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1. Alveolar adenoma: biasanya pada perifer paru, terdiri dari ruang kistik kecil yangdibatasi oleh pneumosit tipe II dan berisi cairan.
2. Bronchial gland adenoma: termasuk oncocytoma dan adenoma kelenjar mucus.3. Papillary adenoma: tumbuh pada perifer paru, terbentuk dari pneumosit tipe II dan
sel clara.
4. Benign metastasizing leiomyoma : Jarang,
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- Disebut juga pulmonary chondroma- Tumor jinak yang terjadi pada orang dewasa.- Biasanya soliter, subpleural- Umumnya, muncul sebagai incidental coin lesion (lingkaran abnormal) dengan
pola popcorn dari gambaran Xray.
- Treatment: excision9. Multiple pulmonary leiomyomatous hamartoma:- Sangat jarang, hampir selalu pada wanita paruh baya dan asimtomatik.- DD : Reaktivasi proliferasi otot polos pada pneumonitis interstisial kronik, benign
metastasizing leiomyoma, leiomyosarcoma, lymphangioleiomyomatosi, proliferasi
otot pulmonal native.
10.Hemangioma- Biasanya pada anakanak- Endobronchial atau parenkim
11.Hemangiomatosis- Dapat muncul dengan gejala dari hipertensi pulmonal atau penyakit paru
interstitial.
- Prognosis buruk; dapat menjadi varian dari penyakit veno-occlusive.- DD: veno-occlusive disease
12.Inflammatory pseudotumor- Juga disebut inflammatory myofibroblastic tumor, plasma cell granuloma- Sering pada anak anak dan orang dewasa yang berusia 30 tahun serta
merupakan tumor paru yang paling sering pada anakanak usia 16 tahun dan
lebih muda.
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- Treatment: eksisi; jarang menyebabkan kematian karena local extension.- Faktor faktor diagnostik yang buruk: metastase, nekrosis >15% dari area
permukaan yang terkena, local recurrence, bizarre giant cells, > 3 tampilan
mikotik/50 HPF, tahap lanjut, high cellularity.
- DD: hemangiopericytoma, carcinoid tumor, plasmacytoma, amyloid tumor,metastatic carcinoma, TB in immunosuppressed patients, organizing
pneumonia, lipid pneumonia, benign and malignant fibrous histiocytoma,
other spindle cell tumors, mycobacterial pseudotumor
13.Inflammatory pseudotumor pada masa kanakkanak- Kebanyakan paling sering lesi paru yang terisolasi pada anak anak dan
asimtomatik.
- Jinak, walaupun beberapa kasus yang jarang telah melaporkan adanyakeganasan.
- Tatalaksana: eksisi atau raditerapi14.Langerhans cell histiocytosis- Also called eosinophilic granuloma, Langerhans cell granulomatosis, histiocytosis
X (H-X), Hand-Schuller-Christian disease, Letterer-Siwe disease
- Usually ages 20-39 years; strongly associated with smokers- 20% with multicentric disease (bone, skin, lymph nodes, spleen, pituitary) have
lung involvement
- 50% of cases only involve lung- Often associated with pneumothorax, Pneumocystis carinii pneumonia- Usually lung disease resolves or stabilizes, but 10-20% may progress to
respiratory failure
- DD: eosinophilic pleuritis (no Langerhans cells although mesothelial cells mayappear similar), reactive Langerhans cells in inflammatory conditions (no sheets
or groups of Langerhans cells), desquamative interstitial pneumonitis
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15.Leiomyoma- Rare, associated with HIV-infected children- DD: spindle cell carcinoma16.Lipoma- Usually endobronchial, usually men ages 50+
17.Lymphangiomyomatosis- Also called lymphangioleiomyomatosis- Rare, unknown etiology, may diffusely involve both lungs- Almost always in women, usually white and of reproductive age; rare cases in
men or postmenopausal women on hormone replacement
- Associated with tuberous sclerosis, renal angiomyolipomas- May involve mediastinal or periaortic lymph nodes- Derived from perivascular epithelioid cells (also angiomyolipomas, clear cell
[sugar] tumor of lung)
- Symptoms: dyspnea with pneumothorax or emphysema, without a smokinghistory
- Have severe impairment of diffusion with air trapping and expanding lungvolumes
- Disease is progressive, prognosis poor, death due to respiratory failure or corpulmonale
- Disease worsened by pregnancy or menstruation, improved post-menopause- Complications: respiratory insufficiency and death, spontaneous pneumothorax,
chylous pleural effusion; may be due to metastases or migration of progenitor
cells
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- Treatment: oophorectomy, hormone manipulation (progesterone or antiestrogens),lung transplantation (but may recur in lung allografts, Hum Path 2003;34:95)
- DD: metastatic endometrial sarcoma, benign metastasizing leiomyoma, idiopathicpulmonary hemosiderosis (all HMB45 negative), micronodular pneumocyte
hyperplasia
18.Micronodular pneumocyte hyperplasia- Associated with tubular sclerosis; may coexist with lymphangioleiomyomatosis- Usually women with shortness of breath- Considered a hamartoma- Not progressive- DD: lymphangioleiomyomatosis19.Paraganglioma- Rare in lung- Usually benign; rarely malignant- May be hyperplastic, not neoplastic- DD: carcinoid tumor (ribbons, festoons, rosettes)20.Sclerosing hemangioma- Also called sclerosing pneumocytoma- Usually adult women, ages 30-50 years, with incidental solitary nodule on chest
Xray
- Apparently derived from type II pneumocytes or with differentiation towardsthese cells
- Almost always benign, 2-4% have nodal metastases that dont appear to affectprognosis (Archives 2003;127:321)
- Sclerosis and hemorrhage are probably secondary changes
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- DD: primary and metastatic carcinoma, clear cell (sugar) tumor, carcinoidtumor, papillary adenoma, alveolar adenoma, epithelioid hemangioendothelioma,
Langerhans cell histiocytosis, meningiomas, meningothelial-like nodules
21.Solitary fibrous tumor- May be intrapulmonary and not pleural- Peaks at ages 50-69 years- Larger tumors associated with hypoglycemia, pleural effusion, pulmonary
osteoarthropathy (particularly if tumors 7cm+)
22.Squamous papilloma- Occur in large bronchi, often with associated tracheal or laryngeal lesions- Due to HPV- Often associated with dysplasia, carcinoma in situ or invasive squamous cell
carcinoma
- May contain mucus cells- DD: mucoepidermoid carcinoma (for papillomas with mucus cells)23.Dysplasia/carcinoma in situ
Dysplasia-general
- Usually associated with bronchial lesions; often present in uninvolved bronchusnear carcinoma
- No distinct criteria for dysplasia versus carcinoma in situ- Expert confirmation recommended before signing out case as carcinoma in situ24.Bronchioalveolar atypical adenomatous hyperplasia- Diagnostic variability exists - also called adenoma, well differentiated
bronchioalveolar carcinoma of Clara cell or type II pneumocyte type
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- Associated with coexisting bronchioloalveolar carcinoma and papillaryadenocarcinoma
