Lung hamartoma is the most frequent benign pulmonary tumor and is composed of several types of tissue: bone, cartilage, connective tissue, fat and muscle. This disease usually presents as a solitary nodule and necessitates thorough differential diagnosis.
Presentation
Lung hamartomas are usually an incidental radiological finding, as they cause no symptoms. However, some patients do have complaints related to the tumor, such as chest pain [1], hemoptysis, cough, wheezing and dyspnea caused by bronchial obstruction [2]. Because these signs overlap with those of pulmonary carcinoma [3], correct diagnosis is crucial, despite their excellent prognosis [4] [5]. Hamartomas have a slow growth rate [6], but rapid enlargement has also been described [7] and are usually single. When multiple, the Carney triad, consisting of an association between pulmonary chondroma, extra-adrenal paraganglioma and leiomyoblastoma [8] or partial Carney triad- hamartoma along with gastric smooth muscle tumors [9] should be considered.
Malignant transformation of lung hamartomas is possible [10], therefore patients must be periodically evaluated. Single nodules occur in men, whereas multiple tumors are characteristic for women. The condition more often occurs in males within their sixth or seventh decade of life [11].
Entire Body System
- Asymptomatic
[…] found at this location but in abnormal quantity, mixture or arrangement INCIDENCE • 0.25% in population • 8% of all solitary pulmonary nodules • Most common benign lung tumor • 5th and 6th decade peak • Male to female ratio of 3:1 CLINICAL • Mostly asymptomatic [learningradiology.com]
Importance Hamartomas are benign lesions that do not need further workup or resection if they are asymptomatic. Since they usually present as an indeterminate nodule on the chest radiograph, a CT is often obtained to exclude malignancy. [mayoclinic.pure.elsevier.com]
Two patients were asymptomatic, 2 had cough and 1 presented with haemoptysis. Pre-operative diagnosis was lung carcinoma in 4 patients. Four patients had lobectomy while 1 had wedge resection. [ajol.info]
Though traditionally done through thoracotomy with the lung in the semi-inflated stage, this video demonstrates the use of this technique through VATS in a 32 years old asymptomatic lady with a deep-seated right upper lobe hamartoma. [ctsnet.org]
Patients can be asymptomatic with peripheral parenchymal hamartomas, with incidental discovery on chest imaging obtained for another reason. [emedicine.medscape.com]
- Soft Tissue Mass
Spirometry with the expected flow/volume curve [ 3, 4 ] findings of a calcifying soft tissue mass on imaging modalities and bronchoscopy confirmed the diagnosis [ 5 ]. [karger.com]
tissue mass, sometimes with calcification CT: May contain intralesional fat, calcifications Radiology images Images hosted on PathOut servers: Contributed by Dr. [pathologyoutlines.com]
On CT scan, these lesions are described as rounded soft tissue masses that frequently exhibit calcification and fat density for adipose tissue. [6] Most measure 1–3 cm in diameter, but they range up to 9 cm. [ccij-online.org]
Figure 1 Chest radiograph showing soft tissue mass within the right hemithorax (A). CT (B) demonstrates that the mass contains low attenuation soft tissue suggestive of fat, and calcification, features in keeping with a teratoma. [cardiothoracicsurgery.biomedcentral.com]
- Surgical Procedure
The operative indication and surgical procedure for PH are still very controversial. [cancerjournal.net]
Inflammatory involvement of neighbouring structures may necessitate a more radical surgical procedure, sometimes including resection. 31) If the lung distal to the obstruction is irreversibly damaged, lobar resection or even pneumonectomy may be indicated [kjim.org]
The treatment of tissue changes such as tumors and cancers, despite best efforts of modern medicine still comparatively limited to chemotherapy in their diverse modifications and surgical procedures. [google.st]
- Epilepsy
Secondary generalised epilepsy may appear. Multiple seizures and progressive cognitive impairment occur. [patient.info]
In case of a Hypothalamic Hamartoma syndrome, a patient suffers from problems like : Multiple seizures, often accompanied with laughter Progressive failure in cognition Impaired social adjustment Early puberty Occasional angry outbursts Epilepsy In case [primehealthchannel.com]
HH itself generates GS and starts process of secondary epileptogenesis responsible for refractory focal or generalized epilepsy ( 1 ). In PTEN hamartoma tumor syndrome (PHTS), pathophysiology is related to function of protein product of PTEN gene. [unboundmedicine.com]
- Swelling
