Table 3.
Summary of the published cases of leiomyosarcoma of the vulva and their characteristics over the past 20 years [32–51].
| Author, yr | No of Pts | Age(yrs) | Localization | Symptoms | Initial diagnosis | Diagnosis | Tumor size | Staging |
|---|---|---|---|---|---|---|---|---|
| Yordanov et al, 2020 | 1 | 73 | Symphysis | Pain syndrome, present for years, rapid growth in the last 3 months | n.s. | Leiomyosarcoma | 70 mm, satellite nodules | cM0 |
| Saquib et al, 2020 | 1 | 63 | Bartholin‘s gland, left | Painless swelling, >1y | Chronic Bartholin‘s gland cyst | Leiomyosarcoma | 28 mm | cM0, cN0 |
| Aljehani et al, 2021 | 1 | 38, pregnant | Bartholin‘s gland, left | Small vulvar mass since 1y, rapid growth in pregnancy, painless | Benign, Bartholin‘s cyst | Leiomyosarcoma | 100 mm | cM0 |
| Korkmaz et al, 2016 | 1 | 65 | Bartholin‘s gland, left | Vulvar lump with progressive enlargement over 6 months | Angiofibroma (after biopsy) | Leiomyosarcoma | 60 mm | cM0 |
| Alnafisah et al, 2016 | 1 | 37 | Vulva, left | Vulvar lump with progressive enlargement | n.s. | Leiomyosarcoma | 50 mm | Initially cM0 cN+ |
| Akrivi et al, 2021 | 1 | 42 | Area of the left Bartholin‘s gland | 6 months, progressive swelling | Chronic Bartholin‘s gland abscess | Leiomyosarcoma | 65 mm | cM0 |
| Mowers et al, 2014 | 1 | 48 | Bartholin‘s gland, left | Induration for 5 years, sudden rapid growth, pain, difficulty urinating | Bartholin‘s Gland cyst | Myxoid leiomyosarcoma | 46 mm | cM0 |
| Sameeta et al, 2019 | 1 | 63 | Right posterior fourchette | 6 months, slow growing | n.s. | Leiomyosarcoma | 21 mm | cM0 |
| Sayeed et al, 2017 | 3 | 72 | n.s. | vulvar mass | n.s. | Leiomyosarcoma | 110 mm | n.s. |
| 56 | n.s. | vulvar mass | n.s. | Leiomyosarcoma | 135 mm | n.s. | ||
| 68 | n.s. | vulvar mass | n.s. | Leiomyosarcoma | 55 mm | n.s. | ||
| Swanson et al, 2020 | 3 | 80 | n.s. | n.s. | n.s. | Leiomyosarcoma | 97 mm | n.s. |
| 31 | n.s. | n.s. | n.s. | Leiomyosarcoma | 25 mm | n.s. | ||
| 80 | n.s. | n.s. | n.s. | Leiomyosarcoma | 52 mm | cM1 (liver) | ||
| Nath et al, 2019 | 1 | 38 | Right upper vulva | Pain, ulceration, gradually growing mass over 8 months | n.s. | Leiomyosarcoma | 80 mm | cM0 |
| Smith et al, 2020 | 1 | 46 | Left labia majora | Painless mass | n.s. | Leiomyosarcoma | 30 mm | cM0 |
| Shankar, 2006 | 1 | 58 | Bartholin‘s gland, right | 4-months history, enlarging lump, painless | Not typical features for Bartholin‘s cyst | Leiomyosarcoma | n.s. | n.s. |
| González-Bugatto et al, 2009 | 1 | 52 | Bartholin‘s gland, left | 1–2 cm nodule for 4 years, rapid growth over past 6 months | Left Bartholin‘s gland cyst | Leiomyosarcoma | 60 mm | cM0 |
| Levy et al, 2014 | 1 | 57 | Perineal region of left labia | Tender and uncomfortable mass, increasing in size over past 4–6 weeks | Left Bartholin‘s gland cyst | Leiomyosarcoma | 25 mm | n.s. |
| Teramae et al, 2014 | 1 | 51 | Right side of vulva | Palpable mass for 20 years, rapid growth over 1 year | Aggressive angiomyxoma of the vulva | Leiomyosarcoma | 135 mm | cM0 |
