Glomangioma of the lung: a case report and review of the literature
© Gebauer et al.; licensee BioMed Central Ltd. 2014
Received: 17 December 2012
Accepted: 11 October 2013
Published: 3 January 2014
Glomangiomas are rare soft tissue tumors originating from the perivascular tissue. The most common localization is in the dermis of the extremities, with a few reports of respiratory tract involvement.
We present the case of a 48-year-old Caucasian female patient with a glomangioma in her left lung. It was diagnosed incidentally as a coin lesion in a chest X-ray performed during preoperative work-up for a gastric Roux-en-Y bypass for alimentary obesity. A computed tomography scan of her chest revealed a lesion in her upper left lung lobe 31mm in diameter. After resection, a histopathological examination presented typical signs of a glomangioma, originating from the pulmonary parenchyma.
Glomangiomas of the lung are extremely rare. However, whenever incidental lesions in the lung parenchyma are found, glomangioma should be taken into diagnostic consideration. To the best of our knowledge, signs of malignancy have not previously been reported in the literature. In fact, this tumor entity shows benign behavior, with a low potential for recurrence after complete resection.
KeywordsGlomangioma Glomus tumor Pulmonary tumor Thoracic surgery
A 48-year-old Caucasian female patient presented with an incidental finding of a solitary pulmonary lesion in a chest X-ray during preoperative work-up for a Roux-en-Y gastric bypass for alimentary obesity. Her medical history was unremarkable, except for obesity (body mass index, 42kg/m2) and arterial hypertension.
Glomangioma are derived from glomus bodies, and comprise less than 2% of all soft tissue tumors [1, 4]. Glomus bodies are arteriovenous anastomoses, which are associated with blood flow and temperature regulation.
The histopathological characteristics of glomangiomas are very close to those of glomus bodies. Both are composed of epithelioid smooth-muscle cells, which are typically arranged in sheets and nests around the blood vessels. Within the glomus tumor family, different types of tumors have been described according to their morphological presentation. The glomus tumor itself consists predominantly of glomus cells, whereas the glomangioma presents with an extremely high density of vascularity, including dilated blood vessels or cavernous blood spaces. In a histological examination, the differential diagnosis must exclude several different tumors, such as carcinoid tumors, hemangiopericytomas, sclerosing hemangiomas, leiomyomas and paragangliomas. In general, each tumor entity can be definitively identified by their specific immunohistochemical staining pattern. For example, carcinoid tumors are sometimes mistaken for glomus tumors because they have a comparable conventional histological presentation. However, carcinoid, but not glomus, tumors are positive for cytokeratin and neuroendocrine markers such as chromogranin A and synaptophysin. Hemangiopericytomas are positive for vimentin and CD34, but negative for cytokeratin and smooth muscle markers. Paragangliomas are typically composed of round epithelioid cells with small nuclei, and express neuroendocrine markers and S-100 protein.
Primary glomangiomas and glomus tumors of the lung are extremely rare - to the best of our knowledge, only 30 cases have been described in the literature [3, 5, 6]. The majority of these cases are reports of glomus tumors; glomangiomas in the lung have been described in only five cases [1, 3, 7].
The biological behavior of glomangiomas seems to be benign according to the follow-up data available. So far, follow-up data is accessible for four patients ranging from 9 to 60 months after surgery, without evidence of a relapse [1, 3]. Malignant glomus tumors have been described in the literature in only six cases. Those tumors showed characteristics of a glomangiosarcoma with infiltrative growth and cellular atypia [5, 8–10]. However, lymph node metastases have not been observed in any of the cases.
In our opinion, due to their uncertain biological behavior, resection of these tumors is warranted once they have been diagnosed. Preoperative imaging and biopsy seldom lead to a conclusive diagnosis, because glomangiomas do not present with specific characteristics in CT or magnetic resonance imaging, and utility of FDG-PET is also of limited value. In none of the reported cases was a correct diagnosis possible preoperatively.
