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GOOD MORNINGGOOD MORNING
VASCULAR AND VASCULAR AND CARTILAGINOUS CARTILAGINOUS
HAMARTOMA OF THE RIBS IN HAMARTOMA OF THE RIBS IN INFANCY: A CASE REPORTINFANCY: A CASE REPORT
Mila Amor T. Villanueva-Reyes, M.D. Mila Amor T. Villanueva-Reyes, M.D. Jose M. Carnate, Jr., M.D.Jose M. Carnate, Jr., M.D.
Ariel M. Vergel de Dios, M.D.Ariel M. Vergel de Dios, M.D.
INTRODUCTIONINTRODUCTION
a rare entity a rare entity affects the ribs of the newbornaffects the ribs of the newborn
few case reportsfew case reports
a.k.a. intra-thoracic mesenchymoma, a.k.a. intra-thoracic mesenchymoma, hamartoma (mesenchymoma) of the chest hamartoma (mesenchymoma) of the chest wall in infancy, infantile osteochondroma wall in infancy, infantile osteochondroma
focal overgrowth of normal skeletal focal overgrowth of normal skeletal elements of the ribselements of the ribs
has a benign coursehas a benign course
recognition is importantrecognition is important
first locally reported case of a vascular first locally reported case of a vascular and cartilaginous hamartoma of the ribsand cartilaginous hamartoma of the ribs
INTRODUCTION
CASE REPORTCASE REPORT
14 day old female14 day old femaledyspneadyspneathickly meconium thickly meconium stainedstainedtachypneictachypneicAPGAR 8, 9APGAR 8, 9
‘‘soft tissue soft tissue calcificationcalcificationwith associated with associated destruction of the destruction of the posterolateral posterolateral segments of the segments of the second to the second to the eighth left ribs’eighth left ribs’
CHEST X-RAY
‘‘huge, mixed-huge, mixed-attenuation, attenuation,
predominantly predominantly cystic mass with cystic mass with
peripheral peripheral calcification in calcification in the left hemi-the left hemi-
thorax, pushing thorax, pushing the mediastinum the mediastinum
to the right, to the right, probably arising probably arising
from, and from, and distorting the distorting the
underlying ribs’underlying ribs’
CT-SCAN
R L
FNAB:’suggestive of a germ cell tumor’ FNAB:’suggestive of a germ cell tumor’ (teratoma)(teratoma)
well-circumscribed mass arising from well-circumscribed mass arising from the posterolateral aspects of the left 3the posterolateral aspects of the left 3rdrd, , 44thth and 5 and 5thth ribs ribs
CASE REPORT
CASE REPORTCASE REPORT
Pathologic Diagnosis:Pathologic Diagnosis:
Vascular and cartilaginous Vascular and cartilaginous hamartoma of the ribs in infancy hamartoma of the ribs in infancy with secondary aneurysmal bone with secondary aneurysmal bone cyst formation.cyst formation.
DISCUSSIONDISCUSSION
Vascular and cartilaginous Vascular and cartilaginous hamartoma of the ribs(VCHR)hamartoma of the ribs(VCHR)
at birth or early infancyat birth or early infancy
expansile, intraosseous lesionexpansile, intraosseous lesioninvolves one or more ribsinvolves one or more ribsbenign behaviorbenign behavior
DISCUSSION
disorderly islands of mature hyaline disorderly islands of mature hyaline cartilage exhibiting endochondral cartilage exhibiting endochondral ossificationossification
intervening fibrous tissues and blood-intervening fibrous tissues and blood-filled vascular channelsfilled vascular channels
DISCUSSION
HISTOGENESISHISTOGENESIS
Not appropriateNot appropriate Implies a neoplastic originImplies a neoplastic origin
“Mesenchymoma”
HISTOGENESISHISTOGENESIS
““focal overgrowth of mature normal cells focal overgrowth of mature normal cells and tissues native to the organ in which it and tissues native to the organ in which it occurs”occurs”
Infancy & childhoodInfancy & childhood Totally benignTotally benign
“Hamartoma”
Vascular & cartilaginous Vascular & cartilaginous hamartoma of the ribshamartoma of the ribs
Islands of mature hyaline cartilage, fibrous Islands of mature hyaline cartilage, fibrous tissues and vascular channelstissues and vascular channels
Birth or early infancyBirth or early infancy Invariably benign behaviorInvariably benign behavior
HISTOGENESISHISTOGENESIS
PROGNOSISPROGNOSIS
Benign behaviorBenign behavior “ “ Malignant Mesenchymoma” with long Malignant Mesenchymoma” with long
post-operative survivalpost-operative survival
DIFFERENTIAL DIFFERENTIAL DIAGNOSESDIAGNOSES
1. Mature cystic teratoma 2. Osteoclastoma 3. Enchondroma4. Osteochondroma5. Hemangioma of the bone6. Aneurysmal Bone Cyst(ABC)
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Cartilage lobulesCartilage lobules
1. Mature Cystic Teratoma
Stratified squamous epitheliumStratified squamous epithelium Sebaceous glandsSebaceous glands Hair folliclesHair follicles
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Multinucleated giant cellsMultinucleated giant cells
2. Osteoclastoma
No bone/cartilage matrix producedNo bone/cartilage matrix produced
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Cartilage lobulesCartilage lobules
3. Enchondroma
Vascular channels not prominentVascular channels not prominent
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Cartilage lobulesCartilage lobules
4. Osteochondroma
Mature bony stalk with cartilaginous Mature bony stalk with cartilaginous capcap
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Blood filled vascular channelsBlood filled vascular channels
5. Hemangioma of the bone
Endothelial-lined vessels separated by Endothelial-lined vessels separated by loose connective tissue stromaloose connective tissue stroma
DIFFERENTIAL DIAGNOSESDIFFERENTIAL DIAGNOSES
Blood-filled vascular channelsBlood-filled vascular channels
6. Primary Aneurysmal bone cyst
No cartilage lobulesNo cartilage lobules
May be due to prominent vascular May be due to prominent vascular channels in VCHR channels in VCHR
From disruption of adjacent vasculature From disruption of adjacent vasculature by primary bone lesions during growth by primary bone lesions during growth (Levy et. al.)(Levy et. al.)
Secondary Aneurysmal bone cyst in VCHR
These conditions should always These conditions should always be kept in mind when presented be kept in mind when presented with tumors of the ribs in infants.with tumors of the ribs in infants.
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