Pancreatoblastoma: A Rare Tumor Still Evolving in Clinical Presentation and Histology

  • Chitra Balasundaram Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
  • Munish Luthra Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
  • Disaya Chavalitdhamrong Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
  • Jonathan Chow Department of Pathology, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
  • Hina Khan Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
  • Paul JH Endres Department of Pathology, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Keywords: Histology, Neoplasm Metastasis, Pancreas, Pancreatoblastoma

Abstract

Context Pancreatoblastoma is a rare neoplasm in adults with a total of only 24 cases that have been reported in the literature. Adult pancreatoblastomas are large tumors and majority are larger than 8 cm at the time of diagnosis. Metastasis is seen in 26% of adults and usually involves the liver and then the lymph nodes. Metastasis is usually observed in cases where the primary tumor measures more than 10 cm. Pancreatoblastoma is named after its resemblance to fetal pancreatic tissue in the seventh week of life. The presence of squamoid corpuscles with a morular appearance is the most characteristic feature of the tumor. Pancreatoblastomas can have mixed features of both endocrine and exocrine cells; however, acinar differentiation is the most prevalent feature. Case report We present a case of a 27-year-old female with a 3.6 cm pancreatoblastoma with metastasis to the liver and lungs as well as to the breast. This case has several distinguishing features from previously reported cases. Such widespread metastasis is unusual given the small size of the primary tumor. Also, metastasis to the breast from a pancreatoblastoma has been previously undescribed in literature. The histological features in our case of pancreatoblastoma were atypical, characterized by the absence of acinar component, supported by the lack of staining for both trypsin and lipase in the tumor, which has not been described in literature. Additionally, the nests of squamous cells in this tumor had a pilomatricoma like morphology as opposed to the morular appearance of the squamoid corpuscles seen in classical cases. Conclusion Pancreatoblastoma can have an atypical clinical picture and a small primary with extensive metastasis to unusual sites may present a diagnostic challenge. Given its rarity, a high index of suspicion is required to correctly diagnose this condition. The histology reported on this case is unique and has not been reported in the literature.

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Author Biographies

Chitra Balasundaram, Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Internal Medicine
Munish Luthra, Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Internal Medicine
Disaya Chavalitdhamrong, Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Internal Medicine
Jonathan Chow, Department of Pathology, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Pathology
Hina Khan, Department of Internal Medicine, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Internal Medicine
Paul JH Endres, Department of Pathology, James J. Peters VA Medical Center, Mount Sinai School of Medicine. Bronx, New York, NY, USA
Department of Pathology

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Primary pancreatic tumor consisting of squamous nests surrounded by primitive appearing cells.
Published
2012-05-10
How to Cite
Balasundaram, C., Luthra, M., Chavalitdhamrong, D., Chow, J., Khan, H., & Endres, P. (2012). Pancreatoblastoma: A Rare Tumor Still Evolving in Clinical Presentation and Histology. JOP. Journal of the Pancreas, 13(3), 301-303. https://doi.org/10.6092/1590-8577/661
Section
CASE REPORTS