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Lung tumor

Carcinoid and related tumors

Carcinoid tumor


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 15 March 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

See subtypes below: central, peripheral

General
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● World Health Organization classification recognizes 4 histologic subtypes of pulmonary neuroendocrine carcinomas (NECs), which include typical carcinoids (TCs), atypical carcinoids (ACs), small cell carcinomas (SCCs), and large cell NECs (LCNECs)
● Also called well-differentiated neuroendocrine carcinoma
● <5% of primary lung tumors
● Locally invasive, rarely metastasizes
● Usually age <40 years old
● No gender predilection, not related to smoking
● Occasionally occurs as part of MEN syndrome
● May infiltrate or spread to local lymph nodes, but doesn’t affect prognosis
● Rarely produces carcinoid syndrome (flushing, diarrhea, cyanosis)
● 10 year survival is 50%
● In children, involves lung or liver, may metastasize regardless of histology (Arch Pathol Lab Med 2003;127:1200)

Gross description
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● Either central (polypoid and endobronchial in major bronchi) or peripheral (solid/nodular)
● Usually well defined, smooth, ivory to pink cut surface, no necrosis

Micro description
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● Nests or trabeculae of medium sized polygonal cells with lightly eosinophilic cytoplasm, low nuclear grade, round to oval finely granular nuclei; may have rosettes or small acinar structures with variable mucin
● Scanty vascular stroma, occasionally amyloid stroma with bone
● No / minimal mitotic activity (<2/10HPF), no necrosis

Micro images
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Left: figure B; right: figure A

Positive stains
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● TTF1 (43-53%, Appl Immunohistochem Mol Morphol 2007;15:407, Am J Clin Pathol 2005;123:394)
● Secretin receptor overexpression (Mod Pathol 2008;21:387)


Central carcinoid tumor

General
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● 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

Radiologic images
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Case of the Week #266

Case reports
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● 30 year old woman with hemoptysis and fever (Case of the Week #266)

Treatment
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● Surgical resection

Gross description
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● Solitary, intrabronchial polypoid mass that may infiltrate bronchial wall, covered by intact mucosa
● Gray-yellow cut surface, cartilage may be present

Micro description
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● 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
● Sox10+ sustentacular cells observed in carcinoid tumours but not in high-grade NECs (Histopathology 2011;58:276)
● May have oncocytic features (abundant granular eosinophilic cytoplasm with numerous mitochondria by EM, Hum Pathol 2011;42:578)

Micro images
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Case of the Week #266:

AE1-AE3
Chromogranin
Synaptophysin
NSE

Virtual slides
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Bronchial carcinoid

Positive stains
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● Keratin, serotonin, neuron-specific enolase, chromogranin A and B, synaptophysin, CD57/Leu7, pancreatic polypeptide, N-CAM

Negative stains
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● Mucin (except in glandular lumina), TTF1 (usually, Hum Pathol 2004;35:825)

Molecular description
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● 1/3 are aneuploid, which doesn’t appear to affect prognosis

Electron microscopy description
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● Dense core secretory granules that vary in shape and size

Differential diagnosis
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Small cell neuroendocrine carcinoma: may to difficult to distinguish if extensively crushed, but PAX5 discriminates low/intermediate grade from high-grade neuroendocrine carcinomas (Am J Clin Pathol 2008;129:556)


Peripheral carcinoid tumor

General
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● Arise in peripheral lung, often beneath the pleura
● Usually asymptomatic and incidental
● Excellent prognosis
● Rare nodal metastases are usually cured by excision

Treatment
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● Lobectomy (since multiple tumors are common)

Gross description
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● Multiple, nonencapsulated, gray-tan nodules, bulging, brown-tan cut surface, not associated with a bronchus

Micro description
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● Disorderly spindle cells resembling smooth muscle
● Moderate pleomorphism and mitotic activity
● Prominent stroma
● Amyloid and melanin often present (Histopathology 2011;59:526)

Positive stains
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● Congo Red (amyloid), TTF1 (usually), calcitonin (often)

End of Lung tumor > Carcinoid and related tumors > Carcinoid tumor


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