Mediastinal and Other Neoplasms (Part 2,

Other Lung Neoplasms)

Jay H. Ryu, MD
Mayo Clinic
Rochester, MN
Conflict of Interest Disclosure
• None
Learning Objectives
(Part 2, Other Lung Neoplasms)

At the conclusion of this presentation, you will be able to:

• Recognize the characteristic presentation of several uncommon
intrathoracic tumors.
• Describe diagnostic approach and management strategies
applied to uncommon intrathoracic tumors.
Outline: Uncommon Lung Neoplasms
• Lung neuroendocrine tumor (NET; carcinoid)
• Salivary gland type carcinomas
• Hamartoma
• MALToma
• Plasmacytoma
• Sarcoma
• Metastatic
2021 WHO Classification of Lung Tumors
1. Epithelial – adenocarcinoma, squamous cell Ca, salivary-gland type
(mucoepidermoid, adenoid cystic), etc.
2. Lung neuroendocrine (NE) neoplasms – NE tumors (carcinoid), NE
carcinomas (small cell, large cell NE), etc.
3. Tumors of ectopic tissues
4. Mesenchymal tumors – hamartoma, chondroma, *diffuse pulmonary
lymphangiomatosis, sarcomas, etc.
5. Hematolymphoid tumors – lymphomas, Langerhans cell
histiocytosis, *Erdheim-Chester, etc.
(*covered in my “Less Common Pulmonary Diseases” talk)
(Nicholson et al. J Thorac Oncol 2022)
Lung NET (carcinoid, typical/atypical)
• Malignant with neuroendocrine differentiation; 2% of all thoracic tumors
but 2nd most common; M = F.
• No association with smoking.
• Sometimes associated with MEN 1 or familial.
• Histologically subclassified: low-, intermediate-grade (higher mitotic
activity and necrosis); high-grade = NE carcinomas.
• Low-grade (typical carcinoid) lung NET (80-90%) - indolent behavior,
nodal spread uncommon; 5-year survival 85-100%.
• Intermediate-grade (atypical carcinoid) lung NET - aggressive,
metastasis to mediastinal nodes in 20-60%; 5-year survival 30-95%.
• High-grade / NE carcinomas: small cell and large cell lung cancer.
Lung NET (carcinoid)
• Low-grade lung NET (typical carcinoid) usually 5th decade; older for
intermediate-grade NET (atypical carcinoid).
• 2/3 in major or lobar bronchi.
• Symptoms from bronchial obstruction (recurrent pneumonias or wheezing)
or vascularity (hemoptysis)
• Occasionally carcinoid or Cushing's syndrome, acromegaly.
• Generally attached to the bronchus by broad base; can be polypoid,
create ball-valve.
CXR, CT: obstructive pneumonia, discrete hilar mass, or peripheral nodule.
Other imaging in selected cases: MRI, gallium Ga-68 DOTATATE or
OctreoScan (somatostatin receptor-based). Low uptake on FDG-PET.
Lung NET, low-grade (typical carcinoid)
67M nonsmoker - hemoptysis
Lung NET (carcinoid)
Management
• Localized lung NET - surgical resection of the tumor with mediastinal
lymphadenectomy.
• Endobronchial laser, if poor surgical candidate.
• Unresectable lung NET – RT ± chemo, ± endobronchial.
• Metastatic lung NET tumors - SSAs, everolimus, cytotoxic
chemotherapy, radiolabeled SSA.
• Control of liver metastases (hepatic-predominant disease) by
surgical resection, embolization therapy, radiofrequency ablation,
or cryoablation.
Prognosis
• 5-yr survival: low-grade 85-100%, intermediate 30-95%.
Salivary Gland Type Carcinomas
• Arise from minor salivary glands in upper aerodigestive tract.
• Account for 0.1 - 0.2% of all thoracic tumors.
• Most common forms in thorax: mucoepidermoid carcinoma and
adenoid cystic carcinoma (ACC).
• Low-grade malignancies; lymph node spread rare to uncommon.
• ACC - “iceberg” lesion.
• No association with smoking.
• Symptoms: cough, hemoptysis, wheezing (asthma-like), or
obstructive pneumonia.
• Imaging: endobronchial mass in trachea or major bronchus.
• Variable FDG-avidity on PET.
 Salivary Gland Type Carcinomas
Mucoepidermoid Carcinoma
• Wide age range, children to
elderly (median 40 years).
• Histologic: 3 components:
mucus-secreting, squamous,
and intermediate cells.
• Characterized by
t(11;19)(q21;p13) chromosomal
translocation.
Management: surgical resection.
Prognosis: excellent if successful
resection; 10-year 87%.
Adenoid Cystic Carcinoma
• Adults (commonly 5th decade).
• Histologic: mixed growth
pattern, perineural invasion.
Management: surgical resection
• Propensity to recur locally,
metastasize; late recurrence
(15-20 years).
• Radiation and palliative laser
therapy for unresectable
tumors; chemo, TKIs.
Prognosis: median survival after
resection ~6 yr.
 Salivary Gland Type Carcinomas
Mucoepidermoid Carcinoma Adenoid Cystic Carcinoma

67M nonsmoker

44F nonsmoker, cough/wheeze x 10 mo

10X

200X
Adenoid Cystic Carcinoma
67M ex-smoker COPD (FEV1 43% pred), chr cough x 4 mo.

