ABSTRACT

Glomus tumors are uncommon benign soft tissue neoplasms. We report the case of a 32-year-old male who presented with right upper lobe atelectasis due to an endobronchial tumor. The patient underwent right upper lobectomy and wedge bronchoplasty. The pathological diagnosis was bronchial glomus tumor. To our knowledge, this presentation (with right upper lobe atelectasis) has never before been reported. Glomus tumor should be considered in the differential diagnosis of endobronchial lesions causing lobar atelectasis.

Keywords: Glomus tumor; Bronchial neoplasms; Thoracic surgical procedures.

RESUMO

Tumores glômicos são raras neoplasias benignas de tecidos moles. Relatamos o caso de um paciente de 32 anos de idade que apresentava atelectasia em lobo superior direito devido a um tumor endobrônquico. O paciente foi submetido a lobectomia superior direita e broncoplastia em cunha. O diagnóstico patológico foi de tumor glômico endobrônquico. Até onde sabemos, essa apresentação com atelectasia em lobo superior direito nunca foi relatada. O tumor glômico deve ser considerado no diagnóstico diferencial de lesões endobrônquicas que causam atelectasia lobar.

Palavras-chave: Tumor glômico; Neoplasias pulmonares; Procedimentos cirúrgicos torácicos.

Introduction

Glomus tumors are uncommon benign soft tissue neoplasms. They are presumed to originate from cells resembling the modified smooth muscle cells of the glomus body.(1,2) Although glomus tumors are most commonly located in the subungual region, they can, occasionally, also be found at sites where glomus bodies are sparse or even absent.(2) The presence of this type of neoplasm in the respiratory tract is rare, and bronchial glomus tumors are even rarer.(1-8) We report the first case of a glomus tumor located in the right upper lobe bronchus and accompanied by right upper lobe atelectasis.

Case report

A 32-year-old male reported fever and dry cough 9 months prior to admission. On that occasion, he received treatment for pneumonia elsewhere, but the dry cough remained. The physical examination revealed right-sided wheezing, and a chest X-ray showed right upper lobe atelectasis (Figure 1a). A chest CT scan revealed an endobronchial lesion occluding the right upper lobe bronchus (Figure 1b), with no mediastinal lymph node enlargement. Fiberoptic bronchoscopy revealed an endobronchial tumor located in the right upper lobe bronchus. The tumor had a smooth, reddish surface and had obstructed the right upper lobe bronchus, without protruding into the right main bronchus. A biopsy provided inconclusive results. The patient underwent right thoracotomy with a presumed diagnosis of carcinoid tumor. Since the right upper lobe was atelectatic and fibrotic, we proceeded to a right upper lobectomy. The intraoperative histological examination demonstrated exiguous bronchial margins, and a wedge bronchoplasty was therefore performed. Radical mediastinal lymph node dissection was not performed. The patient had an uneventful recovery and was discharged on the fourth postoperative day.
The pathological examination of the resected piece revealed a polypoid tumor in the right upper lobe bronchus, measuring 1.7 × 1.0 cm. The microscopic examination revealed a tumor formed by thin-walled vessels surrounded by small cells with round nuclei and clear or eosinophilic cytoplasm (Figures 2a and 2b). The resection margins were tumor-free. Immunohistochemical analysis showed the cells to be positive for smooth muscle actin (1A4) and neuron-specific enolase (NSE). Tests for cytokeratin (AE1/AE3 and CAM 5.2), chromogranin, thyroid transcription factor-1 (TTF-1) and S100 protein were negative. The final diagnosis was bronchial glomus tumor. There was no recurrence during the 14 months of follow-up.







Discussion

Glomus tumors are uncommon benign tumors, constituting about 1.6% of all soft tissue neoplasms.(2) They are presumed to originate from cells resembling the modified smooth muscle cells of the neuromyoarterial glomus or glomus body, which is a form of arteriovenous anastomosis thought to be associated with thermoregulation.(8) Although glomus tumors are most commonly located in the subungual region, other common sites include the palm, wrist, forearm and foot. However, in rare cases, these tumors are also found at sites where glomus bodies are sparse or even absent, such as the viscera.(4)

Several cases of glomus tumor arising from the trachea or the lung parenchyma have been reported.(5,8-10) However, only nine cases of glomus tumor arising from the bronchus have been published in the English-language literature, including the case reported herein.(1-8) To our knowledge, this is the first case report of a bronchial glomus tumor causing right upper lobe atelectasis (Table 1).



