October 2020 — Gastroenterology

A 30 year old woman with history of endometriosis presents to the emergency department with a sudden onset of acute abdominal pain. CT imaging demonstrates a large 20 cm fluid density mass originating in the upper left quadrant of the abdomen.

Figure 1: Gross - Distal pancreas, spleen, and adjacent mass
Figure 2: H&E, 100x
Figure 3: Select immunostains, 100x

What is your diagnosis?

  • Hemangioma
  • Angiomyolipoma
  • Lymphangioma
  • Lymphangiomyoma

The correct answer is...

The correct answer is Lymphangioma.

This lesion consists of variably sized vascular spaces lined by a single layer of endothelial cells which form anastamosing spaces occupied by eosinophilic proteinaceous debris, lymphocytes, and erythrocytes. Also present is an interstitial stroma with fibrosis and lymphoid infiltrate. Rare blood vessels are identified throughout the lesion. The endothelial lining is highlighted by vascular/lymphocytic markers and are HMB45, MelanA, and PAX-8 negative (not shown). These morphologic and immunophenotypic features are consistent with a lymphangioma. A hemangioma would likely contain more abundant smaller vascular spaces; they are also generally better circumscribed. While morphologically consistent with a lymphangiomyoma or angiomyolipoma, these lesions are generally both HMB45 positive.

Lymphangiomas often occur in young children, and the exact pathogenesis is not yet fully determined. They are traditionally classified into four separate categories (capillary, cavernous, cystic, and mixed). Frequently found in more superficial locations, they do rarely occur within the GI tract, most commonly within the small intestine, large intestine, and esophagus. A lymphangioma arising from the pancreas, such as with this lesion, is extremely rare.

1. Wiegand, S., Eivazi, B., Barth, P.J., Von Rautenfeld, D.B., Folz, B.J., Mandic, R., Werner, J.A., 2008. Pathogenesis of lymphangiomas. Virchows Archiv.. doi:10.1007/s00428-008-0611-z
2. Karajgikar, J., Deshmukh, S., 2019. Pancreatic Lymphangioma. Journal of Computer Assisted Tomography.. doi:10.1097/rct.0000000000000818
3. Fujii, M., Saito, H., Yoshioka, M., Shiode, J., 2018. Rare Case of Pancreatic Cystic Lymphangioma. Internal Medicine.. doi:10.2169/internalmedicine.9445-17
4. WHO Classification of Tumours of the Digestive System. International Agency for Research on Cancer, 2019.

Photo of Matthew Woge, M.D. Matthew Woge, M.D.
Resident, Surgical Pathology
Mayo Clinic
Photo of Rondell Graham, M.B.B.S. Rondell Graham, M.B.B.S.
Consultant, Anatomic Pathology
Mayo Clinic
Associate Professor of Laboratory Medicine and Pathology
Mayo Clinic College of Medicine and Science

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This post was developed by our Education and Technical Publications Team.