Multiple seizures, often accompanied with laughter Progressive failure in cognition Impaired social adjustment Early puberty Occasional angry outbursts Epilepsy In case of Hamartomas arising on head or neck, the individual may suffer from a hoarse voice, swelling [primehealthchannel.com]
Head and neck It may present as a swelling or lump in the head or neck, as pain in the chin, neck or face or as hoarseness. Gastrointestinal tract Polyps can occur anywhere in the gut but are most common in the colon. [patient.info]
Respiratoric
- Cough
[…] location but in abnormal quantity, mixture or arrangement INCIDENCE • 0.25% in population • 8% of all solitary pulmonary nodules • Most common benign lung tumor • 5th and 6th decade peak • Male to female ratio of 3:1 CLINICAL • Mostly asymptomatic • Cough [learningradiology.com]
Two patients were asymptomatic, 2 had cough and 1 presented with haemoptysis. Pre-operative diagnosis was lung carcinoma in 4 patients. Four patients had lobectomy while 1 had wedge resection. [ajol.info]
If located endobronchially, they can cause irritation of bronchial mucosa resulting in persistent cough as a presenting symptom. [emedicine.medscape.com]
They can result in symptoms like coughing, Haemoptysis and Dyspnoea. Patients with Haemoptysis cough up blood from the respiratory tract which sounds as a sign of some complicated syndrome but is actually not so harmful. [primehealthchannel.com]
Our patient was symptomatic having cough, hemoptysis, chest pain, and dyspnea. [ccij-online.org]
- Hemoptysis
[…] arrangement INCIDENCE • 0.25% in population • 8% of all solitary pulmonary nodules • Most common benign lung tumor • 5th and 6th decade peak • Male to female ratio of 3:1 CLINICAL • Mostly asymptomatic • Cough • Fever (with postobstructive pneumonia) • Hemoptysis [learningradiology.com]
However, some patients do have complaints related to the tumor, such as chest pain, hemoptysis, cough, wheezing and dyspnea caused by bronchial obstruction. [symptoma.com]
[…] adults 40s to 60s, peripheral lesions more common in men Sites Mostly arise in peripheral lung; endobronchial in < 10% of cases Etiology Thought to arise from primitive mesenchymal tissue Clinical features Asymptomatic, slow growth Occasionally with hemoptysis [pathologyoutlines.com]
Our patient was symptomatic having cough, hemoptysis, chest pain, and dyspnea. [ccij-online.org]
Occasionally, the tumors are central or giant with endobronchial extension causing hemoptysis, bronchial obstruction, coughing, wheezing, expectoration, leukocytosis and fever. [jacpjournal.org]
Neurologic
- Seizure
The associated episodes of laughter develop into seizures that become longer, more severe and sound less natural. Secondary generalised epilepsy may appear. Multiple seizures and progressive cognitive impairment occur. [patient.info]
A minor Hypothalamic Hamartoma makes a sufferer feel like laughing and also gives rise to seizures. Large Hamartoma tumors on the Hypothalamus can give rise to more serious symptoms including changes in behavior and mental ability. [primehealthchannel.com]
HH can cause gelastic (laughing) seizures (GS) and generalized seizures (see “History”). HH itself generates GS and starts process of secondary epileptogenesis responsible for refractory focal or generalized epilepsy ( 1 ). [unboundmedicine.com]
Unlike most such growths, a hypothalamic hamartoma is symptomatic; it most often causes gelastic seizures, and can cause visual problems, other seizures, rage disorders associated with hypothalamic diseases, and early onset of puberty. [en.wikipedia.org]
Some of these include seizures, personality changes, and early onset of puberty in children. Kidneys: Hamartomas may be found incidentally on imaging studies of the kidney, but can occasionally cause symptoms of kidney dysfunction. [verywell.com]
Workup
A lung hamartoma may be located in the peripheral areas (most cases) or may be endobronchial [12]. A thoracic radiography typically describes a small, well-circumscribed, possibly lobulated nodule or mass that may contain fat, bone or calcified areas [13]. The pathognomonic radiological aspect is that of "popcorn" calcium deposits, but stippled or curvilinear patterns can be noticed. Lesions can be found in any pulmonary lobe and usually do not cause cavitation. However, a certain diagnosis cannot be established using only plain radiography. A computer tomography (CT) scan offers an accurate description. 60% of lesions contain fat tissue, localized or disseminated inside the tumor and if fat is present and the CT aspect offers a certainty, no further investigations are needed [14]. High-resolution CT offers a better characterization of the architecture of the pathological process, while dynamic computer tomography scans may describe the hemodynamic features [15].
