A bronchial glomus tumor surgically treated with segmental resection
http://www.100md.com
《血管的通路杂志》
Second Department of Surgery, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata 990-9585, Japan
Abstract
Extracutaneous glomus tumors are unusual and their occurrence in bronchus has been recognized with rarity. We present the clinical and pathological features of a case with bronchial glomus tumor. A 67-year-old man was admitted with a symptom of cough, and his chest CT scan and fiberoptic bronchoscopy demonstrated a localized polypoid tumor in the right superior bronchial trunk. Histology of the biopsy specimen by bronchoscopy was demonstrated as a typical carcinoid tumor. The tumor was surgically resected by a segmental resection of the right superior bronchial trunk and end-to-end anastomosis was followed to reconstruct the airway tract through a standard right side thoracotomy approach. The pathological and immunohistochemical findings resulted in the diagnosis of the tumor as a bronchial glomus tumor.
Key Words: Glomus tumor; Bronchus; Resection; Carcinoid
1. Introduction
The glomus tumor is a distinctive neoplasm arising in the cells resembling the modified smooth muscle cells of the normal glomus body. Its estimated incidence rate is 1.6% in the 500 connective soft tissue tumors. It is distributed most commonly in the subungual region of the finger, and the other sites are the palm, wrist, forearm, and foot. In fact, it is now recognized that the tumor also develops in sites where the normal glomus body may be sparse or even absent. Unusual locations listed include the patella, chest wall, bone, heart, stomach, eyelid, nasal cavity, mediastinum, lung, rectum, uterus, vagina, labia, and mesentery [1].
Several tracheal glomus tumors have been described, so far only three case reports of bronchial glomus tumor have been documented [1–3].
Here, we present the rare case of a bronchial glomus tumor arising from the right superior bronchial trunk.
2. Case report
A 67-year-old man had been complaining of frequent cough. The patient's past medical history was diabetes mellitus as well as cerebral infarction. Pulmonary function tests demonstrated vital capacity (VC) of 3.51 l, 101.4% of predicted value; forced expiratory volume in 1 s (FEV 1.0) of 2.95 l, 84.0% of predicted value. A chest X-ray film on admission showed no abnormality in the bilateral lung fields. Chest CT scan revealed a polypoid tumor in the right superior bronchial trunk (Fig. 1A). Infiltration of the tumor to the bronchial wall was suspected, but lymph node swelling was not evident.
To make a definite diagnosis of the bronchial tumor, a bronchoscopic study was performed. This examination demonstrated a vascular protruding tumor 8 mm in diameter with a smooth surface, covered with intact bronchial mucosa (Fig. 1B). The lesion occupied approximately 80% of the lumen. A biopsy was carefully performed without any complications such as bleeding. The histological examination of the biopsy specimens showed the typical carcinoid.
Without right upper lobectomy, the tumor was surgically resected by a segmental resection of right superior bronchial trunk and an end-to-end anastomosis was followed to reconstruct the airway tract through a standard right side thoracotomy approach.
The surgical specimen of the bronchial segmental resection consisted of a cylinder of bronchus measuring 1.5 cm in length and 3.1 cm in circumference. Projecting into the bronchial lumen was a polypoid firm mass measuring 0.8 cm (Fig. 2A). On the light microscope, the tumor was well circumscribed with intact bronchial epithelium and totally confined within the subepithelial connective tissue (Fig. 2B). The tumor was histologically composed of anastomosing nests and sheets of tumor cells surrounding the dilated vascular space. The tumor cells were uniformly monotonous with bland oval to round nuclei and a clear cytoplasm (Fig. 2C). No cellular atypia or mitoses were observed. There were no insular or trabecular structures resembling carcinoid tumor. Immunohistochemically, most tumor cells showed positive reactivity for -smooth muscle actin, vimentin, and collagen type IV. Staining was negative for synaptophysin, chromogranin A, factor VIII, CD34, Grimelius, Fontann-Masson. The proximal and distal resection margins were free of tumor.
The patient had an uneventful postoperative course and was discharged home in stable condition. He had no respiratory complaints and a fiber optic bronchoscope and CT scan revealed that no signs of anastomotic stenosis and recurrence 8 months following the resection.