- If malignant appearing but noninvasive, recommended to call bronchioloalveolarcarcinoma of nonmucinous type
Carcinoma
25.Carcinoma-general- 95% of lung tumors are bronchogenic carcinoma; also bronchial carcinoids,
mesenchymal, miscellaneous neoplasms
- In US, lung cancer is #1 cause of cancer death in men and women; causes 30% ofcancer deaths in men
- In 2002, 164,000 new cases in US; incidence in men is 74 per 100,000 vs. 31 per100,000 for women
- Peaks at ages 50-69 years; 2% occur before age 40- Young patients (age 40 or less) have higher incidence of 20q gains/amplifications
compared to older patients (56% vs. 8%), Mod Path 2002;15:372
- Cigarette smoking: causes most cases of lung cancer; relative risk of smokers vs.nonsmokers is 10:1; increases to 20:1 for >40 cigarettes/day; risk is strongly
related to number of cigarettes smoked, described in pack years (number of packs
per day x number of years smoking)
- Also associated with carcinomas of lip, tongue, pharynx, larynx, esophagus,bladder, pancreas, kidney, floor of mouth
- 10% of smokers have atypia or hyperplasia of bronchial epithelium- Carcinogens in tobacco smoke are benzo [a] pyrene (an initiator) and phenol
derivatives (promoters)
- Usually associated with squamous cell and small cell carcinoma, less likely withadenocarcinoma
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- Other causes: radiation exposure, uranium (RR with uranium exposure is 4:1 fornonsmokers, 10:1 for smokers vs. general population); asbestos (RR with asbestos
exposure is 5:1 for nonsmokers, 50-90:1 for smokers vs. general population),
exposure to nickel, chromate, coal, mustard gas, arsenic, beryllium, iron, vinyl
chloride, radon radiation, gold miners
- Causes of death for asbestos workers are: 20% lung cancer, 10% mesothelioma,10% GI carcinomas
- Symptoms: cough, weight loss, chest pain, shortness of breath, increased sputumproduction
- Systemic symptoms: Lambert-Eaton myasthenic syndrome (muscle weakness dueto antibodies to neuronal calcium channel), sensory peripheral neuropathy,
acanthosis nigricans, leukemoid reaction, hypertrophic pulmonary
osteoarthropathy (clubbing), superior vena cava syndrome (compression/invasion
of SVC causes venous congestion, circulatory compromise, dusky head, arm
edema), pain in distribution of ulnar nerve and Horners syndrome (enophthalmos,
ptosis, miosis, anhidrosis) due to apical lung tumors called Pancoast tumors
- Classification: broad classification is non-small cell carcinoma (80%) versus smallcell carcinoma (20%)
- 50% of non-small cell carcinomas are metastatic at diagnosis vs. 80% of small cellcarcinomas
- Many have mixed histologic subtypes- Scar cancers: scar is desmoplastic response to tumor- Spread: along bronchus distally and proximally, into lung parenchyma to
mediastinum or pleura, causing pleural seeding, pleural effusion, involvement of
diaphragm and chest wall
- Metastases: 50% have nodal involvement at resection (usually hilar, mediastinaland supraclavicular); also metastases to adrenals (50%), liver (30%), brain, bone;
also opposite lung, pericardium, kidneys
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- Treatment: complete excision for non-small cell lung carcinoma; radiation therapy(usually non-curative), chemotherapy (rarely curative, even for small cell
carcinoma)
- Survival: overall 5 year survival is 10-15%; only 30% have limited disease atdiagnosis making resection for cure an option
- For stage I non-small cell carcinoma, 5 year survival is 60%, poor prognosticfactors are p53+, HER2+, angiolymphatic invasion or tumor size > 3 cm, Hum
Path 2002;33:105
- Poor prognostic factors (clinical): high TNM stage, weight loss >10% of bodyweight, age < 40 years or women (usually are high stage), small cell or giant cell
subtypes, angiolymphatic invasion, pleural effusion, lack of lymphoplasmacytic
reaction; possibly ERCC1 genetic polymorphisms for non-small cell carcinoma
(Clin Lung Cancer 2009;10:118, Clin Cancer Res 2004;10:4939, Eur J
Cardiothorac Surg 2008;33:805)
- Favorable subtypes: non-mucinous bronchioloalveolar, well differentiatedsquamous cell
26.Acinic cell tumorBronchial gland tumor with low grade malignancy
- Very rare at this site, usually in parotid gland, less commonly in submandibularand sublingual glands
- May arise from pluripotent cells of tracheobronchial submucosal serous andmucus glands
- Treatment: surgical excision- DD: metastastic salivary gland tumor, primary lung adenocarcinoma, clear cell
(sugar) tumor of lung, oncocytic carcinoma, bronchial oncocytoma, bronchial
granular cell tumor, metastatic renal cell carcinoma
Adenocarcinoma
- Arises from terminal bronchioles
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- In US, 50% of lung carcinomas in women are adenocarcinoma; is the mostcommon subtype in nonsmokers
- 80% contain mucin- Grow slower than squamous cell; may be associated with scarring- 5 year survival: stage I - 69%, II - 40%, IIIA - 17%, IIIB - 5%, IV - 8%- Peripheral tumors with bronchioloalveolar and invasive areas < 5 mm had low
rates of vascular and pleural invasion and no nodal involvement, Am J Surg
Pathol 2003;27:937
- More likely TTF1 negative in males or smokers- DD: melanoma (may be mucin positive)References: Am J Surg Pathol 2003;27:150, Am J Surg Pathol 2002;26:767 (TTF1)
Sources for IHC testing: Clarient (Pulmotype testing)
Subtypes:
Bronchial surface cell type with little/no mucin
top
8% of adenocarcinoma cases
Gross: cartilage bearing bronchus with polypoid growth or in distal airways
Micro: papillary or tubular groups of tall columnar cells resembling ciliated columnar
cells, but without cilia
EM: numerous mitochondria and smooth surfaced vesicles, basal bodies but usually
no cilia, no secretory granules
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Goblet cell type
top
Gross: lobar pneumonia-like or nodular
Micro: tumor cells resemble goblet cells, with cytoplasmic mucin displacing the
nuclei to basal portion; usually in bronchioloalveolar pattern, rarely in papillary
pattern
Positive stains: lysozyme
Bronchial gland cell type
top
5% of adenocarcinoma cases
Gross: cartilage bearing bronchus with polypoid growth or in distal airways as
nodular tumor
Micro: acini, tubules, ducts, cribriform patterns or solid nests of cuboidal or polygonal
cells, often with mucin; may have signet ring cells; resemble bronchial glands; nests
surrounded by myoepithelial type cells
Clara cell type
top
50% of adenocarcinoma cases
Gross: pleura indented toward area of central fibrosis
Micro: papillary, tubular or bronchioloalveolar patterns of peg-shaped cells or low
columnar cells with tongue-shaped projections; fibrotic focus representing collapsed
alveoli may be present; variable nuclear atypia and mitotic activity; frequent
intranuclear eosinophilic inclusion bodies