Fludeoxyglucose positron emission tomography shows increased tumor avidity for this tracer. Magnetic resonance imaging (MRI) describes the heterogeneous nature and is usually the modality of choice for Carney triad follow-up [16]. Paraganglioma and other neurogenic malignancies are easily confirmed by iodine-131-meta-iodobenzylguanidine scintigraphy.
Severe consequences are associated with tumors causing bronchial obstruction. In these cases, imaging methods may discover bronchiectasis or destruction of the peripheral pulmonary tissue.
In cases where a clear diagnosis is impossible to make using imaging methods, a percutaneous needle aspiration may be required. Ultrasonography is used to guide this process and is valuable in abdominal Carney triad components identification. If unsuccessful, a biopsy should be performed, or tissue may be analyzed after surgical removal. If the tumor is endobronchial, a transbronchial biopsy via a flexible fiberoptic bronchoscopy is an option [11]. Histological analysis shows fibromyxoid stroma, small, cuboidal or columnar bronchial epithelial cells, fat, bone, smooth muscle, and cartilage.
Blood workup may include the carbohydrate antigen 19-9, that is sometimes present in lung hamartomas [17], while molecular biology, if available, may describe abnormalities of 12q14 and 6q21 domains [18] [19]. Furthermore, patients should be periodically monitored for lung cancer, as they have been demonstrated to have 6.6 times increased risk [20].
X-Ray
- Pulmonary Coin Lesion
Chondroid hamartomas account for 7-14% of pulmonary coin lesions. [4] Calcifications are often present in a diffuse or popcorn distribution (popcorn calcification). [9] In the CT scan of chest, well-defined sharply marginated lesion showing multiple intralesional [mjdrdypu.org]
Treatment
A 63-year-old male was admitted for hospital treatment with obvious symptoms and signs of right-sided lobar pneumonia. His treatment was started with a combination of antibiotics Ceftriaxone and Ciprofloxacin. [ncbi.nlm.nih.gov]
A 63-year-old male was admitted for hospital treatment with obvious symptoms and signs of right-sided lobar pneumonia. His treatment was started with a combination of antibiotics Ceftriaxone and Ciprofloxa-cin. [scopemed.org]
Hamartoma Treatment The treatment of Hamartoma tumors depend on the type of the disease that the individual suffers from. In case of a Hamartoma tumor arising on the skin, treatment is generally unnecessary. [primehealthchannel.com]
If the lung nodule has changed in size or shows disease, we will make recommendations for the most appropriate treatment plan. Surgical Treatment for Lung Nodules UW Health offers numerous surgical treatments for lung disease. [uwhealth.org]
Treatment is by surgical resection. The prognosis is excellent. Links: lung cancer [gpnotebook.co.uk]
Prognosis
The prognosis is excellent. Links: lung cancer [gpnotebook.co.uk]
They are treated, if at all, by surgical resection, with an excellent prognosis: generally, the only real danger is the inherent possibility of surgical complications. [en.wikipedia.org]
Because these signs overlap with those of pulmonary carcinoma, correct diagnosis is crucial, despite their excellent prognosis. Hamartomas have a slow growth rate, but rapid enlargement has also been described and are usually single. [symptoma.com]
Differential diagnosis Benign chondroma Fibroma Bronchial lipoma Metastatic teratoma after chemotherapy Prognosis Malignant change has been reported, albeit extremely rarely; malignancies developing within a hamartoma include carcinomas, sarcomas and [e-immunohistochemistry.info]
Etiology
