Sublingual cavernous haemanginoma

Hemangioma cavernoso sublingual

Olga Pérez-Macias Martín^a, Beatriz Peral Cagigal^a, Beatriz Madrigal Rubiales^b, Sara Blanco Sanfrutos^a, Luis Miguel Redondo González^a y Alberto Verrier Hernández^a

^aServicio de Cirugía Oral y Maxilofacial, Hospital del Río Hortega, Valladolid, España
^bServicio de Anatomía Patológica, Hospital del Río Hortega, Valladolid, España

Correspondence

Clinical case

We present the case of a 47-year old female, suffering from a tumour in the left side of the floor of the mouth. The patient complained of a slow but progressive growth over several months, and difficulties in speech and deglutition. She has a history of left submaxillectomy removed in another service, with no anatomopathological report.Clinical examination revealed the presence of a tumour in the floor of the mouth, extended to the submental region, measuring 4.5cm in diameter, hard feeling to palpation and not painful. In the neck examination, we identified an old scar related to a previous maxillectomy and no adenopathies or other lesions.

The orthopantomography did not show any alterations and the intraoral incisional biopsy was labelled as chronic sialadenitis of the sublingual glands. An oro-cervical CT found the presence of a 4x2cm solid mass in left sublingual region, with remodelling of the adjacent mandibular cortical and punctate calcifications, which could correspond to a glandular ectopia.In the absence of a clear diagnosis, an MRI was performed, observing a 6.4x5.8x3.2cm tumour, situated in the left floor extended to the submental zone, with isointense signal on T1 and markedly hyperintense on T2. This, added to the intern calcifications, made us think of a vascular tumour with phleboliths, as the presumptive diagnosis. Considering its vascular origin, a prior arteriography was requested. An arteriography of both external carotids and the selective for both lingual arteries was performed 24hours before surgery; but no afferent vessels leading to the tumour were identified. This fact questioned the radiologic diagnosis. It was finally decided to surgically remove the tumour with the aim of reaching an anatomopathological and definite diagnosis. The patient went through surgery under general anaesthesia and the tumour, including the sublingual gland, was extirpated through the previous cervical scar.Pathology showed a well-demarcated, but not encapsulated tumour, consisting of a proliferation of vascular structures of varying size, mostly widely dilated and congestive, showing a lobular distribution pattern. Vessels were lined by flattened endothelium without atypia, showing immunostaining with CD31 and CD34, with a thin wall underlain by a layer of dense collagen with a lymphoplasmacytic inflammatory component. Parenchyma on the periphery of the tumour was composed by seromucous acini and corresponded to a salivary gland type. There were some hemorrhagic zones. Numerous arterial and venous structures of different caliber appeared with a practically occlusive thrombosis of light, with phenomena of partial recanalisation and thrombus calcification, situated in the periphery of the tumour. The final diagnosis was a left sublingual cavernous hemangioma.

