Pancreatoblastoma

Pancreatoblastoma
SpecialtyOncology

Pancreatoblastoma is a rare type of pancreatic cancer.[1] It occurs mainly in childhood[2] and has a relatively good prognosis.

Symptoms and signs

Children with pancreatoblastoma rarely present with early-stage disease, instead, most present with locally advanced or metastatic disease. Common presenting symptoms include abdominal pain, emesis, and jaundice. A multidisciplinary approach including good clinical history, state of the art imaging, and careful pathology is often needed to establish the correct diagnosis.[3]

Pathology

Resected pancreatoblastomas can be quite large, ranging from 2 centimeters to 20 centimeters in size (1 to 8 inches). They are typically solid, soft masses. Under the microscope, at least two cell types are seen: cells with “acinar” differentiation, and cells forming small “squamoid” nests. The cells with acinar differentiation have some features of the normal acinar cell of the pancreas (the most common cell in the normal pancreas).[4]

Diagnosis

The diagnosis can be done via histological characteristics, as well as the following:[5]

  • Tumor marker alpha-fetoprotein increases
  • Imaging (primary tumor)
    • MRI
    • Ultrasound
    • CT scan

Treatment

Pancreatoblastoma- gross specimen of tumor resected during surgery

If the tumor is operable, the first line of therapy should be surgical resection. Then, after surgical resection, adjuvant chemotherapy should be given, even in stage I disease. In patients with inoperable disease, chemotherapy alone should be given.[6] A multi-disciplinary approach to the treatment, including surgeons, oncologists, pathologists, radiologists, and radiation oncologists, is often the best approach to managing these patients.[3]

See also

  • Grayson Gilbert

References

  1. ↑ "Pancreatoblastoma". Archived from the original on 2004-06-02.
  2. ↑ Naik VR, Jaafar H, Leow VM, Bhavaraju VM (March 2006). "Pancreatoblastoma: a rare tumour accidentally found" (PDF). Singapore Med J. 47 (3): 232–4. PMID 16518559. Archived (PDF) from the original on 2016-03-03. Retrieved 2021-11-28.
  3. 1 2 "Pancreatic Cancer Multi-Disciplinary Clinic at Johns Hopkins University". Archived from the original on 2020-05-03. Retrieved 2021-11-28.
  4. ↑ "Pancreatic Cancer Frequently Asked Questions". Archived from the original on 2020-02-09. Retrieved 2021-11-28.
  5. ↑ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Pancreatoblastoma". www.orpha.net. Archived from the original on 9 October 2015. Retrieved 31 December 2021.
  6. ↑ http://www.orpha.net/data/patho/GB/uk-pancrea.pdf Archived 2016-03-08 at the Wayback Machine -
Classification
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