopsies evidenced the same histophenotypic features. This observation highlights that STs should be considered in preoperative differential diagnosis of pancreas tumors, since they may be treated by limited surgical resection.
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References
Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, Bacchi C, Sin VC, Wong NL, Coady M, Chan JK (2001) Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol 14:563–568
Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, Rimondi P, Andrion A (1994) Clear cell (“sugar”) tumor of the lung is a lesion strictly related to angiomyolipoma—the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology 26:230–236
Cibas ES, Goss GA, Kulke MH, Demetri GD, Fletcher CD (2001) Malignant epithelioid angiomyolipoma (‘sarcoma ex angiomyolipoma’) of the kidney: a case report and review of the literature. Am J Surg Pathol 25:121–126
Folpe AL (2002) Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: Fletcher CDM, Unni KK, Mertens F (eds) Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon, pp 221–222
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW (2000) Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol 24:1239–1246
Govender D, Sabaratnam RM, Essa AS (2002) Clear cell ‘sugar’ tumor of the breast: another extrapulmonary site and review of the literature. Am J Surg Pathol 26:670–675
Heywood G, Smyrk TC, Donohue JH (2004) Primary angiomyolipoma of the pancreas. Pancreas 28:443–445
Kung M, Landa JF, Lubin J (1984) Benign clear cell tumor (“sugar tumor”) of the trachea. Cancer 54:517–519
Langner C, Homayounfar K, Ruten B, Fass J, Ruschoff J (2001) Concomitant occurrence of angiomyolipoma, focal nodular hyperplasia, bile duct adenoma, and cavernous hemangioma in the liver. Pathologe 22:417–423
Lubensky IA, Pack S, Ault D, Vortmeyer AO, Libutti SK, Choyke PL, Walther MM, Linehan WM, Zhuang Z (1998) Multiple neuroendocrine tumors of the pancreas in von Hippel–Lindau disease patients: histopathological and molecular genetic analysis. Am J Pathol 153:223–231
Martignoni G, Pea M, Rigaud G, Manfrin E, Colato C, Zamboni G, Scarpa A, Tardanico R, Roncalli M, Bonetti F (2000) Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions. Am J Surg Pathol 24:889–894
Paradis V, Laurendeau I, Vieillefond A, Blanchet P, Eschwege P, Benoit G, Vidaud M, Jardin A, Bedossa P (1998) Clonal analysis of renal sporadic angiomyolipomas. Hum Pathol 29:1063–1067
Paradis V, Laurent A, Flejou JF, Vidaud M, Bedossa P (1997) Evidence for the polyclonal nature of focal nodular hyperplasia of the liver by the study of X-chromosome inactivation. Hepatology 26:891–895
Rochaix P, Lacroix-Triki M, Lamant L, Pichereaux C, Valmary S, Puente E, Al Saati T, Monsarrat B, Susini C, Buscail L, Delsol G, Voigt JJ (2003) PNL2, a new monoclonal antibody directed against a fixative-resistant melanocyte antigen. Mod Pathol 16:481–490
Sale GE, Kulander BG (1988) ‘Benign’ clear-cell tumor (sugar tumor) of the lung with hepatic metastases ten years after resection of pulmonary primary tumor. Arch Pathol Lab Med 112:1177–1178
Stone CH, Lee MW, Amin MB, Yaziji H, Gown AM, Ro JY, Tetu B, Paraf F, Zarbo RJ (2001) Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med 125:751–758
Tanaka Y, Ijiri R, Kato K, Kato Y, Misugi K, Nakatani Y, Hara M (2000) HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cell ‘sugar’ tumors. Am J Surg Pathol 24:1295–1299
Tazelaar HD, Batts KP, Srigley JR (2001) Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol 14:615–622
Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E, Pederzoli P, Bonetti F (1996) Clear cell “sugar” tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol 20:722–730
Zavala-Pompa A, Folpe AL, Jimenez RE, Lim SD, Cohen C, Eble JN, Amin MB (2001) Immunohistochemical study of microphthalmia transcription factor and tyrosinase in angiomyolipoma of the kidney, renal cell carcinoma, and renal and retroperitoneal sarcomas: comparative evaluation with traditional diagnostic markers. Am J Surg Pathol 25:65–70
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We thank Dr. Lucile Andrac-Meyer, Christiane Bailly, Laurence Lamant, and Talal al-Saati for their kind help.
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Ramuz, O., Lelong, B., Giovannini, M. et al. “Sugar” tumor of the pancreas: a rare entity that is diagnosable on preoperative fine-needle biopsies. Virchows Arch 446, 555–559 (2005). https://doi.org/10.1007/s00428-005-1216-4
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DOI: https://doi.org/10.1007/s00428-005-1216-4