Plueral Malignancy: Radiologic-pathologic

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Plueral Malignancy: Radiologic-pathologic

Correlation

Ritu R. Gill, MD

Pleural Malignancies:

Radiologic-Pathologic

Correlation

Ritu R Gill MD

Brigham and Women’s Hospital

Boston, Massachusetts

Pleural Mass Characteristics

• Obtuse angles with pleural surfaces

• Sharp margins on tangential views

• Discrepant margins on different views

• Discrepant margins on different views

• Incomplete border

• May cross fissures

• Look for osseous and soft tissue--chest wall

WHO Histological Classification

of Pleural Tumors

Mesothelial Tumours Diffuse malignant mesothelioma • Epithelioid mesothelioma • Sarcomatoid mesothelioma • Desmoplastic mesothelioma

Lymphoproliferative Disorders • Primary effusion lymphoma 9678/3 • Pyothorax – associated lymphoma Mesenchymal Tumours E ith li id h i d th li • Desmoplastic mesothelioma • Biphasic mesothelioma • Localized malignant mesothelioma

Other Tumours of Mesothelial Origin

• Well differentiated papillary mesothelioma • Adenomatoid tumour • Epithelioid hemangioendothelioma • Angiosarcoma • Synovial sarcoma • Monophasic

• Biphasic solitary fibrous tumour • Calcifying tumour of the pleura • Desmoplastic round cell tumour

Metastases

• 40% Bronchogenic adenocarcinoma

• 20% Breast cancer

• 10% Lymphoma

• 30% Other primaries

– Thymoma

– Carcinoid

Malignant Pleural Mesothelioma

• 2,000-3,000 cases annually in the U.S.

• Incidence increasing worldwide

• Up to 10% lifetime risk of developing

mesothelioma among asbestos workers

• Asbestos + cigarette smoking Æ synergistic

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Malignant Pleural Mesothelioma

• 3-6:1(men:women) follows exposure

• Latency of 30-40 years (2000-3000/yr)

• Present with SOB, chest pain, cough,

and weight loss

• Right side with SVC, Horner syndrome

• HPO, clubbing

Pathologic Spectrum

A B C D E G I H

Epithelioid 50% Mixed 34% Sarcomatoid 16%

C F I

Prognosis of Malignant Mesothelioma

Prognosis of Malignant Mesothelioma

is Dependent on the Histologic Type

is Dependent on the Histologic Type

Malignant Mesothelioma

80 100 Epithelioid Mixed Sarcomatoid v iv a l

Sug a rb a ke r DJ e t a l, J Tho ra c C a rd io va sc Surg . 1999 Ja n;117(1):54-63

0 24 48 72 96 120 0 20 40 60 WDPM Time (months) P e rc e n t s u rv

Stage

Definition

I

Disease confined to within the capsule of the parietal

pleura; ipsilateral pleura, lung, pericardium, diaphragm,

or chest wall disease limited to previous biopsy site

II

All of stage I with positive intrathoracic (N1 or N2)

lymph nodes and positive resection margins

BWH Staging

III

Local extension of disease into chest wall or

mediastinum; heart, or through the diaphragm and

peritoneum; with or without extrathoracic or

contralateral lymph node involvement (N3)

IV

Distant metastatic disease

Sugarbaker, DJ, Norberto, JJ, Swanson, SJ. “ Surgical staging and work up of patients with diffuse malignant pleural mesothelioma.” Mesotheliomas. Seminars in Thoracic and Cardiovascular Surgery. Ed. Sugarbaker DJ. Volume 9, issue 4, 1997

Radiographic Presentation

• Wide range

• Pleural effusion, small to large

,

g

• Encasement of lung

• Lobulated pleural masses

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Chest Wall and Mediastinum

Involvement

Diaphragmatic Extension

diaphragm

Pulmonary, Lymph Node, and

Liver Involvement

Chest Wall Invasion

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Diaphragm involvement Endothoracic fascia involvement or solitary resectable focus tumor

.01 .05 T2

T2

Accuracy of MPM staging by CT and MRI p     

MRI is superior to CT

Diffusion Weighted Imaging

3orthogonal directions 4b-values (0,250,500,750) Time 6.5 mts HASTE CE DW I ADC ADC = 0.883x 10 10--33mmmm22/s/s

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Following Neoadjuvant Chemotherapy

Pre-chemotherapy Pre-chemotherapy

Post-chemotherapy

Post-chemotherapy

Rotating maximum intensity projection and fusion PET-PT images of the same patient from pre and post chemo-therapy studies show reduced post treatment FDG avidity of the right pleural mass. The patient subsequently under-went successful EPP.