| Tjalma et al, 2005 | 1 | 85 | Right side of vulva | Vulvar discomfort | Soft tissue tumor | Myxoid leiomyosarcoma | 60 mm | n.s. |
| Di Gilio el al, 2004 | 1 | 36, pregnant | Left labia majora | Palpable mass, rapid growth | Left Bartholin‘s gland cyst | Myxoid leiomyosarcoma | 60 mm | cM0 |
| Rawal et al, 2005 | 1 | 81 | Right side of vulva | Exophytic lesion | n.s. | Leiomyosarcoma | 50 mm | cM0 |
| Ulutin et al, 2003 | 3 | 39 | Labia majora | n.s. | n.s. | Leiomyosarcoma | 70 mm | n.s. |
| 37 | Labia majora | n.s. | n.s. | Leiomyosarcoma | 35 mm | n.s. | ||
| 18 | Labia majora | n.s. | n.s. | Leiomyosarcoma | 30 mm | n.s. |
| Author, yr | Surgical therapy | Margins | Microscopy | Grading | Immunohistochemistry | Adjuvant therapy | Follow-up | Ref. No. |
|---|---|---|---|---|---|---|---|---|
| Yordanov et al, 2020 | Wide local excision | n.s. | n.s. | n.s. | SMA +, S-100 –, MyoD – | RT | 3 months, recurrence-free | [32] |
| Saquib et al, 2020 | Excision, 2° left hemi-vulvectomy and left inguinal LNE | Negative margins, pN0 (0/8) | 22 MF/ 10 HPF | G2 | SMA +, Caldesmon +, Desmin +, Ki-67 30%, Vimentin –, S-100 – | None | n.s. | [33] |
| Aljehani et al, 2021 | Tumor resection | n.s. | 9 MF/ 10 HPF | n.s. | SMA +, Caldesmon +, Desmin + | None | 12 months, recurrence-free | [34] |
| Korkmaz et al, 2016 | Local excision | n.s. | 20 MF/ 10 HPF | n.s. | Calponin +, S-100 –, Ki-67 5% | None | 6 months, recurrence-free | [35] |
| Alnafisah et al, 2016 | Wide local excision; 2° left radical vulvectomy plus left inguinal LNE (2° operation after chemotherapy) | Negative margins at wide excision, 2° negative margins but 3.5 mm tumor rest, pN0 (0/3) | n.s. | G3 | SMA +, ER/PR + | CHT, 3 cycles gemcitabine/ docetaxel; again, CHT after 2° operation, 3 cycles gemcitabine/ docetaxel | 15 months: local recurrence, resection and RT; 18 months: lung metastasis, resection | [36] |
| Akrivi et al, 2021 | Wide local excision | Positive margins | 8 MF/ 10 HPF | n.s. | SMA +, Desmin +, Caldesmon +, Vimentin +, ER/ PR +, S-100 –, Myoglobulin – | None | 53 months, recurrence-free | [37] |
| Mowers et al, 2014 | Left radical hemi-vulvectomy; 2° re-excision | Positive margins; negative margins after re-excision | n.s. | G3 | SMA +, Desmin +, ER/PR + | CHT, 6 cycles doxorubicin/ ifosfamide | 18 months, recurrence-free | [38] |
| Sameeta et al, 2019 | Wide local excision | Positive margins | 25 MF/ 10 HPF | G3 | SMA +, Desmin +, ER +, S-100 – | RT | n.s. | [39] |
| Sayeed et al, 2017 | Wide local excision | Positive margins | 8 MF/ 10 HPF | n.s. | n.s. | n.s. | Died of disease | [40] |
| Wide local excision | Positive margins | 34 MF/ 10 HPF | n.s. | n.s. | n.s. | Died of disease | [40] | |
| Wide local excision | Negative margins | 23 MF/ 10 HPF | n.s. | n.s. | n.s. | Died of disease | [40] | |
| Swanson et al, 2020 | Tumor resection | Positive margins | 36 MF/10 HPF | n.s. | SMA +, Caldesmon +, Desmin – | n.s. | 1 month | [41] |
