27 May 2023: Articles
Vulvar Leiomyosarcomas: A Case Series with Clinical Comparison to Uterine Leiomyosarcomas and Review of the Literature
Mistake in diagnosis, Rare disease
Stephanie Verta A* , Zaher Alwafai B , Nika Schleede B , Christine E. Brambs A , Corina Christmann D , Verena Reichert B , Marek Zygmunt A , Birgit Plattner B , Günter Köhler ADOI: 10.12659/AJCR.939402
Am J Case Rep 2023; 24:e939402
Table 2. Summary of the case characteristics of the 8 VLMS of the DKSM.
| Pt Nr | Age (years) | Menopausal state | Presenting symptom | Initial diagnosis | Tumor size | Initial surgery | 2° surgery | R-Status |
|---|---|---|---|---|---|---|---|---|
| 1 | 45 | Premenopausal | Painful growing swelling | Bartholin‘s gland cyst or abscess | 76 mm | TE; not completely removed (R1) | Partial vulvectomy with partial negative margins after resection of the M. sphincter ani | R0 (positive margins after tumor excision, 2° operation) |
| 2 | 52 | Premenopausal | Occlusion of the introitus | chronic Bartholin‘s gland cyst | 47 mm | TE | Re-excision recommended | R1 |
| 3 | 81 | Postmenopausal | n.s. | n.s. | 64 mm | radical vulvectomy with intraoperative frozen section to ensure complete resection | None | R0 |
| 4 | 53 | Premenopausal | n.s. | vulvar tumor | 25 mm | TE | Left hemi-vulvectomy | R0 |
| 5 | 73 | Postmenopausal | n.s. | n.s. | 35 mm | TE; not completely removed (R1) | Re-excision | R0 |
| 6 | 52 | Peri-menopausal | Foreign body sensation, growing tumor | Bartholin‘s gland abscess | 50 mm | marsupialization; tumor not completely removed (R1) | Right hemi-vulvectomy and negative margins after reconstruction by VY-flap plastic | R0 (positive margins after tumor excision, 2° operation) |
| 7 | 68 | Postmenopausal | Growing mass | tumor increasing in size | 25 mm | TE; not completely removed (R1) | Radical right vulvectomy | R0 |
| 8 | 54 | Peri-menopausal | n.s. | Bartholin‘s gland abscess | 33 mm | TE; completely removed | None | R0 |
| n.s. – not specified; TE – tumor excision; R1 – tumor cells found in the resection margin/tumor not completely removed; R0 – negative resection margins/tumor completely removed; pT – pathological tumor size; pNx – unknown pathological lymph node status; L0 – no lymphatic vessel invasion; Lx – unknown lymphatic vessel invasion; V0 – no blood vessel invasion; Vx – unknown blood vessel invasion; Pn0 – no perineural invasion; Pnx – unknown perineural invasion; cM0 – clinically no distant metastasis; MF – mitotic figure; HPF – high-power field; G1/G2/G3 – low/intermediate/high-grade; “+” – positive; (+) – weak positive; SMA – smooth muscle actin; ER – estrogen receptor; PR – progesterone receptor; S-100 – “soluble”-100; Ki-67 – (Kiel)-67 proliferation marker; RT – radiation therapy; EBRT – external beam radiation therapy; Gy – Gray; CHT – chemotherapy. | ||||||||
| Pt Nr | Margins | Tumor stage | Grading | Caldesmon | Desmin | SMA | S-100 | Ki-67 |
| 1 | Resection margin ≥5 mm | pT2 pNx L0 V0 Pn0 cM0 | G2 | + | + | + | n.s. | 5% |
| 2 | Margins partially infiltrated | pT1a pNx Lx Vx Pnx cM0 | G2 | n.s. | n.s. | + | n.s. | 30–70% |
| 3 | n.s. | pT2a pNx L0 V0 Pnx cM0 | G1 | + | n.s. | + | Negative | Slightly increased |
