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InterhospitalPediatric Chest Conference
November 27, 2008King Chulalongkorn Memorial Hospital
History
• ผูปวยเด็กชายไทย อายุ 5 เดือน ที่อยู อ.เมือง จ.ระยอง
• CC : ไข ไอ หอบเหนื่อยมากขึ้น 10 วันกอนมาโรงพยาบาล
• PI : 3 เดือนกอน มารดาสังเกตวาหนาอกดานขวาโตขึ้นรวมกับหายใจ
เร็ว ดูดนมแลวเหนื่อย ตองหยุดดูดเปนพัก ๆ เด็กรองงอแงมากขึ้น
มารดาพาไปรักษาที่คลินิก แพทยแนะนําใหสังเกตอาการตอ
10 วันกอน ผูปวยมีไข ไอ หอบเหนื่อย ไดไปรับการรักษาที่รพ.
ระยอง ตรวจพบBT 38.5oC, tachypnea, ฟงปอดได crepitation both
lungs
History
• CXR: cardiomegaly with mediastinal mass
• Dx: anterior mediastinal mass with pneumonia
• Rx: admit, on oxygen box 10 LPM, Ceftriaxone iv
• 5 วันกอนอาการเหนื่อยหอบไมดีขึ้น มีไข เพิ่ม dose Ceftriaxone (70
mg/kg/day)
• 2 วันกอนอาการเหนื่อยหอบมากขึ้น ซีดลง ม ีrespiratory failure ได
intubation, on ventilator: PIP 24, PEEP 4, rate 50/min, Ti 0.4,
FiO2 1 และขอสงตัวมารับการรักษาตอที่รพ.จุฬาฯ
History
• PH: บตุรคนที่ 2 คลอดปกติ น้ําหนักแรกคลอด 2,370 gm หลังคลอดไมมี
อาการผิดปกติใด ๆ
• G&D: ชันคอ แตยังไมพลิกคว่ํา
Physical examination• BW 6.4 kg(P25-50), Ht 56 cm (P 10), HC 41.5 cm (P 75)
• V/S: BT 37.7 oC, PR 140/min, BP 113/71 mmHg
• GA: A male infant on endotracheal tube, irritable, not pale, no jaundice,
no cyanosis, no edema
• HEENT: AF 2.5 x 2.5 cm, no bulging
pharynx and tonsils not injected
• RS: decreased breath sound Rt lung, coarse crepitation both lungs
• CVS: no active precordium, normal S1S2, no murmur
• Abdomen: soft, liver 2 cm below RCM, spleen not palpable
• NS: pupil 3 mm RTL both, active equal movement, DTR 2+ all
• LN: no superficial lymphadenopathy
Investigation
• CBC: Hb 12 g/dL, Hct 40.4%, Wbc 8,510 /mm3 (N 51%,
L 40%, M 3.6%) plt 328,000/mm3
• U/A: pH 6, spgr 1.025, prot 1+, glucose neg, ketone 2+
wbc 0-1, no Rbc
• Blood chemistry: BUN 6, Cr 0.14 mg/dl
Na 142, K 3.3, Cl 99, HCO3 25 mEq/L
Problem lists
Thai 5-month-old boy with
• Chronic dyspnea for 3 months
• Progressive Rt chest wall enlargement for 3 months
• Pneumonia +/- anterior mediastinal mass with
respiratory failure
CT chest• Large hypovascular multiseptated cystic lesion of the
right hemithorax, likely originating from chest wall,
with expansion and soap-bubble appearance of right
3rd -5th ribs and extension into right hemithorax,
reaching and displacing mediastinum to the left
• RUL atelectasis, contralateral mediastinal shift from
direct pressure effect, there are also segmental
atelectasis in RML, LUL, LLL
Tumor marker
• β-HCG : < 5 (0-5) U/ml
• Alpha-fetoprotein : 9.07 (0-10) IU/ml
Blood chemistry
• TB 0.43,DB 0.1 mg/dL
SGOT 52, SGPT 19, AP 299 U/L
Alb 4, glob 2.6 mg/dL
• Ca 8.6, PO4 3.3 mg/dL
• LDH 1320 U/L (230-460)
Bone marrow aspiration & biopsy23/9/2551
• BMA: no abnormal cell, no blast
• BM biopsy:
– suboptimal specimen due to trauma
– Presence of trilineage marrow with normal maturation
– no granuloma
– no fibrosis
Diagnosis: no histologic evidence of malignancy
EKG
• Normal sinus rhythm rate 150/min
• Normal QRS axis
• Normal PR interval (0.08)
• No chamber enlargement
Problem lists• Rt hemithorax mass with rib 3rd -5th destruction
• Respiratory failure secondary to compression of the lung
Nature of mass ??
Origin: intrapleural or extrapleural mass??
Benign or malignancy ??
Pulmonary involvement ??
Treatment ??
Prognosis ??
