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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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