Table I.
Histological and immunohistochemical features, treatment and clinical outcome of vulvar leiomyosarcoma reported cases.
| Author- Published year | Age (years) | Size (cm) | MFI | MTCN | IHC | Initial treatment | Margins | Local recurrence | Treatment of recurrence | Follow-up | (Refs.) |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Davos and Abell (1976) | 41 | NS | >10 | NS | NS | Wide local excision | NS | NS | - | 28 months, alive, NED | (18) |
| Davos and Abell (1976) | 49 | NS | >10 | NS | NS | Local excision | NS | + | Wide local excision | 9 years, alive, NED | (18) |
| Davos and Abell (1976) | 84 | NS | >10 | NS | NS | Wide local excision | NS | + | No further treatment | 6 months, disseminated metastases, died of disease | (18) |
| Davos and Abell (1976) | 54 | NS | >10 | NS | NS | Local excision | NS | + | Hemivulvectomy; hysterectomy and excision of vaginal mass | 9 years, disseminated metastases, died of disease | (18) |
| Davos and Abell (1976) | 35 | NS | >10 | NS | NS | Local excision | NS | + | Re-excision; radical vulvectomy | 9 years, alive | (18) |
| Møller et al (1990) | 54 | 8 | 15 | Yes- Focal | Desmin: Positive | Radical vulvectomy + inguinofemoral lymphadenectomy | No | No | - | 30 months, alive, NED | (55) |
| Tawfik et al (1994) | 52 | 15 | 25-30 | Yes- Focal | SMA, vimentin, HHF-35, ER, p53: Positive | Local excision + radiotherapy | Infiltrative | No | - | 14 months, alive, NED | (25) |
| Aartsen and Albus-Lutter (1994) | 15 | 7 | NS | NS | NS | Radiotherapy 40 Gy | +; 2.5 years | Wide Local Excision + Radiotherapy 40 Gy | 24 years, alive, NED | (1) | |
| Aartsen and Albus-Lutter (1994) | 41 | 4.5 | NS | NS | NS | Wide local excision | - | - | - | 17 years, died of disease | (1) |
| Aartsen and Albus-Lutter (1994) | 75 | 10 | NS | NS | NS | Radical vulvectomy | NS | 6 months & 3 months | Local excision -Local excision | 4 months, died of disease | (1) |
| Aartsen and Albus-Lutter (1994) | 67 | 10 | NS | NS | NS | Radical vulvectomy | NS | 5 months | Radiotherapy 49 Gy | 7 years, died of disease | (1) |
| Fried-Oginski et al (1995) | 31 | 5 | 4 | Yes- Focal | SMA, muscular actin, vimentin: Positive | Local Excision + 8 weeks later Wide Local Excision | NS | 10 months | Wide Local Excision + Radiotherapy | NS | (56) |
| Nielsen et al (1996) | 33 | 5.5 | 5 | NS | SMA, muscular actin, vimentin, desmin, ER, PR: Positive; keratin, S-100: Negative | Local Excision | Infiltrative | 4 months | Wide local Excision | 36 months, alive, NED | (38) |
| Nielsen et al (1996) | 46 | 10 | 5 | NS | SMA, muscular actin, vimentin, desmin, ER, PR: Positive; keratin, S-100: Negative | Modified Radical Vulvectomy | No | - | - | 30 months, alive, NED | (38) |
| Nielsen et al (1996) | 37 | 5 | 8 | NS | SMA, muscular actin, vimentin, ER, PR: Positive; keratin, S-100, desmin: Negative | Wide Local Excision | Infiltrative | 3 months | Wider Excision; positive margins again; modified radical vulvectomy | 36 months, alive, NED | (38) |
| Nielsen et al (1996) | 67 | 16 | 10 | NS | SMA, muscular actin, vimentin, ER, PR: Positive; keratin, S-100, desmin: Negative | NS | NS | - | Local, radiotherapy chemotherapy | 7 months, metastases to lungs and bones, died of disease | (38) |
| Nielsen et al (1996) | 56 | 5.5 | 5 | NS | SMA, muscular actin, vimentin: Positive; keratin, desmin, S-100, ER, PR: Negative | NS | Infiltrative | NS | NS | NS | (38) |
| Torres et al (2000) | 14 | 6 | - | - | - | Local Excision | - | +; 13 months | Wide Local Excision + Chemotherapy; Wide Local Excision + Radiotherapy 50 Gy; Vulvectomy + Hysterectomy + Radiotherapy 65 Gy | 22 Years, alive, NED | (13) |
| Rubin and Fletcher (2000) | 26 | 1.2 | 6 | Yes, <50% | SMA, desmin: Positive; Cam52, EMA: Negative | Local Excision | Infiltrative | 8 months & 35 months | NS | 62 months, NED | (57) |
| Rubin and Fletcher (2000) | 30 | ND | 5 | No | SMA, HHF: Positive; desmin, Cam52, EMA: Negative | Local Excision | NS | 19 months | NS | 11 years: 39 Local Recurrences, died of disease | (57) |
| Rubin and Fletcher (2000) | 32 | 2 | 10 | No | SMA, desmin: Positive; Cam52, EMA: Negative | Wide Local Excision | NS | - | - | 83 months, alive, NED | (57) |
| Lösch et al (2001) | 38 | - | - | - | ER, PR, Ki-67: Positive; p53: Negative | - | - | - | - | 24 months, alive, NED | (22) |
| Di Gilio et al (2004) | 36 | 6 | 2 | NS | - | Wide Local Excision + Ipsilateral Lymphadenectomy | NS | - | - | 30 months, alive, NED | (10) |
| Dewdney et al (2005) | 36 | 5 | - | - | - | Vulvectomy | - | - | - | 13 months, alive, NED | (3) |
