Vulvar Leiomyosarcomas: A Case Series with Clinical Comparison to Uterine Leiomyosarcomas and Review of the Literature

Stephanie Verta; Zaher Alwafai; Nika Schleede; Christine E. Brambs; Corina Christmann; Verena Reichert; Marek Zygmunt; Birgit Plattner; Günter Köhler

doi:10.12659/AJCR.939402

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 ^A

DOI: 10.12659/AJCR.939402

Am J Case Rep 2023; 24:e939402

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Background Case Reports Discussion Conclusions References:

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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.
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.
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.

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Vulvar Leiomyosarcomas: A Case Series with Clinical Comparison to Uterine Leiomyosarcomas and Review of the Literature

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