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EM: microvilli on free cell surface of peg cells, scattered rough endoplasmic
reticulum, 200-900 nm round, dense, neurosecretory granules
DD: type II alveolar epithelial cell type
Type II alveolar epithelial cell type
top
Gross: solitary nodular of rarely diffusely distributed tumor
Micro: papillary or bronchioloalveolar patterns of cuboidal to low columnar cells with
dome-shaped free cell border; finely vacuolated cytoplasm
EM: cytoplasmic lamellar inclusion bodies
Hepatoid
top
Resembles hepatocellular carcinoma (HCC); more common in gastric
adenocarcinomas
Old age, high serum AFP, aggressive behavior, often with liver metastases
Micro: tubular and papillary adenocarcinoma with hepatic morphology; liver
metastases are difficult to distinguish from HCC
Positive stains: same as HCC - AFP, CK8, CK18, canalicular staining with polyclonalCEA
Positive stains: different from HCC - CK19, CK20, negative for HepPar1, negative
for CK7
Molecular: 4q-, 8p-, Xq+ (seen in hepatocellular carcinoma and hepatoblastoma)
References: Am J Surg Pathol 2003;27:1302
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Adenocarcinoma of fetal lung type
top
Rare, high grade variant is very aggressive
May be a variant of pulmonary blastoma without malignant mesenchymal
components
Associated with upregulation of Wnt signaling pathway, Mod Path 2002;15:617
Micro: irregular tubular structures of columnar epithelial cells with clear cytoplasm
and oval nuclei, optically clear nuclei rich in biotin; resembles fetal lung in
pseudoglandular stage; fibrous stroma without atypia; no morules
Micro images: high grade
DD: well-differentiated fetal adenocarcinoma (has morules)
Adenocarcinoma - well differentiated fetal type
top
Mean ~40 years
Better prognosis than pulmonary blastoma
Variant of bronchioloalveolar carcinoma
Associated with upregulation of Wnt signaling pathway, Mod Path 2002;15:617
Gross: well-defined, non-encapsulated, not related to visible bronchi
Micro: irregular tubular structures of columnar epithelial cells with clear cytoplasm
and oval nuclei, continuous with morular structures; nuclei in morules are optically
clear and rich in biotin; fibrous stroma without atypia
Micro images: image1
Positive stains: chromogranin A, synaptophysin, N-CAM
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Negative stains: p53
DD: adenocarcinoma of fetal type (no morules)
Adenocarcinoma vs. mesothelioma
top
Difficult to differentiate histologically if adenocarcinoma does not produce mucin
Stains recommended to differentiate these 2 tumors: calretinin, cytokeratin 5/6 or
WT1 and CEA, MOC31 (or B72.3, Ber-EP4 or BG8), Am J Surg Pathol
2003;27:1031
Stains that are specific for adenocarcinoma vs. mesothelioma: MOC31 (100% vs.
8%), Ber-EP4 (100% vs. 18%), BG8 (96% vs. 7%), CEA (88% vs. 0%), B72.3 (84%
vs. 0%), TTF1 (74% vs. 0%), CD15/LeuM1 (72% vs. 0%)
Stains that are specific for mesothelioma vs. adenocarcinoma: calretinin (100% vs.
8%), cytokeratin 5/6 (100% vs. 2%), WT1 (93% vs. 0%), thrombomodulin (77% vs.
14%), N-cadherin (73% vs. ?)
Stains frequently positive in both tumors: EMA, E-cadherin, HBME, CD44S,
mesothelin, vimentin
EM: long, slender microvilli in mesothelioma
Adenoid cystic carcinoma
top
May arise from submucus bronchial glands
Usually in large bronchi, may involve the trachea
Frequent metastases to regional lymph nodes and lung parenchyma
Prolonged course, but overall prognosis is poor
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Treatment: radiation therapy (palliative)
Gross: large, polypoid, intrabronchial mass or may grow subepithelially along the
bronchi causing thickened bronchial wall
DD: basaloid carcinoma with adenoid cystic carcinoma-like pattern (microcystic
spaces containing mucin, surrounded by small tumor cells)
Adenosquamous carcinoma
top
Substantial amounts of squamous and glandular differentiation
Dont diagnose if one component is clearly minor
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More favorable prognosis: non-mucinous histology (hobnail cells, Clara cells),
localized disease
This diagnosis is usually limited to non-invasive tumors with lepidic spread (along
alveolar septa); if stromal, vascular or pleural invasion is seen, tumor is classified as
adenocarcinoma, mixed subtype
Gross: peripheral lung, one or more nodules (nodules may coalesce) or pneumonia-
like infiltrate
Gross images: multifocal tumor #1, #2
Micro: tall, columnar cells line up along alveolar septa, project into spaces with
papillary projections, but underlying lung architecture is preserved; variable anaplasia
but usually well differentiated
Composed of mucus-producing goblet cells, Clara cells or type II alveolar epithelial
cells
Mucinous type - composed of well differentiated columnar cells containing mucin
that line respiratory spaces; tumor cells are associated with bronchioles, not bronchi;
usually sharp demarcation between normal and tumor cells
Non-mucinous type - cuboidal cells with bright eosinophilic cytoplasm, prominent
nucleoli and nuclear atypia; apical spouts and hobnail cells often present; cilia are
rare; also PAS+ intranuclear inclusions; associated with interstitial fibrosis (may be
severe) and chronic inflammatory infiltrate; 13% have psammoma bodies
Micro images: noninvasive, nonmucinous (H&E, TTF1, CK7, CK20), mucinous
(H&E, TTF1, CK7, CK20), mixed mucinous and nonmucinous,
Virtual slides: tumor & nodal metastasis
Positive stains: alpha-1-antitrypsin for Clara cells in non-mucinous type, surfactant
for type 2 pneumocytes in non-mucinous type, TTF1 (80% of non-mucinous, 0% of
mucinous), CK7 (94-100%)
Negative stains: CK20 (but 25-90% of mucinous are CK20+)
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EM: mucinous - resemble bronchiolar goblet cells; non-mucinous - resemble Clara
cells or type 2 pneumocytes
DD: metastatic mucinous carcinoma
References: Mod Path 2002;15:538 (TTF1, CK7, CK20), Hum Path 2002;33:915
(CK7, CK20)
DD: metastatic adenocarcinoma
Epithelial-myoepithelial carcinoma
top
Arises from submucosal bronchial glands, mimics the similar salivary gland tumor
Very rare, < 10 cases reported through April 2003
Ages 40-69 years
Low grade malignancy; long interval to recurrence or metastasis
Case report of 73 year old man with bronchial tumor, Archives 2003;127:e177
Gross: intraluminal polypoid mass in bronchus; may invade pulmonary parenchyma
Gross/micro images: image1
Micro: well circumscribed mass, pushing margin; tumor consists of ductlike
structures with inner epithelial cells and outer clear myoepithelial cells; no
myxoid/chondroid stroma, no perineural invasion
Positive stains: epithelial cells - cytokeratin, EMA; myoepithelial cells - S100, muscle
specific actin
Negative stains: HMB45
DD: mucoepidermoid carcinoma, acinic cell carcinoma, pleomorphic adenoma,