Discussion

Angiomas are vascular lesions, localised and benign. Cavernous or deep hemangiomas are situated under subcutaneous tissue. They can be located in any part of the organism, even if 65% of the hemangiomas are found in the head and neck^1,2. They are the most frequent non-epithelial tumours, showing an incidence level of 30% in major salivary glands. Furthermore, they are the most common lesions in salivary glands of those occurring during childhood, especially in their juvenile form³. The parotid gland is the most frequent site of hemangiomas that affect salivary glands (81-85%).^3-5 Incidence levels of these tumours are difficult to quantify because they are rarely biopsied. Even in those that are, it is difficult to distinguish if they derive from a minor salivary gland or from the adjacent tissue, due to the fact that they often intertwine. In our case, glandular acini were observed intermingled with vascular tissue, so we cannot affirm nor exclude an intraglandular origin^3,5. The most common causes of masses in the sublingual gland and submental region are: Ludwig's angina, sublingual congenital dermoid cyst, amyloidosis, ranula, branchial cyst, thyroglossal duct cyst and cystic higroma⁶. If there was an opacity zone in a salivary gland area, our first diagnosis would be sialolithiasis. Changes in the hemangioma blood flow lead to the formation of thrombi and phleboliths. Many cases of hemangiomas with phleboliths have been described in literature in the parotid and submandibular gland, but it is rare that phleboliths are found in the sublingual gland. The first case of this was published by Hazan in 2003⁶. Benign tumours of mesenchymal origin that affect salivary glands, such as hemangiomas, pose difficulties in diagnosis⁴. Basing it only on clinical history and examination, we could suggest the diagnosis as a benign epithelial tumour or sialolithiasis, but a radiological evaluation would be necessary. In conventional x-rays, calcifications like phleboliths will look radiopaque, but this will not help us distinguish between phleboliths and sialolithiasis. Ultrasounds and CT scans are capable to differentiate between extrinsic and intrinsic masses of salivary glands, as well as solid or cystic lesions⁶. However, a CT is less specific in detecting the vascular nature of hemangiomas, sometimes resulting in a false diagnosis of non-vascular lesions. The reason for this lack of specificity is due to the fact that the contrast used in a CT is not a true intravascular agent. Contrast can rapidly diffuse throughout the extravascular space through the numerous pores in the endothelium vessels. In addition, it is eliminated quickly by renal mechanism. After approximately two hours, only 32% of the initial concentration remains circulating. This explains why late filling of the sinusoidal vessels (90-120 minutes) goes unnoticed. For this same reason, conventional arteriography does not always show the true nature of the lesion because the contrast is excreted before late filling of the vessels. In the presented case, this would be one of the possible explanations for the absence of afferent vessels leading to the tumour in the arteriography. Furthermore, in the histological fragment, occlusive phenomena of partial thrombosis and partial calcification of thrombi (phebolithes) are observed. In an MRI, marked hyperintensity in T2 corresponds to calcification zones, whereas isointensity in T1 corresponds to muscle^2,4.

Phebolith formation has been reported as a characteristic feature of hemangiomas, therefore, radiograms are useful aids in the confirmation of a clinical diagnosis⁷. Cavernous hemangiomas, unlike the superficial ones, never completely regress. However, sporadically, some of them can partially regress when they have been exposed to trauma, ulcer or haemorrhage.

Various terms and classes have been suggested to describe these types of lesions. The renaissance began in 1982 when Mulliken and Glowacki⁸ introduced a "biological" classification.

Often deep hemangiomas that are not perceptible within the first few years of life and began to regress are discovered only by chance. Development of flebolith within the hemangioma from a thrombus may cause symptoms and usually leads to diagnosis of the hemangioma.Observation would be the most logical attitude in most of the lesions, but severe haemorrhage, functional compromise or infectious episodes with ulcer or thrombocytopenia, would be some indications of invasive treatment. Therapeutic alternatives include corticoids, sclerosing agents, surgery after embolisation, cryotherapy (useful in superficial lesions, but never in deep ones), laser (especially in the tongue and lips) and finally, radiotherapy, although there is a risk of developing secondary tumours^2,7. Due to the functional alteration and localization, the best therapeutic choice for the patient was surgical extirpation. To conclude, although cavernous hemangioma is a rare pathology in the sublingual gland, it should be considered as one of the possible diagnoses of lesions with calcifications in the sublingual and submandibular region, especially if there are phleboliths⁵.

References

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6. Çancaya H, Ünal Ö, Ugras S. Hemangioma with phleboliths in the sublingual gland: as a cause of submental opacity. Tohoku J Exp Med. 2003; 199:187-91.         [ Links ]

7. Sano K, Ogawa A, Inokuchi T. Buccal hemangioma with phleboliths, Report of two cases. Oral Surg Oral Med Oral Pathol. 1988; 65:151-6.         [ Links ]

8. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristic. Mulliken JB, Glowacki J. Plast Reconst Surg. 1982; 69:412-22.         [ Links ]

Correspondence:
maciasol@hotmail.com
(O. Pérez-Macias Martín)