Etiology The etiology of pulmonary hamartoma is not understood. A summary of the etiologic concepts.. . [mdedge.com]
[…] could be diagnosed as lipoma, myxoma, leiomyoma or fibroadenoma Epidemiology Most common benign neoplasm of lung Generally adults 40s to 60s, peripheral lesions more common in men Sites Mostly arise in peripheral lung; endobronchial in < 10% of cases Etiology [pathologyoutlines.com]
(Etiology) The cause of development of Pulmonary Hamartoma is generally unknown It may be caused by certain genetic defects which are not yet well-characterised Research is currently being undertaken to identify the relevant causal factors What are the [dovemed.com]
Breast: 8 cases of hamartoma per 20,000 mammograms Kidney: renal hamartoma (angiomyolipoma) found in 40–80% of patients with tuberous sclerosis Etiology and Pathophysiology Functional disorder will depend on tissue of origin. [unboundmedicine.com]
[…] causally in the pathogenesis / etiology herein associated tissue changes or associated. [google.st]
Epidemiology
Epidemiology Hamartomas account for 1% to 2% of all pulmonary neoplasms and are the most common benign tumour 3. The incidence in a series of 8000 post-mortems was 0.25%. The average age is 55 years. Endobronchial lesions more commonly occur in men. [e-immunohistochemistry.info]
[…] triad may be a distinct entity Descriptive term sometimes added to describe components, i.e. lipomatous, chondroid, chondromatous, fibrochondromatous, fibroleiomyomatous If lacks cartilage, could be diagnosed as lipoma, myxoma, leiomyoma or fibroadenoma Epidemiology [pathologyoutlines.com]
Epidemiology Pulmonary hamartomas or mesenchymomas are the most common form of benign lung tumours with an incidence of between 0.025% – 0.32% according to different necropsy studies. In a large series they constituted 7–14% of all coin lesions. [humpath.com]
Epidemiology Can be hard to assess because many hamartomas are asymptomatic Incidence Depends on organ system affected Breast hamartomas are not common; estimated incidence is 0.1–0.7%. [unboundmedicine.com]
Epidemiology Incidence It is difficult to give an accurate assessment of incidence, as many skin lesions are simply ignored as 'birthmarks' and many internal lesions are incidental findings. [patient.info]
Pathophysiology
Breast: 8 cases of hamartoma per 20,000 mammograms Kidney: renal hamartoma (angiomyolipoma) found in 40–80% of patients with tuberous sclerosis Etiology and Pathophysiology Functional disorder will depend on tissue of origin. [unboundmedicine.com]
Pathophysiology Pathogenesis Hamartomas occur in the same location as the normal tissue (in the tissue of its origin) as opposed to choristomas, which grow in other tissues (different from its origin). [wikidoc.org]
Prevention
Agents for the prevention and / or treatment of a lesion of mesenchymal origin The invention relates to agents for the prevention and / or treatment of a tissue change, whereby the tissue change comprises tissue of mesenchymal origin, using the means, [google.st]
Minimizing the size of the arterial stump would prevent this complication ( 16 ). [jtd.amegroups.com]
NCI_CTEP-SDC (Cancer Therapy Evaluation Program - Simple Disease Classification) 2015AB NCI_CareLex (Content Archive Resource Exchange Lexicon) 2015AB NCI_DCP (NCI Division of Cancer Prevention Program) 2015AB NCI_DICOM (Digital Imaging Communications [doctor.am]
In case of serious discomforts such as Haemoptysis, you should seek immediate medical treatment to prevent complications. [primehealthchannel.com]
References
- Lien YC, Hsu HS, Li WY, et al. Pulmonary hamartoma. J Chin Med Assoc. 2004;67:21–26.
