A rare case of solitary pulmonary cavernous haemangioma with non-resolving recurrent haemoptysis

Abstract

A 24-year-old male presented with history of recurrent unresolving haemoptysis since 1.5 years. He underwent repeated hospitalisation. Initial computed tomography (CT) scan was suggestive of pneumonitis of the left lower lobe and was treated conservatively with broad-spectrum antibiotics. He had no past history of pulmonary tuberculosis. On presentation at our hospital, a repeat CT pulmonary angiogram was done which showed a low attenuating mass lesion in the left lower lobe with eccentric calcification supplied by branches from the left pulmonary artery. Few dot-like enhancing pulmonary veins are noted within the lesion. He underwent left lower lobectomy. Post-operative period was uneventful and patient was discharged on the ninth post-operative day. On histopathology, the diagnosis was primary haemangioma arising from the lung parenchyma which is a rare benign lung tumour reported worldwide and probably for the first time from India.

Introduction

A cavernous haemangioma is a benign vascular tumour that is extremely uncommon in the lung. Most of the literature is based on single-case reports. Multiple pulmonary and cardiac with pulmonary haemangiomas are still mentioned in the literature but isolated pulmonary haemangioma is a rare occurrence.

Case report

A 24-year-old male presented to the hospital with recurrent haemoptysis since 1.5 years. Patient had 8–10 episodes of haemoptysis. During each episode, he coughed out about half cup of fresh blood with small amount of frothy sputum leading to repeated visits to a local physician. He was admitted and treated conservatively with antibiotics. Patient had no past history of pulmonary tuberculosis. General examination did not reveal any telangiectatic lesions over the face or other parts of the body. Physical examination of respiratory and cardiac system was otherwise normal. No bruit or murmur heard. Pulse oximetry showed 100% saturation in room air.

His initial CT scan of the thorax was suggestive of pneumonitis of the left lower lobe. Despite medical management, haemoptysis recurred. Hence, he was admitted at RG Kar Medical College, Kolkata. A repeat CT pulmonary angiogram was done which was suggestive of low attenuating mass lesion 2.2 × 1.8 cm with eccentric calcification in the left lower lobe, supplied by a branch from the left pulmonary artery along with few dot-like enhancing pulmonary veins noted within the lesion (Fig. 1). Owing to CT features characteristic of haemangioma, no further tissue diagnosis was attempted in the form of lung biopsy and immunohistochemistry. Preoperative checklist was done—pulmonary function test was normal with FEV1 2.1 L. 2D echocardiography showed no obvious abnormality.

Fig. 1.

Low attenuating mass lesion 2.2 × 1.8 cm with eccentric calcification in the left lower lobe, supplied by a branch from the left pulmonary artery along with few dot-like enhancing pulmonary veins noted within the lesion

Initially, the patient was put on conservative management. Preoperative bronchoscopic evaluation revealed no mass lesion but some specks of blood in the left lower lobe bronchus. An attempt of embolisation was done by the interventional cardiologist. But, due to absence of clear window of the feeding vessel, a decision of surgery was taken.

He underwent left posterolateral thoracotomy and left lower lobectomy as it was a mass lesion, malignancy and inflammatory pathology being the other aetiologies to rule out. Intraoperatively, few adhesions of the lung with the parietes were released. A solid approximately 3 × 3cm mass was palpable in the left lower lobe abutting the fissure (Fig. 2). A standard lobectomy was done by securing the vessels followed by excision at the level of the left lower lobar bronchus. No separate feeding vessel was noted. No other lesion was noted in the lung, parietes and mediastinum. No enlarged mediastinal lymph nodes were seen. During the surgery, around 300 mL blood loss was noted. Recovery was uneventful with no air leak and with an intensive treatment unit (ITU) stay of 2 days and hospital stay of 7 days.

Fig. 2.

Intraoperative picture showing adhesions of the left lower lobe with diaphragm. Beneath the adhesions, a solid mass with numerous small feeding vessels was present abutting the oblique fissure

The histopathology revealed the presence of irregular dilated thin-walled vascular channels lined by flattened endothelium containing red blood cells infiltrating between the pulmonary parenchyma; findings consistent with cavernous haemangioma (Fig. 3). The bronchial stump and hilar lymph node showed no obvious pathology. Immunohistochemistry was not recommended by the pathologist owing to characteristic histopathological picture.

Fig. 3.

H&E stain of cavernous haemangioma showing bronchial epithelium lined by smooth muscle surrounded by compressed alveoli along with dilated capillaries containing RBC