17 mo before diagnosis
 Adenoid Cystic Carcinoma
31F
“asthma” x 2 yr
Obstructive lung diseases
• COPD
• Asthma
• Bronchiectasis
• Upper / large airway
lesions
• Some ILDs – LAM,
PLCH, sarcoid
• Some bronchiolitis
• Miscellaneous
Hamartoma
• Most common benign neoplasm in lung (75% of benign lung tumors).
• 4% of SPNs.
• Benign tumor-like malformation - abnormal mixture of tissue elements
(cartilage, connective tissue, smooth muscle, fat, and respiratory
epithelium).
• All patient age groups
• Highest incidence in 4th - 7th decades.
• Asymptomatic
• Grow slowly, if at all.
• Diagnostic confirmation, if needed, by TTNA, bronch or VATS.
Hamartoma
CXR, CT: peripheral, solitary, round
or lobulated opacity; smooth
margins; generally 1-3 cm in size.
• Calcification in ~25%, small
flecks throughout the lesion
(“popcorn” calcification
infrequent).
• Areas of fat density by CT in
~50%.
Fat-Containing Lesions in Adult Thorax

(Molinari et al.
AJR 2011)
Pulmonary MALToma
• Primary pulmonary lymphomas - 0.5 - 1.0% of lung tumors.
• Most common type: extranodal marginal zone lymphoma of mucosa-
associated lymphoid tissue (MALT), ie, MALT lymphoma or MALToma.
• Adults, median age 50-60 yr.
• Most asymptomatic (low-grade, indolent tumor).
• Association with autoimmune diseases, esp., Sjögren's.
• Treatment: observation, rituximab.
• Median survival >10 yr; sometimes dissemination, transformation.
 Pulmonary MALToma
41M incidental nodule

Imaging: focal consolidative, mass-like, often multiple;

sometimes GGOs, cysts.
Plasmacytoma
66F back pain x 4 mo
• Plasma cell neoplasm can present as a
single lesion in bone, or outside bone
(solitary extramedullary – 3% of plasms cell
malignancies).
• Can present in lung as nodule or hilar
mass.
Management: radiation therapy ± surgery.

Prognosis: 10-15% develop multiple

myeloma.
Pulmonary Sarcoma
• Sarcomas comprise <1% of adult malignancies.
• Originate from soft tissue (80%) or bone.
• Sarcoma encountered in lung is more often metastatic than primary.
• Broad histopathologic spectrum pulmonary sarcomatoid tumors:
sarcomatoid carcinoma, sarcomatoid malignant mesothelioma,
leiomyosarcoma, synovial sarcoma, malignant peripheral nerve
sheath tumors, malignant solitary fibrous tumor, epithelioid
hemangioendothelioma, angiosarcoma, Kaposi sarcoma,
inflammatory myofibroblastic tumor, several others.
Pulmonary Artery Sarcoma
39F nonsmoker, DOE x 8 wk
Metastatic Tumors in Lung
• Lungs - 2nd most frequent site of metastases: 20 - 54% at autopsy.
• Most common 1° neoplasms: breast, colon, kidney, uterus, head/neck.
• Patterns of metastases
• Hematogenous – nodules (multiple or single)
• Endobronchial
• Endovascular – tumor emboli
• Lymphangitic
• Pleural – nodular thickening, effusion
Management: palliative, stereotactic radiotherapy, radiofrequency ablation,
cryotherapy, systemic chemotherapy, metastasectomy.
Criteria for pulmonary metastasectomy
• Adequate cardiopulmonary reserve.
• Primary tumor controlled; metastases completely resectable (no
extrathoracic disease) – preferably solitary met.
Lymphangitic Carcinomatosis
53M Cough, DOE x 6 wks

CT: interlobular septal thickening,

beaded/nodular.
Summary
(Part 2, Other Lung Neoplasms)

• Uncommon intrathoracic tumors such as lung NET (carcinoid),

salivary gland type neoplasms, and hamartoma are often
associated with characteristic presenting features, clinical context,
imaging findings.
• Management is guided by type of tumor, extent of disease, clinical
context, and individual patient preferences.