Department of Dermatology, CHU Hassan II, Fez, Morocco
Corresponding author details:
K Issoual
Department of Dermatology
CHU Hassan II
Fez,Morocco
Copyright:
© 2020 K Issoual, et al. This is
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Primary urethral mucosal melanoma is a rare but aggressive melanoma that affects patients
in their sixth and seventh decade of life. The tumor appears as a black nodule or polyp
but can also be amelanotic. One of the prognostic factors is the size of the tumor. Surgery
seems to be the only effective treatment, Treatments add to surgery, more particularly
chemotherapy, seem to improve the prognosis and prevent recurrences, this in the absence
of guidelines and clear consensus in the treatment of mucosal melanomas. The survival rate
largely depends on the lymph node and visceral metastases of the disease.
Melanoma; Vaginal; Urethral; Mucosa; Hematuria
Genital mucosal melanomas are extremely rare, about 1% of all melanoma localizations, most frequently localized on the vulva [1]. Exceptional on the vagina or on the urogenital tract [2], generally these melanomas are diagnosed late due to their localization. Thus, they are associated with a poor prognosis. This article intends to discuss a case of primitive ureter melanoma.
Observations
A female patient 41 years-old, single; complaining of hematuria, presenting a polypoid
and pigmented tumor on the urethral orifice. Clinical examination of the teguments didn’t
show any suspected skin lesion or lymph nodes. The examination under anesthesia showed
a pigmented umefaction on the peri-ureteral area, extending to the urethral orifice, the
ureter and to the vagina, extends over 4 cm intra vaginally. A biopsy confirmed the diagnosis
of melanoma. The cancer is inoperable; the radiological extension assessment revealed
showed the presence of enlarged lymph nodes and metastatic localizations. The patient
was put on chemotherapy in the absence of surgical indication. The evolution was marked
by the patient’s deaths after 6 months of chemotherapy (Figure 1).
Figure1: Clinical image showing a blackish tumor in the
ureteral meat and vaginal
The urogenital tract melanoma is a very aggressive and rare
disease, an early diagnosis is essential to an adequate treatment
strategy, since the scarcity of data and guidelines regarding treatment
measures, is making the management of this disease very difficult
and challenging.
Urogenital tract melanomas are very rare and extremely aggressive. Vagina melanomas
represent about 0.3% of all malignant melanomas end less than 3% of vagina cancers
[3,4]. Urethral localizations are even rarer, there are only 121 cases described in the
literature since 1966 [5,6], generally originate in the distal urethra, They represent only
0.1% of all malignant melanomas and about 2% of malignant tumors affecting female
ureters [7-10]. The physiopathology is not yet quite understood, but many theories and
risk factors are proposed to explain this ailment, among these, the CKIT gene mutation is
highly incriminated [11]. Another theory suggests that the causal factor could be microenvironmental factors and not associated with ultraviolet radiation vue [12]. The disease
affects people between 38 and 90 years old, often in women in their sixth to seventh decade
[13], generally discovered as a polypoid pigmented and hemorrhagic mass, sometimes
ulcerated. Amelanotic tumors have also been reported in patients, which often leads
to misdiagnosis and delayed treatment [3,14]. This often leads to delayed diagnosis,
treatment, and affect badly the prognosis. The confirmation of the diagnosis is based on
immunehistochemistry, which is S-100 +, mélanA +, HMB-45 + and vimentin + [5,15].
The investigation is the same as skin melanoma. Nodal metastasis and distant metastasis
are frequent, 50% and 20% respectively [16]. There are no well-established guidelines concerning the treatment. When the cancer is resectable, surgery is
recommended [17], with surgical safety margins varying between 1
to 2 cm [3]. Radical surgery associated with lymph nodes dissection,
chemotherapy or radiation therapy exclusive or additional to other
treatments, are all options for advanced-stage malignant primitive
vagina melanomas [4,18,19]. The urogenital localization is associated
with very poor prognosis even if it’s a locally evolutive lesion [14].
The current treatment strategy includes surgery, chemotherapy,
immunotherapy, and palliative care [5]. The available data suggest
a survival rate of a relative 5 years survival rate between 0 to 25%,
in case of primitive vagina melanoma regardless of the treatment
strategy [16].
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