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Granular cell tumor (GCT) of the lung is a rare neoplasm comprising 6-10% of all GCT. 

It was first described in the bronchus by Kramer in 1939.

The tumour usually occurs in adults but a few cases have been reported in children.

These tumours are located in the trachea, bronchus  or in the lung parenchyma.

Granular cell tumor is usually found during bronchoscopy performed because of symptoms of pneumonia, lung cancer and hemoptysis or dyspnea.

The tumour often presents as a polypoid mass obstructing the bronchus. The tumours are most often solitary , but in some cases there may multiple lesions.

Pulmonary GCT behave in a benign fashion.

It has been observed that tumours of less than 8 mm are more amenable to endoscopic removal and larger tumors are more likely to infiltrate through the bronchial wall.

Histologically, the endobronchial GCT consisted of submucosal infiltrates of round to oval cells with distinct cell borders, abundant granular cytoplasm and eccentrically placed small round nuclei.

In some cases the cells resemble signet-ring cells.

The tumour often infiltrate into peribronchial tissue and in some cases focally infiltrate  an adjacent lymph node.

Hyalinized thickening of the subepithelial basement membrane is common.

The overlying epithelium often show  squamous metaplasia or ulceration.

There is usually no evidence of cellular atypia , necrosis, hemorrhage and  mitotic activity.

The tumours are positive for S-100 protein, neuron specific enolase, KP1 (CD68) and vimentin. The tumours do not express  desmin, keratin or p53 oncoprotein.

Complete surgical excision is the treatment of choice.

               

Bronchial granular cell tumor in a child: impact of diagnostic delay on the type of surgical resection. J Pediatr Surg. 2006;41(7):1326-8.

Bronchial granular cell tumor is an uncommon tumor in young children. Recurrent pneumonia in the same site should raise suspicion of an obstructing granular cell tumor of the bronchus. We present a 10-year-old girl with an 18-month history of recurrent pneumonia caused by obstruction of the main lingular bronchus by this type of tumor. In our patient, an upper left lobectomy was only technically feasible instead of a lingulectomy alone because of the spread pulmonary inflammation and the depth of bronchial wall invasion by the tumor. Delayed diagnosis resulted in a more extensive pulmonary resection.

Granular cell tumor--a rare, benign respiratory tract neoplasm in the material of the Institute of Tuberculosis and Lung Diseases .Pneumonol Alergol Pol. 2004;72(5-6):187-91.

The granular cell tumor (GCT) is a nodule that arises most commonly in the skin, the breast or the tongue. The vast majority are benign. Approximately 6-10% of granular cell tumors have been reported in the lower respiratory tract. The clinical, pathological and immunohistochemical findings of eleven cases are described in our material consisted of 6 males and 5 females aged from 35 to 58 years (median, 46 years). The GCT were solitary lesions in all our patients. The tumors were located in trachea (6 cases) and in bronchus (5 cases). They were found during bronchoscopy performed because of symptoms of pneumonia, lung cancer and hemoptysis or dyspnea alone. Diameter of the tumors ranged from 0.2-2.5 cm (median 1.2 cm). Six tumors were surgically excised and 5 were endoscopically removed. Pulmonary GCT behave in a benign fashion. It was observed that tumors of less than 8 mm were more amenable to endoscopic removal and larger tumors were more likely to infiltrate through the bronchial wall. Histologically, the GCT showed submucosal infiltrates of round or oval cells with abundant granular cytoplasm. The tumors cells were positive for S-100 protein, neuron specific enolase, CD68 and vimentin. Our immunohistochemical results are consistent with this concept.

Granular cell tumor of the bronchus.Pediatr Pulmonol. 2000;30(5):425-8.

Persistent atelectasis and recurrent pneumonia in the same location should raise suspicion of congenital anomalies or obstructing lesions of the bronchus leading to the affected area. We present an 8-year-old black female with a history of recurrent fever, cough, atelectasis of the right middle and lower lobes, and weight loss for several months. Flexible bronchoscopy revealed a polypoid mass obstructing the bronchus intermedius. Biopsy of the neoplasm demonstrated a granular cell tumor (GCT). The patient had a lobectomy of the right lower and middle lobes. She had no recurrence of the tumor after several years of follow-up.

Granular cell tumours of the lower respiratory tract.Histopathology.1995 Sep;27(3):257-62.

Granular cell tumours rarely involve the lower respiratory tract. We report eight cases surgically resected at our institution. There were four females and four males, aged between 18 to 56 years (mean 40). One tumour associated with a peripheral lung adenocarcinoma was asymptomatic. The other lesions presented with obstructive pneumonitis (3 cases), haemoptysis (2), dyspnea (1) or cough (1). These tumours were tracheal (1) or bronchial (6) and one case was located in the lung parenchyma. Four cases were multicentric with associated lesions located in a bronchus (2), the oesophagus (1) or a mediastinal lymph node (1). All tumours, with the largest diameter ranging from 0.5-4.5 cm, were histologically invasive. The tumours were positive for S-100 protein, neuron specific enolase, KP1 (CD68) and vimentin. No tumour expressed desmin, keratin or p53 oncoprotein. Our study demonstrates that, in spite of marked anatomical and clinical polymorphism, the rare granular cell tumours of the lower respiratory tract have a constant histological appearance. Our observations confirm that large tumours (> 8-10 mm) usually extend beyond the tracheo-bronchial cartilages and, therefore, only surgical treatment may avoid recurrence.

Granular cell tumors of the lung. Clinicopathologic study of 20 cases. Am J Surg Pathol. 1995;19(6):627-35.