Discussion

Benign lung tumours represent 2–5% of primary lung neoplasm with hamartoma making up the vast majority of cases. Cavernous haemangiomas of the lung, on the other hand, are exceedingly rare [1]. To date, approximately 26 cases of pulmonary cavernous haemangioma (PCH) have been reported [2], none from India. It can affect the parenchyma as well as the airway [3]. Clinical presentations depend on the lesion’s location, number and size [3]. Some patients present with clinical respiratory distress, cyanosis, haemoptysis and even heart failure [3]. Some patients show no apparent symptoms [3]. A review of the literature shows that pulmonary cavernous haemangioma affects a wide age range of patients from 7 weeks to 84 years [1]. Haemangiomas affect both sexes equally [1]. Seven of these patients had haemoptysis as the presenting feature and the remaining were asymptomatic or incidentally detected on autopsy. Seven patients out of 26 had multiple lung nodules [1]. Our patient was 24 years old with recurrent haemoptysis and a solitary mass in the left lung lower lobe. The patients of Maeda [6], Sirmali [4] and their associates were both 54-year-old males; one patient was asymptomatic and the other presented with haemoptysis [5]. Both had a solitary lesion in the lung. In patients of Maeda and Sirmali, in the asymptomatic one, the CT scan showed an ill-defined mass, 4 × 3 cm, with tiny calcifications in the left lung hilum; in the other, there was a well-defined mass 4 × 3 cm in the left upper lobe [4]. Both showed dilated blood vessels on histology and both underwent surgical excision [2, 5, 6]. Our patient had a well-defined solitary mass in the left lower lobe which was low attenuating along with specks of calcification at the hilum and in the wall of the mass. He was subjected to lower lobectomy as it was a mass lesion abutting the oblique fissure, with adhesions and with a small feeding vessel from the left pulmonary artery within the fissure. Also, despite characteristic CT picture, malignancy and inflammatory pathology were still being considered as differential diagnoses. This has been well supported by Chen et al. in 2014 in a similar article [3].

Generally, most of PCHs are solitary lesions and there have been no radiological characteristic findings [1]. The preoperative diagnosis is difficult because pulmonary biopsy is often non-diagnostic. There is also a risk of bleeding after fine needle or bronchoscopic biopsy [2]. Therefore, confirmatory diagnosis can be done only by histopathological examination. 3D printing, a relatively new technique that is not widely available, is also being used for the diagnosis of pulmonary vascular lesions [8]. The use of 3D helical computed tomography is limited by time, prolonged breath holding and the inability to visualise large pulmonary arteriovenous malformations (PAVMs). Additionally, there have been case reports of the false-positive diagnosis of PAVMs by computed tomography [7]. So, a contrast-enhanced computed tomography scan with angiogram is a useful, widely available diagnostic tool in such and also to rule out any coexisting process [8]. Magnetic resonance imaging has limited use. Because of limitations and unavailability of 3D printing facility, we did CT pulmonary angiogram.

In infants and young children, intracystic haemorrhage within a congenital cystic adenomatoid malformation may suggest a vascular lesion [9]. PAVMs seen in Osler-Weber-Rendu syndrome (hereditary haemorrhagic telangiectasia (HHT)) are also detected in this population and have systemic manifestations like multiple cutaneous telangiectasia and haemangiomas in various organs [3, 9]. These are slow-flow true PAVMs [3, 5]. Our patient had isolated cavernous haemangioma in the lung with no systemic manifestations in any form, i.e. non-HHT variant.

In older children and adults, Kaposi sarcoma, angiosarcoma and epithelioid haemangioendothelioma must be excluded. Kaposi sarcoma usually occurs in immunocompromised patients and is characterised by a spindle cell proliferation with mild to moderate nuclear atypia and a low mitotic rate surrounding vascular slits filled with extravasated red blood cells [9]. In the lung, it may grow around large blood vessels or display a lymphatic distribution with widening of the alveolar septa, features not favouring PCH [9]. Primary angiosarcomas of the lung are rare and a metastatic lesion should be suspected [9]. Histologically, angiosarcoma is characterised by atypical cells lining anastomosing vascular channels [9]. The sheet-like pattern of growth differentiates it from PCH and in rare cases with widely dilated anastomosing spaces mimicking cavernous haemangioma, in which the presence or absence of cellular atypia is the distinguishing feature [9]. Epithelioid haemangioendothelioma is rare multifocal vascular neoplasm characterised by proliferation of round to oval cells with abundant eosinophilic cytoplasm and large nuclei in a myxoid or hyaline stroma and these spaces should not be confused with the expanded vascular channels identified in PCH [9].

When cavernous haemangioma is suspected, it is necessary to determine whether the lesion is associated with the endothelium. Histologically, they are characterised by the formation of large, dilated vascular channels separated by connective tissue stroma [9]. Masses are sharply defined but not encapsulated, are variably filled with blood and may display intravascular thrombosis and dystrophic calcification, too [9]. There should be infiltration of the pulmonary parenchyma by the haemangiomatous lesion as was found in our patient. The diagnosis is well supported by further immunohistochemistry.

Solitary cavernous haemangioma should be treated with surgical excision [3]. One group has reported using interferon alfa-2a to successfully treat a 7-year-old patient with a PCH associated with respiratory distress and haemoptysis [7]. These cases suggest that recognition of PCH as part of the differential diagnosis of pulmonary lesions is important, as both surgical resection [3–5, 9] and other treatments may be curative.

Conclusion

Cavernous haemangiomas are rare lesions of the lungs. They can be asymptomatic or can present with life-threatening symptoms such as massive haemoptysis. The choice of treatment of pulmonary cavernous haemangioma is surgical resection.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

The above paper has been done with full permission of the ethics committee. A copy of written ethical approval will be provided for review by the Editor-in-Chief of this journal.

Informed consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.