Rare disease
CASE REPORT
Intranasal lobular capillary haemangioma Dipak Ranjan Nayak,1 Ajay M Bhandarkar,1 Archana Shivamurthy,2 Jasmi Joy1 1
Department of Otolaryngology-Head and Neck Surgery, Kasturba Medical College, Manipal University, Manipal, Karnataka, India 2 Department of Pathology, Melaka Manipal Medical College, Manipal University, Manipal, Karnataka, India Correspondence to Dr Ajay M Bhandarkar, [email protected] Accepted 28 September 2014
SUMMARY Lobular capillary haemangioma (LCH) is a benign proliferation of capillaries with a characteristic lobular architecture on microscopy; it has an affinity for mucous membrane and skin of the head and neck. It is extremely rare in the nasal cavity. We present the case of a 45-year-old man who presented with epistaxis without any predisposing factors, which was diagnosed as lobular capillary haemangioma.
BACKGROUND Lobular capillary haemangioma (LCH) is a benign proliferation of capillaries with a characteristic lobular architecture on microscopy; it has an affinity for mucous membrane and skin of the head and neck.1 It is a rare entity in the nasal cavity. Most cases reported in the literature demonstrate a predisposing factor for the development of LCH. We present a case report of de novo development of LCH in a man with no predisposing factors.
CASE PRESENTATION A 45-year-old man presented to the outpatient department of our hospital with intermittent epistaxis of 6 months duration. There was bleeding of approximately 10 mL at every episode, which resolved spontaneously. This was not preceded by upper respiratory infection, bouts of sneezing, trauma or nose picking. The patient was not a known hypertensive or diabetic and did not suffer from a bleeding or a clotting disorder. He had a history of cardiac surgery for ischaemic heart disease 2 years prior and had stopped antiplatelet medication following the surgery 1 year prior to the presenting epistaxis. The rest of his clinical history was otherwise unremarkable. Diagnostic nasal endoscopy revealed a lobular growth of 2×2 cm arising from the left inferior turbinate, which was tender and bled on touch (figure 1). The ear, nose, throat and systemic examinations were unremarkable.
Figure 1 Diagnostic nasal endoscopy showing a lobular growth arising from the inferior turbinate.
OUTCOME AND FOLLOW-UP Histopathological examination demonstrated polypoidal growth surrounded by extensive vascular spaces and inflammatory cells suggestive of lobular capillary haemangioma (figures 3 and 4). The patient is on regular follow-up without any evidence of recurrence.
DISCUSSION Lobular capillary haemangioma, initially termed as ‘Botryomycosis humaine’ was described by Poncet and Dor in 1897.1 2 In 1904, Hartzell3 introduced the term pyogenic granuloma. However, the term pyogenic granuloma was proven to be a misnomer by Stacey Mills in 1980 when he demonstrated that the lesions produced by this pathological entity were neither pyogenic nor did they show a granulomatous appearance; hence it was correctly
INVESTIGATIONS Contrast-enhanced CT of the nose and paranasal sinuses revealed a 2×2 cm isoattenuating mass arising from the inferior turbinate with normal bony architecture (figure 2). Complete blood examination was essentially normal. To cite: Nayak DR, Bhandarkar AM, Shivamurthy A, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014207196
TREATMENT The patient underwent endoscopic excision of the tumour along with electrodessication of the tumour base and the specimen was sent for histopathological examination.
Figure 2 Contrast-enhanced CT showing an isoattenuating lobular mass arising from the left inferior turbinate.
Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
1
Rare disease
Figure 3 H&E staining (×40) showing a polypoidal growth with vascular proliferation. termed as LCH based on the distinct histological characteristics.4 Intranasal LCH is a rare entity. It was first reported in 1940.1 5 The various terminologies that are synonymous with LCH as described by various authors are pregnancy tumour, granuloma gravidarum, pregnancy granuloma, granuloma pyogenicum and telengiectatic polyp, because it is most commonly seen in pregnancy.5–7 LCH is most commonly seen in the third and fifth decades of life.2 It is seen more commonly in women and in men under 18 years of age.1 In women, it has been reported to be associated with pregnancy (2–5%),5 more commonly in the second and third trimesters with spontaneous regression following labour.5 7 In adults over 40 years of age, the male to female ratio is 1:1.5 The cutaneous variant of LCH is more common in men whereas the mucosal variant is more common in women.6 Sixty per cent of LCH occurs in the head and neck region.5 The most common site is the lip (38%) followed by nose (29%), oral mucosa (18%) and tongue (15%). In the nasal cavity, the commonly involved sites are the anterior septum (Little’s area), tip of inferior turbinate and the vestibule. In the oral cavity, the commonly involved sites are gingival, buccal mucosa, tongue and lips.1–3 5 Pre-existing trauma and pregnancy are the most common predisposing factors for the development of LCH. Nasal packing, insect sting and previous nasal surgeries have been described in the literature as the possible traumatic factors, but the underlying pathogenesis in such cases is unknown.1 2 5 6 However, exaggerated inflammatory response due to prolonged irritating traumatic foci leads to hyperplastic tissue formation, which may
be the possible pathogenetic mechanism in trauma.3 Pregnancy and women on oral contraceptives, as a cause, has been well documented and the underlying pathogenesis in the development of LCH is the high level of oestrogen and progesterone, which suggest that the tumour is hormone sensitive.1 2 5 Other pathogenetic mechanisms that may be responsible for LCH are angiogenic growth factor production, arteriovenous malformations and presence of viral oncogenes.5 6 Histopathology of LCH reveals the presence of extensive endothelial proliferation with prominent vascular spaces, lobular arrangement of capillaries, ulceration of the epithelium and a base that is made up of fibrovascular tissue.5–7 Epistaxis, nasal obstruction and protrusion of lesion from the anterior nares are the most common symptoms in LCH. Epistaxis occurs commonly from the Little’s area and the tip of inferior turbinate. The maxillary sinus roof has also been implicated as a source of epistaxis in a few cases. On examination, LCH may be solitary or multiple; and it may be a sessile or pedunculated pinkish mass that bleeds on touch.1 2 5 7 In pregnancy, it usually tends to regress after child birth whereas otherwise it may warrant surgical excision. Differential diagnosis for LCH includes benign lesions like nasal polyps, haemangioma, Wegeners granulomatosis, sarcoidosis, glioma, meningoencephalocele, fibroma, lipoma, nasopharyngeal cyst, osteoma, leiomyoma, histiocytoma or malignant tumours such as aesthesioneuroblastoma, angiosarcoma, squamous cell carcinoma, Kaposi sarcoma, lymphoma, achromic melanoma or adenocarcinoma.1 6–8 Owing to the diverse differential diagnosis for a mass that bleeds on touch, it becomes mandatory to investigate in order to pinpoint a diagnosis of LCH. CT scan demonstrates an iso/non/hyper attenuating soft tissue density with or without bony destruction. It has been noted that LCH usually respects bony architecture. MRI demonstrates T1 hypointensity and T2 hyperintensity in LCH. Bone erosion in LCH is mainly due to compressive devascularisation by the tumour.1 2 5 9 10 Angiography reveals the presence of increased vascularity due to the extensive feeding vessels to the tumour.3 Endoscopic excision with electrodessication of the tumour base is the treatment of choice in LCH.2 3 8 Embolisation has been found to be useful in select cases.5 Other modalities used with lower success rates are curettage, silver nitrate cauterisation and cryotherapy.8 Newer therapeutic measures being tried and tested are laser, application of sclerosing agents and alitretinoin gel.5 8 The recurrence rate of LCH is approximately 16%.3 Most often, it is due to incomplete excision of the tumour.3 5 The tumour, however, has been found to go into complete remission after pregnancy without any form of treatment.5 Complete resection with healthy mucosal margins as demonstrated on frozen section at the time of excision minimises the recurrence.6
Learning points
Figure 4 H&E staining (×200) showing vessels surrounded by inflammatory cells. 2
▸ Lobular capillary haemangioma of the inferior turbinate is uncommon. ▸ Pregnancy and pre-existing trauma are the most important predisposing factors. ▸ Contrast-enhanced CT of the nose and paranasal sinuses is essential to differentiate it from malignant lesions because it mimics malignant tumours. ▸ Endoscopic excision with electrodessication of base is the treatment of choice. ▸ Recurrence is common in case of incomplete excision. Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
Rare disease Acknowledgements The authors would like to thank Dr Balakrishnan for his constant support.
