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       Dr  Sampurna Roy  MD

 

 
               

Chondroid “hamartoma” (adenochondroma, mixed mesenchymoma).

Despite their most popular name (chondroid hamartoma), it is likely that these lesions are benign connective tissue neoplasms rather than tumour-like malformations.  Image Link

They are generally regarded as hamartomas because they consist of a disorganized proliferation of  various connective and epithelial tissues normally found in the lung.

However, their rarity in childhood and continued growth in adult life favour their interpretation as benign neoplastic lesion .

The term adenochondoma implies a benign mixed neoplasm but the role played by the epithelial components may also be questioned.

It is now appreciated that epithelial structures are commonly entrapped in many lung tumors, both primary and metastatic and is likely that this is how the epithelial clefts of the so-called adenochondroma or chondroid hamartoma should be regarded.   Image1 ; Image2

A name that reflects a purely mesenchymal nature appears appropriate and, since these tumours display a variety of connective tissues, the term mixed mesenchymoma has been recommended.

These lesions are fairly common, constituting about 10% of all ‘coin’ lesions in chest radiographs.

The age range is wide but they are rare in children and the peak age incidence is the sixth decade.

Sequential radiographs show that most first develop in adult life and slowly increase in size thereafter.

Most are parenchymal and asymptomatic but about 8% are endobronchial and cause coughing, dyspnoea or haemoptysis.

 There are no significant pathological differences between parenchymal and endobronchial growths.  

Most measure 1 to 3 cm in diameter but they range up to 9 cm.

They are sharply circumscribed but lack in capsule and shell out easily at operation, after which there is little risk of recurrence. Image 

Most are lobulated and the predominant tissue is cartilage, which may calcify or undergo osseous change.  Image1 ; Image2 ; Image3

Other connective tissues commonly found include fat, fibrous tissue and loose mesenchyme with a myxoid appearance.

These various components are usually present in combination. All are well differentiated and  show no feature of malignancy.

Intersecting the connective tissue lobules are clefts lined by ciliated pseudostratified columnar epithelium, which is quite normal cytologically.

The retention of cilia is notable as these are usually lost when such epithelium becomes neoplastic.

These tumours have no malignant potential.

Differential diagnosis:

 The differential diagnosis includes metastatic testicular teratome from which the malignant elements have been ablated by chemotherapy.

 If the proposed nature of chondroid hamartomas (mixed mesenchymomas) outlined above is accepted, they are obviously closely related to benign connective tissue neoplasms composed of a single tissue – lipomas , chondromas ,and fibromas for example – none of which is common in the lungs.

 Like the mixed mesenchymomas, all these neoplasms may either lie entirely within the lung substance or protrude into major bronchi.

Epithelial clefts are not found in single tissue benign mesenchymal tumours, which resemble in every way their counterparts that occur more commonly in other parts of the body.

Like chondroid hamartomas, they appear to have  no malignant potential. In this context it should be noted that the rare primary pulmonary fibrosarcomas , liposarcomas , chondrosarcomas and osteosarcomas are believed to be sarcomatous from their onset.

                 

Carney's Triad:

Carney’s triad is a rare combination of three unusual neoplasms that affects young women. The patients are generally in the second or third decade, the youngest recorded being a girl aged 9 years. The condition is not familial. The three tumours are extra-adrenal paraganglioma (chemodectomas), gastric epithelioid leiomyosarcomas (Gastrointestinal Stromal Tumour) and pulmonary chondromas. All are generally multiple and early recognition of the syndrome is important in view of the malignant potential of the gastric lesions and paragangliomas. The chondromas have no malignant potential.

                    

Abstracts:

Multifocal mesenchymal hamartoma of the chest wall.Respirology. 2006 May;11(3):334-8.

Reactive alveolar epithelium in chondroid hamartoma of the lung.
Acta Cytol. 2005 Mar-Apr;49(2):154-6. Erratum in: Acta Cytol. 2005 Sep-Oct;49(5):127A.

Giant cystic chondroid hamartoma.J Korean Med Sci. 2005 Jun;20(3):509-11.

Rare tracheal chondroid hamartoma masquerading as asthma in a 14-year-old girl.Ann Allergy Asthma Immunol. 2004 May;92(5):576-9.

Pulmonary chondroid hamartoma with unusual presentation.Indian J Chest Dis Allied Sci. 2002 Oct-Dec;44(4):263-6.

Fine needle aspiration biopsy of pulmonary hamartomas. Radiologic, cytologic and immunocytochemical study of 15 cases. Acta Cytol 1995; 39:1167-1174.

Pulmonary chondroid hamartoma presenting as an inflatable neck mass. Case report and clinicopathologic analysis.Arch Otolaryngol Head Neck Surg. 1994 Apr;120(4):440-3

Pulmonary hamartoma. J Thorac Cardiovasc Surg 1992; 104:674-678.

The aspiration cytology of pulmonary hamartomas. Diagn Cytopathol 1989; 5:174-180.

Endobronchial hamartoma. Report of 7 cases. Scand J Thor Cardiovasc Surg 1989; 23:285-287.

Malignant change in a benign pulmonary hamartoma. Thorax 1989; 44:232-233.

Mesenchymoma of the lung (so-called hamartoma): a review of 154 parenchymal and endobronchial cases. Thorax 1987; 42:790-793.

Benign endobronchial mesenchymal tumors. Their relationship to parenchymal pulmonary hamartomas. Am J Surg Pathol 1982; 6:531-540.

Multiple pulmonary chondromatous hamartomas. Hum Pathol 1982; 13:496-497.

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. Cancer 1979; 43:374-382.

 
July 2009 
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