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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 87-89

Giant pulmonary hamartoma: A rare entity


1 Department of Chest, Medinipore Medical College, Medinipur, West Bengal, India
2 Department of Chest, N R S Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Chest, Calcutta Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication23-Jun-2014

Correspondence Address:
Abinash Agarwala
Department of Chest Medicine, Medinipore Medical College,West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-8775.135121

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  Abstract 

Hamartoma is one of the common benign tumor of lung. These lesions represent an abnormal proliferation and mixing of the normal components of the lung tissue. Most hamartomas are asymptomatic. We report a rare case of giant pulmonary hamartoma (12 cm in diameter) occupied almost whole of left hemithorax. Computed tomography guided fine-needle aspiration cytology revealed a benign cartilaginous neoplasm, suggestive of chondroid hamartoma. Tru-cut biopsy of the tumor confirmed the diagnosis. Complete resection of the tumor was performed. Patient was on a regular follow-up postoperatively for 6 months without any clinical and radiological relapse.

Keywords: Fine-needle aspiration cytology, giant chondroid hamartoma, lung


How to cite this article:
Agarwala A, Sengupta A, Basuthakur S. Giant pulmonary hamartoma: A rare entity. J Assoc Chest Physicians 2014;2:87-9

How to cite this URL:
Agarwala A, Sengupta A, Basuthakur S. Giant pulmonary hamartoma: A rare entity. J Assoc Chest Physicians [serial online] 2014 [cited 2021 Dec 7];2:87-9. Available from: https://www.jacpjournal.org/text.asp?2014/2/2/87/135121


  Introduction Top


Pulmonary hamartoma, also known as chondroma or chondroid hamartoma or chondroid adenoma or mesenchymoma, is the most common type of benign lung tumor. It is composed of varying proportions of mesenchymal tissues and entrapped respiratory epithelium. They were considered as developmental abnormalities in the past but now regarded as benign mesenchymal neoplasms. [1],[2],[3],[4],[5],[6] Incidence of pulmonary hamartoma is 0.25%. Most occurs in the sixth decade of life and has two- to four-fold male preponderance. Most often it is an incidental finding as solitary nodule on chest X-ray. [1],[2],[3] They are usually peripheral and <5 cm in diameter. Giant pulmonary hamartomas (>9 cm) are very rare and only 11 cases have been published until date. [7],[8],[9],[10],[11],[12] The present case was a 25-year-old female with a giant (12 cm) hamartoma, extending from periphery to bronchus, in the left lung.


  Case report Top


A 25-year-old female presented with cough, fever, and left-sided chest pain for 8 months duration. She had a single episode of hemoptysis. Physical examination revealed decreased breath sounds on the left-side. Routine blood and biochemical parameters were within the normal limits. Sputum was negative for acid-fast bacilli for consecutive 3 days. Chest X-ray posteroanterior view revealed a large opacity occupying almost whole of the left hemithorax blurring the left cardiopulmonary angle. Computed tomography (CT) scan of thorax showed a large, 12 cm diameter, heterogeneous mass occupying almost entire left hemithorax with extensive nodular (popcorn) calcification and displacing the mediastinum to the right [Figure 1]. Left bronchus was compressed. No mediastinal lymphadenopathy or pleural effusion was documented. Impression was malignant neoplasm.
Figure 1: Computed tomography scan of thorax showing a large, 12 cm diameter, heterogeneous mass occupying almost entire left hemithorax with extensive nodular (popcorn) calcification and displacing the mediastinum to the right

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Computed tomography guided fine-needle aspiration cytology (FNAC) of the lesion was done. Smears were stained by May-Grόnwald-Giemsa and Papanicolaou stains. Smears were moderately cellular comprising of round to ovoid cells with nucleomegaly and anisonucleosis, moderate amount of pale cytoplasm in a chondromyxoid background. There were also sheets of bronchiolar epithelial cells and chondrocytes interspersed with fat cells and foci of calcified spherules [Figure 2]. Based on cytology a strong suspicion of chondroid hamartoma was made. Percutaneous transthoracic needle biopsy showed predominantly cartilaginous tissue and confirmed the diagnosis [Figure 3]a]. Endobronchial biopsy was also done which showed immature cartilaginous cells having acidophilic cytoplasm resembling histiocytes along with plump fibroblast and smooth muscle fibers [Figure 3]b]. The patient was undergone a sternotomy with complete resection of the tumor. The mass was compressing the left lung with no evidence of local invasion. The postoperative recovery period was uneventful and the patient was discharged home. The patient was on regular follow-up in our department and had no signs of relapse until date.
Figure 2: Computed tomography guided fine-needle aspiration cytology from lung mass showing moderately cellular round to ovoid cells with nucleomegaly and anisonucleosis in a chondromyxoid background with sheets of bronchiolar epithelial cells and chondrocytes interspersed with fat cells and foci of calcified spherules suggestive of chondroid hamartoma (Papanicolaou, ×100)

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Figure 3: (a)Percutaneous transthoracic needle biopsy showing predominantly cartilaginous tissue, (b) Endobronchial biopsy showing immature cartilaginous cells having acidophilic cytoplasm resembling histiocytes along with plump fibroblast and smooth muscle fibers suggestive of pulmonary hamartoma

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  Discussion Top


Albrecht in 1904 coined the term "hamartoma" to describe tumor-like malformations resulting from a presumptive developmental abnormality. Goldsworthy in 1934 applied this term to benign lung tumors, which were composed predominantly of a combination of fat and cartilage. Now hamartomas are considered as mesenchymal clonal neoplasms, a view supported by cytogenetic studies, which identified chromosomal bands of recombination located at 6p21 and 14q24 positions.