4
Contributors DRN reviewed the manuscript. AMB prepared the manuscript, reviewed the literature. JJ collected case data and images.
5
Competing interests None.
6
Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
7
REFERENCES
8
1 2 3
Karagama YG, Howarth K, Steel PRM, et al. Lobular capillary haemongioma of the nasal vestibule: a rare entity. Int J Pediatr Otorhinolaryngol 2002;66:71–5. Ifeacho SN, Caulfield HM. A rare cause of paediatric epistaxis: lobular capillary haemangioma of the nasal cavity. BMJ Case Rep 2011;2011:pii: bcr0720103199. Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: a review. J Oral Sci 2006;48:167–75.
9 10
Smith SC, Patel RM, Lucas DR, et al. Sinonasal lobular capillary hemangioma: a clinicopathologic study of 34 cases characterising potential for local recurrence. Head Neck Pathol 2013;7:129–34. Delbrouck C, Chamiec M, Hassid S, et al. Lobular capillary haemangioma of the nasal cavity during pregnancy. J Laryngol Otol 2011;125:973–7. Derkenne R, Coulet O, Varoquaux A, et al. Nasal cavity lobular capillary hemangioma due to insect sting. Eur Ann Otorhinolaryngol Head Neck Dis 2012;129:278–80. Miller FR, D’Agostino MA, Schlack K. Lobular capillary hemangioma of the nasal cavity. Otolaryngol Head Neck Surg 1999;120:783–4. Zarrinneshan AAZ, Zapanta PE, Wall SJ. Nasal pyogenic granuloma. Otolaryngol Head Neck Surg 2007;136:130–1. Lee DG, Lee SK, Chang HW, et al. CT features of lobular capillary hemangioma of nasal cavity. Am J Neuroradiol 2010;31:749–54. Lee GK, Suh KJ, Lee YH, et al. CT findings in two cases of lobular capillary haemangioma of the nasal cavity: focussing on the enhancement pattern. Dentomaxillofac Radiol 2012;41:165–8.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
3
CASE REPORT
Intranasal lobular capillary haemangioma Dipak Ranjan Nayak,1 Ajay M Bhandarkar,1 Archana Shivamurthy,2 Jasmi Joy1 1
Department of Otolaryngology-Head and Neck Surgery, Kasturba Medical College, Manipal University, Manipal, Karnataka, India 2 Department of Pathology, Melaka Manipal Medical College, Manipal University, Manipal, Karnataka, India Correspondence to Dr Ajay M Bhandarkar, [email protected] Accepted 28 September 2014
SUMMARY Lobular capillary haemangioma (LCH) is a benign proliferation of capillaries with a characteristic lobular architecture on microscopy; it has an affinity for mucous membrane and skin of the head and neck. It is extremely rare in the nasal cavity. We present the case of a 45-year-old man who presented with epistaxis without any predisposing factors, which was diagnosed as lobular capillary haemangioma.
BACKGROUND Lobular capillary haemangioma (LCH) is a benign proliferation of capillaries with a characteristic lobular architecture on microscopy; it has an affinity for mucous membrane and skin of the head and neck.1 It is a rare entity in the nasal cavity. Most cases reported in the literature demonstrate a predisposing factor for the development of LCH. We present a case report of de novo development of LCH in a man with no predisposing factors.
CASE PRESENTATION A 45-year-old man presented to the outpatient department of our hospital with intermittent epistaxis of 6 months duration. There was bleeding of approximately 10 mL at every episode, which resolved spontaneously. This was not preceded by upper respiratory infection, bouts of sneezing, trauma or nose picking. The patient was not a known hypertensive or diabetic and did not suffer from a bleeding or a clotting disorder. He had a history of cardiac surgery for ischaemic heart disease 2 years prior and had stopped antiplatelet medication following the surgery 1 year prior to the presenting epistaxis. The rest of his clinical history was otherwise unremarkable. Diagnostic nasal endoscopy revealed a lobular growth of 2×2 cm arising from the left inferior turbinate, which was tender and bled on touch (figure 1). The ear, nose, throat and systemic examinations were unremarkable.
