Pancreatic PEComa

Pancreatic perivascular epithelioid cell tumours (or "Pancreatic PEComas") are a subtype of the larger family of PEComas. Pancreatic PEComas are very rare with <20 cases described.

More common in adults (in contrast to lymphangiomas in the head and neck, which are more common in children). Account for approximately 1% of abdominal lymphangiomas. More common in women

As their name suggests, these tumours arise from perivascular epithelioid cells and occur due to a genetic alteration in the tuberous sclerosis gene complex. The PEComas are a group of tumours, including

  • clear cell "sugar" tumours
  • angiomyolipoma
  • lymphangiomyomatosis

The cells are periodic acid-Schiff stain (PAS) positive and diastases sensitive. Immunohistochemistry markers include: smooth muscle and melanocytic markers (SMA, HMB-45, and HMSA-1) 2-3

CT
  • tend to occur in the head and body of the pancreas
  • well-demarcated
  • hypoenhancing mass with a hypervascular capsule
  • may demonstrate hemorrhage or cystic degeneration
  • fat attenuation may be present
MRI

Signal characteristics include

  • T1: hypointense
  • T2: hyperintense
  • T1 C+: heterogeneous enhancement
  • T1 FS: macroscopic fat intensity may be present (saturates on a fat sat sequence)
Ultrasound
  • nonspecific pancreatic mass
  • heterogeneous echogenicity
  • well-encapsulated

Given the rarity of the lesion, there is no standard treatment

​A pancreatic PEComa should only be considered if a pancreatic mass is well-encapsulated, but since it is so rare, it should never be at the top of a differential. It's probably only reasonably suggested in a patient with tuberous sclerosis and a pancreatic mass.

Other similar pancreatic masses that should be considered before it are

Pancreatic pathology
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Article information

rID: 43084
Synonyms or Alternate Spellings:
  • Pancreatic perivascular epithelioid cell tumours
  • Pancreatic perivascular epithelioid cell tumor
  • Pancreatic perivascular epithelioid cell tumors
  • Pancreatic PEComa
  • Pancreatic PEComas
  • PEComa of the pancreas

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