Abstract
Vascular tumors of the nasal cavity can represent a variety of pathologies. In this case report, we discuss two patients presenting with a large vascular lesion occupying the nasal cavity. Significant bleeding was encountered during the initial attempts for endoscopic surgical resection. One lesion was successfully excised following preoperative embolization while a second following sphenopalatine artery vascular ligation. In both cases, final pathology showed lobular capillary hemangioma (LCH). We present a literature review and discussion of LCH and other vascular tumors that present in the nasal cavity. In addition, we discuss the utility of pre-resection vascular control of these tumors.
1
Introduction
There are numerous types of vascular tumors that can develop in the nasal cavity. Nasal tumors often present with non-specific characteristics of nasal congestion, rhinorrhea, epistaxis, or anosmia. Less common manifestations include vision changes, headaches, local swelling or pain. Imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI) are valuable for surgical planning, but histology is needed for definitive diagnosis. Recommended treatment varies based on the presenting pathology.
Histology includes lobular capillary hemangioma (LCH), hemangiopericytoma, angiofibroma, leiomyoma, glomus tumor, angiosarcoma and kaposi’s sarcoma . In this paper, we discuss two cases of a patient presenting with a nasal cavity mass with final pathology showing LCH as well as provide a review of vascular nasal cavity tumors included in the differential diagnosis.
2
Case report 1
A 60 year-old female presented to clinic with 4 months of left nasal passage obstruction. She had been experiencing intermittent nasal bleeding that occurred with manipulation of the left nasal cavity. She also endorsed posterior nasal drainage and significantly decreased sense of smell. On exam, there was complete obstruction of the left nasal cavity with a vascular appearing mass ( Fig. 1 ).
A CT scan was obtained demonstrating a large nasal cavity mass with remodeling of the surrounding structures and mucosal inflammation within the left paranasal sinuses. The patient was brought to the operating room for resection. In the operating room, any manipulation of the tumor resulted in significant bleeding. After approximately 800 ml of blood loss, the tumor had only been debulked about 1 cm and procedure was aborted. Pathology returned as acute on chronic inflammation and granulation tissue proliferation with no evidence of neoplasm.
Given the significant blood loss and benign preliminary pathology, we allowed 6 weeks to lapse to allow recovery from the blood loss. Subsequently, the patient underwent preoperative embolization of the left internal maxillary artery ( Fig. 2 ) and returned to the operating room the following day for definitive resection. Although approximately 1200 ml of blood was lost and the patient was transfused 3 units of packed red blood cells, bleeding was much less brisk and manageable compared to during the initial surgical attempt. The lesion appeared to be emanating from the superior aspect of the middle turbinate with a prominent arterial supply at that level. Upon resection of the posterior aspect, there was minimal bleeding, consistent with embolization of the internal maxillary artery. Once the arterial supply emanating from the middle turbinate region was cauterized, there was no further significant bleeding from the tumor ( Fig. 3 ). Final pathology showed a proliferative vascular lesion with acute on chronic inflammation consistent with LCH. Patient recovered well and has no evidence of residual or recurrent neoplasm at last follow up of 6 months.
2
Case report 1
A 60 year-old female presented to clinic with 4 months of left nasal passage obstruction. She had been experiencing intermittent nasal bleeding that occurred with manipulation of the left nasal cavity. She also endorsed posterior nasal drainage and significantly decreased sense of smell. On exam, there was complete obstruction of the left nasal cavity with a vascular appearing mass ( Fig. 1 ).
A CT scan was obtained demonstrating a large nasal cavity mass with remodeling of the surrounding structures and mucosal inflammation within the left paranasal sinuses. The patient was brought to the operating room for resection. In the operating room, any manipulation of the tumor resulted in significant bleeding. After approximately 800 ml of blood loss, the tumor had only been debulked about 1 cm and procedure was aborted. Pathology returned as acute on chronic inflammation and granulation tissue proliferation with no evidence of neoplasm.
Given the significant blood loss and benign preliminary pathology, we allowed 6 weeks to lapse to allow recovery from the blood loss. Subsequently, the patient underwent preoperative embolization of the left internal maxillary artery ( Fig. 2 ) and returned to the operating room the following day for definitive resection. Although approximately 1200 ml of blood was lost and the patient was transfused 3 units of packed red blood cells, bleeding was much less brisk and manageable compared to during the initial surgical attempt. The lesion appeared to be emanating from the superior aspect of the middle turbinate with a prominent arterial supply at that level. Upon resection of the posterior aspect, there was minimal bleeding, consistent with embolization of the internal maxillary artery. Once the arterial supply emanating from the middle turbinate region was cauterized, there was no further significant bleeding from the tumor ( Fig. 3 ). Final pathology showed a proliferative vascular lesion with acute on chronic inflammation consistent with LCH. Patient recovered well and has no evidence of residual or recurrent neoplasm at last follow up of 6 months.
