09 March 2022: Articles
Adenoid Cystic Carcinoma of Bartholin’s Gland: A Case Report with Emphasis on Surgical Management
Rare disease
Stephanie Verta A* , Corina Christmann A , Christine E. Brambs ADOI: 10.12659/AJCR.935707
Am J Case Rep 2022; 23:e935707
Table 1. Overview of the case characteristics, therapeutic modalities, treatment toxicity, and follow-up in cases of ACC of the Bartholin’s gland described in the literature references [2,3,5–7,13,15–22,24–26].
| Author, year of publication | Number of patients | Age (years) | Tumor characteristics | Upfront surgery | Margins | Lymphadene ctomy | Upfront radiotherapy | Upfront chemoradiation |
|---|---|---|---|---|---|---|---|---|
| Lo et al, 2019 | 1 | 33 | Primary tumor, 43 mm | Wide local excision | Positive margins | Bilateral inguinal, positive (1/8) | – | – |
| Akbarzadeh-Jahromi et al, 2014 | 1 | 42 | Primary tumor, 50 mm | Local excision | Positive margins | – | – | – |
| Hsu et al, 2013 | 2 | 37 | Primary tumor, 40 mm | Right radical hemi-vulvectomy | Positive margins | Right-side inguinal-femoral, positive (2/12) | – | – |
| 48 | Primary tumor, 15 mm | Right hemi-vulvectomy | Negative margins | Right-side inguinal-femoral, negative | – | – | ||
| Chang et al, 2019 | 1 | 49 | Primary tumor, 15 mm | Partial vulvectomy | n.s. | Left-side inguinal, negative | – | – |
| Yang et al, 2006 | 2 | 43 | Primary tumor, 17mm | Radical hemi-vulvectomy | Negative margins | Left-side inguinal-femoral, negative | – | – |
| 50 | Second local recurrence | Pelvic exenteration | Negative margins | Bilateral inguinal and pelvic, negative | – | – | ||
| DePasquale et al, 1996 | 1 | 77 | Third local recurrence (previous excision and radiotherapy) | Posterior exenteration | Positive margins | – | – | – |
| Bernstein et al, 1983 | 1 | 34 | Primary tumor, 65 mm | Radical vulvectomy | n.s. | – | – | – |
| Rosenberg et al, 1989 | 5 | 77 | Primary tumor, 90 mm | Local excision | Negative margins | – | – | – |
| 65 | Primary tumor, 40 mm | Left hemi-vulvectomy | Positive margins | Bilateral inguinal, negative | – | – | ||
| 42 | Primary tumor, 40 mm | Local excision | Positive margins | – | – | – | ||
| 37 | Primary tumor, 10 mm | Left hemi-vulvectomy | Negative margins | – | – | – | ||
| 32 | Primary tumor, 70 mm | Total vulvectomy, resection of vaginal wall | Positive margins | – | – | – | ||
| Makhija et al, 2018 | 1 | 50 | Primary tumor, 25 mm | Radical vulvectomy | Positive margins | Left-side, inguinal-femoral, negative (0/8) | – | – |
| Agolli et al, 2013 | 1 | 54 | Primary tumor | Radical hemi-vulvectomy | n.s. | Ipsilateral inguinal-femoral | – | – |
| Momeni et al, 2015 | 1 | 62 | Primary tumor, 37 mm | Posterior exenteration | Negative margins | Left-side, inguinal-femoral, negative | – | – |
| Goh et al, 2018 | 1 | 41 | Primary tumor, 20 mm | Local radical excision | n.s. | Bilateral inguinal | – | – |
| Lopez-Varela et al, 2007 | 2 | 58 | Primary tumor, 20 mm | – | – | Left-side, negative | Yes (EBRT, 50.5 Gy plus 6 Gy Boost) | – |
| 67 | Primary tumor, 40 mm | – | – | – | Yes (EBRT, 45 Gy AND Brachytherapy 20 Gy) | – | ||