| Tumor resection | Positive margins | 12 MF/ 10 HPF | n.s. | SMA +, Caldesmon +, Desmin +, S-100 – | n.s. | 1 month | [41] | |
| Tumor resection | Negative margins | 12 MF/ 10 HPF | n.s. | n.s. | CHT | 4 months | [41] | |
| Nath et al, 2019 | Wide local excision | n.s. | n.s. | n.s. | SMA +, Vimentin +, Desmin –, S-100 –, ER/PR – | Chemoradiation | 21 months, recurrence-free | [42] |
| Smith et al, 2020 | Tumor resection, 2° left partial radical vulvectomy, 3° posterior radical vulvectomy plus bilateral inguino-femoral LNE | Positive margins, 2° positive margins, 3° negative margins, pN0 | n.s. | n.s. | SMA +, Desmin +, S-100 –, Caldesmon –, MyoD1 – | n.s. | n.s. | [43] |
| Shankar, 2006 | Wide local excision | n.s. | n.s. | n.s. | SMA +, Desmin + | RT intended but not carried out | 42 months, recurrence-free | [44] |
| González- Bugatto et al, 2009 | Tumor resection, 2° hemi-vulvectomy with ipsilateral inguinal LNE | Positive margins, 2° negative margins, pN0 | 21 MF/ 10 HPF | G3 | SMA +, Vimentin +, Desmin +, ER/PR +, S-100 – | RT, 66.6 Gy; CHT with mesna, epirubicin, ifosfamide and cisplatin | 12 months: local recurrence, wide excision, negative margins; 48 months: disease free | [45] |
| Levy et al, 2014 | Tumor resection, 2° radical excision | Positive margins, 2° negative margins | 16 MF/ 10 HPF | n.s. | SMA +, Desmin + | n.s. | n.s. | [46] |
| Teramae et al, 2014 | Tumor resection | Negative margins | 7 MF/ 10 HPF | n.s. | Desmin + | Adjuvant treatment (CHT or RT) was recommended but not wanted | 32 months, recurrence-free | [47] |
| Tjalma et al, 2005 | Wide local excision | Negative margins | 34 MF/ 10 HPF | n.s. | SMA +, Desmin +, ER/PR partially +, S-100 –, Ki-67 20% | None | 25 months, recurrence-free | [48] |
| Di Gilio el al, 2004 | Tumor resection, 2° wide local excision with ipsilateral superficial inguinal node sampling | Positive margins, 2° negative margins, pN0 | 2 MF/ 10 HPF | n.s. | SMA +, Vimentin +, S-100 – | n.s. | 30 months, recurrence-free | [49] |
| Rawal et al, 2005 | Wide local excision | n.s. | 35–40 MF/10 HPF | n.s. | SMA + | none | 9 months: suspicion of local recurrence, died of unrelated cause | [50] |
| Ulutin et al, 2003 | Radical vulvectomy with groin LNE | Close surgical margin | n.s. | G2 | n.s. | RT, 52.2 Gy | 73 months, recurrence-free | [51] |
| Simple vulvectomy | n.s. | n.s. | G2 | n.s. | None | 150 months, recurrence-free | [51] | |
| Radical vulvectomy | n.s. | n.s. | G1 | n.s. | None | 172 months, recurrence-free | [51] |
n.s. – not specified; cM0 – clinically no distant metastasis; cM1 – clinically distant metastasis; cN0 – clinically no lymph node metastasis; pN0 – pathologically no lymph node metastasis; LNE – lymphadenectomy; MF – mitotic figure; HPF – high-power field; G1/G2/G3 – low/intermediate/high-grade; SMA – smooth muscle actin; ER – estrogen receptor; PR – progesterone receptor; S-100 – “soluble”-100; Myo-D1 – myoblast determination protein 1; Ki-67 – (Kiel)-67 proliferation marker; “+” – positive; “–“ – negative; Gy – Gray; RT – radiation therapy; CHT – chemotherapy.