Granular cell tumor (GCT) of the lung is a rare neoplasm comprising 6-10% of all GCT. Since it was first described in the bronchus by Kramer in 1939, less than 80 cases have been reported. We present the clinicopathologic features of 23 GCT from 20 patients. The patients ranged in age from 20 to 57 years (median, 45 years) and included 10 males and 10 females. Of the 19 patients with available histories, nine (47%) were incidental findigns and 10 (53%) had obstructive symptoms [pneumonia, 7 (37%); atelectasis, 3 (16%)]. Three (16%) had hemoptysis, and one (5%) had weight loss. The GCT were solitary in 15 patients (75%) and multiple in five others (25%). One patient had three endobronchial lesions, and another had one endobronchial and one peripheral pulmonary lesion. Three of the patients had multiple cutaneous GCT (15%). Grossly, they were polypoid or nodular, tan-yellow, and firm. Histologically, the endobronchial GCT consisted of submucosal infiltrates of round to oval cells with abundant granular cytoplasm. The tumor often infiltrated into peribronchial tissue and in one case focally infiltrated an adjacent lymph node. Hyalinized thickening of the subepithelial basement membrane was common; the overlying epithelium often showed squamous metaplasia or ulceration. In those patients with available follow-up, the clinical behavior of lung GCT was benign. Our experience supports a conservative approach to therapy in most cases unless there has been extensive postobstructive lung injury. Potential conservative therapeutic approaches include bronchoscopic extirpation, laser therapy, or sleeve resection. The histogenesis of GCT is not known, although most studies suggest a peripheral nerve sheath origin. Our immunohistochemical results with positive staining for antibodies to S100 (4/4), NSE (3/3), vimentin (4/4), and actin (4/4, focal) are consistent with this concept.

Cytology of bronchial benign granular-cell tumor. Acta Cytol. 1991 Jul-Aug;35(4):381-4.

The cytologic features of a case with multiple bronchial benign granular-cell tumors are reported and compared with those of previously reported cases. Characteristic tumor cells were found in the bronchoscopic brushing smears and in cell block sections (but not smears) prepared from the washing fluid. These findings were confirmed by the bronchial biopsy and histologic study of the resected tumors. A cytologic diagnosis of bronchial granular-cell tumor should not be difficult because the cytologic appearance of the tumor cells is characteristic; however, the possibility of a concomitant tumor, such as adenocarcinoma or small-cell carcinoma, should be considered and excluded.

Bronchial granular-cell tumor. Report of a case with preoperative cytologic diagnosis on bronchial brushings and immunohistochemical studies. Acta Cytol. 1991;35(4):375-80.

A 42-year-old man presented with a polypoid endobronchial mass of the right apical segmental bronchus. Bronchial brushing smears contained clusters of cells exhibiting abundant diffusely granular cytoplasm with indistinct borders. A cytologic diagnosis of granular-cell tumor was rendered. Histologic examination of the upper right lobectomy specimen provided confirmation. Immunohistochemically, the granular cells strongly reacted with the S-100 protein antibody. This case demonstrates that the cytologic diagnosis of bronchial granular-cell tumor is possible if this lesion is considered in the differential diagnosis of lung tumors.

Bronchopulmonary granular cell tumor. Z Erkr Atmungsorgane. 1989; 173 (3): 242-6.

The so-called granular cell tumors, tumors of uncertain or disputed histogenesis, are generally rare, in the bronchial tree extremely rare. With regard to their localization, they are able--like a bronchogenic cancer--to cause mucus retention, bronchiectasis, recurrent pneumonias and atelectasis. A definitive diagnosis and typing of these benign tumors must be made before operation in the interest of an adequate therapy and avoiding overtreatment.

Granular cell tumour of the bronchus: bronchoscopic and clinical features.Thorax. 1986 Dec;41(12):927-31.

Granular cell tumours are uncommon, generally benign neoplasms of uncertain origin that occasionally affect the tracheobronchial tree. Their incidence seems to be increasing, despite the fact that such tumours are rarely suspected on clinical grounds or bronchoscopic appearance. Here we describe three cases of endobronchial granular cell tumours, one of which regressed spontaneously after biopsy, and review previous accounts of their bronchoscopic and clinical features.

Present status of granular cell tumors. Apropos of 8 new cases with tracheobronchial localization.Rev Pneumol Clin. 1985;41(6):358-64.

The authors report 8 new cases of granular cell tumour of Abrikossoff's tumour located in the trachea or bronchus. After briefly recalling the generally accepted features of this disease, they report several new aspects: the obviously under-estimated frequency, the absence of progression, requiring therapeutic abstention and regular follow-up, the epidemiological problems posed by the association with chronic bronchitis or the coexistence with a malignant bronchial tumour, the uncertainties which still surround the histogenesis, which is partly mesenchymal and partly nervous tissue, more particularly Schwann cells, which can be supported by ultrastructural arguments.

Cytomorphology of granular-cell tumor of the bronchus. A case report. Acta Cytol. 1984  Mar-Apr;28(2):129-32.

The cytologic features of a bronchial granular-cell tumor clinically mimicking a bronchogenic carcinoma are presented. Distinctive granular cells similar to the main cellular components of the surgical specimen were found in abundance in bronchial brushings and washings obtained during bronchoscopy. Our findings and conclusions confirm previously published studies. Granular-cell tumors of the bronchus can easily be diagnosed on cytologic examination if the entity is considered in the differential diagnosis of clinically suspected lung tumors.

            

 
June 2008

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