adenoid cystic carcinoma with a tubular pattern, myoepithelioma, myoepithelial
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carcinoma, clear cell (sugar) tumor, metastatic epithelial-myoepithelial carcinoma
(usually parenchymal, not endobronchial), metastatic clear cell carcinoma
Giant cell carcinoma
top
Included in WHO classification of carcinomas with pleomorphic, sarcomatoid, or
sarcomatous elements
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Gross: usually peripheral lung, spherical tumor with well-defined borders and
bulging, fleshy, homogenous gray-white cut surface; no anthracosis; frequently
involves thoracic wall
Micro: large polygonal cells and anaplastic cells growing in solid nests without
obvious squamous or glandular differentiation; vesicular nuclei, prominent nucleoli,
moderately abundant cytoplasm, well defined cell borders
Virtual slides: anaplastic tumor cells
Positive stains: keratin5 (56%), calretinin (38%), thrombomodulin (25%), mesothelin
(13%), TTF1 (variable reports of staining)
EM: glandular (intracellular and extracellular lumina) and squamous (desmosomes,
tonofilaments) features often present although not obvious with H & E staining
Large cell neuroendocrine carcinoma
top
Diagnostic criteria (Rosai): resembles a non-small cell carcinoma, but on closer
inspection has a hint of neuroendocrine architecture confirmed by special stains
Micro: larger tumor cells than atypical carcinoid, high nuclear grade, increased
mitotic activity (11+ per 10 HPF) and necrosis; poorly developed neuroendocrine
architecture with some pallisading or rosette-like structures present
Positive stains: neuroendocrine markers, CD117 (60%), TTF1 (50%)
Lymphoepithelioma-like carcinoma
top
Rare, ~ 100 cases reported
In Taiwan study, most patients were women, Chinese, non-smokers, Am J Surg
Pathol 2002;26:715
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Case report of EBV+ tumor in 25 year old Italian man, Hum Path 2003;34:623
Otherwise rare in Caucasians, and usually EBV-
Tumor size may correlate with EBV serology titer
May have better prognosis than other nonsmall cell carcinomas of lung
Gross: well circumscribed nodules
Micro: syncytial growth of epithelial cells with large vesicular nuclei, prominent
eosinophilic nucleoli, accompanied by marked CD8+ lymphocytic infiltration;
predominantly pushing borders, permeative interface with adjacent lung; occasional
amyloid deposition
Positive stains: EBV, EBER-1, LMP (EBV latent membrane protein), bcl2, patchy
keratin
Negative stains: CD45/LCA (in tumor cells)
DD: metastatic nasopharyngeal carcinoma, lymphoma, inflammatory pseudotumor
Metastastic tumors to lung
top
Lung is a common site of metastases
Usually multiple, bilateral, sharply outlined, rapidly growing, more pleomorphic and
necrotic than lung primaries, negative for TTF1
Often multiple discrete nodules in periphery of lung; also lymphangitis carcinomatosa
(peribronchial and perivascular patterns via lymphatics); rarely intralymphatic
microscopic foci that may cause pulmonary hypertension
Treatment: excision of isolated nodules
Nodular metastases: breast, GI tract, kidney, sarcoma, melanoma
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Lymphangitis carcinomatosis: stomach, breast, choriocarcinoma, pancreas, prostate;
case report with hepatocellular carcinoma, Archives 2003;127:e11
Central cavitation: squamous cell carcinoma of upper aerodigestive tract, colonic
adenocarcinoma, leiomyosarcoma
Intrabronchial masses: breast, kidney, colon
Tumor emboli: breast, stomach, liver, choriocarcinoma
Lepidic pattern: colon, pancreas
Gross images: multiple tumor nodules #1, #2
Gross/micro images: hepatocellular carcinoma
Virtual slides: metastatic adenocarcinoma, metastatic small cell carcinoma
Positive stains: CDX2 (colorectal carcinoma, Am J Surg Pathol 2003;27:141)
Negative stains: surfactant apoprotein
Metastatic endometrial stromal sarcoma
top
Metastases to lung are rare, reports at Am J Surg Pathol 2002;26:440, Am J Surg
Pathol 2002;26:1142
Metastases occur a mean 10 years after diagnosis of uterine endometrial stromal
sarcoma
Excellent prognosis; patients only rarely die of disease
Note: uterine diagnosis often not disclosed to pathologist
Micro: well circumscribed, solid tumor composed of plump spindle cells in short
fascicles, often with hyalinized areas; may have cystic or sex cord-like areas
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Positive stains: vimentin, ER, PR, CD10; variable smooth muscle actin, desmin and
keratin
Negative stains: chromogranin, HMB45
DD: sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis,
endometriosis, hemangiopericytoma, lymphoma
Micropapillary adenocarcinoma
top
Associated with nodal metastases of similar histology, intrapulmonary metastases,
non-smokers
Poorer survival than non-papillary for stage I (79% vs. 93%), Am J Surg Pathol
2003;27:101, Am J Surg Pathol 2002;26:358
Case report of incidental brain metastases with micropapillary structures, Archives
2003;127:e313
Micro: small papillary tufts without a fibrovascular core; associated with varied other
histologic subtypes
Micro images: papillary and micropapillary structures
Positive stains: CK7 (93%), TTF1 (80%), CK20 (13%)
Mucoepidermoid carcinoma
top
May arise from submucus bronchial glands
Usually in large bronchi
May occur in children
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Usually considered to have low malignant potential with recurrence but only rarely
aggressive
Excellent prognosis after surgical removal
Gross: polypoid growth in major bronchi
Micro: low grade or high grade; mucus secreting cells, squamous cells, intermediate
type cells
DD: adenosquamous carcinoma arising from bronchial epithelium
Papillary carcinoma
top
Includes goblet cell (mucin producing) type, Clara cell type, type II pneumocyte type
and bronchial surface epithelial cell type with or without mucin production
Micro: papillary structures with true fibrovascular core should comprise at least 75%
of tumor
Pleomorphic carcinoma
top
Also called sarcomatoid carcinoma, spindle cell carcinoma, giant cell carcinoma,
pseudosarcoma, pulmonary blastoma, and carcinosarcoma
Included under recent WHO classification of carcinomas with pleomorphic,
sarcomatoid, or sarcomatous elements
90% men, 92% smokers
Diagnosis is based on histology, not immunostains
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Presumed epithelial origin, although epithelial and sarcomatous components express
common markers differently, Am J Surg Pathol 2003;27:1203
Nodal metastases common
Stage 1 tumors have same prognosis as other stage 1 non-small cell carcinomas;
pleomorphic tumors at higher stages may have worse prognosis by stage than other
non-small cell carcinomas, Am J Surg Pathol 2003;27:311
