Pancreatoblastoma. A clinicopathologic study and review of the literature - PubMed (original) (raw)
Review
Pancreatoblastoma. A clinicopathologic study and review of the literature
D S Klimstra et al. Am J Surg Pathol. 1995 Dec.
Abstract
Pancreatoblastoma is a rare pancreatic tumor with a distinctive histologic appearance that generally affects infants and young children. We have studied 14 cases of pancreatoblastoma and reviewed 41 previously reported examples. Nine of our cases occurred in children (from newborn to 4 years old; mean, 2.4), and five affected adults (from 19 to 56 years old; mean, 40). There were 8 male cases and 6 female cases. Most patients presented with incidental abdominal masses, although pain, weight loss, and obstructive jaundice were present, but rarely. The tumors were very cellular microscopically, with cytologically uniform epithelial cells arranged in sheets and nests. Well-formed acinar structures were a consistent feature, and several cases contained ectatic ductular formations, rarely exhibiting intracellular mucin. Consistently present were squamoid corpuscles: circumscribed, whorled nests of plump spindle cells with a squamous appearance and occasional keratinization. The stroma was moderate to abundant and frequently quite cellular (especially in the pediatric cases). By immunohistochemistry, the tumors exhibited acinar, endocrine, and ductal differentiation, with positivity for pancreatic enzymes (100%), endocrine markers (82%), and carcinoembryonic antigen (85%). Ultrastructural examination most commonly revealed acinar differentiation, although mucigen granules and neurosecretory granules were also occasionally found. The behavior was variable: 36% of patients developed metastases, especially to the liver. The adult patients did poorly: three of five died of tumor (mean survival, 18 months), and two were alive at 5 and 15 months, respectively. In contrast, five of the six evaluable pediatric patients were alive from 22 months to 22 years after diagnosis, and only one died of tumor after 16 months. Good responses to chemotherapy were noted in the pediatric group.
Similar articles
- Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature.
Du E, Katz M, Weidner N, Yoder S, Moossa AR, Shabaik A. Du E, et al. Arch Pathol Lab Med. 2003 Nov;127(11):1501-5. doi: 10.5858/2003-127-1501-APIAEP. Arch Pathol Lab Med. 2003. PMID: 14567752 Review. - Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.
Klimstra DS, Heffess CS, Oertel JE, Rosai J. Klimstra DS, et al. Am J Surg Pathol. 1992 Sep;16(9):815-37. doi: 10.1097/00000478-199209000-00001. Am J Surg Pathol. 1992. PMID: 1384374 - Pancreatoblastoma.
Chun Y, Kim W, Park K, Lee S, Jung S. Chun Y, et al. J Pediatr Surg. 1997 Nov;32(11):1612-5. doi: 10.1016/s0022-3468(97)90465-6. J Pediatr Surg. 1997. PMID: 9396538 - Pancreatoblastoma in Japan, with differential diagnosis from papillary cystic tumor (ductuloacinar adenoma) of the pancreas.
Horie A, Haratake J, Jimi A, Matsumoto M, Ishii N, Tsutsumi Y. Horie A, et al. Acta Pathol Jpn. 1987 Jan;37(1):47-63. doi: 10.1111/j.1440-1827.1987.tb03133.x. Acta Pathol Jpn. 1987. PMID: 3554891 - Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology.
Balasundaram C, Luthra M, Chavalitdhamrong D, Chow J, Khan H, Endres PJ. Balasundaram C, et al. JOP. 2012 May 10;13(3):301-3. JOP. 2012. PMID: 22572137 Review.
Cited by
- One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches.
Niger M, Prisciandaro M, Antista M, Monica MAT, Cattaneo L, Prinzi N, Manglaviti S, Nichetti F, Brambilla M, Torchio M, Corti F, Pusceddu S, Coppa J, Mazzaferro V, de Braud F, Di Bartolomeo M. Niger M, et al. World J Gastrointest Oncol. 2020 Aug 15;12(8):833-849. doi: 10.4251/wjgo.v12.i8.833. World J Gastrointest Oncol. 2020. PMID: 32879662 Free PMC article. Review. - Immunohistochemical features of the gastrointestinal tract tumors.
Wong HH, Chu P. Wong HH, et al. J Gastrointest Oncol. 2012 Sep;3(3):262-84. doi: 10.3978/j.issn.2078-6891.2012.019. J Gastrointest Oncol. 2012. PMID: 22943017 Free PMC article. - [Acinar cell carcinomas and pancreatoblastomas: related but not the same].
Sipos B, Klöppel G. Sipos B, et al. Pathologe. 2005 Feb;26(1):37-40. doi: 10.1007/s00292-004-0732-3. Pathologe. 2005. PMID: 15614488 German. - Whole-exome sequencing of pancreatic neoplasms with acinar differentiation.
Jiao Y, Yonescu R, Offerhaus GJ, Klimstra DS, Maitra A, Eshleman JR, Herman JG, Poh W, Pelosof L, Wolfgang CL, Vogelstein B, Kinzler KW, Hruban RH, Papadopoulos N, Wood LD. Jiao Y, et al. J Pathol. 2014 Mar;232(4):428-35. doi: 10.1002/path.4310. J Pathol. 2014. PMID: 24293293 Free PMC article. - Pancreatoblastoma in an adult: case report and review of the literature.
Rajpal S, Warren RS, Alexander M, Yeh BM, Grenert JP, Hintzen S, Ljung BM, Bergsland EK. Rajpal S, et al. J Gastrointest Surg. 2006 Jun;10(6):829-36. doi: 10.1016/j.gassur.2005.11.011. J Gastrointest Surg. 2006. PMID: 16769539 Review.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Medical