- Thomas JW, Staerkel GA, Whitman GJ. Pulmonary hamartoma. AJR Am J Roentgenol. 1999;172(6):1643.
- Jacob S, Mohapatra D, Verghese M. Massive chondroid hamartoma of the lung clinically masquerading as bronchogenic carcinoma. Indian J Pathol Microbiol. 2008;51(1):61-2.
- Zakharov V, Schinstine M. Hamartoma of the lung. Diagn Cytopathol. 2008;36(5):331-2.
- Guo W, Zhao YP, Jiang YG, et al. Surgical treatment and outcome of pulmonary hamartoma: a retrospective study of 20-year experience. J Exp Clin Cancer Res. 2008;27:8.
- Hutter J, Reich-Weinberger S, Hutarew G, et al. Giant pulmonary hamartoma--a rare presentation of a common tumor. Ann Thorac Surg. 2006;82(2):e5-7.
- Adachi Y, Araki K, Metsugi H, et al. A case of relatively rapid enlargement of a pulmonary hamartoma. J Jap Assoc Chest Surg. 2012;26:673-6.
- Rodriguez FJ, Aubry MC, Tazelaar HD, et al. Pulmonary chondroma: a tumor associated with Carney triad and different from pulmonary hamartoma. Am J Surg Pathol. 2007;31(12):1844-53.
- Carney JA, Stratakis CA. Familial paraganglioma and gastric stromal sarcoma: a new syndrome distinct from the Carney triad. Am J Med Genet. 2002;108(2):132-9.
- Basile A, Gregoris A, Antoci B, et al. Malignant change in a benign pulmonary hamartoma. Thorax. 1989;44(3): 232-3.
- Pollock AB, Al Hasan M, Roy TM, et al. Pulmonary hamartoma- an algorithmic approach to the diagnosis and management. Clin Pulm Med. 2008;15:35-9.
- Cosío BG, Villena V, Echave-Sustaeta J, et al. Endobronchial hamartoma. Chest. 2002;122(1):202-5.
- Chai JL, Patz EF. CT of the lung: patterns of calcification and other high-attenuation abnormalities. AJR Am J Roentgenol. 1994;162(5):1063-6.
- Klein JS, Braff S. Imaging evaluation of the solitary pulmonary nodule. Clin. Chest Med. 2008;29(1):15-38.
- Jeong YJ, Lee KS, Jeong SY, et al. Solitary pulmonary nodule: characterization with combined wash-in and washout features at dynamic multi-detector row CT. Radiology 2005;237:675–83.
- Erb RE, Delbeke D, Falke TH. Magnetic resonance imaging of Carney''s triad. South Med J. 1994;87(9):951-5.
- Okabayashi K, Hiratsuka M, Noda Y et al. Giant hamartoma of the lung with a high production of carbohydrate antigen 19-9. Ann Thor Surg. 1993;55(2):511-513.
- Johansson M, Dietrich C, Mandahl N et al. Recombinations of chromosomal bands 6p21 and 14q24 characterise pulmonary hamartomas. Br J Cancer. 1003;67(6):1236-41.
- Bini A, Grazia M, Petrella F, et al. Multiple chondromatous hamartomas of the lung. Interact Cardiovasc Thorac Surg. 2002;1:78–80.
- Ribet M, Jaillard-Thery S, Nuttens M. Pulmonary hamartoma and malignancy.J Thor Cardiovasc Surg 1994;107(2):611-4.