3
Case report 2
A 36 year-old female presented with 6 months left sided nasal airway obstruction and left maxillary region pressure with recurrent episodes of sinusitis. The patient was initially taken to the operating room by a community otolaryngologist for resection. Unfortunately, significant intraoperative bleeding was encountered and the procedure was aborted. Tissue specimen sent from this procedure was consistent with a benign vascular lesion. The patient was referred to our institution for further care.
CT showed a soft tissue mass appearing to arise from the left inferior turbinate. Exam showed a vascular lesion along the mid aspect of her inferior turbinate with some extension into the medial aspect of the maxillary sinus. The patient was taken to the operating room for excision of her left nasal mass. Left sphenopalatine artery ligation was initially performed and the lesion was then grossly resected in conjunction with the involved inferior turbinate. At no time was there significant bleeding encountered and the final blood loss was 50 ml. Final pathology returned as a benign vascular lesion consistent with LCH.
4
Discussion
4.1
Vascular tumors of the nasal cavity
Nasal LCHs are benign growths that can occur in the nasal cavity and paranasal sinuses. They arise from vascular endothelial cells and have capillaries arranged in a characteristic lobular pattern . Historically, these lesions were referred to as pyogenic granulomas, but this is a misnomer given that they are neither infectious nor granulomatous. They are the most common vascular tumor of the nasal cavity . These tumors can often grow to a large size and can sometimes be misdiagnosed as nasopharyngeal angiofibroma (NA). Grossly, these lesions appear as red, exophytic masses and frequently present with bleeding.
In the head and neck, the most common presenting location are the lips (38%) . The nasal cavity represents 7 to 29% of head and neck LCH . Within the nasal cavity, the anterior nasal septum and the tip of the turbinate are the most common sites . The etiology of these lesions is unknown. There appears to be a hormonal component as these lesions occur commonly in pregnancy and in patients on oral contraceptive pills . There are also reports of development of LCH following local trauma .
On CT, LCH typically presents as a well-defined soft tissue mass, sometimes demonstrating bony destruction. The mass shows intense enhancement with contrast. MRI shows intermediate signal on T1 and heterogeneous signal with flow voids on T2. The tumor shows intense enhancement with gadolinium . Treatment is total excision, and can often be done endoscopically. Unfortunately, recurrence rate after excision can be as high as 15% .
Hemangiopericytomas clinically present as a slowly expanding whitish and rubbery solitary mass that can often be confused with a nasal polyp. A reported 15 to 25% of hemangiopericytomas occur in the head and neck, and less than 5% appear in the nose and paranasal sinuses . The median age of presentation is 46 years old . Histologically, hemangiopericytomas are proliferations of pericytes, the spindle shaped cells covering the outer wall of capillaries . Nasal hemangiopericytoma can be classified as benign or malignant based on histologic and clinical features. Metastasis can be seen in 5% of cases . Recommended treatment involves wide local excision with negative margins. Local recurrence rate is about 25% . Some research has reported that radiotherapy followed by chemotherapy has positive efficacy for recurrent and invasive sinonasal hemangiopericytomas . Lifelong follow-up is important in these tumors given the risk of recurrence, even after extended periods of time . Tumor grade dictates prognosis, as a higher mortality rate is associated with higher tumor grade .
Angiofibromas include subsets of nasopharyngeal angiofibromas and extranasopharyngeal angiomas. Nasopharyngeal angiofibromas (NA) are well characterized vascular lesions classically found in adolescent males, often between ages 6 to 26. The classic triad of symptoms are unilateral nasal obstruction, epistaxis, and a nasopharyngeal mass. These masses frequently arise at the posterolateral wall near the sphenopalatine foramen and grow into the nasopharynx . Histologically, they appear as non-encapsulated tumors containing vasculature surrounded by a fibroblastic stroma. They lack the muscular layer of the blood vessel and can have profuse bleeding when the lesion is manipulated . Optimal treatment for NA is gross total resection . Radiotherapy may also play a role in treatment of unresectable and/or locally advanced cases . Tumor recurrence rate varies from 10 to 55% .
Extranasopharyngeal angiofibromas (ENA) are angiofibromas that arise at sites other than the nasopharynx including the nasal cavity and paranasal sinuses. It is thought to be a distinct entity from NA. Unlike NAs, ENAs are seen in adults (often between ages 22–28), frequently reported in females, and less vascular . Treatment of ENA is surgical excision. However, unlike NA, the need for preoperative vascular embolization is less frequent given that they tend to have more fibroblastic components and less vascularity in addition to being smaller in size compared to NA .