| Abrao et al, 1985 | 2 | 37 | Primary tumor, 100 mm | Radical vulvectomy | n.s. | Bilateral inguinal-femoral, negative (0/9 and 0/14) | – | – |
| 40 | Primary tumor, 70 mm | Radical vulvectomy | negative margins | Inguinal-crural, negative (0/22) | – | – | ||
| Yoon et al, 2015 | 5 | 54 | Primary tumor, 20 mm | Radical local excision | positive margins | Right-side inguinal, negative | – | – |
| 67 | Primary tumor, 25 mm | Radical local excision | positive margins | – | – | – | ||
| 50 | Primary tumor, 10 mm | Radical left hemi-vulvectomy | negative margins | Left-side inguinal, negative | – | – | ||
| 60 | Primary tumor, 30 mm | Radical local excision | positive margins | – | – | – | ||
| 59 | Primary tumor, 40 mm | Radical local excision | negative margins | Right-side inguinal, negative | – | – | ||
| Takatori et al, 2013 | 1 | 68 | Primary tumor, 20 mm | Tumor resection | positive margins | – | – | – |
| Bernhardt et al, 2018 | 1 | 50 | Local recurrence, 50 mm | Local excision | positive margins | – | – | – |
| 1 | 42 | Primary tumor, 45 mm | Wide local excision | negative margins | Bilateral inguinal, negative | – | – | |
| EBRT – external beam radiation therapy; Gy – Gray; IMRT – intensity-modulated radiotherapy; VMAT – volumetric-modulated arc therapy; n.s. – not specified. | ||||||||
| Author, year of publication | Adjuvant radiotherapy | Adjuvant chemotherapy | Palliative systemic therapy | Palliative radiotherapy | Adverse events, Toxicity | Follow-up | Reference number | |
| Lo et al, 2019 | Yes (EBRT VMAT; 66 Gy to tumor bed, 59.4 Gy to right pudendal nerve) | Yes (Cisplatin weekly, 40 mg/m) | – | – | n.s. | 3 months: recurrence-free | []2 | |
| Akbarzadeh-Jahromi et al, 2014 | Yes | – | – | – | n.s. | 19 months: recurrence free | []3 | |
| Hsu et al, 2013 | Yes (EBRT, 70 Gy) | – | Yes, 1 cycle of Taxol (135 mg/m) and cisplatin (60 mg/m) followed by 2 courses of carboplatin and 2 courses of carboplatin and LipoDox | Yes (EBRT, 24.5 Gy, to bones) | Palliative chemotherapy: anaphylactic shock after Taxol and cisplatin | 42 months: bone and lung metastasis, palliative chemotherapy and palliative radiotherapy; 54 months: died of disease progression | []5 | |
| – | – | Yes, tamoxifen (4 years stable disease) | – | n.s. | 59 months: Lung metastasis; 120 months: died of lung metastasis | []5 | ||
| Chang et al, 2019 | – | – | – | – | None | 19 moths: local recurrence, excision; 42 months: 2° local recurrence, excision; approximately 54 months: recurrence free | []6 | |
| Yang et al, 2006 | Yes (EBRT, 66 Gy) | – | – | – | n.s. | 17 months: recurrence free | []7 | |
| – | – | 6 cycles of cyclophosphamide (400 mg/m), adriamycin (40 mg/m) and cisplatin (60 mg/m) | – | n.s. | 12 months: lung metastasis, excision; 16 months: lung metastasis, palliative chemotherapy | []7 | ||
| DePasquale et al, 1996 | – | – | – | Had received 6 years beforehand EBRT, 45 Gy AND Brachytherapy, 30 Gy | n.s. | 6 months: died of renal failure, not related to disease | []13 | |
| Bernstein et al, 1983 | n.s. | n.s. | – | – | n.s. | 12 months: recurrence free | []15 | |
| Rosenberg et al, 1989 | yes (EBRT, 40 Gy) | – | – | – | n.s. | 68 months: died without evidence of disease | []16 | |
