Submandibular Venous Hemangioma: Case Report and Review of the Literature Adam N. Wallace, MD,1,2 Ross Vyhmeister, BS,2 Mudassar Kamran, MBBS, DPhil,1,2 Sharlene A. Teefey, MD1,2 1 2
Mallinckrodt Institute of Radiology, Barnes Jewish Hospital, St. Louis, MO 63110, USA Washington University School of Medicine, St. Louis, MO 63110, USA
Received 9 December 2013; accepted 21 August 2014
ABSTRACT: Hemangiomas of the submandibular space are very rare. Only 11 cases have been reported in the English literature, all of which were cavernous hemangiomas. In this report, we describe the case of a venous hemangioma in a 70-year-old woman. Ultrasound examination revealed a lobulated, homogeneous, hypoechoic mass, and minimal flow was detected on power Doppler evaluation. The mass and the submandibular gland were surgically excised, and the endothelium was found to be positive for CD31 and D2-40 markers, consistent with venous hemangioma. To our knowledge, this is the first reported case of a venous hemangioma in the subC mandibular space. V 2014 Wiley Periodicals, Inc. J Clin Ultrasound 43:516–519, 2015; Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jcu.22258 Keywords: submandibular space; hemangioma; salivary glands; ultrasonography
emangiomas are benign vascular tumors categorized histologically as racemose, capillary, cavernous, or venous. Both cavernous and venous hemangiomas consist of large, dilated vessels. However, the vascular channels in venous hemangiomas are characterized by thick fibrous walls containing smooth muscle, whereas cavernous hemangiomas consist of thin-walled vascular spaces lined by flattened endothelial cells.1 Hemangiomas of the subman-
Correspondence to: A. N. Wallace C 2014 Wiley Periodicals, Inc. V
dibular space are very rare, with those reported being of the cavernous subtype. In this report, we describe the case of a patient with a venous hemangioma of the submandibular space and discuss the clinical, radiographic, and histopathologic findings.
A 70-year-old woman was initially seen after 1 month of painful swelling in her right submandibular area. Her symptoms did not coincide with food intake; however, on clinical examination, the patient was thought to have a soft, mildly tender, and enlarged right submandibular gland consistent with sialadenitis. She was then referred for sonographic (US) scanning to evaluate for sialolithiasis. US revealed a lobulated mass measuring 3.3 3 2.9 3 2.9 cm. The mass was homogeneous and hypoechoic with linear echogenic septations (Figure 1A). Minimal flow was detected on power Doppler scanning (Figure 1B). The mass was compressible, appeared well circumscribed, and the salivary gland adjacent to the mass was normal in appearance. However, it was unclear whether the mass was arising from the gland or was an extraglandular lesion that was compressing the gland. Primary differential considerations at this time included a primary salivary gland neoplasm and lymphadenopathy. A CT was then performed, on which the mass appeared lobulated and hypoattenuating with mild enhancement. The CT scan also more clearly showed normal submandibular tissue forming a “clawlike” configuration around the mass (Figure JOURNAL OF CLINICAL ULTRASOUND
SUBMANDIBULAR VENOUS HEMANGIOMA
FIGURE 1. Submandibular space venous hemangioma. (A) Sagittal gray-scale sonogram shows a lobulated, homogeneous, hypoechoic mass with linear echogenic septations adjacent to normal salivary gland tissue. (B) Power Doppler sonogram shows minimal detectable flow within the mass. (C) CT image shows a lobulated, hypoattenuating mass with mild enhancement (*) around which normal glandular tissue forms a “clawlike” configuration (arrow).
1C). This finding suggested that the mass was arising from the gland rather than compressing it. In addition, CT showed the mass wrapping around the mylohyoid muscle and abutting the mandible, although no bony erosions were seen. Also of note, the mass did not contain calcifications. The mass was thought to most likely be a primary salivary gland neoplasm, and US-guided fine-needle aspiration (FNA) was recommended to exclude a malignancy. US-guided FNA of the mass was performed however, when sampling was attempted with a 25-gauge needle, blood immediately filled the needle hub. On-site cytopathology confirmed that the sample contained only blood. ConseVOL. 43, NO. 8, OCTOBER 2015
quently, an 18-gauge needle was used to aspirate 10 mL of bloody fluid for cell-block analysis, which also showed predominantly blood with scattered inflammatory cells. The 18gauge FNA was not complicated by bleeding. The decision was made to proceed with surgical excision for a definitive diagnosis and treatment. Intraoperatively, a dark bluish mass was seen arising from the submandibular gland and extending into the floor of the mouth. The mass was adherent to the lingual nerve adjacent to the submandibular gland. As the mass was dissected from the nerve, oozing venous blood was seen. 517
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Evaluation of the mass on surgical pathology revealed dilated, anastomosing vascular spaces with smooth muscle bundles in the vessel walls. The endothelium was diffusely positive for CD31, a vessel marker, and patchily positive for D2-40, a lymphatic marker. These morphologic and immunophenotypic features are consistent with the diagnosis of a venous hemangioma. At her 10-month clinical follow-up visit, the patient was well.