Differential diagnosisExtrapleural lesion : chest wall, rib-Fibroma/Fibrosacroma-Rhabdomyosarcoma-Lipoma/Liposarcoma-PNET (Askin’s tumor)-Chondroma/Chondrosarcoma-Rib: Osteosarcoma, Ewing sarcoma, Harmartoma, Aneurysmal bone cyst
(1o or 2o from Fibrous dysplasia, Lymphangioma, Mesenchymal hamartoma)
Pleural lesion : Lipoma, Liposarcoma, Mesothelioma
Intrapleural lesion-Mediastinum
- benign cystic teratoma(Dermoid cyst), malignant teratoid tumor- Neuroblastoma
- Pulmonary
Malignancy :
-Incidence: common malignancy
-Large mass
-Multiple rib destruction
Benign
-Slow progressive
-No other organ involvement
-No metastasis
-Rib destruction may be due to primary origin at rib
Differential diagnosisExtrapleural lesion : chest wall, rib-Fibroma/Fibrosacroma-Rhabdomyosarcoma-Lipoma/Liposarcoma-PNET (Askin’s tumor)-Chondroma/Chondrosarcoma-Rib: Osteosarcoma, Ewing sarcoma, Harmartoma, Aneurysmal bone cyst
- Primary- Secondary: Lymphangioma, Mesenchymal hamartoma
Pleural lesion : Lipoma, Liposarcoma, Mesothelioma
Mediastinum : Neuroblastoma
Progression
• Respiration :
– On servo300: PIP 16 above PEEP 5, Rate 30, Ti 0.5, FiO2 0.4
CBG: pH 7.48 PO2 88 PCO2 42 HCO3 30.8
• Infection:
– Ceftriaxone 12-22/9/2551
– Fortum + Amikin since 22/9/2551
– W/U
• H/C, TSC : NG
• U/C : yeast
– Start Fluconazole 25/9/2551
Progression
• Consult intervention for core biopsy 24/9/51
• Consult pediatric surgery for incisional biopsy 26/9/51
Fibro-adipose tissue
and small lymphnode
no neoplasm
Benign cartilage (rib portion)
and underlying reactive bone
Progression• Consult pediatric surgery 2nd incisional biopsy 3/10/2551
• Consult pathologist
1. Skeletal muscle
2. Fibrous connective tissue and tiny piece of lung parenchyma
- Fibroadipose tissue, benign cartilage, skeletal muscle and several reactive lymph nodes
- No evidence of malignancy
Diagnosis: Mesenchymal hamartoma of chest wall
Post incisional biopsy
3/10/2551
Progression• Consult CVT for tumor removal 15/10/2551• Operation: Rt thoracotomy with tumor removal
and Rt thoracoplasty (Operative time : 4 hr)• Finding
• procedure
large Rt intrapleural space mass size 15x10 cm
originate from Rt posterior rib cage
extend to Rt thoracic cage with rib 3rd – 5th and RUL involvement
extend to pericardial sac (not invade pericardium)
2 layer mass, well encapsulate of the inner mass
Content: soft tissue, blood clot and hematoma
Tumor removal with rib 3rd – 5th resection
Partial resection of RUL (apex)
Thoracoplasty with cranioplasty tube & PTFE patch
pathology
Gross : well-circumscribed gray-brown mass with previously rupture, measuring 8x6.2x1.5 cm, multiloculated cyst, containing irregular-shaped dark brown material
Microscopic :
cystic part : fibrocollageneous cystic wall infiltrated by mononuclear cells and multinucleated giant cells with wide spread hemorrhage
Solid part : lung parenchyma with pulmonary edema and scattered hemosiderin-laden macrophages, multiple fragments cartilage, trabecular bone and fibrous tissue
Diagnosis : mesenchymal hamartoma of the chest wall
Progression• Post op : no immediate complication
• CXR:
• On ventilator setting: PIP 16 above PEEP 6 rate 30 Ti 0.45 FiO2 0.4
• Infection: Fortum 22/9-6/10/2551, Amikin 22-29/9/2551
Fluconazole 25/9-10/10/2551
9/10/2551: TSC : P.aeruginosa, U/C: Enterobacter
Fortum + Amikin (total course Fortum 14 days, Amikin7 days)
• Extubation: post op day 6 (21/10/51)
no complication
S/P tumor removal with thoracoplasty
15/10/2551
Post op 1 week
Mesenchymal hamartoma of chest wall• Rare case• Case report in neonate or early infancy • Single tumor, extrapleural mass, variable in size• Expansile intraosseous lesion, extended to
compress but not involve the underlying lung • Benign lesion which often suggests malignancy• Typical radiologic finding• Diagnosis based on histopathology• Self limit process• Good prognosis
Natural history & clinical presentation
Australasina radiology(2003) 47,78-82
Natural history• At least 78 case have been reported• Onset
– Lesion were discovered at birth about 55 %– Most detected during the first year of life– Late diagnosis was reported in age 4-13 years
• Origin– Usually arises from the posterior or lateral portions of
the rib with projection into the thoracic cavity• Site
– Usually unifocal but multifocal lesions were reported– Bilaterality was described only 5 cases
J Med Assoc Thai vol.90 No.11 2007
Clinical presentation• No respiratory symptom
Chest wall mass/deformities• Mild respiratory symptoms• Severe respiratory distress
J Med Assoc Thai vol.90 No.11 2007
Radiological appearance• CXR:
