INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by proliferation of morphologically distinguishable smooth muscle cells in the lymphatics and lymph nodes of the pulmonary parenchyma in most cases. Extrapulmonary LAM is a rare condition and is found to occur concurrently, before or after pulmonary LAM, and show strong association with tuberous sclerosis. DISCUSSION: The literature regarding extrapulmonary LAM without associated pulmonary LAM is limited due to the extreme rarity of the cases. We hereby describe clinical, pathological and radiological features of primary pancreatic LAM presenting clinicoradiologically as pseudocyst of pancreas in a 43-year-old lady. CONCLUSION: The present case is unique as LAM in pancreas without associated pulmonary LAM has never been reported in the literature before.
INTRODUCTION:Lymphangioleiomyomatosis (LAM) is a rare disease characterized by proliferation of morphologically distinguishable smooth muscle cells in the lymphatics and lymph nodes of the pulmonary parenchyma in most cases. Extrapulmonary LAM is a rare condition and is found to occur concurrently, before or after pulmonary LAM, and show strong association with tuberous sclerosis. DISCUSSION: The literature regarding extrapulmonary LAM without associated pulmonary LAM is limited due to the extreme rarity of the cases. We hereby describe clinical, pathological and radiological features of primary pancreatic LAM presenting clinicoradiologically as pseudocyst of pancreas in a 43-year-old lady. CONCLUSION: The present case is unique as LAM in pancreas without associated pulmonary LAM has never been reported in the literature before.
Authors: K Matsui; A Tatsuguchi; J Valencia; Z x Yu; J Bechtle; M B Beasley; N Avila; W D Travis; J Moss; V J Ferrans Journal: Hum Pathol Date: 2000-10 Impact factor: 3.466
Authors: Vilkesh R Jaiswal; Julie Baird; Jason Fleming; David Scott Miller; Suash Sharma; Kyle Molberg Journal: Arch Pathol Lab Med Date: 2003-07 Impact factor: 5.534