Hemangiomas of the submandibular space are very rare Only 11 cases have been reported in the English literature, all of which were cavernous hemangiomas.2–5 Of these 11 cases, 7 (64%) occurred in women and 4 (36%) occurred in men. This gender distribution reflects that of hemangiomas in general, which are twice as common in women as in men.5 The mean age of the patients in the reported cases was 40.7 years old (range, 16–65 years); thus, our patient is the oldest in whom this tumor has been reported. All previously reported patients with submandibular hemangioma presented with acute or subacute submandibular swelling, which was painful in five cases (45%). These findings clinically mimic sialadenitis. However, whereas symptoms of sialadenitis are typically exacerbated by food intake,6 only one reported patient with a submandibular hemangioma experienced postprandial symptom exacerbation.5 Thus, as in our case, symptoms that are not exacerbated by food may raise the possibility of a soft tissue tumor, such as a hemangioma. On physical examination, hemangiomas are generally soft, compressible lesions, in contrast to most malignant tumors, which tend to be more firm.7 Radiologic diagnosis of a salivary gland hemangioma is difficult. US or CT is typically performed in patients with suspected sialadenitis to evaluate for sialolithiasis. On US, the echogenicity of hemangiomas varies, and it may be difficult to delineate the margins of the tumor from adjacent normal tissue. Nonthrombosed superficial hemangiomas and high-flow vascular malformations with arteriovenous shunting are more likely to have demonstrable blood flow on power Doppler US. These blood-filled spaces will be effaced with manual compression, which also demonstrates the softness of these lesions. However, low-flow lesions such as venous hemangiomas may not have detectable flow. Additionally, the slow blood flow and dilated vascular chan518
nels in venous and cavernous hemangiomas predispose to thrombosis and phlebolith formation, which can be mistaken for sialolithiasis.8 In the absence of phlebolith formation or significant detectable flow on power Doppler US, as in our case, the US and single-phase CT appearances of a hemangioma may resemble those of a primary salivary gland tumor. MRI can suggest the diagnosis of a cavernous or venous hemangioma. On T2-weighted images, the vascular spaces with stagnant blood appear as high-signal-intensity lobules with interspersed low-signal-intensity areas representing fibrosis or thrombus. On T1-weighted images, these tumors have signal intensity that is intermediate between muscle and fat.7 Nuclear medicine imaging with 99mTc red blood cell scintigraphy can also make the diagnosis.9 A radiologic diagnosis may obviate the need for FNA, which in all reported cases was nondiagnostic. It also avoids the potential complication of a hematoma.2 Hemangiomas may arise from the gland proper or involve the gland secondarily from invasion of subcutaneous blood vessels.3,10 In our case, the venous hemangioma appeared radiographically to arise from the gland proper. Intraoperatively, the hemangioma could not be resected from the submandibular gland, and both were removed. Most venous hemangiomas are treated by complete surgical excision because these tumors are typically large and do not regress.5 In summary, a venous hemangioma may simulate other benign neoplasms or sialadenitis. Consideration of this tumor in making a differential diagnosis of swelling in the submandibular area may facilitate a radiographic diagnosis and avoid performing a nondiagnostic FNA. REFERENCES 1. Beham A, Fletcher CD. Intramuscular angioma: a clinicopathological analysis of 74 cases. Histopathology 1991;18:53. 2. Cho JH, Nam IC, Park JO, et al. Clinical and radiologic features of submandibular triangle hemangioma. J Craniofac Surg 2012;23:1067. 3. Chuang CC, Lin HC, Huang CW. Submandibular cavernous hemangiomas with multiple phleboliths masquerading as sialolithiasis. J Chin Med Assoc 2005;68:441. 4. Kumar S, Gupta AK, Bakshi J. Submandibular gland hemangioma: clinicopathologic features and a review of the literature. Ear Nose Throat J 2010; 89:E14. 5. McMenamin M, Quinn A, Barry H, et al. Cavernous hemangioma in the submandibular gland JOURNAL OF CLINICAL ULTRASOUND
SUBMANDIBULAR VENOUS HEMANGIOMA masquerading as sialadenitis: case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:146. 6. Bar T, Zagury A, London D, et al. Calcifications simulating sialolithiasis of the major salivary glands. Dentomaxillofac Radiol 2007;36:59. 7. Vilanova JC, Barcelo J, Smirniotopoulos JG, et al. Hemangioma from head to toe: MR imaging with pathologic correlation. Radiographics 2004;24: 367.
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8. Cankaya H, Unal O, Ugras S, et al. Hemangioma with phleboliths in the sublingual gland: as a cause of submental opacity. Tohoku J Exp Med 2003;199:187. 9. Bradley M, Stewart I, King W, et al. The role of ultrasound and 99mTc RBC scintigraphy in the diagnosis of the salivary gland haemangioma. Br J Oral Maxillofac Surg 1991;29:164. 10. el-Khashab MM, el-Kafrawy AH, el-Shalaby N. Hemangiomas of the parotid gland. Egypt Dent J 1972;18:189.