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. 2020 Feb 13;13(2):e231926. doi: 10.1136/bcr-2019-231926

Primary pulmonary glomus tumour: a diagnostic challenge

Vikram Singh 1, Vikas Kumar 2, Harkant Singh 3, Nandita Kakkar 1,
PMCID: PMC7046395  PMID: 32060108

Abstract

Glomus tumour is uncommon benign tumour, which derives from the glomus cells present around arteriovenous anastomosis. These tumours are found most commonly in the deep dermis of the extremities. Primary pulmonary glomus tumour is extremely rare and often misdiagnosed. Knowledge about the existence of glomus tumour in the lung, its classic histomorphology and judicious use of immunohistochemistry can help us in clinching the correct diagnosis.

Keywords: lung cancer (oncology), pathology, surgical oncology

Background

We present a rare case of a primary pulmonary glomus tumour, in a 36-year-old woman, including its pathological features, and diagnostic utility of immunohistochemistry (IHC).

Case presentation

A 36-year-old woman came to the hospital with complaints of cough with expectoration. She was a non-smoker and had no history of respiratory diseases. On clinical examination, there was reduction of breath sound on the left side of the chest.

Investigations

Radiological findings are noted, in form of chest roentgenograph with abnormal round to oval relatively homogeneous, retrocardiac double density in the left lower lobe of the lung, approximately measuring 5×5 cm (figure 1A). CT scan of chest showed a large smoothly marginated heterogeneously enhancing mass in the left lower lobe of the lung, measuring 5×4×3.5 cm with no calcification and cystic area with in suggestive of benign lesion favouring carcinoid (figure 1B). There was no hilar or mediastinal lymphadenopathy present. Fibre optic bronchoscopy of the left lower lobe showed lateral basal segment completely occluded by the growth. Endobronchial biopsy was taken preoperatively that revealed a round cell tumour. IHC was attempted and tumour was negative for neuroendocrine markers (synaptophysin and chromogranin) and also for pan cytokeratin. In view of smooth muscle actin (SMA) immunopositivity, diagnosis of small round cell tumour favouring glomus tumour was offered.

Figure 1.

Figure 1

Radiological findings and image of the gross specimen (A) chest roentgenograph showing abnormal round to oval relatively homogeneous, retrocardiac double density in the left lower lobe of the lung, approximately measuring 5×5 cm (B) CT scan shows a large smoothly marginated heterogeneously enhancing mass in the left lower lobe of the lung with no calcification and cystic area (C) cut surface of lobectomy specimen showing a well circumscribed, greyish white tumour measuring 5×4×3.5 cm

Differential diagnosis

Prior to surgery the differential diagnosis of primary lung neoplasm with round cell morphology favouring glomus tumour over epithelioid gastrointestinal stromal tumour (GIST)was kept.

Treatment

Left lower lobectomy via left posterolateral thoracotomy was done. Intraoperatively, there was a large 5×5 cm mass seen with in the parenchyma of left lower lobe, without any extension into the left main bronchus. We received a lobectomy specimen measuring 11×6×3 cm. Overlying pleura was shiny. On serial slicing, a well-circumscribed, greyish white tumour was identified, measuring 5×4×3.5 cm (figure 1C). It was not involving the bronchus, overlying pleura or surgical resection margin. On microscopic examination, a well encapsulated tumour was appreciated arranged in the form of variably sized lobules (figure 2A). Within these lobules, tumour cells were arranged in sheets, trabeculae and in form of nests with intervening thin vascular network. The individual tumour cells were relatively monomorphic with distinct cell border, round to oval punched out nucleus with dispersed chromatin, inconspicuous nucleoli and moderate eosinophilic cytoplasm (figure 2B). Mitoses were not seen. Adjacent lung parenchyma showed fibrosis, focal intra-alveolar haemorrhage and pulmonary oedema (figure 2C). On performing IHC, these tumour cells were positive for alpha SMA (figure 2D) and vimentin(figure 2E), but negative for synaptophysin (figure 2F), chromogranin(figure 2G), pan cytokeratin (figure 2H), S-100 (figure 2I), CD 34(figure 2J), TTF-1 (figure 2K) and CD 117. Ki 67 index (figure 2L) was less than 2%. Based on the histology and IHC findings, case was diagnosed as a glomus tumour. Sections examined from the hilar lymph nodes showed reactive lymphoid hyperplasia.

Figure 2.

Figure 2

Photomicrographs of H&E and IHC stain (A) a well encapsulated tumour seen in the lung, arranged in the form of variably sized lobules (H&E stain, ×10); (B) tumour cells with distinct cell border, round to oval punched out nucleus with dispersed chromatin, inconspicuous nucleoli and moderate eosinophilic cytoplasm (H&E Stain, ×40) (C) pulmonary oedema noted in the adjacent lung parenchyma (H&E stain, ×20); (D–L)- photomicrograph showing IHC findings—tumour cells were positive for SMA (D) and vimentin (E), but negative for synaptophysin (F), chromogranin(G), pan cytokeratin (2 hours), S-100 (2I), CD 34 (J) andTTF-1(K). Ki 67 index (L) was less than 2%. IHC, immunohistochemistry; SMA, smooth muscle actin.

Outcome and follow-up

Postoperative period was event free. The patient is presently asymptomatic and on regular follow-up for past 1 year.

