- Case report
- Open Access
Malignant glomus tumor of the lung with multiple metastasis: a rare case report
© Wang et al.; licensee BioMed Central. 2015
Received: 28 June 2014
Accepted: 23 December 2014
Published: 7 February 2015
Malignant glomus tumor, or glomangiosarcoma, is a very rare mesenchymal neoplasm that, when seen, occurs in visceral organs. Despite having histologic features of malignancy, these tumors usually do not metastasize. However, when metastasis occurs, this disease is often fatal. Our report presents the case of a 59-year-old female patient with a highly aggressive and widely metastatic glomus tumor of the lung.
Glomus tumors are uncommon benign neoplasms that customarily originate from glomus bodies in the dermis or subcutis of the extremities. Extracutaneous glomus tumors occur but are very rare, especially in visceral organs such as the stomach, mediastinum, trachea, lung, ileum, and kidney [1-5]. A few cases of glomangiosarcoma in visceral organs have been reported; nevertheless, these all involved local invasion and metastasis occurred scarcely . Herein, we report a case with a highly aggressive and widely metastatic malignant glomus tumor of the lung.
Immunohistochemical characteristics of the tumor cells
Tumor cells from lung
Tumor cells from stomach
Rare weakly positive
Rare weakly positive
Rare weakly positive
Rare weakly positive
Ki-67 labeling index
Glomus tumors typically present as solitary or multicentric lesions in the dermis or subcutis, with characteristic subungual location. Extracutaneous presentations occur but are rare, especially in the visceral organs, where glomus bodies are sparse or even absent, including the respiratory tract, gastrointestinal tract, and mediastinum. Within the respiratory tract, the trachea is the most frequent site of involvement. Primary glomus tumors of the lung, especially with metastasis, are extremely rare. To the best of our knowledge, only three cases have been reported in the English literature. The first case of a glomangiomyoma of the lung with widespread metastasis was reported by Gaertner et al.  in 2000, in which the patient developed widespread metastatic disease to the lungs, upper mediastinum, brain, liver, and subcutaneous tissue of the lower limb after surgery, then she received chemotherapy and ultimately died 68 weeks after surgery. A decade later, Liu et al.  reported another case of primary malignant glomus tumor in the lung with multiple metastasis to the left lung, visceral pleura, adjacent bronchi, and the vessel walls. The patient also underwent surgery and chemotherapy, but died 4 days after surgery due to pulmonary failure. This was followed by Hohenforst-Schmidt et al. , who reported a case of glomus tumor in the left hilum of the lung, but multiple lung metastasis were evident at the 6-year follow-up visit; the patient was submitted to a pneumonectomy of the left lung and was disease free within in the surgical limits.
Surgical resection is curative and remains the mainstay of treatment in reported cases of malignant glomus tumor diagnosed with or without local metastasis , and long term follow-up is greatly recommended. Milia et al.  reported a successful outcome with combined radiotherapy and chemotherapy in a case of malignant glomus tumor in a 40-year-old man presenting with a lesion in the upper cervical region. In our case, the patient lost the opportunity for surgery given the widespread metastasis and did not undergo radiotherapy or chemotherapy because of asthenia, resulting in fatality.
Cases of primary pulmonary glomus tumors with metastasis
Gaertner et al. 
Lungs, mediastinum, brain, liver, lower limb
DOD at 68 weeks
Liu et al. 
Fever, dry cough, hemoptysis
Left lung, visceral pleura, the vessel walls, bronchi
DOD at 4 days
Hohenforst-Schmidt et al. 
FOD in surgical limits, N/A
Wang et al.
Cough, hemoptysis, melena
Lungs, stomach, jejunum, ileum, mediastinal nodes
DOD at 20 weeks
Written informed consent was obtained for publication of this report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Lamba G, Mohammed Rafiyath S, Kaur H, Khan S, Singh P, Hamilton AM, et al. Malignant glomus tumor of kidney: the first reported case and review of literature. Hum Pathol. 2011;42:1200–3.View ArticlePubMedGoogle Scholar
- Abu-Zaid A, Azzam A, Amin T, Mohammed S. Malignant glomus tumor (glomangiosarcoma) of intestinal ileum: a rare case report. Case Reports Pathol. 2013;2013:305321.Google Scholar
- Bo Yun H, Se Hyung K, Jin Young C, Sung Eun R, Min Woo L, So Yeon K, et al. Gastroduodenal glomus tumors: differentiation from other subepithelial lesions based on dynamic contrast-enhanced CT findings. AJR. 2011;197:1351–9.View ArticleGoogle Scholar
- Shang Y, Huang Y, Huang H-d, Bai C, Dong Y-c, Zhao L-j, et al. Removal of glomus tumor in the lower tracheal segment with a flexible bronchoscope: report of two cases. Inter Med. 2010;49:865–9.View ArticleGoogle Scholar
- Kleontas A, Barbetakis N, Asteriou C, Nikolaidou A, Baliaka A, Kokkori I, et al. Primary glomangiosarcoma of the lung: a case report. J Cardiothorac Surg. 2010;5:76.View ArticlePubMed CentralPubMedGoogle Scholar
- Kayal JD, Hampton RW, Sheehan DJ, Washington CV. Malignant glomus tumor: a case report and review of the literature. Dermatol Surg. 2001;27:837–40.PubMedGoogle Scholar
- Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol. 2001;25:1–12.View ArticlePubMedGoogle Scholar
- 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;24:1105–14.View ArticlePubMedGoogle Scholar
- Li L, Li H, Yongjian D, YanQing D. Malignant glomus tumour of the lung. Pathology. 2010;42:6.View ArticleGoogle Scholar
- Hohenforst-Schmidt W, Woitow M, Zarogoulidis P, Machairiotis N, Gschwendtner A, Huang H, et al. Glomus tumor in the lung parenchyma. J Thorac Dis. 2012;4:663–6.PubMed CentralPubMedGoogle Scholar
- Khoury T, Balos L, McGrath B, Wong MKK, Cheney RT, Tan D. Malignant glomus tumor: a case report and review of literature, focusing on its clinicopathologic features and immunohistochemical profile. Am J Dermatopathol. 2005;27:428–31.View ArticlePubMedGoogle Scholar
- Milia ME, Turri L, Beldi D, Deantonio L, Pareschi R, Krengli M. Multidisciplinary approach in the treatment of malignant paraganglioma of the glomus vagale: a case report. Tumori. 2011;97:225–8.PubMedGoogle Scholar
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.