Lymphangioleiomyomatosis

Case contributed by Liz Silverstone
Diagnosis certain

Presentation

Abdominal discomfort worse before menstruation.

Patient Data

Age: 30 years
Gender: Female

Initial CT

ct
  • water-attenuation mass extending along the right iliac vessels and insinuating between and enveloping normal structures without mass-effect

  • few small lung cysts

  • no effusions

  • no lymphadenopathy

  • no AMLs

2 years later

ct
  • innumerable scattered thin-walled small lung cysts

  • normal intervening lung parenchyma

  • right pneumothorax

13 years later

ct
  • scattered lung cysts which have increased in size. and number

  • hyperattenuating bilateral pleural thickening due to prior VATS pleurodesis

  • left pneumothorax

Case Discussion

Diagnosis of sporadic lymphangioleiomyomatosis (s-LAM) confirmed.

Abdominal lymphangioleiomyoma was found to vary in size with her menstrual cycle but reduced in size following initiation of sirolimus therapy.

Lymphatic abnormalities are a feature of s-LAM.

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