Pre-chemotherapy Post-chemotherapy

“ Course” of MPM

• Pleural effusion dominant early

• Progressive encasement of lung

• Lobulated pleural masses late

• Invasion of lung

• Lung nodules and distant spread

Solitary Fibrous Tumor

• Benign mesothelioma

• Benign fibrous tumor of pleura

• Localized fibrous tumor of pleura

• Solitary fibrous tumor

Solitary Fibrous Tumor

• Rare

• Symptoms relate to size: cough, chest

pain, dyspnea

pain, dyspnea

• Paraneoplastic syndrome: clubbing,

hypertrophic osteo-arthropathy,

hypoglycemia

Solitary Fibrous Tumor

• Visceral pleura 80% • Parietal pleura 20% • Encapsulated, pedunculated • Nodular, whorled

appearance

• CD34 positive; calretinin and WT-1 negative

• Hemorrhage, necrosis and

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CD34

Calretinin

WT1

Solitary fibrous tumor of pleura

Liposarcoma

• Typically large, infiltrative, and asymptomatic

• No evidence to suggest they arise from pre-existing lipomas • Heterogeneous masses on CT oge

with soft tissue and fat component. They tend to measure less than 50 HU (mean CT values) on pre and post intravenous contrast enhancement images • On MRI, they tend to have high

signal intensity on T2WI, because of myxoid degeneration, while low signal is common and have variable enhancement post gadolinium administration

Normal adipose tissue Well-differentiated liposarcoma

De-differentiated liposarcoma

Synovial Sarcoma

• Typically occurs in adolescents and young adults between the ages of 15 and 40 years • It is believed to originate from

primitive pleuripotent mesenchyme capable of synovial differentiation • Because of its rarity, it is often

mistaken on imaging and histology for sarcomatoid mesothelioma • Molecular studies for the X:18 translocation are useful for differentiation

• Focally positive: EMA, AE1/AE3, Cam5.2

– Negative: CD34, S-100, GFAP – FISH: positive for SYT gene

rearrangement

Lymphoma

• Both primary and

secondary forms can

involve the pleura.

Follicular and B cell

lymphoma tend to be

more common

• There can be associated

lymphadenopathy, as well

as chest wall and marrow

involvement. Tend to be

low on T1WI and iso to

high signal on T2WI

• Diffuse homogenous

enhancement

LCA

CD20 (B)

CD10

CD3 (T)

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Conclusion

• Metastases are 95% of pleural

lesions

• Focal pleural tumor can be favorable

DX

• Malignant pleural mesothelioma and

Adenocarcinomatosis may look alike

Resources

• 1. Pelucchi C, Malvezzi M, La Vecchia C, Levi F, Decarli A, Negri E. The mesothelioma epidemic in Western Europe: an update. Br J Cancer 2004 Mar 8; 90(5):1022-4.

• 2. Miller BH, Rosado-de-Christenson ML, Mason AC, Fleming MV, White CC, Krasna MJ. From the archives of the AFIP—Malignant pleural mesothelioma: radiologic-pathologic correlation. RadioGraphics 1996; 16:613–644. • 3. Kucuksu N, Thomas W, Ezdinli EZ. Chemotherapy of malignant diffuse

mesothelioma. Cancer 1976; 37:1265–1274.

• 4. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981; 47:2678– 2689.

• 5. Suter M, Gebhard S, Boumghar M, Peloponisios N, Genton CY. Localized fibrous tumours of the pleura: 15 new cases and review of the literature. Eur J Cardiothorac Surg 1998;

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