Pulmonary hamartomas are most common benign tumors of the lung. They usually measure 1-5 cm in diameter, whereas those reported as giant hamartomas measure between 9 and 30 cm. [1],[3] Most of them are seen in female patients in contrast to the usual hamartomas. The age range is wide, 30-63 years, mean age of 48.9. Some of the patients are younger than the expected age. Youngest age reported in literature is 30 years. Petheram and Heard have reported a huge hamartoma with a diameter of 30 cm. [9] This patient was 32-year-old male. Most of the giant hamartomas reported are localized in the right lung. When located in the left lung, they occupy mostly the lower lobe. None of the cases had endobronchial lesion. Our case is a 25-year-old female with a giant hamartoma involving almost whole of the left lung with endobronchial lesion.

The peripheral pulmonary hamartomas are usually asymptomatic. Occasionally, the tumors are central or giant with endobronchial extension causing hemoptysis, bronchial obstruction, coughing, wheezing, expectoration, leukocytosis and fever. Our case also had cough, fever, and chest pain for 8 months duration and an episode of hemoptysis due to the giant size of the tumor and endobronchial extension with compression of bronchus.

Usually diagnoses of hamartomas are incidental. They constitute about 8% of all "coin" lesions in chest radiographs appearing as a solitary, round nodules sometimes with punctate or popcorn calcification. CT scan showing specific popcorn-type calcification (30%) is almost pathognomonic. [1],[2],[3],[4] Our case also had typical popcorn calcification.

Fine-needle aspiration cytology is diagnostic of majority of chondroid hamartomas (90%) and hence widely used. Cytological criteria for diagnosis are the presence of mature cartilage, myxoid connective tissues, sheets of bronchiolar epithelium and adipose tissue. Sometimes marked anisonucleosis and large intranuclear inclusion in the epithelial or cartilaginous component may lead to erroneous diagnosis of malignancy.

Histopathological examination usually shows a predominant chondroid differentiation (80%), with fibroblastic (12%), fatty (5%) and osseous (3%) differentiation making the rest. Endobronchial lesions tend to have more fat (9%). Myxomatous connective tissue, smooth muscle, blood vessels and other mesenchymal elements may also be seen.

Most tumors grow slowly (average of 3 mm/year) during follow-up. Surgical treatment is the gold standard in symptomatic hamartomas, including enucleation, lobectomy, wedge resection, segmentectomy, and pneumonectomy.


  Conclusion Top


This rare entity must be kept in mind as it often leads to false interpretation of malignancy. Correct diagnosis is vital for proper management and FNAC is usually enough for a diagnosis.

 
  References Top

1.van den Bosch JM, Wagenaar SS, Corrin B, Elbers JR, Knaepen PJ, Westermann CJ. Mesenchymoma of the lung (so called hamartoma): A review of 154 parenchymal and endobronchial cases. Thorax 1987;42:790-3.  Back to cited text no. 1
    
2.Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996;71:14-20.  Back to cited text no. 2
    
3.Tomashefski JF Jr. Benign endobronchial mesenchymal tumors: Their relationship to parenchymal pulmonary hamartomas. Am J Surg Pathol 1982;6:531-40.  Back to cited text no. 3
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4.Bateson EM. So-called hamartoma of the lung - A true neoplasm of fibrous connective tissue of the bronchi. Cancer 1973;31:1458-67.  Back to cited text no. 4
[PUBMED]    
5.Xiao S, Lux ML, Reeves R, Hudson TJ, Fletcher JA. HMGI(Y) activation by chromosome 6p21 rearrangements in multilineage mesenchymal cells from pulmonary hamartoma. Am J Pathol 1997;150:901-10.  Back to cited text no. 5
    
6.Tallini G, Vanni R, Manfioletti G, Kazmierczak B, Faa G, Pauwels P, et al. HMGI-C and HMGI(Y) immunoreactivity correlates with cytogenetic abnormalities in lipomas, pulmonary chondroid hamartomas, endometrial polyps, and uterine leiomyomas and is compatible with rearrangement of the HMGI-C and HMGI(Y) genes. Lab Invest 2000;80:359-69.  Back to cited text no. 6
    
7.Darke CS, Day P, Grainger RG, Smith GH. The bronchial circulation in a case of giant hamartoma of the lung. Br J Radiol 1972;45:147-50.  Back to cited text no. 7
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8.D'Altorio RA. Case of the fall season. Giant pulmonary hamartoma. Semin Roentgenol 1975;10:253-4.  Back to cited text no. 8
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9.Petheram IS, Heard BE. Unique massive pulmonary hamartoma. Case report with review of hamartomata treated at Brompton Hospital in 27 years. Chest 1979;75:95-7.  Back to cited text no. 9
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10.Okabayashi K, Hiratsuka M, Noda Y, Hanagiri T, Mitsudomi T, Shirakusa T, et al. Giant hamartoma of the lung with a high production of carbohydrate antigen 19-9. Ann Thorac Surg 1993;55:511-3.  Back to cited text no. 10
    
11.Kervancioglu R, Bayram MM, Elbeyli L. Giant pulmonary hamartoma. AJR Am J Roentgenol 1997;168:573.  Back to cited text no. 11
    
12.Fujino S, Tezuka N, Sawai S, Kontani K, Inoue S, Mori A. Giant hamartoma of the lung. Jpn J Thorac Cardiovasc Surg 1998;46:1229-31.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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