Whether an anatomical lobe resection with lymphadenectomy or an atypical wedge resection is the adequate method for surgical treatment of glomangioma remains unanswered. In our opinion, the wedge resection seems to be adequate if no malignancy is seen in the frozen sections. Otherwise oncological surgical standards should be applied similar to the treatment of non-small cell lung cancer.
Glomangiomas of the lung are rare tumors, and only a few cases have been reported in the literature. Even though glomangiomas are typically benign, invasive subtypes, such as glomangiosarcoma, have been reported. Glomangiomas of the lung are usually diagnosed incidentally and should always be resected as the behavior cannot be predicted by preoperative examinations. While a complete surgical resection of the tumor is mandatory, a pulmonary wedge resection should be adequate. Even though intraoperative frozen sections may help to determine tumor-free surgical resection margins, a definitive diagnosis from frozen sections is often not possible.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
18Fluorodeoxyglucose positron emission tomography.
Thanks to Michael Tachezy and Guido Sauter for revising the manuscript. None of the authors were funded for this particular case presentation.
- Gaertner EM, Steinberg DM, Huber M, Hayashi T, Tsuda N, Askin FB, Bell SW, Nguyen B, 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 Pathol. 2000, 24 (8): 1105-1114. 10.1097/00000478-200008000-00008.View ArticlePubMedGoogle Scholar
- Dalfior D, Parisi A, Cannizzaro C, Bontempini L, Di Pace C, Menestrina F, Dalla Palma P, Barbareschi M: Pulmonary glomus tumor. Int J Surg Pathol. 2008, 16 (1): 81-84. 10.1177/1066896907304520.View ArticlePubMedGoogle Scholar
- Santambrogio L, Nosotti M, Palleschi A, Gazzano G, De Simone M, Cioffi U: Primary pulmonary glomangioma: a coin lesion negative on PET study. Case report and literature review. Thorac Cardiovasc Surg. 2011, 59 (6): 380-382. 10.1055/s-0030-1250577.View ArticlePubMedGoogle Scholar
- Ariizumi Y, Koizumi H, Hoshikawa M, Shinmyo T, Ando K, Mochizuki A, Tateishi A, Doi M, Funatsu M, Maeda I, Takagi M: A primary pulmonary glomus tumor: a case report and review of the literature. Case Rep Pathol. 2012, 2012: 782304-PubMedPubMed CentralGoogle Scholar
- Hohenforst-Schmidt W, Woitow M, Zarogoulidis P, Machairiotis N, Gschwendtner A, Huang H, Dramba V, Lorenz K, Goeckenjan G, Koebe HG, Altmannsberger HM, Brachmann J: Glomus tumor in the lung parenchyma. J Thorac Dis. 2012, 4 (6): 663-666.PubMedPubMed CentralGoogle Scholar
- Huang Y, Chen K, Sun K, Cui J, Chen Y, Min X, Liu J, Wang J: A primary pulmonary glomus tumor complicated with hyperpyrexia and anemia. Ann Thorac Surg. 2013, 95 (2): e29-e31. 10.1016/j.athoracsur.2012.08.117.View ArticlePubMedGoogle Scholar
- Tang CK, Toker C, Foris NP, Trump BF: Glomangioma of the lung. Am J Surg Pathol. 1978, 2 (1): 103-109. 10.1097/00000478-197803000-00011.View ArticlePubMedGoogle 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): 1-12. 10.1097/00000478-200101000-00001.View ArticlePubMedGoogle Scholar
- Hishida T, Hasegawa T, Asamura H, Kusumoto M, Maeshima A, Matsuno Y, Suzuki K, Kondo H, Tsuchiya R: Malignant glomus tumor of the lung. Pathol Int. 2003, 53 (9): 632-636. 10.1046/j.1440-1827.2003.01521.x.View ArticlePubMedGoogle Scholar
- Gould EW, Manivel JC, Albores-Saavedra J, Monforte H: Locally infiltrative glomus tumors and glomangiosarcomas. A clinical, ultrastructural, and immunohistochemical study. Cancer. 1990, 65 (2): 310-318. 10.1002/1097-0142(19900115)65:2<310::AID-CNCR2820650221>3.0.CO;2-Q.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.