| yes (EBRT, 20 Gy + 20 Gy left side) | – | – | – | n.s. | 28 months: recurrence free | []16 | ||
| yes (EBRT, 20 Gy + 20 Gy left side) | – | – | – | n.s. | 52 months: recurrence free | []16 | ||
| Yes (EBRT, 29 Gy) | – | – | – | n.s. | 162 months: recurrence free | []16 | ||
| Yes (EBRT Vulva 16 Gy, pelvis 54 Gy) | – | – | – | n.s. | 57 months: recurrence free | []16 | ||
| Makhija et al, 2018 | Yes (EBRT, 50.4 Gy) | Yes (Cisplatin 5-weekly, 150mg) | – | – | Grade I mucositis | 6 months: recurrence free | []17 | |
| Agolli et al, 2013 | Yes (EBRT, 56 Gy) | – | – | – | n.s. | 20 months: recurrence free | []18 | |
| Momeni et al, 2015 | Yes (EBRT, 50.4 Gy) | – | – | – | n.s. | 3 months: recurrence-free | []19 | |
| Goh et al, 2018 | Yes (EBRT VMAT; 63 Gy) | – | – | – | n.s. | 9 months: recurrence free | []20 | |
| Lopez-Varela et al, 2007 | – | – | Yes | Yes | Long-term adverse effect: stenosis of the vulva, treated by perineoplasty | 44 months: lung metastasis, palliative chemotherapy; 54 months: local recurrence, excision, palliative radiotherapy; 142 months: died of disease | []21 | |
| – | – | – | – | Diarrhea, constipation, and temporary incontinence | 4 months: died of not related disease | []21 | ||
| Abrao et al, 1985 | – | – | Yes, Adriamycin (50 mg/m) and cyclophosphamide (750 mg/m) | – | n.s. | 9 months: local recurrence, excision; 16 months: second local recurrence, excision; 19 months: lung metastasis, palliative chemotherapy | []24 | |
| – | – | Yes, adriamycin (50 mg/m) and cyclophosphamide (750 mg/m) | – | n.s. | 42 months: local recurrence, excision; 45 months: 2° local recurrence, excision; 48 months: lung metastasis; 51 months: 3° local recurrence, excision and palliative chemotherapy | []24 | ||
| Yoon et al, 2015 | Yes | – | Yes, 6 cycles of adriamycin and cisplatin | – | n.s. | 7 months: lung metastasis, excision; 8 months: lung metastasis, excision and palliative chemotherapy; 71 months: stable disease | []25 | |
| Yes | – | – | – | n.s. | 106 months: recurrence free | []25 | ||
| Yes | – | – | – | n.s. | 137 months: recurrence free | []25 | ||
| – | – | Yes, 6 cycles of cyclophosphamide, adriamycin, and cisplatin (71 months); 4 cycles of ifosfamide (132 months) | – | n.s. | Local recurrence after 24, 46, 60 and 182 months; distant metastasis after 71, 132 and 189 months; 224 months: progressive disease | []25 | ||
| – | – | – | – | n.s. | n.s. | []25 | ||
| Takatori et al, 2013 | Yes (EBRT, 59.4 Gy) | yes (concomitant Irinotecan, 50 mg/m2×5) | – | – | Grade 2 neutropenia and Grade 2 anemia; Grade 1 nausea and Grade 2 radiation dermatitis | 24 months: recurrence free | []26 | |
| Bernhardt et al, 2018 | Yes (EBRT, bimodality treatment: photon IMRT plus carbon ion (C12) boost) | – | – | – | Low toxicity: mild erythema, moist vaginal and inguinal desquamation, diarrhea | n.s. | []22 | |
| – | – | – | – | Local wound dehiscence with secondary healing | 8 months: recurrence free | |||
| EBRT – external beam radiation therapy; Gy – Gray; IMRT – intensity-modulated radiotherapy; VMAT – volumetric-modulated arc therapy; n.s. – not specified. | ||||||||