Solitary oral angiokeratoma: an occasional diagnosis for vascular lesions
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1
University of Turin, Department of Oncology, Italy
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2
Ospedale San Luigi Gonzaga, Pathology Division, Italy
Aim. Angiokeratoma (AK) is a benign, capillary, vascular tumour characterized by the association with epithelial hyperplasia. Solitary or multiple red to purple papillary lesions can be observed with variable size, shape and location. At the histomorphological assessment, dilated blood vessels located in papillary dermis are commonly observed, resulting in superficial vascular ectasia with an overlying epithelial hyperplasia. The microscopic features of oral AK are identical to those of oral lymphangioma circumscriptum (LC), and AK may represent the hematic counterpart of LC.
Due to clinical features and lesions distribution, AK are classified as: 1) corporis diffusum AK showing generalized and systemic involvement, associated with Fabry disease or Anderson-Fabry disease, a group of enzymatic deficiency disorders; 2) Mibelli’s AK characterized by bilateral lesions, involving the dorsal surfaces of fingers and toes; 3) Fordyce type AK due to the presence of scrotal or vulvar lesions; 4) solitary or multiple papular AK, most often observed on lower extremities; 5) circumscriptum AK, a congenital or infantile form often occurring on the trunk and extremities. A simplified classification sets apart isolated primary AKs and secondary AKs associated to generalized systemic disorder (Fabry disease and fucosidosis).
Isolated oral AKs are usually observed on the dorsal tongue. The elective treatment of oral AK is surgical excision, which can be performed using a traditional cold scalpel or taking advantage of other techniques as electrodessication, cautery, diathermy, cryotherapy or laser ablation. Alternatively, a wait and see approach can be chosen reserving the excisional option to cases characterized by increasing lesion size or bleeding. Nevertheless, looking at cases reported in the literature it must be kept in mind that an ablative treatment prevents a definitive diagnosis of AK as it can just be achieved thanks to histological examination.
Materials and Methods. A 67-year-old Caucasian man was referred to our Oral Medicine and Oral Oncology Clinic on July 2017 because of the occasional finding of a red slightly exophytic lesion at the left posterior margin of tongue. The little red-brownish sessile tumor with papillary surface, not exceeding 6 mm in greatest diameter, was detected by the patient himself, it was asymptomatic and it had a slow enlargement during the last 3 weeks. On palpation it was elastic, non-painful, and non-bleeding. According to the 2018 International Society for the Study of Vascular Anomalies (ISSVA) Classification, the observed clinical aspect was consistent with a benign vascular tumour or an unclassified vascular anomaly. Nevertheless, the patient had a personal medical history positive for a Stage IV prostate cancer with lung metastases, so that a potential distant tongue metastasis could not be ruled out just on the basis of the clinical examination.
Results. Due to the small dimension of the mass, a complete excision was performed under local anesthesia using a 980 nm near infrared diode laser with a 330 micrometers sterile optical fiber. Emission modality was set on continuous wave, power ranging from 2 to 2.5 W. The effect of the thermal damage, produced by diode laser, was measured on the sample revealing an average value of involvement equal to 500 µm on the epithelium and 200 µm in the stromal area. Such evidences did not affect the pathological assessment which revealed a papillary mass mainly composed by cavernous vascular proliferation, containing red blood cells with small thrombi. Such vessels were covered with a squamous epithelium with hyperkeratosis and acanthosis. In the light of such pathological features consistent with AK and in absence of any known enzymatic deficiency disorder, the patient underwent further skin examinations. No other skin lesions were observed and a final diagnosis of solitary AK affecting the tongue was achieved. Healing was uneventful without any scarring.
Discussion. Oral AK appear most often as single, erythematous, shiny papillary lesions, which may show some whitish superficial areas. It is firm on palpation, non-tender with just occasional bleeding. On clinical examination, hemangioma, lymph-hemangioma, focal-epithelial hyperplasia, or nevus have to be considered for a differential diagnosis.
Of note, AK and LC of the oral mucosa have identical clinical and microscopic features; only the presence of small thrombi may suggest a hematic rather than lymphatic pathogenesis. A clear differentiation between such entities can be achieved thanks to a distinct immunohistochemical profile of their endothelial cells. Nevertheless, it is purely academic as AK and LC share a good prognosis after excision, without risk for recurrence or malignant transformation.
When compared to reactive vascular tumours, AK has been reported to be characterized by a self-maintenance ability in absence of external stimuli. It seems that the co-expression of VEGF and VEGF-R1/2 in endothelial cells could be involved in the onset and self-maintenance of AK.
In 2009 a classification of oral AK was proposed aiming to differentiate primary or secondary AK depending on the presence of a concurrent generalized systemic disorder. Other than this, clinicians should also consider the concurrent presence/absence of cutaneous AKs and the number of oral AK observed (single/multiple lesions).
A single oral AK not related to systemic diseases may be treated by diathermy, cryotherapy, or surgical excision. Factors that support an active treatment are: patient psychological distress, itching or bleeding. Other than this the need for a definitive histological diagnosis has to be considered. In the present case, clinical history and characteristics favored the diagnosis of a small vascular tumour, but a distant metastasis due to the known prostate cancer could not be ruled out in absence of a pathological assessment. Such situation also drove the choice of the treatment modality, excluding diathermy, cryotherapy or a laser-assisted photocoagulation. An excision was performed using a diode laser in order to reduce edema and intraoperative bleeding, typical of vascular lesions.
A pathological report making a diagnosis of AK or LC related to the excision of a single lesion from an adult patient not affected by enzymatic deficiency disorder does not provide any helpful information to the clinician. Such entities could be simply described as vascular tumours characterized by superficial vascular ectasia with epithelial hyperplasia.
References
1. Ranjan N, Mahajan VK. Oral angiokeratomas: proposed clinical classification. Int J Dermatol. 2009 Jul; 48(7):778-81
2. Fernández-Aceñero MJ, Rey Biel J, Renedo G. Solitary angiokeratoma of the tongue in adults. Rom J Morphol Embryol. 2010;51(4):771-3
3. Katsoulas N, Tosios KI, Argyris P, Koutlas IG, Sklavounou A. Lymphangioma circumscriptum, angiokeratoma, or superficial vascular ectasia with epithelial hyperplasia? Oral Surg Oral Med Oral Pathol Oral Radiol. 2014 Aug;118(2):e53-7
Keywords:
Angiokeratoma,
Vascular neoplasm,
Tongue,
Hyperplasia,
international society for the study of vascular anomalies
Conference:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine., Ancona, Italy, 19 Oct - 20 Oct, 2018.
Presentation Type:
Poster Presentation
Topic:
Oral Diseases
Citation:
Giordano
L,
Presta
R,
Val
M,
Ruga
E,
Marci
V and
Pentenero
M
(2019). Solitary oral angiokeratoma: an occasional diagnosis for vascular lesions.
Front. Physiol.
Conference Abstract:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine..
doi: 10.3389/conf.fphys.2019.27.00068
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Received:
04 Nov 2018;
Published Online:
09 Dec 2019.
*
Correspondence:
Dr. Lia Giordano, University of Turin, Department of Oncology, Turin, Piedmont, 10124, Italy, lia.giordano@edu.unito.it