– mass centered on one or more ribs– Involved ribs show expansion and destruction,
might be displacement and distortion of adjacent ribs
– Variable calcificaition– Large mass compress the underlying lung or
mediastinal displacement and scoliosis
Australasina radiology(2003) 47,78-82
Radiological appearance• CT chest
– Large cystic spaces with fluid levels– Extensive matrix mineralization calcified
soft-tissue mass– Rib expansion and destruction– Aneurysmal bone cyst-like fluid levels– Compression of the underlying lung, with or
without mediastinal shift
Australasina radiology(2003) 47,78-82Pediatr Surg Int (2006) 22:398-400
CT: confirm the ribs as the site of origin of the mass
Pathologic finding• Grossly: mixed solid and cystic lesion
– Visible cysts with mucoid to bloody contents– Solid areas composed of cartilage, fibrous tissue and bone
• Histology:– Disorganized admixture of disparate but well differentiated skeletal
elements in variably cellular background stroma– Skeletal element :
• Cartilage is abundant• Often : evidence of enchondral ossification or bone trabeculae
formation commonly contain hematopoietic marrow– Stroma : oval or spindle mesenchymal cell, no mitoses and atypia
Focal stromal hypercellularity mistaken from sarcomas– Aneurysmal bone cyst: osteoclast-like giant cells, blood filled spaces,
hemosiderin, foam cells and fibromembranous spetae
Australasina radiology(2003) 47,78-82
Pathologic finding
• Immunohistochemistry:– positive for S-100 in chondrocytes and stromal cell
with chondroid differentiation
– Factor VIII staining of small vascular spaces
– No significant staining with either cytokertin or muscle specific actin
– Not necessary to establish the diagnosis
Pediatr Surg Int (2006) 22:398-400
Diagnosis
• Typical radiologic finding• Base on : histology
– Fine needle aspiration– Biopsy– Resection
Treatment• Symptomatic patient : surgery• Asymptomatic patient : controversial
– En bloc excision or– Conservative
• Some patients : regressed without definitive surgical excision
• No role of chemotherapy or radiation
Pediatr Surg Int (2006) 22:398-400
Complication
• Hemorrhage • Scoliosis
Pediatr Surg Int (2006) 22:398-400
Prognosis• Good prognosis• Four deaths are reported
– Three : occurred immediately after birth due to severe respiratory compromise resulting from pulmonary compression by large masses
– One: result of infection after chemotherapy for “embryonal sarcoma” (Diagnosis by FNA)
Australasina radiology(2003) 47,78-82J Med Assoc Thai vol.90 No.11 2007
Fatal bilateral congenital MHCW• Term female infant, BW 2680 gms
• Respiratory distress at 2 hr after birth
• CXR: bilateral masses involving the posterior 6th-8th ribs with ribs deformity
• Operation: thoracotomy with partial removal of the left chest mass at 12 hours-age
• Post op : progressive dyspnea and expired at 29 hours-age
• Autopsy: well circumscribed masses, measured Lt 7x6x4cm, Rt6x5x4 cm, both projected into the thoracic cavities and severelycompressed both lungs
histolygy: mesenchymal hamartoma , showed marked bilateral pulmonary atelectasis and massive amniotic fluid aspiration, anoxic change of brain
J Med Assoc Thai vol.90 No.11 2007
Outcome
• 16 years F/U• Two case : no surgical treatment
no recurrenceno any other problems
Radiology 1972;104:107-9J Surg Oncol 1982;21:267-70
Outcome • 7-year follow up• 1-year-old female : anterior chest wall mass, no respiratory distress• CXR: deformities(expansion and distortion) of 6th-8th ribs of
posterolateral chest wall• CT: mass with some bony density on the Rt anterior chest wall• Microscopic from biopsy specimens: mesenchymal hamartoma• Operative finding: tumor 5x4x3 cm involved the sternum ant
cartilage of ribs 5th-8th , did not invade the lung, diaphragm, or pleura• Operation: total excision and chest wall repaired with a Marlex
mesh• Microscopic review: well circumscribed mass showed typical
features of a mesenchymal hamartoma• Postoperative course : uneventful• F/U 7 years without a local recurrence or evidence of any other
problems• Last CT: 6th rib show residual masses had disappeared, 8th rib had
not enlargedPediatr Surg Int (1993) 8:521-22
Mesenchymal hamartoma of chest wall
• 1960s-1970s: reported as other types of tumor: benign mesenchymoma, osteochondrosarcoma, osteochondroma and malignant mesenchymoma
• 1979 McLeod and Dahlin : objected term “ hamartoma ”
• 1986 Odell and Benjamin : first used term “mesenchymal hamartoma”
• Recent report has used the term “aneurysmal bone cyst secondary to infantile cartilaginous hamartoma of rib”
Australasina radiology(2003) 47,78-82Pediatr Surg Int (2006) 22:398-400