Figure 1 Diagnostic nasal endoscopy showing a lobular growth arising from the inferior turbinate.
OUTCOME AND FOLLOW-UP Histopathological examination demonstrated polypoidal growth surrounded by extensive vascular spaces and inflammatory cells suggestive of lobular capillary haemangioma (figures 3 and 4). The patient is on regular follow-up without any evidence of recurrence.
DISCUSSION Lobular capillary haemangioma, initially termed as ‘Botryomycosis humaine’ was described by Poncet and Dor in 1897.1 2 In 1904, Hartzell3 introduced the term pyogenic granuloma. However, the term pyogenic granuloma was proven to be a misnomer by Stacey Mills in 1980 when he demonstrated that the lesions produced by this pathological entity were neither pyogenic nor did they show a granulomatous appearance; hence it was correctly
INVESTIGATIONS Contrast-enhanced CT of the nose and paranasal sinuses revealed a 2×2 cm isoattenuating mass arising from the inferior turbinate with normal bony architecture (figure 2). Complete blood examination was essentially normal. To cite: Nayak DR, Bhandarkar AM, Shivamurthy A, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014207196
TREATMENT The patient underwent endoscopic excision of the tumour along with electrodessication of the tumour base and the specimen was sent for histopathological examination.
Figure 2 Contrast-enhanced CT showing an isoattenuating lobular mass arising from the left inferior turbinate.
Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
1
Rare disease
Figure 3 H&E staining (×40) showing a polypoidal growth with vascular proliferation. termed as LCH based on the distinct histological characteristics.4 Intranasal LCH is a rare entity. It was first reported in 1940.1 5 The various terminologies that are synonymous with LCH as described by various authors are pregnancy tumour, granuloma gravidarum, pregnancy granuloma, granuloma pyogenicum and telengiectatic polyp, because it is most commonly seen in pregnancy.5–7 LCH is most commonly seen in the third and fifth decades of life.2 It is seen more commonly in women and in men under 18 years of age.1 In women, it has been reported to be associated with pregnancy (2–5%),5 more commonly in the second and third trimesters with spontaneous regression following labour.5 7 In adults over 40 years of age, the male to female ratio is 1:1.5 The cutaneous variant of LCH is more common in men whereas the mucosal variant is more common in women.6 Sixty per cent of LCH occurs in the head and neck region.5 The most common site is the lip (38%) followed by nose (29%), oral mucosa (18%) and tongue (15%). In the nasal cavity, the commonly involved sites are the anterior septum (Little’s area), tip of inferior turbinate and the vestibule. In the oral cavity, the commonly involved sites are gingival, buccal mucosa, tongue and lips.1–3 5 Pre-existing trauma and pregnancy are the most common predisposing factors for the development of LCH. Nasal packing, insect sting and previous nasal surgeries have been described in the literature as the possible traumatic factors, but the underlying pathogenesis in such cases is unknown.1 2 5 6 However, exaggerated inflammatory response due to prolonged irritating traumatic foci leads to hyperplastic tissue formation, which may
be the possible pathogenetic mechanism in trauma.3 Pregnancy and women on oral contraceptives, as a cause, has been well documented and the underlying pathogenesis in the development of LCH is the high level of oestrogen and progesterone, which suggest that the tumour is hormone sensitive.1 2 5 Other pathogenetic mechanisms that may be responsible for LCH are angiogenic growth factor production, arteriovenous malformations and presence of viral oncogenes.5 6 Histopathology of LCH reveals the presence of extensive endothelial proliferation with prominent vascular spaces, lobular arrangement of capillaries, ulceration of the epithelium and a base that is made up of fibrovascular tissue.5–7 Epistaxis, nasal obstruction and protrusion of lesion from the anterior nares are the most common symptoms in LCH. Epistaxis occurs commonly from the Little’s area and the tip of inferior turbinate. The maxillary sinus roof has also been implicated as a source of epistaxis in a few cases. On examination, LCH may be solitary or multiple; and it may be a sessile or pedunculated pinkish