Giant cell tumors frequently metastasize to small intestine
Carcinosarcoma: carcinoma component is combined with a sarcoma, the latter
consisting of heterologous elements, such as malignant cartilage, bone, or muscle;
both components appear to have a common origin, Am J Surg Pathol 2002;26:510
Gross: 2-17 cm, necrosis and hemorrhage common
Micro: non small cell lung carcinoma with at least 10% neoplastic spindle or giant
cells, usually with epithelial cells; epithelial component 10-85%, usually
adenocarcinoma or large cell carcinoma, also squamous cell carcinoma; usually
poorly differentiated; spindle cells resemble MPNST, MFH or fibrosarcoma; giant
cells usually bizarre with multilobulated nuclei, abundant eosinophilic cytoplasm
accompanied by heavy neutrophilic infiltrate with occasional ingested white blood
cells; stroma often myxoid, frequent inflammatory infiltrate, collagen fibers;
numerous mitotic figures; necrosis common; vascular invasion in 58%
Positive stains: sarcomatoid component - CK7 (63%), TTF1 (43%), surfactant protein
A (6%); epithelial component - CK7 (76%), TTF1(59%), surfactant protein A (39%)
Negative stains: CK20
Molecular: extensive allelic loss in carcinosarcomas at 3p, 5q, 17p
Pulmonary blastoma
top
Also called embryoma
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Considered a subtype of pleomorphic carcinoma / carcinosarcoma
Usually in adults (mean age 43 years), 20% less than 20 years old
Metastases common; 2/3 die within 2 years
In infants/children, epithelial component is benign appearing or minimal; stroma may
be rhabdomyoblastic or chondroid; tumors may be cystic and may involve pleura and
lung
Case report of 16 year old white boy, Archives 2002;126:875
Gross: peripheral, solitary, well circumscribed, large
Micro: biphasic tumor in which epithelial and mesenchymal components have a
primitive, fetal-type appearance; well formed tubular glands surrounded by cellular
stroma of embryonal appearance; resembles Wilms tumor and fetal lung at 10-16
weeks; glandular cells are tall, columnar, often with clear cytoplasm and subnuclear
and supranuclear cytoplasmic vacuoles; morules with ground-glass nuclei are
common; stroma may differentiate towards striated muscle, smooth muscle, cartilage
Micro images: biphasic tumor
Positive stains: PAS (glycogen in epithelial cells)
Sebaceous carcinoma
top
Case report of 78 year old man with slow growing, endobronchial tumor, Am J Surg
Pathol 2002;26:795
Micro: sebaceous differentiation with lobulated and infolded architecture, light and
dark zones composed of basaloid cells with sharp cytoplasmic borders, vesicular
nuclei and cytoplasm varying from scant to abundant with small vesicles
Positive stains: oil red O (lipid)
Negative stains: PAS-diastase, mucicarmine
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DD: metastatic tumor from head and neck, mucoepidermoid carcinoma, squamous
cell carcinoma with sebaceous differentiation
Small cell carcinoma
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Also called undifferentiated carcinoma, oat cell carcinoma
10-20% all of lung carcinoma, 45,000 new cases per year in US
Usually men, median age 60 years, 99% occur in smokers, very aggressive with early
mediastinal lymph node involvement
Apparently derived from primitive cells of basal bronchial epithelium with partial
differentiation towards neuroendocrine cells
Associated with paraneoplastic syndromes due to production of ADH (hyponatremia),
ACTH (Cushings syndrome), parathyroid hormone (hyperparathyroidism), calcitonin
(hypocalcemia), gonadotropins (gynecomastia), serotonin (carcinoid syndrome),
encephalomyelitis, sensory neuropathy, Lambert-Eaton syndrome
Diagnosis is based on H & E staining, not the presence of neuroendocrine markers
Some pathologists report the presence of a large cell component or classify as
undifferentiated or squamous cell carcinoma of small cell type if small tumor cells
with hyperchromatic, coarsely granular or vesicular nuclei, small but distinct nucleoli,
scanty but identifiable cytoplasm, distinct cell border, primarily negative for
neuroendocrine stains
5 year survival (n=55): stage I - 33%, II - 44%, IIIA - 22%, IIIB - 0%, IV - 12%
Biopsies often crushed; cytology may be helpful
Flow cytometry: CD56+ (neural cell adhesion molecule), CD45-, which differentiates
from lymphoma, Archives 2003;127:461
Treatment: chemotherapy, radiation; cure rates of 15-25% for limited disease; most
live 1 year; preoperative chemotherapy and surgery if T1-2, N0-1, M0
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Poor prognostic factors: elevated serum LDH, alkaline phosphatase, albumin,
hemoglobin, white blood count
Gross: usually central/hilar; white-tan, soft, friable, extensive necrosis; peripheral
nodules have fairly well-defined border and fleshy cut surface
Gross images: central tumor #1, #2, spreading along bronchi
Micro: sheets, ribbons, clusters, rosettes or peripheral pallisading of small to medium
sized (2-4x neutrophils) round/oval cells with minimal cytoplasm, salt and pepper
chromatin without prominent clumps, hyperchromatic, indistinct nucleoli, nuclear
molding, smudging, frequent mitotic figures; Azzopardi phenomena (basophilic
nuclear chromatin spreading to wall of blood vessels), indistinct cell borders; stroma
is scanty, vascular, delicate; no glands, replacement of epithelium is less common
than subepithelial growth; necrosis and apoptotic debris are common
More cytoplasm is present in cells in metastases or resections than in small biopsies
May have larger cells with similar morphology, small mixtures of squamous cell
carcinoma or adenocarcinoma; rarely scattered giant cells, prominent nucleoli
Micro images: flow, H&E, stains (not necessarily lung), classic; H&E, CD117
Virtual slides: small cell carcinoma, primary tumor, bone marrow metastasis, liver
metastasis
Positive stains: pan-keratin (100%, dot like pattern), CK-BB (91%), TTF1 (89%),
histamine decarboxylase (78%, Mod Path 2003;16:72), neuron specific enolase
(77%), CD117 (75%, 50% after chemotherapy), chromogranin (58%, may be weak),
synaptophysin (57%), calretinin (49%), thrombomodulin (27%), keratin5 (27%),
CD57/Leu7 (variable), gastrin releasing peptide, N-CAM/CD56, bcl-2 (variable)
Negative stains: CD3, CD20, CD45, CD99/MIC2, pancreatic polypeptide, vimentin,
mesothelin, p63
EM: occasional round, membrane bound, dense core neurosecretory granules, 100-
200 nm in diameter; may have bundles of tonofibrils; may form glandular spaces
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DD: atypical carcinoid tumor (less nuclear atypia,
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Central cases appear to arise from bronchial epithelial dysplasia; peripheral cases
usually lack dysplasia
Peripheral cases with alveolar space filling pattern have better prognosis, no