| 4 | Resection margin >10 mm | pT1a pNx Lx Vx Pnx cM0 | G2 | n.s. | + | + | n.s. | 60% |
| 5 | Resection margin 3 mm | pT1a pNx Lx Vx Pnx cM0 | G1 | n.s. | + | + | n.s. | n.s. |
| 6 | n.s. | pT2a pNx Lx Vx Pnx cM0 | G2 | Focally + | + | + | Negative | 30% |
| 7 | Resection margin ≥10 mm | pT1a pNx Lx Vx Pnx cM0 | G3 | Negative | Negative | + | Nuclear + in 10% | 80% |
| 8 | n.s. | pT1a pNx L0 V0 Pnx cM0 | G2 | n.s. | + | (+) | Negative | 50% |
| n.s. – not specified; TE – tumor excision; R1 – tumor cells found in the resection margin/tumor not completely removed; R0 – negative resection margins/tumor completely removed; pT – pathological tumor size; pNx – unknown pathological lymph node status; L0 – no lymphatic vessel invasion; Lx – unknown lymphatic vessel invasion; V0 – no blood vessel invasion; Vx – unknown blood vessel invasion; Pn0 – no perineural invasion; Pnx – unknown perineural invasion; cM0 – clinically no distant metastasis; MF – mitotic figure; HPF – high-power field; G1/G2/G3 – low/intermediate/high-grade; “+” – positive; (+) – weak positive; SMA – smooth muscle actin; ER – estrogen receptor; PR – progesterone receptor; S-100 – “soluble”-100; Ki-67 – (Kiel)-67 proliferation marker; RT – radiation therapy; EBRT – external beam radiation therapy; Gy – Gray; CHT – chemotherapy. | ||||||||
| Pt Nr | Mitosis rate | ER | PR | Recommendation for adjuvant therapy | RT | CHT | Endocrine therapy | Follow-up |
| 1 | 16 MF/10 HPF | n.s. | n.s. | RT | EBRT with boost; 25×2 Gy with a boost to the former tumor bed of 5×2 Gy (total of 60 Gy) | None | none | 16 months |
| 2 | Frequent MF | n.s. | n.s. | Could not be made due to missing information on re-excision | none | None | none | 106 months |
| 3 | 5 MF/10 HPF | + | + | RT | n.s. | n.s. | n.s. | 24 months |
| 4 | >10 MF/10 HPF | (+) | (+) | None | None | None | None | 76 months |
| 5 | n.s. | n.s. | n.s. | RT | n.s. | n.s. | n.s. | 48 months |
| 6 | n.s. | n.s. | n.s. | RT | EBRT, total dose of 60 Gy (single dose 2 Gy) to tumor region and regional lymph nodes | None | None | 80 months |
| 7 | 33 MF/10 HPF | (+), 5% | Negative | RT | applied (no further information) | None | None | 70 months |
| 8 | 13 MF/10 HPF | n.s. | n.s. | None | None | None | None | 95 months |
| n.s. – not specified; TE – tumor excision; R1 – tumor cells found in the resection margin/tumor not completely removed; R0 – negative resection margins/tumor completely removed; pT – pathological tumor size; pNx – unknown pathological lymph node status; L0 – no lymphatic vessel invasion; Lx – unknown lymphatic vessel invasion; V0 – no blood vessel invasion; Vx – unknown blood vessel invasion; Pn0 – no perineural invasion; Pnx – unknown perineural invasion; cM0 – clinically no distant metastasis; MF – mitotic figure; HPF – high-power field; G1/G2/G3 – low/intermediate/high-grade; “+” – positive; (+) – weak positive; SMA – smooth muscle actin; ER – estrogen receptor; PR – progesterone receptor; S-100 – “soluble”-100; Ki-67 – (Kiel)-67 proliferation marker; RT – radiation therapy; EBRT – external beam radiation therapy; Gy – Gray; CHT – chemotherapy. | ||||||||