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Pulmonary hamartoma

A pulmonary hamartoma is a benign neoplasm composed of cartilage, connective tissue, muscle, fat, and bone. It is one of the commonest benign tumours of the lung 11 and accounts for approximately 8 % of all lung neoplasms 5 and 6 % of solitary pulmonary nodules 6.

Epidemiology 

Patients usually present in the 4th and 5th decades of life. There is a recognised male predilection ( M:F being ~ 2-3:1). Most lesions are diagnosed incidentally.

Clinical presentation

Pulmonary hamartomas are usually asymptomatic and found incidentally when imaging the chest for other reasons. It can occasionally present with haemoptysis, bronchial obstruction and cough (especially endobronchial types) 6.

Pathology

Hamartomas may be chondromatous or leiomyomatous (the former being more common) or a mixture. They are unencapsulated, lobulated tumours with connective tissue septa.

As with any hamartoma it is composed of tissues that normally constitute in the lung and bronchi. The tissue elements, although mature, are disorganized. On rare occasion, they contain principally fatty tissue, in which case they are called lipomatous hamartomas or endobronchial lipomas. 

Location

The vast majority of pulmonary hamartomas are located peripherally within the lungs (> 90%), with endobronchial representing only 1.4-10% of such lesions 10-11.

Typically, hamartomas manifest incidentally as solitary nodules in the periphery of the lung.

Variants
Associations

An unusual association is with Gastrointestinal stromal tumours (GIST) and extra-adrenal paragangliomas. This is knowns as the Carney triad

Radiographic features

Often discovered incidentally. They are typically well-circumscribed nodules or masses (usually small) with either smooth or lobulated margins. Approximately 60% have fat 7 and approximately 20-30% have calcification / ossification (pop-corn like) 3. Cavitation is not seen.

Size is variable, and they can be large (>10cm) but in most cases they are less than 2.5 - 4 cm in diameter 10,13.

Growth occurs but is very slow, with a typical volume doubling time (VDT) of over 400 days 9.

Plain film

Chest radiography is non-specific, demonstrating a soft tissue attenuation, well-circumscribed mass with either smooth or lobulated margins. Calcification (classically popcorn type) may be seen, which can suggest the diagnosis. Fat is difficult to identify with certainty, although the lesion is typically of low density for its size.

CT

CT is far superior in detecting intra-lesional fat and calcification. The reported prevalence of calcification in hamartomas on CT varies from 5 to 50% while Fat may be identified in up to 50% of hamartomas at CT. The fat compoments may be localized or generalized within the nodule.

Fat can be recognised by comparing it to subcutaneous fat, and will typically have a Hounsfield measurement of -40 to -120HU 10. Presence of fat in a well circumscribed solitary pulmonary nodule which does not demonstrate significant growth is essentially pathognomonic of a pulmonary hamartoma and no further investigations are required 7. Unfortunately fat can only be identified in 60% of lesions. Thin section CT is therefore essential to avoid missing small foci of fat.

Calcification is typically dispersed in the form of multiple clumps throughout the lesion in a popcorn configuration 3.

FDG-PET

Although uncommon, increased avidity may be seen on FDG-PET 7. Thus, further investigation of slow-growing, fat-containing masses is unwarranted, confusing, and contributes to patient dose.

MRI
  • T1 - heterogeneous signal
    • mainly intermediate signal 8
    • foci of high signal represent fat
    • low signal regions representing fibrous or calcific material
  • T2 -
    • high signal due to fat and cartilaginous components
    • low signal regions representing fibrous or calcific material

  • T1 C+ (Gd) - heterogeneous enhancement is seen

Treatment and prognosis

Malignant transformation is exceedingly rare, and a peripheral small hamartoma with no atypical features can be safely left alone, with infrequent follow-up to exclude growth.

In atypical cases, or in cases of endobronchial hamartomas causing distal complications, surgical resection is curative. Rigid transbronchial resection is most frequently employed in the later 12

Differential diagnosis

The differential is somewhat dependent on whether fat or calcification is identifiable within the lesion.

If fat is visualised then the differential is narrow, with almost all cases representing pulmonary hamartoma. See - differential of fat containing solitary pulmonary nodule.

Presence of calcification also significantly narrows the differential, but to a lesser degree. See differential of a solitary pulmonary nodule with calcification.

If neither fat nor calcification is present, then the differential is that of a solitary pulmonary nodule and is significantly broader. See - differential of a solitary pulmonary nodule.

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