lymphatic invasion or nodal metastases, Am J Surg Pathol 2003;27:978
Symptoms: bronchial obstruction (pneumonitis, atelectasis)
Cytology: often positive in sputum
5 year survival after resection: stage I-64%, stage II-48%, stage IIIA-28%, stage IIIB-
6%, stage IV-0%
Gross: usually central portion of lung affecting larger bronchi but may be peripheral;invades peribronchial soft tissue, lung parenchyma and nearby lymph nodes; may
compress pulmonary artery and vein; peripheral tumors often have nodular growth
with central necrosis and cavitation; surrounding lung may exhibit lipid pneumonia,
bronchopneumonia, atelectasis; calcification is unusual
Gross images: cavitating tumor #1, #2, tumor obstructing bronchus; endobronchial
tumor, tumor extending to pleura
Micro: sheets or islands of large polygonal malignant cells containing keratin
(individual cells or keratin pearls) and intercellular bridges; adjacent bronchial
dysplasia or carcinoma in situ is common; at advancing tumor border, tumor cells
usually destroy alveoli or fill alveolar spaces; rarely spread beneath basement
membrane; may have focal areas of intracytoplasmic mucin; rarely oncocytes, foreign
body giant cells (reacting to keratin), pallisading granulomas, extensive neutrophilic
infiltration, lepidic growth pattern at tumor periphery, clear cell change (glycogen);
Classify as well, moderately or poorly differentiated based on amount of
keratinization present in predominant component
Peripheral tumor types: alveolar space filling (tumor cells fill alveoli but dont destroy
elastic septa), expanding type (growth destroys elastic septa) or mixture
Variants: small cell (small tumor cells with focal keratinization, distinct nucleoli,
sharply outlined tumor nests, less necrosis than small cell carcinoma), clear cell
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(numerous clear tumor cells containing glycogen), well-differentiated papillary,
basaloid (see below), spindle cell (see below)
Micro images: moderately differentiated, well differentiated, with keratin pearls
Micro images (Mod Path subscribes): cytology
Virtual slides: bronchial tumor, esophageal metastasis; keratinizing-moderately
differentiated
Positive stains: keratin, keratin5 (87-100%), EMA, thrombomodulin (87-100%),
S100, CD15, CEA, p53, p63, HPV, mesothelin (16-31%)
Negative stains: vimentin (usually), TTF1 (usually)
EM: abundant tonofilaments, complex desmosomes, basal lamina
DD: squamous metaplasia with atypia (Hum Path 2002;33:1052)
References: Hum Path 2002;33:921 (p63)
Sources for IHC testing: Clarient (Pulmotype testing)
Early lung carcinoma of hilar type
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Arises proximal to sub segmental bronchi (i.e. major bronchi), confined to bronchial
wall with no lymph node metastases
Are usually squamous cell carcinomas
May be polypoid, nodular, superficially infiltrating or mixed
Longitudinal mucosal folds show changes at tumor border; superficial tumor has
thickened and fused folds
Five year survival is 90% or more if no second squamous cell carcinoma present
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Early squamous cell carcinoma of peripheral type
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Defined as tumor 2 cm or less in peripheral lung with no lymph node or distal
metastases
Only rarely identified in practice, since these tumors grow rapidly
Often have glandular cell characteristics
Basaloid squamous cell carcinoma
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Very aggressive clinical course
Spindle cell squamous cell carcinoma
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Also called sarcomatoid carcinoma
Carcinoid and related tumors
Carcinoid tumor
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Also called well-differentiated neuroendocrine carcinoma
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Occasionally occurs as part of MEN syndrome
May infiltrate or spread to local lymph nodes, but doesnt affect prognosis
Rarely produces carcinoid syndrome (flushing, diarrhea, cyanosis)
10 year survival is 50%
In children, involve lung or liver, may metastasize regardless of histology or
classification as carcinoid vs. neuroendocrine carcinoma, Archives 2003;127:1200
Gross: either central (polypoid and endobronchial in major bronchi) or peripheral
(solid/nodular); usually well defined, smooth, ivory to pink cut surface, no necrosis
Micro: nests or trabeculae of medium sized polygonal cells of low nuclear grade,
round to oval finely granular nuclei and lightly eosinophilic cytoplasm, may have
rosettes or small acinar structures with variable mucin; scanty vascular stroma,
occasionally amyloid stroma with bone; no/minimal mitotic activity or necrosis
Micro images: B-carcinoid, A-carcinoid
Positive stains: TTF-1 (43-53%, Appl Immunohistochem Mol Morphol 2007;15:407,
AJCP 2005;123:394)
Central carcinoid tumor
Most common type, usually slow growing, associated with obstruction, infection,
hemorrhage
Usually adults, but also most common lung tumor of children
5% metastasize, usually to regional lymph nodes; rarely distant osteoblastic
metastases to bone
10 year survival 70%
Cytology often negative since tumor is covered by mucosa
Treatment: surgical resection
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Gross: solitary, intrabronchial polypoid mass that may infiltrate bronchial wall,
covered by intact mucosa; gray-yellow cut surface, cartilage may be present
Gross images: bronchial carcinoid
Micro: nests or cords of uniform, bland cells with central nuclei and moderate
granular cytoplasm, prominent vasculature, stroma may be delicate fibrovascular,
hyalinized or exhibit calcification; angiolymphatic invasion common; rarely mitotic
figures, rosettes or papillary architecture, endocrine atypia, melanin granules
May have paraganglioma appearance with S100+ sustentacular cells
May have oncocytic features (abundant granular eosinophilic cytoplasm with
numerous mitochondria by EM)
Virtual slides: bronchial carcinoid
Positive stains: keratin, serotonin, neuron-specific enolase, chromogranin A and B,
synaptophysin, CD57/Leu7, pancreatic polypeptide, N-CAM
Negative stains: mucin (except in glandular lumina), TTF1 (usually, Hum Path
2004;35:825)
Molecular: 1/3 are aneuploid, which doesnt appear to affect prognosis
EM: dense core secretory granules that vary in shape and size
DD: small cell carcinoma (if extensively crushed)
Peripheral carcinoid tumor
Arise in peripheral lung, often beneath the pleura
Usually asymptomatic and incidental
Excellent prognosis
Rare nodal metastases are usually cured by excision
Treatment: lobectomy (since multiple tumors are common)
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Gross: multiple, nonencapsulated, gray-tan nodules, bulging, brown-tan cut surface,