3. Discussion
Glomus tumors are derived from cells of the neuromyoarterial glomus or glomus body. Ultrastructurally, glomocytes have features of smooth muscle. Histologically, they are subdivided into three general categories based on the prominence of glomocytes, vessels and smooth muscle: (i) the glomus tumor proper type, (ii) a glomaganglioma type, and (iii) a glomangiomyoma type [4,5]. Glomus tumors are usually benign, although malignant glomus tumors have been reported in the literature [6]. The glomus tumor of the present patient was benign with the proper subtype.
Because of their endoscopic and histologic similarity to other kinds of tumors, differential diagnoses such as carcinoid tumor and hemangiopericytoma should be considered especially for glomus tumors in unusual locations [5,7]. Three of the reported 19 tracheobronchial glomus tumors [1,2,8], and this bronchial tumor were initially diagnosed as carcinoid tumors [3]. Immunohistochemical staining for specific marker proteins gives useful information for this differential diagnosis. Glomus tumors show positive stainings for vimentin, collagen type IV and -smooth muscle actin as seen in the present case, and are usually negative for cytokeratin, chromogranin, myoglobin, vascular/endothelial markers such as factor VIII-related antigen, CD34, and Ulex lectin and neuroendocrine markers [4,5]. In this case, although the histological findings were similar to those of a carcinoid tumor, endoscopic resection and immunohistochemical examination, which lead to a more accurate diagnosis, should be initially performed. The characteristic immunohistochemical findings supported the diagnosis of a glomus tumor.
In cutaneous glomus tumors, paroxyams of pain radiating away from the lesion are the most common complaint. Most reported glomus tumors in the respiratory tract have been painless, but have dyspnea due to tracheal stenosis or hemoptysis. In this case, the patient had a cough due to bronchial stenosis.
Most glomus tumors are essentially benign and can be adequately treated by simple excision. Of the 19 reported tracheal or bronchial cases, 14 cases were surgically resected without recurrence. Only 4 cases were treated with endobronchial excision [1,2,8]. In some cases, tumor extended through the tracheal wall into the soft tissue, the treatment of tracheobronchial glomus tumors should be a surgical resection [9,10]. The surgical treatment for those should be a limited resection with negative margins and no formal mediastinal lymph node dissection [3].
In the present case, the patient underwent a segmental resection of the right superior bronchial trunk and an end-to-end anastomosis was performed without right upper lobectomy because the tumor had been initially diagnosed as a typical carcinoid and in its patients lymphnode and distant metastasis is unusual.
In summary, we have described in detail our case with a primary bronchial glomus tumor. Although bronchial and tracheal glomus tumors are rare, they should be kept in mind as one differential diagnostic possibility.
References
Oizumi S, Kon Y, Ishida T, Yamazaki K, Itoh T, Ogura S, Dosaka-Akita H, Morikawa T, Shimizu M, Katoh H, Kawakami Y. A rare case of bronchial glomus tumor. Respiration 2001; 68:95–98.
Okitsu H, Oho K, Koshiishi Y, Abe S, Takakura H, Amemiya R, Naito J, Hayata Y, Kodama T. A case of endobronchial glomus tumor. J Jap Soc Bronchol 1985; 7:312–317.
Lange TH, Magee MJ, Boley TM, Bell SW, Hazelrigg SR. Tracheobronchial glomus tumor. Ann Thorac Surg 2000; 70:292–295.
Gaertner EM, Steinberg DM, Huber M, Hayashi T, Tsuda N, Askin FB, Bell SW, Nguyen Ba, Colby TV, Nishimura SL, Miettinen M, Travis WD. Pulmonary and mediastinal glomus tumors – report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review. Am J Surg Patho 2000; 24:1105–1114.
Yilmaz A, Bayramgurler B, Aksoy F, Yagcituncer L, Selvi A, Uzman O. Pulmonary glomus tumor: a case initially diagnosed as carcinoid tumor. Respirology 2002; 7:369–371.
Hiruta N, Kameda N, Tokudome T, Tsuchiya K, Nonaka H, Hatori T, Akima M, Miura M. Malignant glomus tumor: a case report and review of the literature. Am J Surg Pathol 1997; 21:1096–1103.
Heard BE, Dewar A, Firmin RK, Lennox SC. One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy. Thorax 1982; 37:97–103.
Mojaverian A, Ost D. Tracheobronchial glomus tumor: case report and review of literature. J Bronchol 2004; 11:188–193.
Gowan RT, Shamji FM, Perkins DG, Maziak DE. Glomus tumor of the trachea. Ann Thorac Surg 2001; 72:598–600.
