Vulvar melanoma is a rare gynaecological cancer with poor prognosis due to its high metastatic potential. • The only potentially curative option for localized disease is complete surgical resection with negative margins. • Sentinel lymph node biopsy is only recommended in order to direct adjuvant treatment. • Analysis for targetable mutations should be incorporated into routine clinical testing for vulvar melanoma. • Immunotherapy with anti-PD1 or anti-CTLA4 should be considered in metastatic or non-resectable vulvar melanoma.
Uterine leiomyosarcoma is a rare malignant tumor of smooth muscle origin. We describe the case of a 64-year-old female with intravenous uterine leiomyosarcomatosis with the extension of the tumor mass into the inferior vena cava and right atrium. As initial tissue diagnosis of the tumor obtained from the uterine and right atrial masses suggested intravenous leiomyomatosis, surgical resection was carried out using a one-stage procedure via a laparotomy and median sternotomy with cardiopulmonary bypass and circulatory arrest. Subsequent histology revealed uterine leiomyosarcoma with an intravenous spread, which to our knowledge is only the second case that has been described.
Objectives
The aims of the study were to evaluate clinicopathologic features, management, and outcomes in vulval melanoma and to review the literature.
Materials and Methods
Data were collected retrospectively on patients with vulval melanoma from 2001 to 2017 in 5 gynecological oncology cancer centers (Bristol, Taunton, Truro, Plymouth, and Cheltenham). SPSS software was used for univariate and multivariate statistical analysis. Disease-specific median survival was calculated using Kaplan-Meier curves.
Results
Forty-four patients with vulval melanoma were included, with a median age of 71 years. Forty-three of 44 had wide local excision with full inguinal lymphadenectomy if abnormal lymph nodes. Seven patients had sentinel lymph nodes. However, 2 patients with negative sentinel lymph nodes had distant recurrences within 16 months.
On univariate analysis, presence of ulceration (p = .012), perineural invasion (p = .03), and area of lesion (p = .016) were associated with risk of recurrence but only presence of microsatellites (p = .01) was associated with risk of death.
There were 31 deaths (70%): 29 (94%) of 31 from melanoma and 28 (64%) of 44 recurrences: 17 local (10 groin, 7 vulval) and 9 distant. Overall median survival was 32.5 months (95% CI, 17.8–46.5 months) and median recurrence-free survival 12.6 months (95% CI, 7.7–17.4 months).
Conclusions
This retrospective multicenter study highlights the high recurrence rate and poor prognosis of vulval melanoma. Lymph node surgery did not make any difference to recurrence-free survival or overall survival. The presence of microsatellites was associated with a statistically increased risk of death.
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