Keywords
ulcer - vulvar neoplasms - hemangioma - biopsy
Palavras-chave
úlcera - neoplasias vulvares - hemangioma - biópsia
Introduction
Hemangiomas are proliferative soft-tumor lesions marked by increased cell turnover.
They are the product of a derangement in angiogenesis that allows for the unsuppressed
proliferation of vascular elements. These tumors usually appear after birth, grow
rapidly and involute over the years.[1] A total of 60% of hemangiomas are situated in the cervicofacial region.[2] The remaining section can occur at various locations in the body, including the
vulva. However, vascular tumors are rarely found in the female genital tract.[3] In fact, the female genital tract is an unusual location for hemangiomas, they are
seldom found in this site.[3]
[4]
[5] Therefore, there are very few reports on the condition on the literature.[4]
Although rare, we must be aware of the diagnosis of hemangiomas in the inferior female
genital tract, as they might be a reason for a gynecologic consultation[3] and can cause functional or emotional disability.[4] We report here a rare case of a vulvar hemangioma that presented as a genital ulcer
causing functional impairment and bleeding.
Case Report
The patient is a 52-year-old female , married, self-employed, non-smoker, with prediabetes,
dyslipidemia and premenopausal, with no use of hormonal contraception. The patient
reported a genital lesion for the past 3 years and the use of various treatments involving
creams and ointments, even though there was no diagnosis associated with dyspareunia
and intermittent bleeding. Physical examination revealed a solitary vulvar ulcer ([Fig. 1]). The cervix had no abnormalities according to the colposcopy. No abnormalities
were present in the routine blood tests and examinations of the patient. Initially,
the investigation began by discarding all possible infectious causes of genital ulcers,
such as syphilis, herpes and chancroid, which all proved to be negative.
Fig. 1 Examination of the vulva with a lesion between the vaginal vestibule and the perineal
region.
There was suspicion of a manifestation of Behcet disease; therefore, we initiated
the treatment with systemic corticosteroids. The first response was partial, the patient
presented a decrease in the itching and burning sensations, but the bleeding did not
cease. Furthermore, the pathergy test result was negative. Due to the patient complaints
of pain and sexual dysfunction, it was decided to conduct a surgical excision of the
lesion. The biopsy examination showed a proliferation of tortuous vessels in the submucosa
([Fig. 2]) and thus, the pathological diagnosis was reported as a hemangioma.
Fig. 2 This figure shows a proliferation of tortuous vessels in the submucosa, with variation
in size. (A) Detail of a tortuous vessel and its endothelial lining formed by a single
layer of some flattened cells. Some more swollen, without nuclear atypia. (400x magnification).
(B) Some of them are larger and full of erythrocytes, while others are smaller and
located in the periphery. (400x magnification). (C) Field of observation demonstrating
the superficiality of these tortuous
vessels and their close relation to the mucosa (stratified squamous epithelium). (200x
magnification). (D) Nude area (without epithelial lining) composed of acute inflammatory
cells (polymorphonucleated), chronic (lymphocytes and plasma cells) and erythrocytes,
characterizing ulcerated lesion. (100x magnification). All using hematoxylin-eosin
staining.
Discussion
The patient presented a hemangioma, which was clinically manifested as a bleeding
ulcer. In fact, the hemangioma can manifest as an ulceration and bleed, both occurring
most often in younger patients.[5] Vulvar hemangiomas can cause functional and emotional disability,[4] pain, cosmetic problems, and sexual dysfunction,[5] the last three of which were complaints from our patient. For this reason, it is
important to diagnose it correctly and to treat it adequately.
In this case, the first manifestation was a red, painful and persistent lesion followed
by an ulcer complicated by life-threatening hemorrhage that was not responsive to
conventional treatments and which showed no signs of involution for more than 3 years.
Despite the negative evidence, over this time, an appropriated gynecological exam
had been realized as well as the syndromic management, which is recommended by the
Brazilian Ministry of Health. However, no final diagnostic was obtained, and no satisfactory
results were observed.