| Rewal et al (2005) | 81 | 5 | 35-40 | Yes | SMA: Positive | Wide Local Excision | NS | 9 months | - | Died of unrelated cause (cardiac failure) | (28) |
| Shankar et al (2006) | 58 | 3 | NS | NS | Actin, desmin: Positive | Wide Local Excision | NS | - | - | 42 months, alive, NED | (11) |
| González-Bugatto et al (2009) | 52 | 6 | 21 | No | SMA, Vimentin, Desmin, ER, PR: Positive; S-100, CK, CD31, p53, c-erbB-2, Leukocyte Common Antigen: Negative | Hemivulvectomy + ipsilateral lympadenectomy + radiotherapy 66.6 Gy + chemotherapy | Yes | +; 12 months | Wide local excision | 4 years, alive, NED | (12) |
| Salehin et al (2011) | 71 | 2 | 10 | NS | SMA, Ki-67: Positive | Hemivulvectomy + hysterectomy + salpingo- oophorectomy + inguinal lymphadenectomy | No | NS | NS | NS | (4) |
| Mowers et al (2014) | 48 | 4 & 10 (5 years later) | NS | NS | SMA, desmin, ER, PR: Positive | Incision and drainage | Infiltrative | +; 5 years | Partial excision and marsupialization; Left radical Hemivulvectomy + hysterectomy + bilateral salpingo- oophorectomy + chemotherapy | 18 months, alive, NED | (26) |
| Levy et al (2014) | 57 | 2.5 | 16 | NS | SMA: Positive; Desmin: Positive | Local excision + Radical excision | Infiltrative - Then, no residual disease | NS | NS | NS | (16) |
| Teramae et al (2014) | 51 | 13.5 | 7-10 | NS | Desmin, Ki-67: Positive | Local excision | No | - | - | 32 months, alive, NED | (44) |
| Alnafisah and Alfieri (2016) | 37 | 5 | NS | NS | SMA, ER, PR: Positive | Wide local excision + Chemotherapy; Left radical vulvectomy + ipsilateral inguinal lymphadenectomy | Residual disease | 15 months | Left hemivulvectomy + ipsilateral iliac, inferior epigasric and obturator lymphadenectomy | Lung metastatic disease | (14) |
| Korkmaz et al (2016) | 65 | 6 | 20 | Yes | Calponin, Ki-67: Positive; S-100: Negative | Local Excision | NS | NS | - | 6 months, alive, NED | (7) |
| Sayeed et al (2018) | 72 | 11 | 8 | Yes | - | Wide local excision | Infiltrative | NS* | NS* | aDied of disease | (58) |
| Sayeed et al (2018) | 56 | 13.5 | 34 | Yes | - | Wide local excision | Infiltrative | NS* | NS* | aDied of disease | (58) |
| Sayeed et al (2018) | 68 | 5.5 | 23 | Yes | - | Wide local excision | No | NS* | NS* | aDied of disease | (58) |
| Nath et al (2019) | 38 | 8 | 4-5 | Yes | SMA, vimentin: Positive; CK, desmin, S-100, HMB-45, CD34, ER, PR: Negative | Wide local excision | - | - | - | 21 months, alive, NED | (9) |
| Sameeta et al, (2019) | 63 | 2.1 | 25 | No | SMA, desmin, ER: Positive; S-100, cytokeratin AE1/AE3: Negative | Wide local excision + radiotherapy | Infiltrative (focal) | NS | NS | NS | (59) |
| Saquib et al (2020) | 63 | 2.8 | 22 | Yes- Focal | SMA, desmin, caldesmon, Ki-67: Positive; CD117, CD4, vimentin, S100: Negative | Local excision; ipsilateral lymphadenectomy + ipsilateral hemivul- vectomy | Infiltrative (focal); Negative: After the second operation | - | - | NS | (60) |
| Smith et al (2020) | 46 | 3 | NS | NS | SMA, desmin: Positive; cytokeratin AE1/AE3, p40, S100, myogenin, myo-D1, caldesmon, CD34, STAT-6, TLE1: Negative | Local excision; ipsilateral partial radical vulvectomy; posterior radical vulvectomy, bilateral inguino-femoral lympadenectomy | Yes; after 3 operations: Negative | NS | NS | NS | (61) |
| Swanson et al (2020) | 80 | 5.2 | 12 | Yes | NS | Resection + chemotherapy | No | NS | NS | Liver metastasis at the time of resection | (62) |
| Swanson et al (2020) | NS | 9.7 | 36 | No | SMA: Diffusely positive; h-Caldesmon: Multifocally positive; Desmine: Negative | Resection | Infiltrative | NS | NS | NS | (62) |
| Swanson et al (2020) | NS | 2.5 | 12 | No | Desmin, h-Caldesmon and SMA: Diffusely positive; S100 protein: Negative | Resection | Infiltrative | NS | NS | NS | (62) |
| Akrivi et al (present case) | 42 | 6.5 | 8-10 | No | Desmin, Caldesmon, Vimentin, SMA, ER, PR: Positive; S100, myoglobulin, Keratin-116, CD117, CD34, CD31: Negative | Wide local excision | Focally infiltrative margin | No | Wide local Excision | 53 months, alive, NED | - |
MFI, Mitotic figure index per ten high power fields; MTCN, Microscopic tumor cell necrosis; IHC, immunohistochemistry; NED, no evidence of disease; NS, not specified;
aIn the study by Saveed et al metastasis to lung occurred in 2 cases, at 1 and 36 months, respectively, and in the 3rd case occurred extensive local reccurence of the pelvis at 41 months. One patient was treated with adjuvant radiotherapy after recurrence and 2 patients were treated with chemotherapy after lung metastasis. All of the three patients died of the disease at 4, 36 and 56 months.