The bronchoscopic appearance of the lesion led us to the initial diagnosis of carcinoid tumor, a misdiagnosis that has also been reported by other authors.(2,4,7) In the previous reports, pulmonary resection was not necessary for the treatment of the endobronchial tumor.(1-8) However, in the present case, the tumor had obstructed the right upper lobe bronchus for at least 9 months, leading to pulmonary infection and parenchymal destruction. These findings led us to proceed to a right upper lobectomy with wedge bronchoplasty in order to achieve tumor-free resection margins. A thorough mediastinal lymph node dissection was not performed because of endotracheal tube dislodgement leading to respiratory instability at the end of the surgical procedure. However, in cases in which carcinoid tumor is suspected, at least a systematic lymph node dissection should be performed.

In the case presented here, the immunohistochemical analysis revealed positivity for smooth muscle actin and NSE, and negativity for cytokeratin, chromogranin, TTF-1 and S100 protein. The main differential diagnosis of such an endobronchial lesion is carcinoid tumor, which is positive for chromogranin and TTF-1.(2) Although malignant behavior has been reported, glomus tumors are benign neoplasms and rarely recur after being completely excised.(2,11)

Despite being a rare pathologic entity, bronchial glomus tumor should be considered in the differential diagnosis of endobronchial lesions causing right upper lobe atelectasis.


References


1. Okitsu H, Oho K, Koshiishi Y, Abe S, Takakura H, Amemiya R, et al. A case of endobronchial glomus tumor [Article in Japanese]. J Jpn Soc Bronchol. 1985;7(3):312-7.

2. Gaertner EM, Steinberg DM, Huber M, Hayashi T, Tsuda N, Askin FB, et al. Pulmonary and mediastinal glomus tumors--report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review. Am J Surg Pathol. 2000;24(8):1105-14.

3. Oizumi S, Kon Y, Ishida T, Yamazaki K, Itoh T, Ogura S, et al. A rare case of bronchial glomus tumor. Respiration. 2001;68(1):95-8.

4. Yilmaz A, Bayramgurler B, Aksoy F, Tuncer LY, Selvi A, Uzman O. Pulmonary glomus tumour: a case initially diagnosed as carcinoid tumour. Respirology. 2002;7(4):369-71.

5. Vailati P, Bigliazzi C, Casoni G, Gurioli C, Saragoni L, Poletti V. Endoscopic removal of a right main bronchus glomus tumor. Monaldi Arch Chest Dis. 2004;61(2):117-9.

6. De Weerdt S, Noppen M, De Boosere E, Goossens A, Remels L, Meysman M, et al. Cough, fatigue and fever. Eur Respir J. 2004;23(5):786-9.

7. Takahashi N, Oizumi H, Yanagawa N, Sadahiro M. A bronchial glomus tumor surgically treated with segmental resection. Interact Cardiovasc Thorac Surg. 2006;5(3):258-60.

8. Akata S, Yoshimura M, Park J, Okada S, Maehara S, Usuda J, et al. Glomus tumor of the left main bronchus. Lung Cancer. 2008;60(1):132-5.

9. De Cocker J, Messaoudi N, Waelput W, Van Schil PE. Intrapulmonary glomus tumor in a young woman. Interact Cardiovasc Thorac Surg. 2008;7(6):1191-3.

10. Katabami M, Okamoto K, Ito K, Kimura K, Kaji H. Bronchogenic glomangiomyoma with local intravenous infiltration. Eur Respir J. 2006;28(5):1060-4.

11. Hishida T, Hasegawa T, Asamura H, Kusumoto M, Maeshima A, Matsuno Y, et al. Malignant glomus tumor of the lung. Pathol Int. 2003;53(9):632-6.



Study carried out in the Department of General Thoracic Surgery and in the Department of Pathology, Santa Marcelina Hospital, São Paulo, Brazil.
Correspondence to: Artur Eugênio de Azevedo-Pereira. Alameda Jauaperi, 1123, apto. 157, Indianópolis, CEP 04523-015, São Paulo, SP, Brazil.
Tel 55 11 7549-1017. Fax: 55 11 3069-5690. E-mail: artur.de.azevedo@hotmail.com
Financial support: None.
Submitted: 24 November 2009. Accepted, after review, 15 March 2010.



About the authors

Artur Eugênio de Azevedo-Pereira
General Thoracic Surgeon. Department of General Thoracic Surgery,

Santa Marcelina Hospital, São Paulo, Brazil.
Moacyr Pezati Rigueiro
Physician. Department of Pathology, Santa Marcelina Hospital, São Paulo, Brazil.

Fernando Conrado Abrão
General Thoracic Surgeon. Department of General Thoracic Surgery, Santa Marcelina Hospital, São Paulo, Brazil.