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« Back to Contents VULVAR CANCER

LiFE re

Literature for ENYGO

Vulvovaginal adenocarcinoma / melanoma / sarcoma

Editor Anna Dückelmann Sanchez et al. presented a retrospective study on epidemiology and
disease-specific survival in primary genitourinary melanoma cases
Descriptive summary using a large population-based cohort. Synoptically, patients with
primary genitourinary melanoma present with advanced stage and
Most cases of primary vaginal carcinoma are squamous cell carci- have a poor prognosis. Women have worse disease-specific survival
nomas. Other histologic types such as adenocarcinoma are usually compared to men. Disease-specific survival is negatively associat-
metastatic lesions, mainly originating from the uterus. Sadatomo et ed with advanced age at diagnosis, higher stage, and lymph node
al. presented a rare case of isolated vaginal metastasis from rectal involvement.
cancer, diagnosed by magnetic resonance imaging (MRI). Isolated
vaginal metastases are an indication for surgical resection and Hu et al. described an exceptional case of myeloid sarcoma occur-
adjuvant chemotherapy. In colorectal cancer, MRI is a useful tool to ring on the vulvar mucosa as the presenting sign of acute myeloid
evaluate the vagina prior to therapy. leukaemia (AML) following bone marrow transplantation for myelo-
dysplastic syndrome. The patient received systemic chemotherapy
McCluggage gave a comprising overview about non-HPV-related ad- with cytarabine and idarubicin. Treatment led to a marked reduction
enocarcinomas of the lower female genital tract, the most common in the size of the vulvar mass, but the patient died of septic shock
of which is so-called gastric type. Carleton et al. concentrated on the and respiratory failure 3 months after diagnosis. Authors concluded
immunohistochemical profile of this kind of tumour. Adenocarcino- that leukaemic infiltration should be suspected in any patient with
mas exhibiting gastric differentiation are uncommon but represent a history of myeloid leukaemia or myelodysplastic syndrome who
the most common non-human papillomavirus (HPV)-related variant presents with a mass involving the skin or external mucosal surface.
of cervical and vaginal adenocarcinoma. They exhibit considerable
morphologic overlap with adenocarcinomas originating outside the Presenting an interesting case of a 32-year-old patient with epithe-
female genital tract and could be distinguished, inter alia, by PAX8 lioid sarcoma of the vulva, Han et al. stressed the fact that dis-
staining. ease-free-survival-time and mortality in the proximal-type epithelioid
sarcoma are age-dependent according to literature. Younger patients
Van Rosmalen et al. presented a case of a mucinous adenocarci- might have a better prognosis than older patients. Early diagnosis
noma of the vulva with neuroendocrine differentiation based on with definite surgical treatment is absolutely essential for improve-
immunohistochemical analysis, treated by partial vulvectomy. ment of prognosis.

Stefanović et al. reported on a 60-year-old woman presenting with
a palpable tumour near the vaginal introitus and recurrent vaginal
bleeding. Biopsy revealed a melanoma, which was treated with wide
local excision and adequate safety margins. There is general accept-
ance that radical surgery does not improve the prognosis of primary
malignant melanoma of the vagina (PMMV). Todo et al., however,
argued for radical surgery in PMMV, formulating a scoring system of
surgical radicality. This score quantitatively evaluates the radicality
of the initial surgery by giving a certain number of points for the re-
sected organs. According to the authors, the therapeutic significance
of radical surgery for PMMV has not been evaluated appropriately in
previous studies because of the lack of comparability among groups
and differences in the definitions of surgical radicality. They called
for a multicentre cooperative study to validate the clinical impact of
their score on the prognosis of PMMV.

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International Journal of Gynecological Cancer, Volume 26, Supplement #1