Menaissy YM, Gal AA, Mansour KA. Glomus tumor of the trachea. Ann Thorac Surg 2000; 70:295–297.(Nobumasa Takahashi, Hiroy)
Abstract
Extracutaneous glomus tumors are unusual and their occurrence in bronchus has been recognized with rarity. We present the clinical and pathological features of a case with bronchial glomus tumor. A 67-year-old man was admitted with a symptom of cough, and his chest CT scan and fiberoptic bronchoscopy demonstrated a localized polypoid tumor in the right superior bronchial trunk. Histology of the biopsy specimen by bronchoscopy was demonstrated as a typical carcinoid tumor. The tumor was surgically resected by a segmental resection of the right superior bronchial trunk and end-to-end anastomosis was followed to reconstruct the airway tract through a standard right side thoracotomy approach. The pathological and immunohistochemical findings resulted in the diagnosis of the tumor as a bronchial glomus tumor.
Key Words: Glomus tumor; Bronchus; Resection; Carcinoid
1. Introduction
The glomus tumor is a distinctive neoplasm arising in the cells resembling the modified smooth muscle cells of the normal glomus body. Its estimated incidence rate is 1.6% in the 500 connective soft tissue tumors. It is distributed most commonly in the subungual region of the finger, and the other sites are the palm, wrist, forearm, and foot. In fact, it is now recognized that the tumor also develops in sites where the normal glomus body may be sparse or even absent. Unusual locations listed include the patella, chest wall, bone, heart, stomach, eyelid, nasal cavity, mediastinum, lung, rectum, uterus, vagina, labia, and mesentery [1].
Several tracheal glomus tumors have been described, so far only three case reports of bronchial glomus tumor have been documented [1–3].
Here, we present the rare case of a bronchial glomus tumor arising from the right superior bronchial trunk.
2. Case report
A 67-year-old man had been complaining of frequent cough. The patient's past medical history was diabetes mellitus as well as cerebral infarction. Pulmonary function tests demonstrated vital capacity (VC) of 3.51 l, 101.4% of predicted value; forced expiratory volume in 1 s (FEV 1.0) of 2.95 l, 84.0% of predicted value. A chest X-ray film on admission showed no abnormality in the bilateral lung fields. Chest CT scan revealed a polypoid tumor in the right superior bronchial trunk (Fig. 1A). Infiltration of the tumor to the bronchial wall was suspected, but lymph node swelling was not evident.
To make a definite diagnosis of the bronchial tumor, a bronchoscopic study was performed. This examination demonstrated a vascular protruding tumor 8 mm in diameter with a smooth surface, covered with intact bronchial mucosa (Fig. 1B). The lesion occupied approximately 80% of the lumen. A biopsy was carefully performed without any complications such as bleeding. The histological examination of the biopsy specimens showed the typical carcinoid.
Without right upper lobectomy, the tumor was surgically resected by a segmental resection of right superior bronchial trunk and an end-to-end anastomosis was followed to reconstruct the airway tract through a standard right side thoracotomy approach.
The surgical specimen of the bronchial segmental resection consisted of a cylinder of bronchus measuring 1.5 cm in length and 3.1 cm in circumference. Projecting into the bronchial lumen was a polypoid firm mass measuring 0.8 cm (Fig. 2A). On the light microscope, the tumor was well circumscribed with intact bronchial epithelium and totally confined within the subepithelial connective tissue (Fig. 2B). The tumor was histologically composed of anastomosing nests and sheets of tumor cells surrounding the dilated vascular space. The tumor cells were uniformly monotonous with bland oval to round nuclei and a clear cytoplasm (Fig. 2C). No cellular atypia or mitoses were observed. There were no insular or trabecular structures resembling carcinoid tumor. Immunohistochemically, most tumor cells showed positive reactivity for -smooth muscle actin, vimentin, and collagen type IV. Staining was negative for synaptophysin, chromogranin A, factor VIII, CD34, Grimelius, Fontann-Masson. The proximal and distal resection margins were free of tumor.
The patient had an uneventful postoperative course and was discharged home in stable condition. He had no respiratory complaints and a fiber optic bronchoscope and CT scan revealed that no signs of anastomotic stenosis and recurrence 8 months following the resection.
3. Discussion
Glomus tumors are derived from cells of the neuromyoarterial glomus or glomus body. Ultrastructurally, glomocytes have features of smooth muscle. Histologically, they are subdivided into three general categories based on the prominence of glomocytes, vessels and smooth muscle: (i) the glomus tumor proper type, (ii) a glomaganglioma type, and (iii) a glomangiomyoma type [4,5]. Glomus tumors are usually benign, although malignant glomus tumors have been reported in the literature [6]. The glomus tumor of the present patient was benign with the proper subtype.
