case of the day 12.01

8/6/2019 Case of the day 12.01

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Case of the dayCase of the day

Nguyen Ngoc TrangNguyen Ngoc Trang

Radiology DeptRadiology Dept


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ClinicalClinical

EndoscopeEndoscope Appendectomy at Viet TiepAppendectomy at Viet TiepHospital since 10 days.Hospital since 10 days.

Then occurred abdominal painThen occurred abdominal pain Viet DucViet DucHospital with Dx bowel occlusion postHospital with Dx bowel occlusion post--operationoperation

ASP: dilation bowel with air, some hydroaericASP: dilation bowel with air, some hydroaericlevelslevels

Abdominal ultrasound and CTAbdominal ultrasound and CT--scanner:scanner:retroperitoneal mass, may be bilateral adrenalretroperitoneal mass, may be bilateral adrenalmassmass


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DxDx

LymphangioleiomyomatosisLymphangioleiomyomatosis

(LAM)(LAM)LAM + TSCLAM + TSC

Tuberous Sclerosis ComplexTuberous Sclerosis Complex

(TSC)(TSC)


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LymphangioleiomyomatosisLymphangioleiomyomatosis

(LAM)(LAM) LymphangioleiomyomatosisLymphangioleiomyomatosis (LAM)(LAM) isis

characterizedcharacterized byby proliferationproliferation ofof abnormalabnormal

appearingappearing smoothsmooth musclemuscle cellscells (LAM(LAM cells)cells)inin thethe lungs,lungs, thethe kidneys,kidneys, andand thethelymphaticlymphatic systemsystem.. TheThe diagnosisdiagnosis of ofpulmonarypulmonary LAMLAM isis usuallyusually mademade withwith

clinicalclinical historyhistory andand computedcomputed tomographictomographic(CT)(CT) demonstrationdemonstration ofof pulmonarypulmonary cystscysts ororlunglung biopsybiopsy


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Transverse highTransverse high--spatialspatial--resolution CT image demonstrates thinresolution CT image demonstrates thin--walled lungwalled lungparenchymal cysts (arrow indicates one of many) with intervening normalparenchymal cysts (arrow indicates one of many) with intervening normal

parenchyma.parenchyma.


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Abdominal findings may be present in more than 70% ofAbdominal findings may be present in more than 70% ofpatientspatients with LAM, and the most common abdominal finding iswith LAM, and the most common abdominal finding is

renalrenal angiomyolipoma.angiomyolipoma.Angiomyolipomas are often small (


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In a patient with a history ofLAM, the absence of fat in aIn a patient with a history ofLAM, the absence of fat in a renal lesion shouldrenal lesion shouldnot preclude the diagnosis of angiomyolipoma, as fat is not detectable in up tonot preclude the diagnosis of angiomyolipoma, as fat is not detectable in up to

5% of these tumors5% of these tumors


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Proliferation of smooth muscle cells in the lymph vessels mayProliferation of smooth muscle cells in the lymph vessels may produce cysticproduce cysticmasses consistent with dilatations of the abdominalmasses consistent with dilatations of the abdominal lymph vessels due tolymph vessels due to

lymphatic obstruction; these are knownlymphatic obstruction; these are known as lymphangioleiomyomas. These lowas lymphangioleiomyomas. These low--attenuation retroperitonealattenuation retroperitoneal masses occur in up to 20% of patients. They maymasses occur in up to 20% of patients. They may

be thickbe thick-- or thinor thin--walled and can vary in size depending on diet orwalled and can vary in size depending on diet orgravitationalgravitational factorsfactors


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LymphadenopathyLymphadenopathyEnlarged lymph nodes are described in up to 40% of cases. LymphEnlarged lymph nodes are described in up to 40% of cases. Lymph nodes can measurenodes can measure

up to 4 cm in diameter. Some lymph nodes containup to 4 cm in diameter. Some lymph nodes contain lowlow--attenuation areas, which indicateattenuation areas, which indicatethe presence of chylousthe presence of chylous lymph collections, or hamartomatous hyperattenuating areaslymph collections, or hamartomatous hyperattenuating areas

thatthat enhance after contrast material administrationenhance after contrast material administration


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Chylous AscitesChylous AscitesOverdistention of lymphatic cysts may result in intraperitonealOverdistention of lymphatic cysts may result in intraperitoneal rupture andrupture andchylous ascites. This is an unusual abdominal complicationchylous ascites. This is an unusual abdominal complication ofLAM that canofLAM that can

occur in the absence of pleural effusion.occur in the absence of pleural effusion. Chylous ascites may be more frequentChylous ascites may be more frequent

after transplantation.after transplantation.


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Tuberous Sclerosis ComplexTuberous Sclerosis Complex

(TSC)(TSC) TuberousTuberous sclerosissclerosis complexcomplex (TSC)(TSC) isis anan

autosomalautosomal dominantdominant disorderdisorder characterizedcharacterized bybyhamartomas,hamartomas, seizures,seizures, andand mentalmental retardationretardation..

TSCTSC isis causedcaused byby mutationmutation ofof twotwo genes,genes, TSCTSC11oror TSCTSC22;; itsits occurrenceoccurrence cancan bebe sporadicsporadic(approximately(approximately 6060%% ofof cases)cases) oror inheritedinherited

LAMLAM hashas beenbeen consideredconsidered aa formeforme frustefruste ofof TSCTSC

UpUp toto aa thirdthird ofof womenwomen withwith TSCTSC maymay exhibitexhibitlunglung cysts,cysts, thethe hallmarkhallmark featurefeature ofof LAMLAM;; thesethesepatientspatients havehave beenbeen categorizedcategorized asas havinghavingTSC/LAMTSC/LAM


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TSC + LAMTSC + LAM

Nilo A. Avila, Sporadic Lymphangioleiomyomatosis and Tuberous Sclerosis ComplexNilo A. Avila, Sporadic Lymphangioleiomyomatosis and Tuberous Sclerosis Complex

with Lymphangioleiomyomatosis: Comparison of CT Features;with Lymphangioleiomyomatosis: Comparison of CT Features;RadiologyRadiology2007;242:2772007;242:277--285285


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TSC + LAMTSC + LAM




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TSC + LAMTSC + LAM




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TSC + LAMTSC + LAM




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Case 1Case 1 F38F38

MSCT axial view shows focal lesions of angiomyolipomaof low density with slight

central enhancement in the arterial (A), portal (B) anddelayed (C) phases.

A typical lipoma is visible in the soft tissues of the backon the right side


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Case 2Case 2 F23F23

Patient with tuberous sclerosiscomplex. The frontal reconstructionreveals giant, bilateralangiomyolipomas of the kidneys, twolesions of angiomyolipoma in the liverand a small amount of fluid in thepelvis

MSCT in pulmonary window shows

small cysts in the pulmonaryparenchyma and the leftpneumothorax detected by CT

case of the day 12.01

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