Alveolar adenoma

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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 c

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