Discussion

The term glomus (Latin for ‘ball’ or spherical mass) was first described by Hoyer in 1877. Glomus tumour originates from the modified smooth muscle cells present in the wall of arteriovenous anastomosis involved with temperature regulation.1

The classic location of the glomus tumour is the subungual region, but it can occur elsewhere in the skin, soft tissues, nerves, stomach, nasal cavity and trachea.2

Glomus tumour arising in respiratory system is rare, in majority of cases trachea was most common site.3

Gaertner et al published a case series of five case comprising of four primary lung and one mediastinal glomus tumour. In their series, one of the primary glomus tumour was frankly malignant and reported as glomangiosarcomas.4

Patients of primary glomus tumours of lung are usually asymptomatic or present with history of cough as seen in this index case. In majority of patients, these tumours usually reveal a lung nodule, which are often found incidentally as a coin lesion on a chest roentgenograph, which leads to further investigation by the means of a CT and positron emission tomography (PET) scan similar to our case. Prior to surgery, the most common differential diagnoses for these lesions are non-small cell lung cancers. The CT scan often reveals a solitary lesion and the PET scan shows low or no metabolic activity. No cases have associated mediastinal lymphadenopathy or metastasis and therefore they often proceed to surgical resection.

The rarity of pulmonary and mediastinal glomus tumours, coupled with histological similarity to more common primary and metastatic lesions, makes this a challenging and easily overlooked diagnosis. Though predominantly benign, rarely glomus tumours can demonstrate aggressive and/or malignant clinical and histological features.Histologically, typical glomus tumours are subcategorised as solid glomus tumour, glomangioma or glomangiomyoma, depending on the relative prominence of glomus cells, vascular structures and smooth muscle. Malignant glomus tumours (glomangiosarcomas) are exceedingly rare and requires histological presence of frankly sarcomatous area in addition to benign glomus tumour as described in few published case reports .5 6

The majority of the glomus tumour are entirely benign and local recurrence is very uncommon. Folpe et al proposed the following criteria for malignant glomus tumour comprising deep location, size ≥20 mm, atypical mitotic figures, moderate to high nuclear grade and ≥5 mitotic figures per 50 high power fields.7

The possible list of differential diagnosis of pulmonary glomus tumour includes carcinoid tumour, haemangiopericytoma (HPC), sclerosing haemangioma, epithelioid GIST, leiomyoma and paraganglioma. In lung, carcinoids are often confused with glomus tumours for being more common and sharing a similar morphology, although on IHC, they show positivity for cytokeratin and neuroendocrine markers such as chromogranin A and synaptophysin and thus helps in differentiation from glomus tumour, as seen in our case. Glomus tumours are most typically positive for αSMA, vimentin and negative for CD31, cytokeratin and S100.8 Mravic et al found CD34 positivity in 32% of cases, which ranged from focal to diffuse in distribution.8

Sclerosing haemangiomas are positive for TTF-1, surfactant apoprotein A and cytokeratin. Paragangliomas are typically composed of round epithelioid cells with small nuclei and express neuroendocrine markers and S-100 protein. Solitary fibrous tumour (SFT)/HPC belongs to same family and often can be confused with glomus tumour. NGFI-A binding protein 2 (NAB2)-signal transducer and activator of transcription 6 (STAT6) fusion gene is the hallmark of SFTs/HPCs family. NAB2-STAT6 fusion gene could be rapidly detected by STAT6 IHC, which exhibits strong nuclear expression. The diffuse CD34, staining pattern and nuclear STAT-6 expression on IHC is helpful in differentiating SFT from glomus tumours.9

In primary glomus tumour, surgical resection is main stay of treatment, however, the treatment modality depends on the extent of the disease. Surgical sleeve resection is particularly applicable to tumours of the main stem bronchi and trachea. Video-assisted thoracoscopic resection can also be done and has better postoperative pain profile and shorter hospital stay than open procedure. The extent of resection depends on the pulmonary segments involved. Wedge resection is done if the tumour is small and localised10 otherwise segmentectomy, lobectomy or pneumonectomy may be required.10–12 However, if the tumour is completely confined to the bronchus, it can be managed by bronchoscopic techniques like extraction by the forceps, cryotherapy or electrocoagulation.13 The treatment of benign tumour requires complete, not necessarily wide, excision unlike for malignant tumour that necessitates wide excision.14

The glomus tumour is usually benign with good long-term prognosis without recurrence. However, the malignant tumours show recurrence and local invasiveness with rare distant metastasis.

Learning points.

  • Glomus tumour is an extremely rare tumour of the lung.

  • This tumour is often misdiagnosed, as a result of their rarity and the variety of the clinical symptoms.

  • Knowledge about the existence of glomus tumour in the lung, classic histomorphology and judicious use of immunohistochemistry can help us in clinching the correct diagnosis.

  • The glomus tumour is usually benign with good long-term prognosis without recurrence. However, the malignant tumours show recurrence and local invasiveness with rare distant metastasis.

Footnotes

Contributors: VS and NK are pathologist, conceptualised and prepared the manuscript. HS and VK are treating cardiothoracic surgeon and supervised the manuscript.

Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

Competing interests: None declared.

Patient consent for publication: Obtained.

Provenance and peer review: Not commissioned; externally peer reviewed.

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