not associated with a bronchus
Micro: disorderly spindle cells resembling smooth muscle; moderate pleomorphism
and mitotic activity; prominent stroma; amyloid and melanin often present
Positive stains: Congo Red (amyloid), TTF-1 (usually), calcitonin (often)
Atypical carcinoid tumor
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Also called moderately differentiated neuroendocrine carcinoma
Diagnostic criteria (Rosai): resembles a carcinoid but with atypical features
More aggressive than typical carcinoid tumors: nodal metastases in 70% vs. 5%
5 year survival is 49-69%
Micro: carcinoid tumors with increased mitotic activity (2-10 per 10 HPF), nuclear
pleomorphism or foci of necrosis
Positive stains: more intense neuroendocrine staining than small cell carcinoma; also
positive for pancreatic polypeptide
EM: numerous large neurosecretory granules
Tumorlet
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Nodular proliferation of small spindle cells near bronchioles
May be associated with bronchiectasis or other causes of scarring
Almost always benign
By definition 5 mm or less
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Positive stains: same as carcinoid
EM: same as carcinoid
Lymphoma and lymphoid infiltrates
BALT lymphoma
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Lymphoma of Bronchus Associated Lymphoid Tissue
Rare type of low-grade, primary pulmonary MALT lymphoma
Often need gene rearrangement studies to diagnose
Indolent with good prognosis
Case report of 41 year old woman with clonality by gene rearrangement but not by
flow cytometry, Archives 2003;127:115
Micro: centrocyte-like lymphocytic proliferation in bronchiolar submucosa with
lymphoepithelial lesions, reactive lymphocytes and plasma cells; bland appearance
Micro images: H&E with lymphoepithelial lesions
DD: reactive hyperplasia
Burkitts lymphoma
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Case report of 33 year old man with cystic fibrosis and Burkitts lymphoma after
double lung transplant, Am J Surg Pathol 2003;27:818
Diffuse large B cell lymphoma
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Presents as large parenchymal mass accompanied by necrosis, occasionally by
intrabronchial mass
May evolve from MALT lymphoma or arise without preexisting lesions
5 year survival is 50%
Micro: pleomorphic large lymphoid cells, usually noncleaved
Positive stains: CD20
Negative stains: CD3, CD5, CD26
Follicular bronchitis / bronchiolitis
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Associated with immunodeficiency (congenital, AIDS), rheumatoid arthritis and
Sjogrens syndrome
Mild shortness of breath
Treatment: steroids, uncertain if chemotherapy if helpful
Micro: lymphoid follicles and plasma cells around distal bronchi and bronchioles that
infiltrate fibromuscular wall and may compress the lumen; alveoli relatively
unaffected
Hodgkins lymphoma
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Very rare (
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Case report of localized pulmonary consolidation in 21 year old woman, Archives
2003;127:e49
Usually good prognosis, but fatal cases occur
Gross: nodular or multinodular lesions in upper lung
Micro: typical Reed-Sternberg cells, mononuclear cells, lacunar variants in
background of mixed inflammatory infiltrate; rarely granulomatous inflammation
simulating tuberculosis
Micro images: 3A: CD15; 3B: CD30
Positive stains: CD15, CD30
Negative stains (Reed-Sternberg like cells): CD45, CD20, CD3, EMA
DD: tuberculosis, Wegeners granulomatosis, organizing pneumonia, T cell
lymphoma, anaplastic lymphoma
Lymphomatoid granulomatosis
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Also called angioimmunoproliferative lesion
Lymphoproliferative disorder that is malignant per se or likely to become malignant,
resembles post-transplant lymphoproliferative disorders
Middle aged men and women with well defined, bilateral rounded mass densities on
chest Xray
Associated with transplant-related immunosuppression, Sjogrens syndrome, HIV
80% of cases have extrapulmonary involvement (skin, CNS, kidneys, liver, spleen,
adrenal glands, heart, GI tract)
Rarely, there is only extrapulmonary involvement
Usually no hilar or mediastinal lymph node involvement
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May be an EBV related, B cell proliferation; T cells present are usually reactive
May have reversal of T helper / suppressor ratio
Large number of atypical lymphoid cells is a poor prognostic factor
Median survival 14 months; death due to sepsis, destruction of lung tissue; similar
infiltrates found in other organs
Treatment: chemotherapy
Micro: nodular inflammatory infiltrate of large atypical lymphoid cells (prominent
nuclei, mitotic activity), plasma cells, immunoblasts, involving walls of pulmonary
vessels (angioinvasive, angiocentric, angiodestructive); no multinucleated giant cells
Positive stains: EBV (50-70%)
DD: Wegeners granulomatosis (giant cells, necrosis)
MALT lymphoma
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Lymphoma of Mucosal Associated Lymphoid Tissue - a type of marginal zone
lymphoma
Also called lymphoma of bronchial associated lymphoid tissue (BALT)
Called pseudolymphoma in older literature
Etiology: in pediatric HIV patients with lymphoid interstitial pneumonitis,chemokines and cytokines may recruit inflammatory cells, either representing an early
stage of MALT or providing a microenvironment for the evolution of a monoclonal
B-cell population (Mod Path 2001;14:929)
In adults, lung MALT is usually low-grade, median age 68 years (range 34-88), often
associated with autoimmune disorders, monoclonal gammopathies, hepatitis C,
Helicobacter pylori gastritis; 44% involve mediastinal nodes
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Usually indolent with excellent prognosis; may recur locally, rarely transforms, few
die of disease
20% have monoclonal gammopathy, 30% have pleural effusions
Flow cytometry of tumor cells is useful in phenotyping; may even be helpful on
peripheral blood
Case reports: 41 year old woman with multiple lung nodules, negative flow cytometry
but clonal IgH gene rearrangement (Arch Pathol Lab Med 2003;127:115)
Treatment: local resection for limited disease, chemoradiotherapy for advanced
disease
Gross: solitary discrete mass, occasionally multiple nodules
Gross images: contributed by anonymous - discrete tan mass #1; #2; #3
Micro: nodular pattern of monotonous, mature lymphocytes with germinal centers
that infiltrate overlying epithelium (lymphoepithelial lesions) and around vessels,
pleura and alveolar septa; cells may be monocytoid or resemble centrocytes; also
reactive plasma cells, variable fibrosis and epithelioid granulomas; also colonization
of germinal centers by tumor cells; rarely granulomatous vasculitis