mass that bleeds on touch.1 2 5 7 In pregnancy, it usually tends to regress after child birth whereas otherwise it may warrant surgical excision. Differential diagnosis for LCH includes benign lesions like nasal polyps, haemangioma, Wegeners granulomatosis, sarcoidosis, glioma, meningoencephalocele, fibroma, lipoma, nasopharyngeal cyst, osteoma, leiomyoma, histiocytoma or malignant tumours such as aesthesioneuroblastoma, angiosarcoma, squamous cell carcinoma, Kaposi sarcoma, lymphoma, achromic melanoma or adenocarcinoma.1 6–8 Owing to the diverse differential diagnosis for a mass that bleeds on touch, it becomes mandatory to investigate in order to pinpoint a diagnosis of LCH. CT scan demonstrates an iso/non/hyper attenuating soft tissue density with or without bony destruction. It has been noted that LCH usually respects bony architecture. MRI demonstrates T1 hypointensity and T2 hyperintensity in LCH. Bone erosion in LCH is mainly due to compressive devascularisation by the tumour.1 2 5 9 10 Angiography reveals the presence of increased vascularity due to the extensive feeding vessels to the tumour.3 Endoscopic excision with electrodessication of the tumour base is the treatment of choice in LCH.2 3 8 Embolisation has been found to be useful in select cases.5 Other modalities used with lower success rates are curettage, silver nitrate cauterisation and cryotherapy.8 Newer therapeutic measures being tried and tested are laser, application of sclerosing agents and alitretinoin gel.5 8 The recurrence rate of LCH is approximately 16%.3 Most often, it is due to incomplete excision of the tumour.3 5 The tumour, however, has been found to go into complete remission after pregnancy without any form of treatment.5 Complete resection with healthy mucosal margins as demonstrated on frozen section at the time of excision minimises the recurrence.6
Learning points
Figure 4 H&E staining (×200) showing vessels surrounded by inflammatory cells. 2
▸ Lobular capillary haemangioma of the inferior turbinate is uncommon. ▸ Pregnancy and pre-existing trauma are the most important predisposing factors. ▸ Contrast-enhanced CT of the nose and paranasal sinuses is essential to differentiate it from malignant lesions because it mimics malignant tumours. ▸ Endoscopic excision with electrodessication of base is the treatment of choice. ▸ Recurrence is common in case of incomplete excision. Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
Rare disease Acknowledgements The authors would like to thank Dr Balakrishnan for his constant support.
4
Contributors DRN reviewed the manuscript. AMB prepared the manuscript, reviewed the literature. JJ collected case data and images.
5
Competing interests None.
6
Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
7
REFERENCES
8
1 2 3
Karagama YG, Howarth K, Steel PRM, et al. Lobular capillary haemongioma of the nasal vestibule: a rare entity. Int J Pediatr Otorhinolaryngol 2002;66:71–5. Ifeacho SN, Caulfield HM. A rare cause of paediatric epistaxis: lobular capillary haemangioma of the nasal cavity. BMJ Case Rep 2011;2011:pii: bcr0720103199. Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: a review. J Oral Sci 2006;48:167–75.
9 10
Smith SC, Patel RM, Lucas DR, et al. Sinonasal lobular capillary hemangioma: a clinicopathologic study of 34 cases characterising potential for local recurrence. Head Neck Pathol 2013;7:129–34. Delbrouck C, Chamiec M, Hassid S, et al. Lobular capillary haemangioma of the nasal cavity during pregnancy. J Laryngol Otol 2011;125:973–7. Derkenne R, Coulet O, Varoquaux A, et al. Nasal cavity lobular capillary hemangioma due to insect sting. Eur Ann Otorhinolaryngol Head Neck Dis 2012;129:278–80. Miller FR, D’Agostino MA, Schlack K. Lobular capillary hemangioma of the nasal cavity. Otolaryngol Head Neck Surg 1999;120:783–4. Zarrinneshan AAZ, Zapanta PE, Wall SJ. Nasal pyogenic granuloma. Otolaryngol Head Neck Surg 2007;136:130–1. Lee DG, Lee SK, Chang HW, et al. CT features of lobular capillary hemangioma of nasal cavity. Am J Neuroradiol 2010;31:749–54. Lee GK, Suh KJ, Lee YH, et al. CT findings in two cases of lobular capillary haemangioma of the nasal cavity: focussing on the enhancement pattern. Dentomaxillofac Radiol 2012;41:165–8.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Nayak DR, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207196
3
