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].
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]
Treatment is by surgical resection. The prognosis is excellent. Links: lung cancer [gpnotebook.co.uk]
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]
Prognosis
The prognosis is excellent. Links: lung cancer [gpnotebook.co.uk]
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]
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]
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]
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]
Hodges suggested four etiologic theories; congenital malformation, hyperplasia of normal structure, neoplasia and response to inflammation. 18) The theory of congenital origin is accepted by most authors. [kjim.org]
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]
Linguistic Variant - Portuguese, Brazil) 2015AB LNC-RU-RU (LOINC Linguistic Variant - Russian, Russia) 2015AB LNC-TR-TR (LOINC Linguistic Variant - Turkish, Turkey) 2015AB LNC-ZH-CN (LOINC Linguistic Variant - Chinese, China) 2015AB MCM (Glossary of Clinical Epidemiologic [doctor.am]
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
Minimizing the size of the arterial stump would prevent this complication ( 16 ). [jtd.amegroups.com]
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]
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
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- 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.