Micro images: contributed by anonymous - monotonous population of small-
intermediate cells; lymphoepithelial lesion
Figure 1-CT with multiple pulmonary nodules; 2A-lymphocytic proliferations with
some germinal centers; 2B-lymphoepithelial lesions associated with bronchial mucosa
Positive stains: CD20, CD43
Negative stains: CD5, CD10, CD23
DD: benign lymphoid aggregates / reactive disorder: architecture is preserved,
associated with immunosuppression and collagen vascular disease; not monoclonal by
flow cytometry or PCR
References: Am J Surg Pathol 2001;25:997, Am J Surg Pathol 2002;26:76
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Nasal type NK-T cell lymphoma
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Case report of orbital tumor with lung metastases, Hum Path 2003;34:290
Plasmacytoma
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Associated with nodal or bone involvement and myeloma
Micro: mature and atypical plasma cells
SLL/CLL
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Small lymphoblastic lymphoma / chronic lymphocytic leukemia
Usually asymptomatic pulmonary nodules or infiltrates in elderly
Involves the lung at autopsy in 30% of cases, may cause significant pulmonary
impairment
Gross: well defined, encapsulated mass, homogenous gray cut surface
Micro: monomorphic infiltrate with pseudofollicles
DD: reactive lymphoid infiltrate (polymorphic lymphocytes, germinal centers, otherinflammatory cells)
Waldenstrm macroglobulinemia
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B cell neoplasm (usually lymphoplasmacytic lymphoma) plus serum IgM monoclonal
paraprotein
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Usually elderly patients with indolent clinical course
Median survival 5 years
Involves bone marrow (100%), liver, spleen, lymph nodes (15-20%); also
hyperviscosity syndrome in 15%
Lung an unusual site, Am J Surg Pathol 2003;27:1104.
Diagnosis: serum protein electrophoresis
Other malignancies
Angiosarcoma
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Presents as mass or diffuse pulmonary infiltrates
DD: metastatic tumor
Desmoplastic small round cell tumor
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Case report in 22 year old man, Archives 2002;126:1226
Micro: nests of small round tumor cells within a cellular and vascular collagenous
stroma
Micro images: H&E, stains
Positive stains: AE1/AE3, EMA, CAM5.2, vimentin, desmin, neuron-specific
enolase, WT1
Negative stains: thrombomodulin, calretinin
Molecular: t(11;22)(p13;q12) [EWS-WT1 gene fusion]
Molecular images: RT-PCR
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EM: intracytoplasmic whorls of intermediate filaments
EM images: cytoplasmic perinuclear aggregates of intermediate filaments
Epithelioid hemangioendothelioma
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Formerly called intravascular bronchioloalveolar tumor
80% women, usually young adults
Neoplastic, but usually not metastatic
Progressive growth, usually remains within thoracic cavity, may cause death from
respiratory insufficiency
Other sites: liver, bone
10% have peripheral eosinophilia
Poor prognosis if vascular spread, pleural involvement, severe symptoms
Case history: Archives 2003;127:e319
Gross: multiple round, well demarcated nodules < 2 cm, often in lower lung, with a
gray-white peripheral rim; may spread along pleura or pericardium and resemble
mesothelioma
Micro: central hyalinized stroma, eosinophilic amorphous material or coagulative
necrosis with variable calcification surrounded by thin rim of plump eosinophilicendothelial cells; clusters fill alveoli, apparently through pores of Kohn, and
occasionally bronchioles, arteries, veins; nuclei are bland, round/oval, may have
cytoplasmic vacuoles; no/minimal mitoses; lung architecture preserved
Gross/micro images: H&E, D-Factor VIII
Positive stains: Factor VIII, CD31, CD34; variable ER and PR
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EM: endothelial features - well developed basal lamina, pinocytotic vesicles,
occasional Weibel-Palade bodies
DD: metastatic tumor from liver or other sites (destroy lung architecture, high mitotic
rate), sclerosing hemangioma (destroys lung architecture, negative vascular markers)
Hemangiopericytoma
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Rare, often a misdiagnosis
Micro: blunt spindle cells with scant cytoplasm, separated by arborizing vascular
spaces arranged in a stag-horn pattern
DD: metastatic endometrial stromal sarcoma
Histiocytic-dendritic neoplasm
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Case report (letter), Am J Surg Pathol 2002;26:1373
HIV associated polymorphic lymphoproliferative disorders
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Resemble posttransplant lymphoproliferative disorders in solid organ transplant
recipients, Am J Surg Pathol 2003;27:293
Kaposis sarcoma
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Usually associated with AIDS
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Tumor follows lymphatic channels
Leiomyosarcoma
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Usually is metastatic, not primary
Melanoma
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Rare, must rule out metastatic disease
Rhabdomyosarcoma
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Pleomorphic subtype more common in adults, embryonic subtype more common in
children
Synovial sarcoma
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Rare
Case report with SYT-SSX2 and rapidly progressive course, Archives 2003;127:e201
Case report of poorly differentiated tumor with rhabdoid features, Archives
2003;127:e160
Associated with chest pain, hemoptysis, dyspnea, cough, fever
40-55% die of disease after 20 years follow-up, due to metastases to liver, CNS, bone
or invasion of adjacent organs
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Tumors with SYT-SSX2 fusion proteins have better prognosis with 89% 5 year
survival vs. 42% for SYT-SSX1 fusion protein
Micro: monophasic spindle cells or biphasic with epithelial and spindle cell
component; monophasic tumors have compact fascicles of hyperchromatic spindle
cells with hemangiopericytoma-like areas; often punctuated by small arteries and
capillaries in an irregular distribution; poorly differentiated tumors have small, round
blue cells resembling Ewings sarcoma/PNET; rarely has rhabdoid features
Micro images: H&E, bcl2, CD99, CD117, poorly differentiated tumor: H&E and
vimentin
Positive stains: CD117 (in dispute)
Molecular: t(X;18)(p11.2;icq11.2); produces either SYT-SSX1 or SYT-SSX2 fusion
genes
DD: monophasic tumor resembles fibrosarcoma, hemangiosarcoma, leiomyosarcoma,
spindle cell carcinoma, carcinosarcoma or sarcomatoid carcinoma (primary or
metastatic)
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