Pancreatic PEComa
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Article Attributes
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.
Clinical presentation
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
Pathology
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
Radiographic features
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
- T1: hypointense
- T2: hyperintense
- T1 C+: heterogeneous enhancement
- T1 FS: macroscopic fat intensity may be present (saturates on a fat sat sequence)
US
- nonspecific pancreatic mass
- heterogeneous echogenicity
- well-encapsulated
Treatment and prognosis
Given the rarity of the lesion, there is no standard treatment
Differential diagnosis
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 endocrine tumour ("islet cell" tumor)
- solid-pseudopapillary tumour (SPT)
- metastasis to the pancreas
- also rare, but much more likely than a pancreatic PEComa
-<p><strong>Pancreatic perivascular epithelioid cell tumours</strong> (or "<strong>Pancreatic PEComas</strong>") are a subtype of the larger family of <a title="PEComas" href="/articles/perivascular-epithelioid-cell-tumours">PEComas</a>. Pancreatic PEComas are very rare with <20 cases described.</p><h4>Clinical presentation</h4><p>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</p><h4>Pathology</h4><p>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</p><ul>- +<p><strong>Pancreatic perivascular epithelioid cell tumours</strong> (or "<strong>Pancreatic PEComas</strong>") are a subtype of the larger family of <a href="/articles/perivascular-epithelioid-cell-tumours">PEComas</a>. Pancreatic PEComas are very rare with <20 cases described.</p><h4>Clinical presentation</h4><p>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</p><h4>Pathology</h4><p>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</p><ul>
-</ul><h4>Treatment and prognosis</h4><p>Given the rarity of the lesion, there is no standard treatment</p><h4>Differential diagnosis</h4><p>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. Other similar pancreatic masses that should be considered before it are</p><ul>-<li><a title="Pancreatic endocrine tumor" href="/articles/endocrine-tumours-of-the-pancreas">pancreatic endocrine tumour ("islet cell" tumor)</a></li>-<li><a title="SPEN" href="/articles/solid-pseudopapillary-tumour-of-the-pancreas-1">solid-pseudopapillary tumour (SPT)</a></li>- +</ul><h4>Treatment and prognosis</h4><p>Given the rarity of the lesion, there is no standard treatment</p><h4>Differential diagnosis</h4><p>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.</p><p>Other similar pancreatic masses that should be considered before it are</p><ul>
- +<li><a href="/articles/endocrine-tumours-of-the-pancreas">pancreatic endocrine tumour ("islet cell" tumor)</a></li>
- +<li><a href="/articles/solid-pseudopapillary-tumour-of-the-pancreas-1">solid-pseudopapillary tumour (SPT)</a></li>
References changed:
- 1. Manning MA, Srivastava A, Paal EE et-al. Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1-Benign Tumors: From the Radiologic Pathology Archives. Radiographics. 2016;36 (1): 123-41. <a href="http://dx.doi.org/10.1148/rg.2016150212">doi:10.1148/rg.2016150212</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/26761535">Pubmed citation</a><span class="auto"></span>
- 2. Hirabayashi K, Nakamura N, Kajiwara H et-al. Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature. Pathol. Int. 2009;59 (9): 650-5. <a href="http://dx.doi.org/10.1111/j.1440-1827.2009.02421.x">doi:10.1111/j.1440-1827.2009.02421.x</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19712133">Pubmed citation</a><span class="auto"></span>
- 3. Martignoni G, Pea M, Reghellin D et-al. PEComas: the past, the present and the future. Virchows Arch. 2008;452 (2): 119-32. <a href="http://dx.doi.org/10.1007/s00428-007-0509-1">doi:10.1007/s00428-007-0509-1</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234444">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18080139">Pubmed citation</a><span class="auto"></span>
Tags changed:
- pancreas
- pecoma
Systems changed:
- Gastrointestinal