Because of their endoscopic and histologic similarity to other kinds of tumors, differential diagnoses such as carcinoid tumor and hemangiopericytoma should be considered especially for glomus tumors in unusual locations [5,7]. Three of the reported 19 tracheobronchial glomus tumors [1,2,8], and this bronchial tumor were initially diagnosed as carcinoid tumors [3]. Immunohistochemical staining for specific marker proteins gives useful information for this differential diagnosis. Glomus tumors show positive stainings for vimentin, collagen type IV and -smooth muscle actin as seen in the present case, and are usually negative for cytokeratin, chromogranin, myoglobin, vascular/endothelial markers such as factor VIII-related antigen, CD34, and Ulex lectin and neuroendocrine markers [4,5]. In this case, although the histological findings were similar to those of a carcinoid tumor, endoscopic resection and immunohistochemical examination, which lead to a more accurate diagnosis, should be initially performed. The characteristic immunohistochemical findings supported the diagnosis of a glomus tumor.
In cutaneous glomus tumors, paroxyams of pain radiating away from the lesion are the most common complaint. Most reported glomus tumors in the respiratory tract have been painless, but have dyspnea due to tracheal stenosis or hemoptysis. In this case, the patient had a cough due to bronchial stenosis.
Most glomus tumors are essentially benign and can be adequately treated by simple excision. Of the 19 reported tracheal or bronchial cases, 14 cases were surgically resected without recurrence. Only 4 cases were treated with endobronchial excision [1,2,8]. In some cases, tumor extended through the tracheal wall into the soft tissue, the treatment of tracheobronchial glomus tumors should be a surgical resection [9,10]. The surgical treatment for those should be a limited resection with negative margins and no formal mediastinal lymph node dissection [3].
In the present case, the patient underwent a segmental resection of the right superior bronchial trunk and an end-to-end anastomosis was performed without right upper lobectomy because the tumor had been initially diagnosed as a typical carcinoid and in its patients lymphnode and distant metastasis is unusual.
In summary, we have described in detail our case with a primary bronchial glomus tumor. Although bronchial and tracheal glomus tumors are rare, they should be kept in mind as one differential diagnostic possibility.
References
Oizumi S, Kon Y, Ishida T, Yamazaki K, Itoh T, Ogura S, Dosaka-Akita H, Morikawa T, Shimizu M, Katoh H, Kawakami Y. A rare case of bronchial glomus tumor. Respiration 2001; 68:95–98.
Okitsu H, Oho K, Koshiishi Y, Abe S, Takakura H, Amemiya R, Naito J, Hayata Y, Kodama T. A case of endobronchial glomus tumor. J Jap Soc Bronchol 1985; 7:312–317.
Lange TH, Magee MJ, Boley TM, Bell SW, Hazelrigg SR. Tracheobronchial glomus tumor. Ann Thorac Surg 2000; 70:292–295.
Gaertner EM, Steinberg DM, Huber M, Hayashi T, Tsuda N, Askin FB, Bell SW, Nguyen Ba, Colby TV, Nishimura SL, Miettinen M, Travis WD. Pulmonary and mediastinal glomus tumors – report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review. Am J Surg Patho 2000; 24:1105–1114.
Yilmaz A, Bayramgurler B, Aksoy F, Yagcituncer L, Selvi A, Uzman O. Pulmonary glomus tumor: a case initially diagnosed as carcinoid tumor. Respirology 2002; 7:369–371.
Hiruta N, Kameda N, Tokudome T, Tsuchiya K, Nonaka H, Hatori T, Akima M, Miura M. Malignant glomus tumor: a case report and review of the literature. Am J Surg Pathol 1997; 21:1096–1103.
Heard BE, Dewar A, Firmin RK, Lennox SC. One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy. Thorax 1982; 37:97–103.
Mojaverian A, Ost D. Tracheobronchial glomus tumor: case report and review of literature. J Bronchol 2004; 11:188–193.
Gowan RT, Shamji FM, Perkins DG, Maziak DE. Glomus tumor of the trachea. Ann Thorac Surg 2001; 72:598–600.
Menaissy YM, Gal AA, Mansour KA. Glomus tumor of the trachea. Ann Thorac Surg 2000; 70:295–297.(Nobumasa Takahashi, Hiroy)