Vascular leiomyomas, or angioleiomyomas, are benign soft tissue tumors arising from smooth muscle. These tumors often occur in the uterus (95%), skin (3%) and gastrointestinal tract (1.5%) . The nasal cavity accounts for 3% of vascular leiomyomas . Like LCH, angioleiomyomas growth is thought to be affected by sex hormones, with recent reports of estrogen and progesterone receptors being expressed in angioleiomyomas . Nasal angioleiomyomas were previously shown to have a 2:1 prevalence in females and most commonly occur between age 40 and 60 . A recent review reported that both genders are affected equally with a mean age of 56 and 55 years for women and men, respectively . Grossly, they appear as a small solitary cutaneous mass often arising from the inferior turbinate, nasal septum, and nasal vestibule . The turbinates are affected most frequently, with most lesions presenting as sessile or polypoid well-circumscribed, non-encapsulated masses ranging from 0.2 to 4 cm . There are various hypotheses to the origin of vascular leiomyomas in the nasal cavity given the relative rarity of smooth muscle in the nasal cavity. Tumors are thought to originate from undifferentiated mesenchymal cells or from smooth muscles contained in blood vessels or piloerector muscle . Treatment is total local excision with recurrence rates being very low .
Glomus tumors are benign proliferations of glomus body, a specialized arteriovenous shunt thought to have thermoregulatory function. Nasal and paranasal glomus tumors are extremely rare, with less than 20 cases currently reported in the literature . There also appears to be a female predominance for glomus tumors . Histologically, they appear as nests of glomus cells clustered surrounding vessels with typically absent mitotic figures. CD34, a hematopoietic progenitor cell antigen, may be positive on immunohistochemical staining and helpful in the differential diagnosis . Although not thoroughly researched in nasal glomus tumors, MRI of glomus tumors elsewhere on the body show a characteristic well-defined mass with intermediate/low signal on T1, hyperintense signal on T2, and intense enhancement with gadolinium . Complete excision is considered curative with minimally reported recurrence rates of 10% .
Angiosarcoma is a very rare malignant vascular tumor arising from the sinonasal site. They represent 1% of all sarcomas . Only 24 cases have been reported in the literature since 1974 . Grossly, these tumors appear as polypoid, nodular, violaceous, and red masses with areas of hemorrhagic necrosis . These tumors can be aggressive with invasion into the surrounding brain, orbit, and infratemporal fossa. Histology shows dense vasculature lined with atypical endothelial cells with surrounding fibrous stoma. There is often microscopic evidence of necrosis and hemosiderin deposits . Given the poor prognosis , therapy often includes surgery followed by radiation, but there is no standard protocol due to the limited number of cases. Five year survival rate is 22% and distal metastasis is seen in the first 24 months in 30% of cases .
Kaposi’s sarcoma (KS) is another vascular neoplasm that can arise in the nasal cavity. KS is a virally mediated low-grade vascular tumor that is most often seen in patients with acquired immunodeficiency syndrome. Head and neck KS most commonly involves the oral mucosa but can also be found in the pharynx, larynx, and tonsil. There are only a few cases published on KS found in the nasal mucosa. Lesions start as a flat collection of vessels that eventually turn into a more nodular lesion of vascular channels surrounded by spindle cells . Treatment in HIV positive patients involves highly active antiretroviral therapy drugs. Other treatments include intralesional or systemic chemotherapy .
4.2
Role of preoperative vascular control
Most vascular lesions that present in the nasal mucosa are small and can be safely excised without preoperative embolization or vascular ligation. For larger hypervascular lesions, preoperative embolization is an option that has been utilized to reduce lesion size, decrease the probability of significant blood loss, improve operative visualization and reduce operative time . Embolization is frequently done through a transarterial approach where arterial blood supply to the tumor is selectively embolized. This can be done safely for vessels arising from branches of the external carotid artery. Embolization should not be done on branches of the internal carotid artery due to the risk of severe vascular complications to the brain or ophthalmic artery .
There is debate on whether preoperative embolization is necessary for safe and complete excision of vascular lesions . Preoperative transarterial embolization may have no significant effect on blood loss, though this may be explained by incomplete embolization due to complex vasculature and arteriovenous shunting within the tumor . Another technique originally described in 1994 is direct intratumoral embolization . This approach, which utilizes direct injection of an embolization agent into the tumor, has been successfully applied to nasal vascular tumors .
Endoscopic arterial ligation for control of bleeding has been most studied in spontaneous epistaxis, but has also been used in control of bleeding during nasal surgery. The two main arterial targets are the sphenopalatine (SPA) and anterior ethmoid artery . SPA ligation is effective in controlling severe posterior nasal cavity bleeding in 88% to 98% of cases . Anterior ethmoid ligation is often done in conjunction with SPA ligation for additional control of anterior bleeding . Arterial ligations have been shown to be valuable during various nasal surgeries and significantly reduce the rate of postoperative epistaxis and the need for nasal packing .
Preoperative embolization has been most widely described in nasopharyngeal angiofibromas. There have been several cases reported of embolization utilized in the treatment of LCH occurring in the head and neck . LCH has a hormonal component and often occurs during pregnancy. Embolization has been found to be useful in this population of patients who require intervention during pregnancy and cannot afford extended time under anesthesia and/or significant blood loss . Others have argued that endoscopic surgery alone is the treatment of choice for LCH and preoperative embolization is unnecessary . In our experience, preoperative embolization made an otherwise unresectable tumor resectable. Vascular ligation lead to minimal blood loss in a tumor previously aborted for hemorrhage.
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