The syndromic management of genital ulcers is recommended as an initial treatment,
given the number of differential diagnoses and the practical difficulties in establishing
a clear-cut etiologic diagnosis. It is prudent for healthcare providers to initially
consider every female genital ulcer as a sexually transmitted disease (STD), which
are: syphilis, chancroid, lymphogranuloma venereum, donovanosis and genital herpes.[6]
Therefore, our approach was to initially screen the patient for STDs; these all proved
to be negative, which excluded these diseases from our differential diagnoses. Even
with proper laboratory analysis, no pathogen is found in up to 25% of the patients
with genital ulcers.[7]
Continuing the investigation of the etiology of the ulcer, we considered that it could
be caused by a non-sexually transmitted disease,[6] such as Behcet disease.[8] Considering the possibility of this vasculitis, we performed a systemic corticosteroid
treatment. However, there was no complete regression of symptoms. Subsequently, the
biopsy of an ulcer, which did not respond to initial therapy, was the recommended
course of action.[6]
[9] Therefore, with the biopsy of the patient's ulcer, it was possible to reach a definite
diagnosis of vulvar hemangioma ([Fig. 2]).
As most hemangiomas present spontaneous involution, treatment may be reserved for
lesions that present complications, such as functional impairment, pain, ulceration,
and bleeding, as the ones presented by our patient; however, there are others, like
unusually rapid growth, infection, and cosmetic concerns.[2] In our case, the discomfort of our patient with her symptoms and her sexual dysfunction
led to our decision to treat her.
The initial approach in our case was a conservative treatment with systemic corticosteroids
to minimize the inflammatory process, as we assumed we were dealing with a manifestation
of Behcet disease. Fortunately, these medications are effective in inducing rapid
involution in massive hemangiomas. The success rate varies from 30 to 90%.[2] The therapy was based on daily doses of Prednisone 20 mg. The patient responded
partially with a significant improvement in the itching and burning symptoms. However,
the ulcer did not involute as expected, and she was still suffering from bleeding
and dyspareunia.
It is important to highlight that during this period, the biopsy was suggested and
refused by the patient, who accepted it only upon knowledge that it could be a malignant
neoplasm.
The surgical excision of hemangiomas of the female genitalia is reserved for symptomatic
lesions refractory to medical management.[10] The procedure was performed for a curative purpose, to exclude malignancy and to
obtain a definite diagnosis. A circular excision and purse-string closure are considered
the best surgical approach for hemangiomas.[11] The resection of the lesion and reconstruction of the minor labia eliminated the
symptoms and provided the cosmetic improvement required by the patient. A definite
diagnosis of hemangioma was reached after pathological examination of the lesion.
The proliferation of large vessels full of erythrocytes in the submucosa, involving
the dermis are pathognomonic of hemangioma and excluded the possibility of being an
ordinary inflammatory process ([Fig. 2]-[A]). A second surgical approach was necessary in this case because of a residual vulvar
lesion. The biopsy and histological examination of this tissue were compatible with
a Bartholin cyst
Vulvar hemangioma is an extremely rare pathology in adults; however, infantile hemangioma
is one of the most common benign soft-tissue tumors in infants. The treatment of infantile
hemangiomas has been changing from surgical incision to the use of oral corticosteroids,
cryosurgery, laser, radiation, propranolol, and anticancer drugs vincristine and bleomycin.[12]
[13]
[14]
More recent reports also suggest alternative modalities for the treatment of this
disease, such as the use of curcumin, indicating that the effectiveness of curcumin
in hemangioma may be associated with its potent antiproliferative and apoptotic activities
in hemangioma endothelial cells.[15]
Conclusion
To conclude, this case represents a rare situation of a vulvar hemangioma that presented
as a genital ulcer, associated with psychological and physical dysfunction for the
patient. The symptoms of itching, pain and sexual impairment were significant, and
the delay in the diagnosis and resolution of the problem were a source of distress
for the patient and her husband. We emphasize the importance of following the steps
of the differential diagnosis and proceeding with a surgical approach only if necessary.
